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Please enter a valid web address * About * Blog * Projects * Help * Donate * Contact * Jobs * Volunteer * People * Sign up for free * Log in Search metadata Search text contents Search TV news captions Search radio transcripts Search archived web sites Advanced Search * About * Blog * Projects * Help * Donate Donate icon An illustration of a heart shape * Contact * Jobs * Volunteer * People Full text of "@ MBmedicalbookneiro " See other formats THIRD EDITION NEUROLOGIC INTERVENTIONS MARTIN KESSLER ELSEVIER Neurologic Interventions for Physical Therapy THIRD EDITION Suzanne "Tink" Martin, PT, PhD Professor and Associate Chair, Department of Physical Therapy, University of Evansville, Evansville, Indiana Mary Kessler, PT, MHS Associate Dean, College of Education and Health Sciences, Director Physical Therapist Assistant Program, Associate Professor, Department of Physical Therapy, University of Evansville, Evansville, Indiana SAUNDERS | ELSEVIER Table of Contents Cover image Title page Copyright Contributors Dedication Preface Acknowledgments Section 1: Foundations Chapter 1: The Roles of the Physical Therapist and Physical Therapist Assistant in Neurologic Rehabilitation Introduction The role of the physical therapist in patient management The role of the physical therapist assistant in treating patients with neurologic deficits The physical therapist assistant as a member of the healthcare team Chapter 2: Neuroanatomy Introduction Major components of the nervous system Reaction to injury Chapter 3: Motor Control and Motor Learning Introduction Motor control Issues related to motor control Motor learning Theories of motor learning Stages of motor learning Chapter 4: Motor Development Introduction Developmental time periods Influence of cognition and motivation Developmental concepts Developmental processes Motor milestones Typical motor development Posture, balance, and gait changes with aging Section 2: Children Chapter 5: Positioning and Handling to Foster Motor Function Introduction Children with neurologic deficits General physical therapy goals Function related to posture Physical therapy intervention Positioning and handling interventions Preparation for movement Interventions to foster head and trunk control Adaptive equipment for positioning and mobility Functional movement in the context of the child’s world Chapter 6: Cerebral Palsy Introduction Incidence Etiology Classification Functional classification Diagnosis Pathophysiology Associated deficits Physical therapy examination Physical therapy intervention Chapter 7: Myelomeningocele Introduction Incidence Etiology Prenatal diagnosis Clinical features Physical therapy intervention Chapter 8: Genetic Disorders Introduction Genetic transmission Categories Down syndrome CRI-DU-Chat syndrome Prader-willi syndrome and angelman syndrome Arthrogryposis multiplex congenita Osteogenesis imperfecta Cystic fibrosis Spinal muscular atrophy Phenylketonuria Becker muscular dystrophy Fragile X syndrome Rett syndrome Autism Spectrum Disorder Genetic disorders and intellectual disability Section 3: Adults Chapter 9: Proprioceptive Neuromuscular Facilitation Introduction History of proprioceptive neuromuscular facilitati Basic principles of PNF Biomechanical considerations Patterns Proprioceptive neuromuscular facilitation techniques Developmental sequence Proprioceptive neuromuscular facilitation and motor learning Chapter 10: Cerebrovascular Accidents Introduction Etiology Medical intervention Recovery from stroke Prevention of cerebrovascular accidents Stroke syndromes ical findings: Patient impairments Treatment planning Complications seen following stroke Acute care setting tant Early physical therapy intervention Midrecovery to late recovery Chapter 11: Traumatic Brain Injuries Introduction Classifications of brain injuries Secondary problems Patient examination and evaluation Patient problem areas Physical t ipy intervention: acute care Physical therapy interventions during inpatient rehabilitation Integrating physical and cognitive components of a task into treatment interventions Discharge planning Chapter 12: Spinal Cord Injuries Introduction Etiology Naming the level of injury Mechanisms of injury Medical intervention Pathologic changes that occur following injury Types of lesions Clinical manifestations of spinal cord injuries Resolution of spinal shock Complications Functional outcomes Physical therapy intervention: acute care Physical therapy interventions during inpatient rehabilitation Body-weight-supported treadmill Discharge planning Chapter 13: Other Neurologic Disorders Introduction Parkinson disease Multiple sclerosis Amyotrophic lateral sclerosis Guillain-barré syndrome Postpolio syndrome Index Copyright ELSEVIER SAUNDERS 3251 Riverport Lane St. Louis, MO 63043 NEUROLOGIC INTERVENTIONS FOR PHYSICAL THERAPY, THIRD EDITION ISBN: 978-1-4557-4020-8 Copyright © 2016 by Saunders, an imprint of Elsevier Inc. Previous editions copyrighted 2007, 2000 All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in writing from the publisher. Permissions may be sought directly from Elsevier’s Health Sciences Rights Department in Philadelphia, PA, USA: phone: (+ 1) 215 239 3804, fax: (+ 1) 215 239 3805, e-mail: healthpermissions@elsevier.com. You may also complete your request online via the Elsevier homepage (http://www.elsevier.com), by selecting ‘Customer Support’ and then ‘Obtaining Permissions.’ Notice Knowledge and best practice in this field are constantly changing. As new research and experience broaden our knowledge, changes in practice, treatment, and drug therapy may become necessary or appropriate. Readers are advised to check the most current information provided (i) on procedures featured or (ii) by the manufacturer of each product to be administered, to verify the recommended dose or formula, the method, and duration of administration, and contraindications. It is the responsibility of the practitioner, relying on their own experience and knowledge of the patient, to make diagnoses, to determine dosages and the best treatment for each individual patient, and to take all appropriate safety precautions. To the fullest extent of the law, neither the Publisher nor the Editor assumes any liability for any injury and/or damage to persons or property arising out of or related to any use of the material contained in this book. The Publisher International Standard Book Number: 978-1-4557-4020-8 Executive Content Strategist: Kathy Falk Content Development Specialist: Brandi Graham Publishing Services Manager: Julie Eddy Senior Project Manager: Richard Barber Designer: Ryan Cook Printed in the United States of America Last digit is the print number: 987654321 eee Working together 3 | ™ to grow libraries in ELSEVIER Book Aid developing countries www.elsevier.com e www.bookaid.org Contributors Maghan C. Bretz, PT, MPT St Mary’s Rehabilitation Institute, Adjunct Instructor, Department of Physical Therapy, Evansville, Indiana, Evolve videos Terry Chambliss, PT, MHS Physical Therapist, Evansville, Indiana, Proprioceptive Neuromuscular Facilitation 10 Dedication To my husband, Terry, who has always been there with love and support, and to my parents who were always supportive of my educational endeavors. Tink To Craig, my husband, who continues to provide me with love, support, and encouragement to pursue this and all of my other professional goals, and to Kyle and Kaitlyn, who still like to see their photographs in print. A final word of thanks to my parents, John and Judy Oerter, who have always encouraged me to work hard and strive for excellence. You have always believed in me and my ability to succeed. Mary 11 Preface We are gratified by the very positive responses to the first two editions of the Neurologic Interventions for Physical Therapy text. In an effort to make a good reference even better, we have taken the advice of reviewers and our physical therapist and physical therapist assistant students to complete a third edition. The sequence of chapters still reflects a developmental trend with motor development, handling and positioning, and interventions for children coming before the content on adults. Chapters on specific pediatric disorders and neurologic conditions seen in adults remain as well as introductory chapters on physical therapy practice and the role of the physical therapist assistant. The review of basic neuroanatomy structure and function and the chapter on proprioceptive neuromuscular facilitation have been updated and continue to provide foundational knowledge. The intervention components of each chapter have been enhanced to emphasize function and the use of current best evidence in the physical therapy care of these patients. Concepts related to neuroplasticity and task-specific training are also included. All patient cases have been reworked again to reflect current practice and are formatted in a way to assist students with their documentation skills. We continue to see that the text is used by students in both physical therapist assistant and doctor of physical therapy programs, and this certainly has broad appeal. However, as we indicated in our last preface, we continue to be committed to addressing the role of the physical therapist assistant in the treatment of children and adults with neurologic deficits. On the contrary, the use of the textbook by physical therapy students should increase the understanding of and appreciation for the psychomotor and critical-thinking skills needed by all members of the rehabilitation team to maximize the function of patients with neurologic deficits. The Evolve site continues to be enhanced as we try to insert additional resources for faculty and students. An instructor Test Bank and PowerPoint slides have been added in this third edition. Also, newly added video clips of interventions as well as gait and proprioceptive neuromuscular facilitation will allow students to increase their understanding of the subject matter and to be better prepared for the neurologic portion of their certification exam. The mark of sophistication of any society is how well it treats the young and old, the most vulnerable segments of the population. We hope in some small measure that our continuing efforts will make it easier to unravel the mystery of directing movement, guiding growth and development, and relearning lost functional skills to improve the quality of life for the people we serve. 12 Acknowledgments I again want to acknowledge the dedication and hard work of my colleague, friend, and co-author, Mary Kessler. Mary’s focus on excellence is evident in the updated adult chapters. Special thanks to Dawn Welborn-Mabrey for her marvelous pediatric insights. Thank you to past contributors, Dr. Pam Ritzline, Mary Kay Solon, Dr. Donna Cech, and Terry Chambliss. Thank you to the students at the University of Evansville. You are really the reason this book happened in the first place and the reason it has evolved into its present form. I want to acknowledge the work of those at Elsevier, especially Brandi Graham, for seeing us through the timely completion of the third edition. Tink I must thank my good friend, mentor, colleague, and co-author, Tink Martin. Without Tink, none of these editions would have been completed. She has continued to take care of many of the details, always keeping us focused on the end result. Tink’s ongoing encouragement and support have been most appreciated. A special thank you to all of the students at the University of Evansville. They are the reason that we originally started this project, and they have continued to encourage and motivate us to update and revise the text. Additional thanks must be extended to all of the individuals who have assisted us over the last 20 years, including Dr. Catherine McGraw, Maghan Bretz, Sara Snelling, Dr. Pam Ritzline, Mary Kay Solon, Janet Szczepanski, Terry Chambliss, Suzy Sims, Beth Jankauski, and Amanda Fisher. Every person mentioned has contributed to the overall excellence and success of this text. Mary 13 SECTION 1 Foundations 14 CHAPTER 1 15 The Roles of the Physical Therapist and ater Therapist Assistant in Neurologic Rehabilitation Objectives After reading this chapter, the student will be able to: ¢ Discuss the International Classification of Functioning, Disability, and Health (ICF) and its relationship to physical therapy practice. ¢ Explain the role of the physical therapist in patient/client management. ¢ Describe the role of the physical therapist assistant in the treatment of adults and children with neurologic deficits. 16 Introduction The practice of physical therapy in the United States continues to change to meet the increased demands placed on service provision by reimbursement entities and federal regulations. The profession has seen an increase in the number of physical therapist assistants (PTAs) providing physical therapy interventions for adults and children with neurologic deficits. PTAs are employed in outpatient clinics, inpatient rehabilitation centers, extended-care and pediatric facilities, school systems, and home healthcare agencies. Traditionally, the rehabilitation management of adults and children with neurologic deficits consisted of treatment derived from the knowledge of disease and interventions directed at the amelioration of patient signs, symptoms, and functional impairments. Physical therapists and physical therapist assistants help individuals “maintain, restore, and improve movement, activity, and functioning, thereby enhancing health, well-being, and quality of life” (APTA, 2014). Physical therapy is provided across the lifespan to children and adults who “may develop impairments, activity limitations, and participation restrictions” (APTA, 2014). These limitations develop as a consequence of various health conditions and the interaction of personal and environmental factors (APTA, 2014). Sociologist Saad Nagi developed a model of health status that has been used to describe the relationship between health and function (Nagi, 1991). The four components of the Nagi Disablement Model (disease, impairments, functional limitations, and disability) evolve as the individual loses health. Disease is defined as a pathologic state manifested by the presence of signs and symptoms that disrupt an individual’s homeostasis or internal balance. Impairments are alterations in anatomic, physiologic, or psychological structures or functions. Functional limitations occur as a result of impairments and become evident when an individual is unable to perform everyday activities that are considered part of the person’s daily routine. Examples of physical impairments include a loss of strength in the anterior tibialis muscle or a loss of 15 degrees of active shoulder flexion. These physical impairments may or may not limit the individual's ability to perform functional tasks. Inability to dorsiflex the ankle may prohibit the patient from achieving toe clearance and heelstrike during ambulation, whereas a 15-degree limitation in shoulder range may have little impact on the person’s ability to perform self-care or dressing tasks. According to the disablement model, a disability results when functional limitations become so great that the person is unable to meet age-specific expectations within the social or physical environment (Verbrugge and Jette, 1994). Society can erect physical and social barriers that interfere with a person’s ability to perform expected roles. The societal attitudes encountered by a person with a disability can result in the community’s perception that the individual is handicapped. Figure 1-1 depicts the Nagi classification system of health status. Pathology Alteration Ditficulty performing Significant Societal of structure routine tasks functional limn@ation disadvantage and function cannot perform of disability expected tasks FIGURE 1-1 Nagi classification system of health status. The second edition of the Guide to Physical Therapist Practice incorporated the Nagi Disablement Model into its conceptual framework of physical therapy practice. The use of this model has directed physical therapists (PTs) to focus on the relationship between impairment and functional limitation and the patient’s ability to perform everyday activities. Increased independence in the home and community and improvements in an individual's quality of life are the expected outcomes of physical therapy interventions (APTA, 2003). However, as our practice has evolved, current practice guidelines recognize the critical roles PTs and PTAs play in providing “rehabilitation and habilitation, performance enhancement, and prevention and risk-reduction services” for patients and the overall population (APTA, 2014). As physical therapy professionals, it is important that we understand our role in optimizing patient function. The second edition of the Guide to Physical Therapist Practice (APTA, 2003) defined function as “those activities identified by an individual as essential to support physical, social, and psychological well-being and to create a personal sense of meaningful living.” Function is related to 17 age-specific roles in a given social context and physical environment and is defined differently for a child of 6 months, an adolescent of 15 years, and a 65-year-old adult. Factors that contribute to an individual's functional performance include personal characteristics, such as physical ability, emotional status, and cognitive ability; the environment in which the adult or child lives and works, such as home, school, or community; and the social expectations placed on the individual by the family, community, or society. The World Health Organization (WHO) developed the International Classification of Functioning, Disability, and Health (ICF), which has been endorsed by the American Physical Therapy Association (APTA). This system provides a more positive framework and standard language to describe health, function, and disability and has been incorporated into the third edition of the Guide to Physical Therapist Practice. Figure 1-2 illustrates the ICF model. Health is much more than the absence of disease; rather, it is a condition of physical, mental, and social well-being that allows an individual to participate in functional activities and life situations (WHO, 2013; Cech and Martin, 2012). A biopsychosocial model is central to the ICF and defines a person’s health status and functional capabilities by the interactions between one’s biological, psychological, and social domains (Figure 1-3). This conceptual framework recognizes that two individuals with the same diagnosis might have very different functional outcomes and levels of participation based on environmental and personal factors. Health condition (disorder or disease) Body functions and structures Participation Activities Environmental factors FIGURE 1-2 Model of the International Classification of Functioning, Disability, and Health (ICF). (From Cech D, Martin S. Functional Movement Development Across the Life Span, ed 3, St Louis, 2012, Elsevier.) Affect Motivation Cognitive ability Social roles Cultural roles Sensorimotor tasks FIGURE 1-3 The three domains of function—biophysical, psychological, sociocultural—must operate independently as well as interdependently for human beings to achieve their best possible functional status. (From Cech D, Martin S: Functional movement development across the life span, ed 3. St Louis, 2012, Elsevier.) The ICF also presents functioning and disability in the context of health and organizes the information into two distinct parts. Part 1 addresses the components of functioning and disability as 18 they relate to the health condition. The health condition (disease or disorder) results from the impairments and alterations in an individual’s body structures and functions (physiologic and anatomical processes). Activity limitations present as difficulties performing a task or action and encompass physical as well as cognitive and communication activities. Participation restrictions are deficits that an individual may experience when attempting to meet social roles and obligations within the environment. Functioning and disability are therefore viewed on a continuum where functioning encompasses performance of activities, and participation and disability implies activity limitations and restrictions in one’s ability to participate in life situations. Part 2 of the ICF information recognizes the external environmental and internal personal factors which influence a person’s response to the presence of a disability and the interaction of these factors on one’s ability to participate in meaningful activities (APTA, 2014; WHO, 2013). All factors must be considered to determine their impact on function and participation (O’Sullivan, 2014; Cech and Martin, 2012). The ICF is similar to the Nagi Model; however, the ICF emphasizes enablement rather than disability (Cech and Martin, 2012). In the ICF model, there is less focus on the cause of the medical condition and more emphasis directed to the impact that activity limitations and participation restrictions have on the individual. As individuals experience a decline in health, it is also possible that they may experience some level of disability. Thus, the ICF “mainstreams the experience of disability and recognizes it as a universal human experience” (ICF, 2014). Various functional skills are needed in domestic, vocational, and community environments. Performance of these skills enhances the individual's physical and psychological well-being. Individuals define themselves by what they are able to accomplish and how they are able to participate in the world. Performance of functional tasks not only depends on an individual’s physical abilities and sensorimotor skills but is also affected by the individual’s emotional status (depression, anxiety, self-awareness, self-esteem), cognitive abilities (intellect, motivation, concentration, problem-solving skills), and ability to interact with people and meet social and cultural expectations (Cech and Martin, 2012). Furthermore, individual factors such as congenital disorders and genetic predisposition to disease, demographics (age, sex, level of education, and income), comorbidities, lifestyle choices, health habits, and environmental factors (including access to medical and rehabilitation care and the physical and social environments) may also impact the individual’s function and his or her quality of life (APTA, 2014). 19 The role of the physical therapist in patient management As stated earlier, physical therapists are responsible for providing rehabilitation, habilitation, performance enhancement, and preventative services (APTA, 2014). Ultimately, the PT is responsible for performing a review of the patient’s history and systems and for administering appropriate tests and measures in order to determine an individual’s need for physical therapy services. If after the examination the PT concludes that the patient will benefit from services, a plan of care is developed that identifies the goals, expected outcomes, and the interventions to be administered to achieve the desired patient outcomes (APTA, 2014). The steps the PT utilizes in patient/client management are outlined in the third edition of the Guide to Physical Therapist Practice and includes examination, evaluation, diagnosis, prognosis, interventions, and outcomes. The PT integrates these elements to optimize the patient’s outcomes, including improving the health or function of the individual or enhancing the performance of healthy individuals. Figure 1-4 identifies these elements. In the examination, the PT collects data through a review of the patient’s history and a review of systems and then administers appropriate tests and measures. The PT then evaluates the data, interprets the patient’s responses, and makes clinical judgments relative to the chronicity or severity of the patient’s problems. Within the evaluation process, the therapist establishes a physical therapy diagnosis based on the patient’s level of impairment and functional limitations. Use of differential diagnosis (a systematic process to classify patients into diagnostic categories) may be used. Once the diagnosis is completed, the PT develops a prognosis, which is the predicted level of improvement and the amount of time that will be needed to achieve those levels. Patient goals are also a component of the prognosis aspect of the evaluation. The development of the plan of care is the final step in the evaluation process. The plan of care includes short- and long-term goals and specific interventions to be administered, as well as the expected outcomes of therapy and the proposed frequency and duration of treatment. Goals and outcomes should be objective, measureable, functionally oriented, and meaningful to the patient. Intervention is the element of patient management in which the PT or the PTA interacts with the patient through the administration of “various physical interventions to produce changes in the [patient’s] condition that are consistent with the diagnosis and prognosis” (APTA, 2014). Intervention are organized into 9 categories: “patient or client instruction (used with every patient); airway clearance techniques, assistive technology, biophysical agents; functional training in self- care and domestic, work, community, social, and civic life; integumentary repair and protection techniques; manual therapy techniques; motor function training; and therapeutic exercise” (APTA, 2014). Reexamination of the patient includes performance of appropriate tests and measures to determine if the patient is progressing with treatment or if modifications are needed. The final component related to patient management is review of patient outcomes. The PT must determine the impact selected interventions have had on the following: disease or disorder, impairments, activity limitations, participation, risk reduction and prevention, health, wellness, and fitness, societal resources, and patient satisfaction (APTA, 2014). Other aspects of patient/client management include the coordination (the working together of all parties), communication, and documentation of services provided. 20 EVALUATION DIAGNOSIS PROGNOSIS INTERVENTION OUTCOMES FIGURE 1-4 The elements of patient/client management. (From American Physical Therapy Association: Guide to Physical Therapist Practice 3.0. Alexandria, VA, 2014, APTA.) PTAs assist only with the intervention component of care (Clynch, 2012). All interventions performed by the PTA are directed and supervised by the PT. These interventions may include “procedural intervention(s), associated data collection, and communication—including written documentation associated with the safe, effective, and efficient completion of the task” (Crosier, 2010). All other tasks remain the sole responsibility of the PT. 21 The role of the physical therapist assistant in treating patients with neurologic deficits There is little debate as to whether PTAs have a role in treating adults with neurologic deficits, as long as the individual needs of the patient are taken into consideration and the PTA follows the plan of care established by the PT. Physical therapist assistants are the only healthcare providers who “assist a physical therapist in the provision of selected interventions” (APTA, 2014). The primary PT is still ultimately responsible for the patient, both legally and ethically, and the actions of the PTA relative to patient management (APTA, 2012a). The PT directs and supervises the PTA when the PTA provides interventions selected by the PT. The APTA has identified the following responsibilities as those that must be performed exclusively by the PT (APTA, 2012a): 1. Interpretation of referrals when available 2. Initial examination, evaluation, diagnosis, and prognosis 3. Development or modification of the plan of care, which includes the goals and expected outcomes 4, Determination of when the expertise and decision-making capabilities of the PT requires the PT to personally render services and when it is appropriate to utilize a PTA 5. Reexamination of the patient and revision of the plan of care if indicated 6. Establishment of the discharge plan and documentation of the discharge summary 7. Oversight of all documentation for services rendered APTA policy documents also state that interventions that require immediate and continuous examination and evaluation are to be performed exclusively by the PT (APTA, 2012b). Specific examples of these interventions have changed recently. PTs and PTAs are advised to refer to APTA policy documents, their state practice acts, and the Commission on Accreditation in Physical Therapy Education (CAPTE) guidelines for the most up-to-date information regarding interventions that are considered outside the scope of practice for the PTA. Practitioners are also encouraged to review individual state practice acts and payer requirements for supervision requirements as they relate to the PT/PTA relationship (Crosier, 2011). Before directing the PTA to perform specific components of the intervention, the PT must critically evaluate the patient’s condition (stability, acuity, criticality, and complexity) consider the practice setting in which the intervention is to be delivered, the type of intervention to be provided, and the predictability of the patient’s probable outcome to the intervention (APTA, 2012a). In addition, the knowledge base of the PTA and his or her level of experience, training, and skill level must be considered when determining which tasks can be directed to the PTA. The APTA has developed two algorithms (PTA direction and PTA supervision; Figures 1-5 and 1-6) to assist PTs with the steps that should be considered when a PT decides to direct certain aspects of a patient’s care to a PTA and the subsequent supervision that must occur. Even though these algorithms exist, it is important to remember that communication between the PT and PTA must be ongoing to ensure the best possible outcomes for the patient. PTAs are also advised to become familiar with the Problem-Solving Algorithm Utilized by PTAs in Patient/Client Intervention (Figure 1-7) as a guide for the clinical problem-solving skills a PTA should employ before and during patient interventions (APTA, 2007). Unfortunately, in our current healthcare climate, there are times when the decision as to whether a patient may be treated by a PTA is determined by productivity concerns and the patient’s payer source. An issue affecting some clinics and PTAs is the denial of payment by some insurance providers for services provided by a PTA. Consequently, decisions regarding the utilization of PTAs are sometimes determined by financial remuneration and not by the needs of the patient. 22 FIGURE 1-5 PTAdirection algorithm. (From Crosier J: PT direction and supervision algorithms, PT in Motion 2(8):47, 2010.) 23 Provide rented Cortera to morvior meee Prete reed On omereraate potertchent ont CR 08 Cate Grecton Wo he PTA eeplany em Pe PTA §— proceed as ncicated, Grection Wo Be PTA FIGURE 1-6 PTA supervision algorithm. (From Crosier J: PT direction and supervision algorithms, PT in Motion 2(8):47, 2010.) Problem-Solving Algorithm Utilized by PTAs in Pationt/Client Intervention (See Contoiing Assumptions on previous page.) aI See | a Shoe ELE FIGURE 1-7 Problem solving algorithm utilized by PTAs in patient/client intervention. (From American Physical Therapy Association: A normative model of physical therapist assistant education, Version 2007, Alexandria, VA, 2007, APTA, p. 85.) Although PTAs work with adults who have had cerebrovascular accidents, spinal cord injuries, 24 and traumatic brain injuries, some PTs still view pediatrics as a specialty area of practice. This narrow perspective is held even though PTAs work with children in hospitals, outpatient clinics, schools, and community settings, including fitness centers and sports-training facilities. Although some areas of pediatric physical therapy are specialized, many areas are well within the scope of practice of the generalist PT and PTA (Miller and Ratliffe, 1998). To assist in resolving this controversy, the Pediatric Section of APTA developed a draft position statement outlining the use of PTAs in various pediatric settings. The original position paper stated that “physical therapist assistants could be appropriately utilized in pediatric settings with the exception of the medically unstable, such as neonates in the ICU” (Section on Pediatrics, APTA, 1995). This document was revised in 1997 and remains available from the Section on Pediatrics. The position paper states that “the physical therapist assistant is qualified to assist in the provision of pediatric physical therapy services under the direction and supervision of a physical therapist” (Section on Pediatrics, APTA, 1997). It is recommended that PTAs should not provide services to children who are physiologically unstable (Section on Pediatrics, APTA, 1997). In addition, this position paper also states that “delegation of physical therapy procedures to a PTA should not occur when a child’s condition requires multiple adjustments of sequences and procedures due to rapidly changing physiologic status and/or response to treatment” (Section on Pediatrics, APTA, 1997). The guidelines proposed in this document follow those suggested by Dr. Nancy Watts in her 1971 article on task analysis and division of responsibility in physical therapy (Watts, 1971). This article was written to assist PTs with guidelines for delegating patient care activities to support personnel. Although the term delegation is not used today because of the implications of relinquishing patient care responsibilities to another practitioner, the principles of patient/client management, as defined by Watts, can be applied to the provision of present-day physical therapy services. PTs and PTAs unfamiliar with this article are encouraged to review it because the guidelines presented are still appropriate for today’s clinicians and are referenced in APTA documents. 25 The physical therapist assistant as a member of the healthcare team The PTA functions as a member of the rehabilitation team in all treatment settings. Members of this team include the primary PT; the physician; speech, occupational, and recreation therapists; nursing personnel; the psychologist; case manager; and the social worker. However, the two most important members of this team are the patient and his or her family. In a rehabilitation setting, the PTA is expected to provide interventions to improve the patient’s functional independence. Relearning motor activities, such as bed mobility, transfers, ambulation skills, stair climbing, and wheelchair negotiation, if appropriate, are emphasized to enhance the patient’s functional mobility. In addition, the PTA participates in patient and family education and is expected to provide input into the patient's discharge plan. Patient and family instruction includes providing information, education, and the actual training of patients, families, significant others, or caregivers and is a part of every patient’s plan of care (APTA, 2014; APTA, 2003). As is the case in all team activities, open and honest communication among all team members is crucial to maximize the patient’s participation and achievement of an optimal functional outcome. The rehabilitation team working with a child with a neurologic deficit usually consists of the child; his or her parents; the various physicians involved in the child’s management and other healthcare professionals, such as an audiologist and physical and occupational therapists; a speech language pathologist; and the child’s classroom teacher. The PTA is expected to bring certain skills to the team and to the child, including knowledge of positioning and handling, use of adaptive equipment, management of abnormal muscle tone, knowledge of developmental activities that foster acquisition of functional motor skills and movement transitions, knowledge of family- centered care and the role of physical therapy in an educational environment. Additionally, interpersonal communication and advocacy skills are beneficial as the PTA works with the child and the family, as well as others. Family teaching and instruction are expected within a family- centered approach to the delivery of various interventions embedded into the child’s daily routine. Because the PTA may be providing services to the child in his or her home or school, the assistant may be the first to observe additional problems or be told of a parent’s concern. These observations or concerns should be communicated immediately to the supervising PT. Due to the complexity of patient’s problems and the interpersonal skill set needed to work with the pediatric population and their families, most clinics require prior work experience before employing PTAs and PTs in these treatment settings (Clynch, 2012). PTs and PTAs are valuable members of a patient’s health-care team. To optimize the relationship between the two and to maximize patient outcomes, each practitioner must understand the educational preparation and experiential background of the other. The preferred relationship between PTs and PTAs is one characterized by trust, understanding, mutual respect, effective communication, and an appreciation for individual similarities and differences (Clynch, 2012). This relationship involves direction, including determination of the tasks that can be directed to the PTA, supervision because the PT is responsible for supervising the assistant to whom tasks or interventions have been directed and accepted, communication, and the demonstration of ethical and legal behaviors. Positive benefits that can be derived from this preferred relationship include more clearly defined identities for both PTs and PTAs and a more unified approach to the delivery of high-quality, cost-effective physical therapy services. Chapter summary Changes in physical therapy practice have led to an increase in the number of PTAs and greater variety in the types of patients treated by these clinicians. PTAs are actively involved in the treatment of adults and children with neurologic deficits. After a thorough examination and evaluation of the patient's status, the primary PT may determine that the patient’s intervention or a portion of the intervention may be safely performed by an assistant. The PTA functions as a member of the patient’s rehabilitation team and works with the patient to maximize his or her ability to participate in meaningful activities. Improved function in the home, school, or community remains as the primary goal of our physical therapy interventions. 26 Review questions 1. Discuss the ICF model as it relates to health and function. 2. List the factors that affect an individual’s performance of functional activities. 3. Discuss the elements of patient/client management. 4. Identify the factors that the PT must consider before utilizing a PTA. 5. Discuss the roles of the PTA when working with adults or children with neurologic deficits. 27 References American Physical Therapy Association. Guide to physical therapist practice. ed 2 Alexandria, VA: APTA; 2003 pp 13-47, 679. American Physical Therapy Association: Direction and supervision of the physical therapist assistant, 2012a, HOD P06-05-18-26. Available at: www.apta.org/uploadedFiles/APTAorg/About_Us/Policies/Practice/DirectionSupervisionPT Accessed January 5, 2014. American Physical Therapy Association: Procedural interventions exclusively performed by physical therapists, 2012b, HOD P06-00-30-36. Available at: www.apta.org/uploadedFiles/APTAorg/About_Us/Policies/Practice? ProceduralInterventions.pdf. Accessed January 5, 2014. American Physical Therapy Association (APTA). Guide to physical therapist practice 3.0. ed 3 Alexandria, VA: APTA; 2014. Available at: http://guidetoptpractice.apta.org Accessed September 24, 2014. American Physical Therapy Association Education Division. A normative model of physical therapist professional education, version 2007. Alexandria, VA: APTA; 2007 pp 84-85. Cech D, Martin S. Functional movement development across the life span. ed 3 Philadelphia: Saunders; 2012 pp 1-13. Clynch HM. The role of the physical therapist assistant regulations and responsibilities. Philadelphia: FA Davis; 2012 pp 23, 43-76. Crosier J. PTA direction and supervision algorithms. PTinMotion. 2010. Available at: www.apta.org/PTinMotion/2010/9PTAsToday Accessed January 7, 2014. Crosier J. The PT/PTA relationship: 4 things to know. February 2011. Available at: www.apta.org/PTAPatientCare Accessed January 7, 2014. International classification of functioning, disability, and health (ICF), World Health Organization. Available at: www.who.int/classifications/icf/en/. Accessed January 5, 2014. Miller ME, Ratliffe KT. The emerging role of the physical therapist assistant in pediatrics. In: Ratliffe KT, ed. Clinical pediatric physical therapy. St Louis: Mosby; 1998:15-22. Nagi SZ. Disability concepts revisited: Implications for prevention. In: Pope AM, Tarlox AR, eds. Disability in America: toward a national agenda for prevention. Washington, DC: National Academy Press; 1991:309-327. O'Sullivan SB. Clinical decision making planning and examination. In: O’Sullivan SB, Schmitz TJ, Fulk GD, eds. Physical rehabilitation assessment and treatment. ed 6 Philadelphia: Davis; 2014:1-29. Section on Pediatrics, American Physical Therapy Association. Draft position statement on utilization of physical therapist assistants in the provision of pediatric physical therapy. Sect Pediatr Newsl. 1995;5:14-17. Section on Pediatrics, American Physical Therapy Association. Utilization of physical therapist assistants in the provision of pediatric physical therapy. Alexandria, VA: APTA; 1997. Verbrugge L, Jette A. The disablement process. Soc Sci Med. (38):1994;1-14. Watts NT. Task analysis and division of responsibility in physical therapy. Phys Ther. (51):1971;23-35. World Health Organization. How to use the ICF: a practical manual for using the international classification of functioning, disability and health (ICF), 2013. 2013 Geneva. 28 CHAPTER 2 phe Neuroanatomy Objectives After reading this chapter, the student will be able to: ¢ Differentiate between the central and peripheral nervous systems. ¢ Identify significant structures within the nervous system. ¢ Understand primary functions of structures within the nervous system. ¢ Describe the vascular supply to the brain. ¢ Discuss components of the cervical, brachial, and lumbosacral plexuses. 30 Introduction The purpose of this chapter is to provide the student with a review of neuroanatomy. Basic structures within the nervous system are described and their functions discussed. This information is important to physical therapists (PTs) and physical therapist assistants (PTAs) who treat patients with neurologic dysfunction because it assists clinicians with identifying clinical signs and symptoms. In addition, it allows the PTA to develop an appreciation of the patient’s prognosis and potential functional outcome. It is, however, outside the scope of this text to provide a comprehensive discussion of neuroanatomy. The reader is encouraged to review neuroscience and neuroanatomy texts for a more in-depth discussion of these concepts. 31 Major components of the nervous system The nervous system is divided into two parts, the central nervous system (CNS) and the peripheral nervous system (PNS). The CNS is composed of the brain, the cerebellum, the brain stem, and the spinal cord, whereas the PNS comprises all of the components outside the cranium and spinal cord. Physiologically, the PNS is divided into the somatic nervous system and the autonomic nervous system (ANS). Figure 2-1 illustrates the major components of the CNS. Cerebrum + ~/ Cerebral mr NN hemispheres wa : — . re > = . Diencephalon , ya Brain stem ae) | = Midbrain and cerebellum “SA. Pons Medulla a y | | ' } | | Spinal region Peripheral region _—, | | FIGURE 2-1 Lateral view of the regions of the nervous system. Regions are listed on the left, and subdivisions are listed on the right. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) The nervous system is a highly organized communication system. Nerve cells within the nervous system receive, transmit, analyze, and communicate information to other areas throughout the body. For example, sensations, such as touch, proprioception, pain, and temperature, are transmitted from the periphery as electrochemical impulses to the CNS through sensory tracts. Once information is processed within the brain, it is relayed as new electrochemical impulses to peripheral structures through motor tracts. This transmission process is responsible for an individual's ability to interact with the environment. Individuals are able to perceive sensory experiences, to initiate movement, and to perform cognitive tasks as a result of a functioning nervous system. Types of Nerve Cells The brain, brain stem, and spinal cord are composed of two basic types of nerve cells called neurons and neuroglia. Three different subtypes of neurons have been identified based on their function: (1) 32 afferent neurons; (2) interneurons; and (3) efferent neurons. Afferent or sensory neurons are responsible for receiving sensory input from the periphery of the body and transporting it into the CNS. Interneurons connect neurons to other neurons. Their primary function is to process information or transmit signals (Lundy-Ekman, 2013). Efferent/Somatic or motor neurons transmit information to the extremities to signal muscles to produce movement. Neuroglia are nonneuronal supporting cells that provide critical services for neurons. Different types of neuroglia (astrocytes, oligodendrocytes, microglia, and ependymal cells) have been identified in the CNS. Figure 2-2 depicts the types of neuroglia. Astrocytes are responsible for maintaining the capillary endothelium and as such provide a vascular link to neurons. Additionally, astrocytes contribute to the metabolism of the CNS, regulate extracellular concentrations of neurotransmitters, and proliferate after an injury to create a glial scar (Fitzgerald et al., 2012). Oligodendrocytes wrap myelin sheaths around axons in the white matter and produce satellite cells in the gray matter that participate in ion exchange between neurons. Microglia are known as the phagocytes of the CNS. They engulf and digest pathogens and assist with nervous system repair after injury. Ependymal cells assist with the movement of cerebrospinal fluid through the ventricles as these cells line the ventricular system (Fitzgerald et al., 2012). Schwann and satellite cells provide similar functions in the PNS. FIGURE 2-2 The four types of neuroglia cells: astrocytes, microglia, oligodendrocytes, and ependymal cells. (From Copstead LEC, Banasik JL: Pathophysiology: biological and behavioral perspectives, ed 2, Philadelphia, 2000, WB Saunders.) Neuron Structures As depicted in Figure 2-3, a typical neuron consists of a cell body, dendrites, and an axon. The dendrite is responsible for receiving information and transferring it to the cell body, where it is processed. Dendrites bring impulses into the cell body from other neurons. The number and arrangement of dendrites present in a neuron vary. The cell body or soma is composed of a nucleus and a number of different cellular organelles. The cell body is responsible for synthesizing proteins and supporting functional activities of the neuron, such as transmitting electrochemical impulses and repairing cells. Cell bodies that are grouped together in the CNS appear gray and thus are called gray matter. Groups of cell bodies in the PNS are called ganglia. The axon is the message- sending component of the nerve cell. It extends from the cell body and is responsible for transmitting impulses from the cell body to target cells that can include muscle cells, glands, or other neurons. 33 Dendrites Myelin sheath Nodes of Ranvier FIGURE 2-3 Diagram of a neuron. Synapses Synapses are the connections between neurons that allow different parts of the nervous system to communicate with and influence each other. The synaptic cleft is the intercellular space between the axon terminal and the postsynaptic target cell and is the site for interneuronal communication. Neurotransmitters Neurotransmitters are chemicals that are transported from the cell body and are stored in the axon terminal. Upon activation (depolarization) of the neuron, an action potential is transmitted along the axon and when it reaches the axon terminal, it causes the release of the neurotransmitter into the synaptic cleft. The neurotransmitter then binds with a receptor to elicit a change in activity of the receptor (Lundy-Ekman, 2013). An in-depth discussion of neurotransmitters is beyond the scope of this text. We will, however, discuss some common neurotransmitters because of their relationship to CNS disease. Furthermore, many of the pharmacologic interventions available to patients with CNS pathology act by facilitating or inhibiting neurotransmitter activity. Common neurotransmitters include acetylcholine, glutamate, g-aminobutyric acid (GABA), dopamine, serotonin, and norepinephrine. Acetylcholine conveys information in the PNS and is the neurotransmitter used by all neurons that synapse with skeletal muscle fibers (lower motor neurons) (Lundy-Ekman, 2013). Acetylcholine also plays a role in regulating heart rate and other autonomic functions. Glutamate is an excitatory neurotransmitter and facilitates neuronal change during development. Excessive glutamate release is also thought to contribute to neuron destruction after an injury to the CNS. GABA is the major inhibitory neurotransmitter of the brain and glycine is the major inhibitory neurotransmitter of the spinal cord. Dopamine influences motor activity, motivation, general arousal, and cognition. Serotonin plays a role in “mood, behavior, and 34 inhibits pain” (Dvorak and Mansfield, 2013). Norepinephrine is used by the ANS and produces the “fight-or-flight response” to stress (Fitzgerald et al., 2012; Lundy-Ekman, 2013). Axons Once information is processed, it is conducted to other neurons, muscle cells, or glands by the axon. Axons can be myelinated or unmyelinated. Myelin is a lipid/protein that encases and insulates the axon. Oligodendrocytes are the cells in the CNS that produce myelin, whereas Schwann cells wrap myelin around axons in the PNS. The presence of a myelin sheath increases the speed of impulse conduction, thus allowing for increased responsiveness of the nervous system. The myelin sheath surrounding the axon is not continuous; it contains interruptions or spaces within the myelin called the nodes of Ranvier. The nodes allow for impulse conduction of the action potential as these areas control ion flow. As the impulse travels down the myelinated axon, it appears to jump from one node to the next. New action potentials are generated at each node, thus creating the appearance that the impulse skips from one node to the next. This process is called saltatory conduction and increases the velocity of nervous system impulse conduction (Figure 2-4). Unmyelinated axons send messages more slowly than myelinated ones (Lundy-Ekman, 2013). Myelin Node of Ranvier B 4 ++ ™ —— - FIGURE 2-4 Saltatory conduction, or the process by which an action potential appears to jump from node to node along an axon. A, A depolarizing potential spreads rapidly along the myelinated regions of the axon, then slows when crossing the unmyelinated node of Ranvier. B, When an action potential is generated at a node of Ranvier, the depolarizing potential again spreads quickly across myelinated regions, appearing to jump from node to node. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) White Matter Areas of the nervous system with a high concentration of myelin appear white because of the fat content within the myelin. Consequently, white matter is composed of axons that carry information away from cell bodies. White matter is found in the brain and spinal cord. Myelinated axons are bundled together within the CNS to form fiber tracts. Gray Matter Gray matter refers to areas that contain large numbers of nerve cell bodies and dendrites. Collectively, these cell bodies give the region its grayish coloration. Gray matter covers the entire surface of the cerebrum and is called the cerebral cortex. The cortex is estimated to contain 50 billion neurons— approximately 500 billion neuroglial cells and a significant capillary network (Fitzgerald et al., 2012). Gray matter is also present deep within the spinal cord and is discussed in more detail later in this chapter. Fibers and Pathways Major sensory or afferent tracts carry information to the brain, and major motor or efferent tracts relay transmissions from the brain to smooth and skeletal muscles. Sensory information enters the CNS through the spinal cord or by the cranial nerves as the senses of smell, sight, hearing, touch, taste, heat, cold, pressure, pain, and movement. Information travels in fiber tracts composed of axons that ascend in a particular path from the sensory receptor to the cortex for perception, association, and interpretation. Motor signals descend from the cortex to the spinal cord through 35 efferent fiber tracts for muscle activation. Fiber tracts are designated by their point of origin and by the area in which they terminate. Thus, the corticospinal tract, the primary motor tract, originates in the cortex and terminates in the spinal cord. The lateral spinothalamic tract, a sensory tract, begins in the gray matter of the spinal cord and ascends in the lateral aspect of the cord to terminate in the thalamus. A more thorough discussion of motor and sensory tracts is presented later in this chapter. Brain The brain consists of the cerebrum, which is divided into two cerebral hemispheres (the right and the left), the cerebellum, and the brain stem. The surface of the cerebrum or cerebral cortex is composed of depressions (sulci) and ridges (gyri). These convolutions increase the surface area of the cerebrum without requiring an increase in the size of the brain. The outer surface of the cerebrum is composed of gray matter approximately 2 to 4mm thick, whereas the inner surface is composed of white matter fiber tracts (Fitzgerald et al., 2012). Information is conveyed by the white matter and is processed and integrated within the gray matter, although there are also several nuclei within the cerebral hemispheres that interconnect with the cortex and/or each other. Supportive and Protective Structures The brain is protected by a number of different structures and substances to minimize the possibility of injury. First, the brain is surrounded by a bony structure called the skull or cranium. The brain is also covered by three layers of membranes called meninges, which provide additional protection. The outermost layer is the dura mater. The dura is a thick, fibrous connective tissue membrane that adheres to the cranium. The dural covering has two distinct projections: the falx cerebri, which separates the cerebral hemispheres, and the tentorium cerebelli, which provides a separation between the posterior cerebral hemispheres and the cerebellum. The area between the dura mater and the skull is known as the epidural space. The next or middle layer is the arachnoid. The space between the dura and the arachnoid is called the subarachnoid space. The cerebral arteries are located here. The third protective layer is the pia mater. This is the innermost layer and adheres to the brain itself. The cranial meninges are continuous with the membranes that cover and protect the spinal cord. Cerebrospinal fluid bathes the brain and circulates within the subarachnoid space. Figure 2-5 shows the relationship of the skull with the cerebral meninges. 36 Arachnoid Subarachnoid space Pia Dura Cerebral mater mater hemisphere FIGURE 2-5 Coronal section through the skull, meninges, and cerebral hemispheres. The section shows the midline structures near the top of the skull. The three layers of meninges, the superior sagittal sinus, and arachnoid granulations are indicated. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) Lobes of the Cerebrum The cerebrum is divided into four lobes—frontal, parietal, temporal, and occipital—each having unique functions, as shown in Figure 2-6, A. The hemispheres of the brain, although apparent mirror images of one another, have specialized functions as well. This sidedness of brain function is called hemispheric specialization or lateralization. 37 B FIGURE 2-6 The brain. A, Left lateral view of the brain, showing the principal divisions of the brain and the four major lobes of the cerebrum. B, Sensory homunculus. C, Primary and association sensory and motor areas of the brain. (A from Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders; B and C from Cech D, Martin S: Functional movement development across the life span, ed 3, St Louis, 2012, Elsevier.) Frontal lobe The frontal lobe contains the primary motor cortex. The frontal lobe is responsible for voluntary control of complex motor activities. In addition to its motor responsibilities, the frontal lobe also exhibits a strong influence over cognitive functions, including judgment, attention, awareness, abstract thinking, mood, and aggression. The principal motor region responsible for speech (Broca’s area) is located within the frontal lobe. In the left hemisphere, Broca’s area plans movements of the mouth to produce speech. In the opposite hemisphere, this same area is responsible for nonverbal communication, including gestures and adjustments of the individual's tone of voice. Parietal lobe The parietal lobe contains the primary sensory cortex. Incoming sensory information is processed within this lobe and meaning is provided to the stimuli. Perception is the process of attaching meaning to sensory information and requires interaction between the brain, body, and the individual’s environment (Lundy-Ekman, 2013). Much of our perceptual learning requires a functioning parietal lobe. Specific body regions are assigned locations within the parietal lobe for this interpretation. This mapping is known as the sensory homunculus (Figure 2-6, B). The parietal lobe also plays a role in short-term memory functions. Temporal lobe The temporal lobe contains the primary auditory cortex. Wernicke’s area of the temporal lobe is the highest center for interpretation of all the sensory systems and allows an individual to hear and comprehend spoken language. Visual perception, musical discrimination, and long-term memory capabilities are all functions associated with the temporal lobe. Occipital lobe 38 The occipital lobe contains the primary visual cortex. The eyes take in visual signals concerning objects in the visual field and relay that information. The visual association cortex is extensive and is located throughout the cerebral hemispheres. Association Cortex Association areas are regions within the parietal, temporal, and occipital lobes that horizontally link different parts of the cortex. For example, the sensory association cortex integrates and interprets information from all the lobes receiving sensory input and allows individuals to perceive and attach meaning to sensory experiences. Additional functions of the association areas include personality, memory, intelligence, and the generation of emotions (Lundy-Ekman, 2013). Figure 2-6, C depicts association areas within the cerebral hemispheres. Motor Areas of the Cerebral Cortex The primary motor cortex, located in the frontal lobe, is primarily responsible for contralateral voluntary control of the upper and lower extremity and facial movements. Thus, a greater proportion of the total surface area of this region is devoted to neurons that control these body parts. Other motor areas include the premotor area, which controls muscles of the trunk and anticipatory postural adjustments, the supplementary motor area which controls initiation of movement, orientation of the eyes and head, and bilateral, sequential movements, and Broca’s area, which is “responsible for planning movements of the mouth during speech and the grammatical aspects of language” (Lundy-Ekman, 2013). Hemispheric Specialization The cerebrum can be further divided into the right and left cerebral hemispheres. Gross anatomic differences have been demonstrated within the hemispheres. The hemisphere that is responsible for language is considered the dominant hemisphere. Approximately 95% of the population, including all right-handed individuals, are left-hemisphere dominant. Even in individuals who are left-hand dominant, the left hemisphere is the primary speech center in about 50% of these people (Geschwind and Levitsky, 1968; Gilman and Newman, 2003; Guyton, 1991; Lundy-Ekman, 2013). Table 2-1 lists primary functions of both the left and right cerebral hemispheres. Table 2-1 Behaviors Attributed to the Left and Right Brain Hemispheres Behavior Left Hemisphere Right Hemisphere Cognition/intellect Processing information in a sequential, linear manner Processing information in a simultaneous, holistic, or gestalt manner Observing and analyzing details Grasping overall organization or pattern Perception/cognition | Processing and producing language, processing verbal cues and __| Processing nonverbal stimuli (environmental sounds, visual cues, speech intonation, complex instructions shapes, and designs) Visual-spatial perception Drawing inferences, synthesizing information Academic skills Reading: sound-symbol relationships, word recognition, reading | Mathematical reasoning and judgment comprehension Alignment of numerals in calculations Performing mathematical calculations Motor and task Planning and sequencing movements Sustaining a movement or posture, consistency in movement performance erformance Performing movements and gestures to command. Behavior and emotions] Organization, Ability to self-correct, judgment, awareness of disability and safety concerns Expressing positive emotions Expressing negative emotions and perceiving emotion (Adapted from O’Sullivan SB: Stroke. In O’Sullivan SB, Schmitz TJ, editors: Physical rehabilitation assessment and treatment, ed 4, Philadelphia, 2001, FA Davis; O’Sullivan SB: Stroke. In O’Sullivan SB, Schmitz TJ, Fulk GD, editors: Physical rehabilitation, ed 6, Philadelphia, 2014, FA Davis.) Left Hemisphere Functions The left hemisphere has been described as the verbal or analytic side of the brain. The left hemisphere allows for the processing of information in a sequential, organized, logical, and linear manner. The processing of information in a step-by-step or detailed fashion allows for thorough analysis. For the majority of people, language is produced and processed in the left hemisphere, specifically the frontal and temporal lobes. The left parietal lobe allows an individual to recognize words and to comprehend what has been read. In addition, mathematical calculations are performed in the left parietal lobe. An individual is able to sequence and perform movements and gestures as a result of a functioning left frontal lobe. A final behavior assigned to the left cerebral hemisphere is the expression of positive emotions, such as happiness and love. Common 39 impairments seen in patients with left hemispheric injury include an inability to plan motor tasks (apraxia); difficulty in initiating, sequencing, and processing a task; difficulty in producing or comprehending speech; memory impairments; and perseveration of speech or motor behaviors (O'Sullivan, 2014). Right Hemisphere Functions The right cerebral hemisphere is responsible for an individual’s nonverbal and artistic abilities. The right side of the brain allows individuals to process information in a complete or holistic fashion without specifically reviewing all the details. The individual is able to grasp or comprehend general concepts. Visual-perceptual functions including eye-hand coordination, spatial relationships, and perception of one’s position in space are carried out in the right hemisphere. The ability to communicate nonverbally and to comprehend what is being expressed is also assigned to the right parietal lobe. Nonverbal skills including understanding facial gestures, recognizing visual-spatial relationships, and awareness of body image are processed in the right side of the brain. Other functions include mathematical reasoning and judgment, sustaining a movement or posture, and perceiving negative emotions, such as anger and unhappiness (O'Sullivan, 2014). Specific deficits that can be observed in patients with right hemisphere damage include poor judgment and safety awareness, unrealistic expectations, denial of disability or deficits, disturbances in body image, irritability, and lethargy. Hemispheric Connections Even though the two hemispheres of the brain have discrete functional capabilities, they perform many of the same actions. Communication between the two hemispheres is constant, so individuals can be analytic and yet still grasp broad general concepts. It is possible for the right hand to know what the left hand is doing and vice versa. The corpus callosum is a large group of axons that connect the right and left cerebral hemispheres and allow communication between the two cortices. Deeper Brain Structures Subcortical structures lie deep within the brain and include the internal capsule, the diencephalon, and the basal ganglia. These structures are briefly discussed because of their functional significance to motor function. Internal Capsule The internal capsule contains the major projection fibers that run to and from the cerebral cortex. All descending fibers leaving the motor areas of the frontal lobe travel through the internal capsule, a deep structure within the cerebral hemisphere. The internal capsule is made up of axons that project from the cortex to the white matter fibers (subcortical structures) located below and from subcortical structures to the cerebral cortex. The capsule is shaped like a less-than sign (<) and has five regions. The anterior limb connects to the frontal cerebral cortex, the genu contains the motor fibers that are going to some of the brain stem motor nuclei, the posterior limb carries sensory signals relayed from the thalamus to the parietal cortex and the frontal signals of the corticospinal tract. The other two limbs relay visual and auditory signals from the thalamus to the occipital and temporal lobes, respectively. A lesion within this area can cause contralateral loss of voluntary movement and conscious somatosensation, which is the ability to perceive tactile and proprioceptive input. The internal capsule is pictured in Figure 2-7. 40 Corona radiata Caudate nucious White matior Corobral cortex Thatamus Corpus __ j callosum I Putamen Corona ib nea internal _ e Globus capsule ~~ pallidus (A Sy . Amygdala Mamilary body Subdthalamic nucleus Substanda nigra capsule Rf. oculomotor nerwo 8 : rene Pons Medulla Pyramid Olive Cerebellum FIGURE 2-7 The cerebrum. A, Diencephalon and cerebral hemispheres. Coronal section. B, A deep dissection of the cerebrum showing the radiating nerve fibers, the corona radiata, that conduct signals in both directions between the cerebral cortex and the lower portions of the central nervous system. (A from Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, WB Elsevier; B from Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) Diencephalon The diencephalon is situated deep within the cerebrum and is composed of the thalamus, epithalamus, and subthalamus. The diencephalon is the area where the major sensory tracts (dorsal columns and lateral spinothalamic) and the visual and auditory pathways synapse. The thalamus consists of a large collection of nuclei and synapses. In this way, the thalamus serves as a central relay station for sensory impulses traveling upward from other parts of the body and brain to the cerebrum. It receives sensory signals and channels them to appropriate regions of the cortex for interpretation. Moreover, the thalamus relays sensory information to the appropriate association areas within the cortex. Motor information received from the basal ganglia and cerebellum is transmitted to the correct motor region through the thalamus. Hypothalamus The hypothalamus is a group of nuclei that lie at the base of the brain, underneath the thalamus. The hypothalamus regulates homeostasis, which is the maintenance of a balanced internal environment. This structure is primarily involved in automatic functions, including the regulation of hunger, thirst, digestion, body temperature, blood pressure, sexual activity, and sleep-wake cycles. The hypothalamus is responsible for integrating the functions of both the endocrine system and the ANS through its regulation of the pituitary gland and its release of hormones. Basal Nuclei Another group of nuclei located at the base of the cerebrum comprise the basal ganglia. The basal ganglia form a subcortical structure made up of the caudate nucleus, putamen, globus pallidus, substantia nigra, and subthalamic nuclei. The globus pallidus and putamen form the lentiform nucleus, and the caudate and putamen are known as the neostriatum. The nuclei of the basal ganglia influence the motor planning areas of the cerebral cortex through various motor circuits. Primary responsibilities of the basal ganglia include the regulation of posture and muscle tone and the control of volitional and automatic movement. In addition to the caudate and putamen’s role in motor control, the caudate nucleus is involved in cognitive functions. The most common condition that results from dysfunction within the basal ganglia is Parkinson disease. The substantia nigra, a nucleus that is part of the basal ganglia, “loses its ability to produce dopamine, a neurotransmitter necessary to normal function of basal ganglia neurons” (Fuller et al., 2009). This can lead to symptoms of Parkinson disease, which can include bradykinesia (slowness initiating movement), akinesia (difficulty in initiating movement), tremors, rigidity, and postural instability. Limbic System The limbic system is a group of deep brain structures in the diencephalon and cortex that includes parts of the thalamus and hypothalamus and a portion of the frontal and temporal lobes. The hypothalamus and the amygdala play a role in the control of primitive emotional reactions, 41 including rage and fear. The amygdala relays signals to the limbic system. The limbic system guides the emotions that regulate behavior and is involved in learning and memory. More specifically, the limbic system appears to control memory, pain, pleasure, rage, affection, sexual interest, fear, and sorrow. Cerebellum The cerebellum controls balance and complex muscular movements. It is located below the occipital lobe of the cerebrum and is posterior to the brain stem. It fills the posterior fossa of the cranium. Like the cerebrum, it also consists of two symmetric hemispheres and a midline vermis. The cerebellum is responsible for the integration, coordination, and execution of multijoint movements. The cerebellum regulates the initiation, timing, sequencing, and force generation of muscle contractions. It sequences the order of muscle firing when a group of muscles work together to perform a movement such as stepping or reaching. The cerebellum also assists with balance and posture maintenance and has been identified as a comparator of actual motor performance to that which is anticipated. The cerebellum monitors and compares the movement requested, for instance, the step, with a movement actually performed (Horak, 1991). Brain Stem The brain stem is located between the base of the cerebrum and the spinal cord and is divided into three sections (Figure 2-8). Moving cephalocaudally, the three areas are the midbrain, pons, and medulla. Each of the different areas is responsible for specific functions. The midbrain connects the diencephalon to the pons and acts as a relay station for tracts passing between the cerebrum and the spinal cord or cerebellum. The midbrain also houses reflex centers for visual, auditory, and tactile responses. The pons contains bundles of axons that travel between the cerebellum and the rest of the CNS and functions with the medulla to regulate breathing rate. It also contains reflex centers that assist with orientation of the head in response to visual and auditory stimulation. Cranial nerve nuclei can also be found within the pons, specifically, cranial nerves V through VII, which carry motor and sensory information to and from the face. The medulla is an extension of the spinal cord and contains the fiber tracts that run through the spinal cord. Motor and sensory nuclei for the neck and mouth region are located within the medulla, as well as the control centers for heart rate and respiration. Reflex centers for vomiting, sneezing, and swallowing are also located within the medulla. Corpus callosum PARIETAL LOBE Cingulate gyrus FRONTAL LOBE —— ZEN Beau \ LIMBIC LOBE OCCIPITAL LOBE Hippocampus Thalamus DIENCEPHALON { iia Hypothalamus mygdala Pituitary gland Midbrain CEREBELLUM BRAIN STEM Pons SPINAL CORD Medulla FIGURE 2-8 Schematic midsagittal view of the brain shows the relationship between the cerebral cortex, cerebellum, spinal cord, and brain stem, and the subcortical structures important to functional movement. (From Cech D, Martin S: Functional movement development across the life span, ed 3, St Louis, 2012, Elsevier.) The reticular formation is also situated within the brain stem and extends vertically throughout its length. The system maintains and adjusts an individual's level of arousal, including sleep-wake cycles. In addition, the reticular formation facilitates the voluntary and autonomic motor responses 42 necessary for certain self-regulating, homeostatic functions and is involved in the modulation of muscle tone throughout the body. Spinal Cord The spinal cord has two primary functions: coordination of motor information and movement patterns and communication of sensory information. Subconscious reflexes, including withdrawal and stretch reflexes, are integrated within the spinal cord. Additionally, the spinal cord provides a means of communication between the brain and the peripheral nerves. The spinal cord is a direct continuation of the brain stem, specifically the medulla. The spinal cord is housed within the vertebral column and extends approximately to the level of the intervertebral disc between the first two lumbar vertebrae. The spinal cord has two enlargements—one that extends from the third cervical segment to the second thoracic segment and another that extends from the first lumbar to the third sacral segment. These enlargements accommodate the great number of neurons needed to innervate the upper and lower extremities located in these regions. At approximately the vertebral L1 level, the spinal cord becomes a cone-shaped structure called the conus medullaris. The conus medullaris is composed of sacral spinal segments. Below this level, the spinal cord becomes a mass of spinal nerve roots called the cauda equina. The cauda equina consists of the nerve roots for spinal nerves L2 through 55. Figure 2-9 depicts the spinal cord and its relation to the brain. A thin filament, the filum terminale, extends from the caudal end of the spinal cord and attaches to the coccyx. In addition to the bony protection offered by the vertebrae, the spinal cord is also covered by the same protective meningeal coverings, as in the brain. THE BRAIN iF -_ / rf [fA Frontal lobe AA Frontal lobe Motor area Parietal lobe Sensory area Occipital lobe i 5 Temporal lobe Cerebellum Medulla Cervical segment THE SPINAL CORD Thoracic segment Conus medullaris Lumbar segment aa; Sacral segment Dural sac containing cauda equina and filum terminale FIGURE 2-9 The principal anatomic parts of the nervous system. (From Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) 43 Internal Anatomy The internal anatomy of the spinal cord can be visualized in cross-sections and is viewed as two distinct areas. Figure 2-10, A illustrates the internal anatomy of the spinal cord. Like the brain, the spinal cord is composed of gray and white matter. The center of the spinal cord, the gray matter, is distinguished by its H-shaped or butterfly-shaped pattern. The gray matter contains cell bodies of motor and sensory neurons and synapses. The upper portion is known as the dorsal or posterior horn and is responsible for transmitting sensory stimuli. The lower portion is referred to as the anterior or ventral horn (Figure 2-10, B). It contains cell bodies of lower motor neurons, and its primary function is to transmit motor impulses. The lateral horn is present at the T1 to L2 levels and contains cell bodies of preganglionic sympathetic neurons. It is responsible for processing autonomic information. The periphery of the spinal cord is composed of white matter. The white matter is composed of sensory (ascending) and motor (descending) fiber tracts. A tract is a group of nerve fibers that are similar in origin, destination, and function. These fiber tracts carry impulses to and from various areas within the nervous system. In addition, these fiber tracts cross over from one side of the body to the other at various points within the spinal cord and brain. Therefore, an injury to the right side of the spinal cord may produce a loss of motor or sensory function on the contralateral side. Dorsal gray Dorsal white horn columns Ventral gray hom ——} — = : Ny i4 Ventral white column aC ol > ~ Spinal pia mater Lateral white column POSTERIOR Lateral gray horn Dorsal root filaments Subarachnoid space Spinal arachnoid Spinal dura mater a Ventral root filaments A | ANTERIOR GRAY MATTER WHITE MATTER Dorsal horn Dorsal column Lateral horn Lateral column Ventral horn Anterior column B FIGURE 2-10 The spinal cord. A, Structures of the spinal cord and its connections with the spinal nerve by way of the dorsal and ventral spinal roots. Note also the coverings of the spinal cord, the meninges. B, Cross-section of the spinal cord. The central gray matter is divided into horns and a commissure. The white matter is divided into columns. (A from Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) 44 Major Afferent (Sensory) Tracts Two primary ascending sensory tracts are present in the white matter of the spinal cord. The dorsal or posterior columns carry information about position sense (proprioception), vibration, two-point discrimination, and deep touch. Figure 2-10 shows the location of this tract. The fibers of the dorsal columns cross in the brain stem. Pain and temperature sensations are transmitted in the spinothalamic tract located anterolaterally in the spinal cord (Figure 2-11). Fibers from this tract enter the spinal cord, synapse, and cross within three segments. Sensory information must be relayed to the thalamus. Touch information has to be processed by the cerebral cortex for discrimination to occur. Light touch and pressure sensations enter the spinal cord, synapse, and are carried in the dorsal and ventral columns. Dorsal columns ¥ \ Lateral Fasciculus gracilis Posterior fissure corticospinal tract descending to skeletal muscle for voluntary movement Fasciculus cuneatus Posterior spinocerebellar tract Rubrospinal tract descending for posture and muscle coordination Anterior spinocerebellar tract ascending from proprioceptors in muscle and tendons for position sense Lateral spinothalamic tract ascending for pain ibe Reticulospinal tract temperature (fibers scattered) Vestibulospinal tract Anterior corticospinal tract Tectospinal tract Anterior median fissure FIGURE 2-11 Cross-section of the spinal cord showing tracts. (From Gould BE: Pathophysiology for the health-related professions, Philadelphia, WB Saunders, 1997.) Major Efferent (Motor) Tract The corticospinal tract is the primary motor pathway and controls skilled movements of the extremities. This tract originates in the frontal lobe from the primary and premotor cortices, descends through the internal capsule, and continues to finally synapse on anterior horn cells in the spinal cord. This tract also crosses from one side to the other in the brain stem. A common indicator of corticospinal tract damage is the Babinski sign. To test for this sign, the clinician takes a blunt object, such as the back of a pen and runs it along the lateral border of the patient’s foot (Figure 2- 12). The sign is present when the great toe extends and the other toes splay. The presence of a Babinski sign indicates that damage to the corticospinal tract has occurred. 45 —— = B FIGURE 2-12 Babinski sign. A, Normal. Stroking from the heel to the ball of the foot along the lateral sole, then across the ball of the foot, normally causes the toes to flex. B, Developmental or pathologic. Babinski sign in response to the same stimulus. In people with corticospinal tract lesions, or in infants younger than 7 months old, the great toe extends. Although the other toes may fan out, as shown, movement of the toes other than the great toe is not required for the Babinski sign. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier, 2013.) Other Descending Tracts Other descending motor pathways that affect muscle tone are the rubrospinal, lateral and medial vestibulospinal, tectospinal, and medial and lateral reticulospinal tracts. The rubrospinal tract originates in the red nucleus of the midbrain and terminates in the anterior horn, where it synapses with lower motor neurons that primarily innervate the upper extremities. Fibers from this tract facilitate flexor motor neurons and inhibit extensor motor neurons. Proximal muscles are primarily affected, although the tract does exhibit some influence over more distal muscle groups. The rubrospinal tract has been said to assist in the correction of movement errors. The lateral vestibulospinal tract assists in postural adjustments through facilitation of proximal extensor muscles. Regulation of muscle tone in the neck and upper back is a function of the medial vestibulospinal tract. The medial reticulospinal tract facilitates limb extensors, whereas the lateral reticulospinal tract facilitates flexors and inhibits extensor muscle activity. The tectospinal tract provides for orientation of the head toward a sound or a moving object. Anterior Horn Cell An anterior horn cell is a large neuron located in the gray matter of the spinal cord. An anterior horn cell sends out axons through the ventral or anterior spinal root; these axons eventually become peripheral nerves and innervate muscle fibers. Thus, activation of an anterior horn cell stimulates skeletal muscle contraction. Alpha motor neurons are a type of anterior horn cell that innervate skeletal muscle. Because of axonal branching, several muscle fibers can be innervated by one 46 neuron. A motor unit consists of an alpha motor neuron and the muscle fibers it innervates. Gamma motor neurons are also located within the anterior horn. These motor neurons transmit impulses to the intrafusal fibers of the muscle spindle and assist with maintenance of muscle tone. Muscle Spindle The muscle spindle is the sensory organ found in skeletal muscle and is composed of motor and sensory endings and muscle fibers. These fibers respond to stretch and therefore provide feedback to the CNS regarding the muscle’s length. The easiest way to conceptualize how the muscle spindle functions within the nervous system is to review the stretch reflex mechanism. Stretch or deep tendon reflexes can easily be facilitated in the biceps, triceps, quadriceps, and gastrocnemius muscles. If a sensory stimulus, such as a tap, on the patellar tendon is applied to the muscle and its spindle, the input will enter through the dorsal root of the spinal cord to synapse on the anterior horn cell (alpha motor neurons). Stimulation of the anterior horn cell elicits a motor response, such as reflex contraction of the quadriceps (extension of the knee), as information is carried through the anterior root to the skeletal muscle. An important note about stretch or deep tendon reflexes is that their activation and subsequent motor response can occur without higher cortical influence. The sensory input entering the spinal cord does not have to be transmitted to the cortex for interpretation. This has clinical implications, because it means that a patient with a cervical spinal cord injury can continue to exhibit lower extremity deep tendon reflexes despite lower extremity paralysis. PNS The PNS consists of the nerves leading to and from the CNS, including the cranial nerves exiting the brain stem and the spinal roots exiting the spinal cord, many of which combine to form peripheral nerves. These nerves connect the CNS functionally with the rest of the body through sensory and motor impulses. Figure 2-13 provides a schematic representation of the PNS and its transition to the CNS. Posterior root Spinal cord segment Primary sensory cell body Dorsal root ganglion Posterior horn Posterior primary ramus Anterior prienary ramus { \ 5°) R _ _ > < cae Brain « s* . —= > Pe | \ orn \ / ‘ - P j \ ’ -* Anterior hom j/ ‘ ¥ - y \ i Cel body AD ‘ 14>" u \ ‘ ‘ Sympathetic ‘ chain gangtion Spinal cord —- Perineurium y Epineurium - “A Nerve bundle 4 (tascicte) Motor Endoneurium Node of Myelin Rarvier sheath Motor end plate FIGURE 2-13 Schematic representation of the peripheral nervous system and the transition to the central nervous system. The PNS is divided into two primary components: the somatic (body) nervous system and the ANS. The somatic or voluntary nervous system is concerned with reactions to external stimulation. This system is under conscious control and is responsible for skeletal muscle contraction by way of the 31 pairs of spinal nerves. By contrast, the ANS is an involuntary system that innervates glands, 47 smooth (visceral) muscle, and the myocardium. The primary function of the ANS is to maintain homeostasis, an optimal internal environment. Specific functions include the regulation of digestion, circulation, and cardiac muscle contraction. Somatic Nervous System Within the PNS are 12 pairs of cranial nerves, 31 pairs of spinal nerves, and the ganglia or cell bodies associated with the cranial and spinal nerves. The cranial nerves are located in the brain stem and can be sensory or motor nerves, or mixed. Primary functions of the cranial nerves include eye movement, smell, sensation perceived by the face and tongue, auditory and vestibular functions, and innervation of the sternocleidomastoid and trapezius muscles. See Table 2-2 for a more detailed list of cranial nerves and their major functions. Table 2-2 Cranial Nerves Related Function Connection to Brain |i | Oculomotor | Moves eye up, down, medially; raises upper eyelid; constricts pupil; adjusts the shape of the lens of the eye | Midbrain (anterior) IV Trochlear Moves eye medially and down Midbrain (posterior) Benen prs ec Ben prs a Regulates viscera, swallowing, speech, taste Medulla Pxt | Accessory | Elevates shoulders, turns head Spinal cord and medulla XII Hypoglossal Moves tongue Medulla Vill Vestibulocochlear} Sensation of head position relative to gravity and head movement; hearing Between pons and medulla Ix Glossopharyngeal} Swallowing, salivation, taste Medulla (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St. Louis, 2013, Elsevier.) The spinal nerves consist of 8 cervical, 12 thoracic, 5 lumbar, and 5 sacral nerves and 1 coccygeal nerve. Cervical spinal nerves C1 through C7 exit above the corresponding vertebrae. Because there are only 7 cervical vertebrae, the C8 spinal nerve exits above the T1 vertebra. From that point on, each succeeding spinal nerve exits below its respective vertebra. Figure 2-14 shows the distribution and innervation of the peripheral nerves. 48 DERMATOMES . PERIPHERAL NERVES Pa, DERMATOMES | C2 \ Posterior rami of cervical —WY NR, le J Cervical cutaneous —————} bac CO 1 of, » pai a }+—~to™ Amery Intercostobrachial cutaneous YY | 4 y— Lateral brachial cutaneous NV | | {| Media! brachial cutaneous—\\ 4 | — Anterior thoracic rami 1} V Posterior brachial cutaneous —— \\ : Lateral thoracic rami. | Posterior thoracic rami———+——++——+— Medial antebrachial cutaneous ———,_} Posterior lumbar rami Musculocutaneous: | | Posterior antebrachial cutaneous—— | Uf : lioinguina > —- 4 | ’ Ulnar 1 /| 4 ) Radial ‘ | \ + Median . } y | NU — N= Lumboinguinal tit \ i¥ Posterior sacral rami \ | \ Lateral femoral cutaneous } \\o Anterior femoral cutaneous | NY ; Obturator \ \ Posterior femoral cutaneous ———— | {> Common peroneal | Saphenous Superficial ‘aiid | | y | \L4—t} | \ } Sural 4 | AP \L4- We f \ y | a } c Deep peroneal y J FIGURE 2-14 Dermatomes and cutaneous distribution of peripheral nerves. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 3, Philadelphia, 2007, WB Saunders.) Spinal nerves, consisting of sensory (posterior or dorsal root) and motor (anterior or ventral root) components, exit the intervertebral foramen. The region of skin innervated by sensory afferent fibers from an individual spinal nerve is called a dermatome. Myotomes are a group of muscles innervated by a spinal nerve. Once through the foramen, the spinal nerve divides into two primary rami. This division represents the beginning of the PNS. The dorsal or posterior rami innervate the paravertebral muscles, the posterior aspects of the vertebrae, and the overlying skin. The ventral or anterior primary rami innervate the intercostal muscles, the muscles and skin in the extremities, and the anterior and lateral trunk. The 12 pairs of thoracic nerves do not join with other nerves and maintain their segmental relationship. However, the anterior primary rami of the other spinal nerves join together to form local networks known as the cervical, brachial, and lumbosacral plexuses (Guyton, 1991). The reader is given only a brief description of these nerve plexuses, because a detailed description of these structures is beyond the scope of this text. Cervical plexus The cervical plexus is composed of the C1 through C4 spinal nerves. These nerves primarily innervate the deep muscles of the neck, the superficial anterior neck muscles, the levator scapulae, and portions of the trapezius and sternocleidomastoid. The phrenic nerve, one of the specific nerves within the cervical plexus, is formed from branches of C3 through C5. This nerve innervates the diaphragm, the primary muscle of ventilation, and is the only motor and main sensory nerve for this muscle (Guyton, 1991). Figure 2-15 identifies components of the cervical plexus. 49 To rectus lateralis ileal = C1 To rectus capitis anterior and Lesser occipita' a longus capitis To vagus \V To longus capitis and longus colli Great auricular ——— To longus capitis, longus colli, and scalenus medius To geniohyoid , d ss To thyrohyoid To sternocleidomastoid To levator scapulee Transverse cutaneous nerve of neck — AN To levator scapulae : YA C5 To scalenus medius N Phrenic nerve Supraclavicular FIGURE 2-15 The cervical plexus and its branches. (From Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) Brachial plexus The anterior primary rami of C5 through T1 form the brachial plexus. The plexus divides and comes together several times, providing muscles with motor and sensory innervation from more than one spinal nerve root level. The five primary nerves of the brachial plexus are the musculocutaneous, axillary, radial, median, and ulnar nerves. Figure 2-16 depicts the constituency of the brachial plexus. These five peripheral nerves innervate the majority of the upper extremity musculature, with the exception of the medial pectoral nerve (C8), which innervates the pectoralis muscles; the subscapular nerve (C5 and C6), which innervates the subscapularis; and the thoracodorsal nerve (C7), which supplies the latissimus dorsi muscle (Guyton, 1991). 50 From C4 | Lt Dorsal scapular nerve cs To phrenic nerve Suprascapular nerve ‘ J To scaleni Nerve to subclavius t— C.6 To scaleni Lateral pectoral nerve C.7 To scaleni Long thoracic nerve Posterior cord c8 A| & T i Musculocutaneous nerve o/ Y baat G7 TA a Lateral cord From T.2 Radial jadial nerve First intercostal nerve Z 2 Thoracodorsal Medial Median nerve nerve pectoral nerve ul é Lower ner nerve _ subscapular nerve Medial Medial cutaneous / Medial cutaneous cord Upper subscapular nerve of forearm nerve of arm nerve FIGURE 2-16 The brachial plexus and its branches. (From Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) The musculocutaneous nerve innervates the forearm flexors. The elbow, wrist, and finger extensors are innervated by the radial nerve. The median nerve supplies the forearm pronators and the wrist and finger flexors, and it allows thumb abduction and opposition. The ulnar nerve assists the median nerve with wrist and finger flexion, abducts and adducts the fingers, and allows for opposition of the fifth finger (Guyton, 1991). Lumbosacral Plexus Although some authors discuss the lumbar and sacral plexuses separately, they are discussed here as one unit, because together they innervate lower extremity musculature. The anterior primary rami of L1 through S3 form the lumbosacral plexus. This plexus innervates the muscles of the thigh, lower leg, and foot. This plexus does not undergo the same separation and reuniting as does the brachial plexus. The lumbosacral plexus has eight roots, which eventually form six primary peripheral nerves: obturator, femoral, superior gluteal, inferior gluteal, common peroneal, and tibial. The sciatic nerve, which is frequently discussed in physical therapy practice, is actually composed of the common peroneal and tibial nerves encased in a sheath. This nerve innervates the hamstrings and causes hip extension and knee flexion. The sciatic nerve separates into its components just above the knee (Guyton, 1991). The lumbosacral plexus is shown in Figures 2-17 and 2-18. ol From 12th thoracic Hiohypogastric Hioinguinal Genitofemoral Lateral cutaneous of thigh To psoas and iliacus To sacral plexus Femoral nerve Obturator FIGURE 2-17 The lumbar plexus and its branches, especially the femoral nerve. (From Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) a2 Superior gluteal nerve Inferior gluteal nerve To piriformis Parasympathetic branch Parasympathetic branches S.4 Sciatic nerve —Yf A Common peroneal nerve y S.5 Tibial nerve a Pudendal nerve Posterior femoral Pelvic cutaneous nerve parasympathetic To levator ani, coccygeus, and sphincter ani externus FIGURE 2-18 The sacral plexus and its branches, especially the sciatic nerve. (From Guyton AC: Basic neuroscience: anatomy and physiology, ed 2, Philadelphia, 1991, WB Saunders.) nerves Peripheral Nerves Two major types of nerve fibers are contained in peripheral nerves: motor (efferent) and sensory (afferent) fibers. Motor fibers have a large cell body with multiple branched dendrites and a long axon. The cell body and the dendrites are located within the anterior horn of the spinal cord. The axon exits the anterior horn through the white matter and is located with other similar axons in the anterior root, which is located outside the spinal cord in the intervertebral foramen. The axon then eventually becomes part of a peripheral nerve and innervates a motor end plate in a muscle. The sensory neuron, however, has a peripheral axon that innervates the receptors in the skin, muscle, or viscera. This travels in the peripheral nerve and its cell body is the dorsal root ganglion. The central axons of these cells form the dorsal roots that enter the spinal cord. An example is the Golgi tendon organ, which is innervated by a large myelinated axon (Figure 2-19). Golgi tendon organs are encapsulated nerve endings found at the musculotendinous junction. They are sensitive to tension within muscle tendons and transmit this information to the spinal cord. The axon travels through the dorsal (posterior) root of a spinal nerve and into the spinal cord through the dorsal horn. The axon may terminate at this point, or it may enter the white matter fiber tracts and ascend to a different level in the spinal cord or brain stem. Thus, a sensory neuron sends information from the periphery to the spinal cord. 53 Vertebral lamina Dura mater Arachnoid | eiae Piamater Dorsal ramus Ventral ramus Dorsal root ganglion Ventral root Rami communicantes Vertebral body A Gray matter: White matter: Dorsal horn Dorsal column Lateral horn Lateral column Ventral horn Anterior column B 54 Afferent axon Efferent axon Abductor digiti minimi muscle FIGURE 2-19 A, Spinal region: horizontal section, including vertebra, spinal cord and roots, the spinal nerve, and rami. Afferent and efferent neurons are illustrated on the left side. The spinal nerve is formed of axons from the dorsal and ventral roots. The bifurcation of the spinal nerve into dorsal and ventral rami marks the transition from the spinal to the peripheral region. B, Cross-section of the spinal cord. The central gray matter is divided into horns and a commissure. The white matter is divided into columns. C, Afferent and efferent axons in the upper limb. A single segment is illustrated. The arrows illustrate the direction of information in relation to the central nervous system. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) Autonomic Nervous System Functions of the ANS include the regulation of “circulation, respiration, metabolism, secretion, body temperature, and reproduction” (Lundy-Ekman, 2013). Control centers for the ANS are located in the hypothalamus and the brain stem. The ANS is composed of motor neurons located within spinal nerves that innervate smooth muscle, cardiac muscle, and glands, which are also called effectors or target organs. The ANS is divided into the sympathetic and parasympathetic divisions. Both the sympathetic and parasympathetic divisions innervate internal organs, use a two- neuron pathway and one-ganglion impulse conduction, and function automatically. Autoregulation is achieved by integrating information from peripheral afferents with information from receptors within the CNS. The two-neuron pathway (preganglionic and postganglionic neurons) provides the connection from the CNS to the autonomic effector organs. Cell bodies of the preganglionic neurons are located within the brain or spinal cord. The myelinated axons exit the CNS and synapse on the neurons in the peripheral ganglia. The axons of these cell bodies form the unmyelinated postganglionic axons, whereas innervate the target cell of the effector organ (Farber, 1982; Lundy- Ekman, 2013). Figure 2-20 provides a schematic representation of this organization, while Figure 2- 21 shows the influence of the sympathetic and parasympathetic divisions on effector organs. 55 CENTRAL NERVOUS SYSTEM EFFECTOR ORGANS Motoneu: Somatic wee ACH Skeletal muscle Preganglionic Postganglionic a Sympathetic $< a gp er Smooth muscle, glands clei =e acl) Sweat glands* Preganglionic st ionic Parasympatheti¢, @ ACC each Smooth muscle, glands Preganglionic To circulation Epinephrine (80%) Adren; oo ———_———_—> é , mints vi Norepinephrine (20%) Adrenal medulla FIGURE 2-20 Organization of the autonomic nervous system. (From Cech D, Martin S: Functional movement development across the life span, ed 3, St Louis, 2012, Elsevier.) Brain stem Parasympathetic fibers — CRANIAL NERVES III, Vil, IX, X Phrenic nerve to diaphragm — RESPIRATION ARMS Intercostal muscles — RESPIRATION Sympathetic nervous system — * HEART * BLOOD VESSELS * TEMPERATURE LEGS * EXTERNAL GENITALIA FIGURE 2-21 Functional areas of the spinal cord. (From Gould BE: Pathophysiology for the health-related professions, Philadelphia, 1997, WB Saunders.) The sympathetic fibers of the ANS arise from the thoracic and lumbar portions of the spinal cord. Axons of preganglionic neurons terminate in either the sympathetic chain or the prevertebral ganglia located in the abdomen. The sympathetic division of the ANS assists the individual in 56 responding to stressful situations and is often referred to as the “fight-or-flight response.” Sympathetic responses help the individual to prepare to cope with the stimulus by maintaining an optimal blood supply. Activation of the sympathetic system stimulates smooth muscle in the blood vessels to contract, thereby causing vasoconstriction. Norepinephrine, also known as noradrenaline, is the major neurotransmitter responsible for this action. Consequently, heart rate and blood pressure are increased as the body prepares for a fight or to flee a dangerous situation. Blood flow to muscles is increased as it is diverted from the gastrointestinal tract. The parasympathetic division maintains vital bodily functions or homeostasis. The parasympathetic division receives its information from the brain stem, specifically cranial nerves II] (oculomotor), VII (facial), IX (glossopharyngeal), and X (vagus), and from lower sacral segments of the spinal cord. The vagus nerve is a parasympathetic preganglionic nerve. Motor fibers within the vagus nerve innervate the myocardium and the smooth muscles of the lungs and digestive tract. Activation of the vagus nerve can produce the following effects: bradycardia, decreased force of cardiac muscle contraction, bronchoconstriction, increased mucous production, increased peristalsis, and increased glandular secretions. Efferent activation of the sacral components results in emptying of the bowel and bladder and arousal of sexual organs. Acetylcholine is the chemical transmitter responsible for sending nervous system impulses to effector cells in the parasympathetic division. Acetylcholine is used for both divisions at the preganglionic synapse and dilates arterioles. Thus, activation of the parasympathetic division produces vasodilation. When an individual is calm, parasympathetic activity decreases heart rate and blood pressure and signals a return of normal gastrointestinal activity. Figures 2-22 and 2-23 show the influence of the sympathetic and parasympathetic divisions on effector organs (Lundy-Ekman, 2013). Eyelid Pupillary dilation Facial artery Arteries of upper limb Trachea Superior cervical Middle cervical Stetate ganglion Arteries of lower limb Pancreas intestine External 3) genitals FIGURE 2-22 Efferents from the spinal cord to sympathetic effector organs. A, Direct, one-neuron connections to the adrenal medulla. B, Two-neuron pathways to the periphery and thoracic viscera, with synapses in paravertebral ganglia. C, Two-neuron pathways to the abdominal and pelvic organs, with synapses in outlying ganglia. Note that all sympathetic presynaptic neurons originate in the thoracic cord and the lumbar cord. (From Lundy-Ekman L: 57 Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) Ciliary muscle pupil K/ Lacrimal gland &) Salivary gland Trachea Heart Stomach Liver | €/— Pancreas ie Kidney fs (\ Intestine = _—— s4 |} of 7 External genitals FIGURE 2-23 Parasympathetic outflow through cranial nerves III, VII, IX, and X and S2—S4. Note that all parasympathetic preganglionic neurons originate in the brainstem or the sacral spinal cord. (From Lundy-Ekman, L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) Higher levels within the CNS also exert influence over the ANS. The region most closely associated with this control is the hypothalamus, which regulates functions such as digestion and controls heart and respiration rates. Cerebral Circulation A final area that must be reviewed when discussing the nervous system is the circulation to the brain. The cells within the brain completely depend on a continuous supply of blood for glucose and oxygen. The neurons within the brain are unable to carry out glycolysis and to store glycogen. It is therefore absolutely essential that these neurons receive a constant supply of blood. Knowledge of cerebrovascular anatomy is the basis for understanding the clinical manifestations, diagnosis, and management of patients who have sustained cerebrovascular accidents and traumatic brain injuries. Anterior Circulation All arteries to the brain arise from the aortic arch. The first major arteries ascending anteriorly and laterally within the neck are the common carotid arteries. The carotid arteries are responsible for supplying the bulk of the cerebrum with circulation. The right and left common carotid arteries bifurcate just behind the posterior angle of the jaw to become the external and internal carotids. The external carotid arteries supply the face, whereas the internal carotids enter the cranium and supply the cerebral hemispheres, including the frontal lobe, the parietal lobe, and parts of the temporal and 58 occipital lobes. In addition, the internal carotid artery supplies the optic nerves and the retina of the eyes. At the base of the brain, each of the internal carotids bifurcate into the right and left anterior and middle cerebral arteries. The middle cerebral artery is the largest of the cerebral arteries and is most often occluded. It is responsible for supplying the lateral surface of the brain with blood and also the deep portions of the frontal and parietal lobes. The anterior cerebral artery supplies the superior border of the frontal and parietal lobes. Both the middle cerebral artery and the anterior cerebral artery make up what is called the anterior circulation to the brain. Figures 2-24 and 2-25 depict the cerebral circulation. Anterior cerebral artery Anterior communicating artery Internal carotid artery Posterior cerebral artery Middle cerebral artery Superior Posterior communicatin cerebellar artery 9 artery Basilar artery Anterior inferior cerebellar artery Posterior inferior cerebellar artery Vertebral artery FIGURE 2-24 Arterial supply to the brain. The posterior circulation, supplied by the vertebral arteries is labeled on the left. The anterior circulation, supplied by the internal carotids, is labeled on the right. The watershed area, supplied by small anastomoses at the ends of the large cerebral arteries, is indicated by dotted lines. (From Lundy- Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) 59 Anterior cerebral artery Posterior Posterior cerebral artery Middle cerebral artery B FIGURE 2-25 Arterial supply to the cerebral hemispheres. The large cerebral arteries: anterior, middle, and posterior. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 2, St Louis, 2002, Elsevier.) Posterior Circulation The posterior circulation is composed of the two vertebral arteries, which are branches of the subclavian. The vertebral arteries supply blood to the brain stem and cerebellum. The vertebral arteries leave the base of the neck and ascend posteriorly to enter the skull through the foramen magnum. The two vertebral arteries supply the medulla and upper spinal cord and fuse to form the basilar artery. The basilar artery supplies the pons, cerebellum and then divides into the right and left posterior cerebral arteries. The posterior cerebral artery connects to the carotid system via the posterior communicating artery. Both of these supply the structures of the midbrain. The posterior cerebral artery then continues to supply the occipital and temporal lobes. The anterior and posterior communicating arteries, which are branches of the carotid, are interconnected at the base of the brain and form the circle of Willis. This connection of blood vessels provides a protective mechanism to the structures within the brain. Because of the circle of Willis, failure or occlusion of one cerebral artery does not critically decrease blood flow to that region. Consequently, the occlusion can be circumvented or bypassed to meet the nutritional and metabolic needs of cerebral tissue. 60 Reaction to injury What happens when the CNS or the PNS is injured? The CNS and the PNS are prone to different types of injury, and each system reacts differently. Within the CNS, artery obstruction of sufficient duration produces cell and tissue death within minutes. Neurons that die because they are deprived of oxygen do not possess the capacity to regenerate. Neurons in the vicinity of damage are also at risk of injury secondary to the release of glutamate, an excitatory neurotransmitter. At normal levels, glutamate assists with CNS functions; however, at higher levels glutamate can be toxic to neurons and can promote neuronal death. The presence of excessive glutamate also facilitates calcium release, which ultimately produces excitotoxicity including the liberation of calcium- dependent digestive enzymes, cellular edema, cell injury, and death (Lundy-Ekman, 2013). For many years, it was thought that brain injuries were permanent and that there was little opportunity for repair. This viewpoint is no longer considered accurate as our understanding of neural plasticity has evolved. Neuroplasticity is the brain’s ability to adapt and for neurons “to alter their structure and function in response to a variety of internal and external pressures, including behavioral training” (Kleim and Jones, 2008). Neural regeneration, activation of previously inactive areas, and axonal and collateral sprouting can all lead to improved brain function. As clinicians, we must design treatment sessions that will maximize CNS recovery. Conversely, peripheral nerve injuries often result from means other than vascular compromise. Common causes of peripheral nerve injuries include stretching, laceration, compression, traction, disease, chemical toxicity, and nutritional deficiencies. Patient findings can include paresthesia (pins and needles sensations), sensory loss, and muscle weakness. The response of a peripheral nerve to the injury is different from that in the CNS. If the cell body is destroyed, regeneration is not possible. The axon undergoes necrosis distal to the site of injury, the myelin sheath begins to pull away, and the Schwann cells phagocytize the area, producing Wallerian degeneration (Figure 2-26). If the damage to the peripheral nerve is not too significant and involves only the axon, regeneration is possible. Axonal sprouting from the proximal end of the damaged axon can occur. The axon regrows at the rate of 1.0 mm per day, depending on the size of the nerve fiber (Dvorak and Mansfield 2013). To have return of function, the axon must grow and reinnervate the appropriate muscle. Failure to do so results in degeneration of the axonal sprout. The rate of recovery from a peripheral nerve injury depends on the age of the patient and the distance between the lesion and the destination of the regenerating nerve fibers. A discussion of the physical therapy management of peripheral nerve injuries is beyond the scope of this text. 61 . Presynaptic axon \. (o \ [20 Pa \ = INES terminals retract—>—_ _/ 24 — A q = J \ < 7 all O \ { Chromatolysis of us L~ rN cell body ———— é ‘i \ { \ _ y- N ‘\ / Axon lesion ~ Myelin degeneration ———— Distal axon and terminal degenerates Muscle fibers FIGURE 2-26 Wallerian degeneration. A, Normal synapses before an axon is severed. B, Degeneration following severance of an axon. Degeneration following axonal injury involves several changes: (1) the axon terminal degenerates; (2) myelin breaks down and forms debris; and (3) the cell body undergoes metabolic changes. Subsequently, (4) presynaptic terminals retract from the dying cell body, and (5) postsynaptic cells degenerate. (From Lundy-Ekman L: Neuroscience: fundamentals for rehabilitation, ed 4, St Louis, 2013, Elsevier.) Injury to a motor neuron can result in variable findings. If an individual experiences damage to the corticospinal tract from its origin in the frontal lobe to its end within the spinal cord, the patient is classified as having an upper motor neuron injury. Clinical signs of an upper motor neuron injury include spasticity (velocity-dependent, increased resistance to passive stretch), hyperreflexia, the presence of a Babinski sign, and possible clonus. Clonus is a repetitive stretch reflex that is elicited by passive dorsiflexion of the ankle or passive wrist extension. If the injury is to the anterior horn cell, the motor nerve cells of the brain stem, the spinal root, or the spinal nerve, the patient is recognized as having a lower motor neuron injury. Clinical findings of this type of injury include flaccidity, marked muscle atrophy, muscle fasciculations, and hyporeflexia. Chapter summary An understanding of the structures and functions of the nervous system is necessary for physical therapists and physical therapist assistants. This knowledge assists practitioners in working with patients with neuromuscular dysfunction, because it allows the therapist to have a better appreciation of the patient’s pathologic condition, deficits, and potential capabilities. In addition, an understanding of neuroanatomy is helpful when educating patients and their families regarding the patient’s condition and possible prognosis. Review questions 1. Describe the major components of the nervous system. 62 2. What is the function of the white matter? 3. What are some of the primary functions of the parietal lobe? 4, What is Broca’s aphasia? 5. Discuss the primary function of the thalamus. 6. What is the primary function of the corticospinal tract? 7. What is an anterior horn cell? Where are these cells located? 8. Discuss the components of the PNS. 9. Where is the most common site of cerebral infarction? 10. What are some clinical signs of an upper motor neuron injury? 63 References Dvorak L, Mansfield PJ. Essentials of neuroanatomy for rehabilitation. Boston: Pearson; 2013 pp 50-74, 141-143. Farber SD. Neurorehabilitation: a multisensory approach. Philadelphia: WB Saunders; 1982 pp 1- Be. FitzGerald MJT, Gruener G, Mtui E. Clinical neuroanatomy and neuroscience. St Louis: Elsevier; 2012 pp 78, 97-110, 299. Fuller KS, Winkler PA, Corboy JR. Degenerative diseases of the central nervous system. In: Goodman CC, Fuller KS, eds. Pathology for the physical therapist. 3 ed. St Louis: Saunders/Elsevier; 2009:1439. Geschwind N, Levitsky W. Human brain: Left-right asymmetries in temporal speech regions. Science. 1968;161:186-187. Gilman 5, Newman SW. Manter and Gatz’s essentials of clinical neuroanatomy and neurophysiology. ed 10 Philadelphia: FA Davis; 2003 pp 1-11, 61-63, 147-154, 190-203. Guyton AC. Basic neuroscience: anatomy and physiology. ed 2 Philadelphia: WB Saunders; 1991 pp 1-24, 39-54, 244-245. Horak FB. Assumptions underlying motor control for neurologic rehabilitation. In: Contemporary management of motor control problems: proceedings of the II step conference; Alexandria, VA: Foundation for Physical Therapy; 1991:11-27. Kleim JA, Jones TA. Principles of experience-dependent neural plasticity: implications for rehabilitation after brain damage. J Speech Lang Hearing Res. 2008;51:5225-S239. Lundy-Ekman L. Neuroscience: fundamentals for rehabilitation. ed 4 St Louis: Elsevier; 2013 pp 35, 36, 53-65, 70-77, 153-170, 416-426. O'Sullivan SB. Stroke. In: O’Sullivan SB, Schmitz TJ, Fulk GD, eds. Physical rehabilitation. 4 ed. Philadelphia: FA Davis; 2014:659. 64 CHAPTER 3 65 Motor Control and Motor Learning Objectives After reading this chapter, the student will be able to: 1. Define motor control, motor learning, and neural plasticity. 2. Understand the relationship among motor control, motor learning, and motor development. 3. Differentiate models of motor control and motor learning. 4. Understand the development of postural control and balance. 5. Discuss the role of experience and feedback in motor control and motor learning. 6. Relate motor control, motor learning, and neural plasticity principles to therapeutic intervention. 66 Introduction Motor abilities and skills are acquired during the process of motor development through motor control and motor learning. Once a basic pattern of movement is established, it can be varied to suit the purpose of the task or the environmental situation in which the task takes place. Early motor development displays a fairly predictable sequence of skill acquisition through childhood. However, the ways in which these motor abilities are used for function are highly variable. Individuals rarely perform a movement exactly the same way every time. Variability must be part of any model used to explain how posture and movement are controlled. Any movement system must be able to adapt to the changing demands of the individual mover and the environment in which the movement takes place. The individual mover must be able to learn from prior movement experiences. Different theories of motor control emphasize different developmental aspects of posture and movement. Development of postural control and balance is embedded in the development of motor control. Understanding the relationship among motor control, motor learning, and motor development provides a valuable framework to understand the treatment of individuals with neurologic dysfunction at any age. Motor development is a product as well as a process. The products of motor development are the milestones of the developmental sequence and the kinesiologic components of movement such as head and trunk control necessary for these motor abilities. These products are discussed in Chapter 4, The process of motor development is the way in which those abilities emerge. The process and the product are affected by many factors such as time (age), maturation (genes), adaptation (physical constraints), and learning. Motor development is the result of the interaction of the innate or built-in species blueprint for posture and movement and the person’s experiences with movement afforded by the environment. Sensory input is needed for the mover to learn about moving and the results of moving. This sensory input contributes to perceptual development because perception is the act of attaching meaning to sensation. Motor development is the combination of the nature of the mover and the nurture of the environment. Part of the genetic blueprint for movement is the means to control posture and movement. Motor development, motor control, and motor learning contribute to an ongoing process of change throughout the life span of every person who moves. 67 Motor control Motor control, the ability to maintain and change posture and movement, is the result of a complex set of neurologic and mechanical processes. Those processes include motor, cognitive, and perceptual development. Motor control begins with the control of self-generated movements and proceeds to the control of movements in relationship to changing demands of the task and the environment. Control of self-movement largely results from the development of the neuromotor systems. As the nervous and muscular systems mature, movement emerges. The perceptual consequences of self-generated movements drive motor development (Anderson et al., 2014). Motor control allows the nervous system to direct what muscles should be used, in what order, and how quickly, to solve a movement problem. The infant's first movement problem relates to overcoming the effects of gravity. A second but related problem is how to move a larger head as compared with a smaller body to establish head control. Later, movement problems are related to controlling the interaction between stability and mobility of the head, trunk, and limbs. Control of task-specific movements, such as stringing beads or riding a tricycle, depends on cognitive and perceptual abilities. The task to be carried out by the person within the environment dictates the type of movement solution that is going to be needed. Because the motor abilities of a person change over time, the motor solutions to a given motor problem may also change. The motivation of the individual to move may also change over time and may affect the intricacy of the movement solution. An infant encountering a set of stairs sees a toy on the top stair. She creeps up the stairs but then has to figure out how to get down. She can cry for help, bump down on her buttocks, creep down backward, or even attempt creeping down forward. A toddler faced with the same dilemma may walk up the same set of stairs one step at a time holding onto a railing, and descend in sitting holding the toy, or may be holding the toy with one hand and the railing with the other and descend the same way she came up the stairs. An older child will walk up and down without holding on, and an even older child may run up those same stairs. The relationship among the task, the individual, and the environment is depicted graphically in Figure 3-1. All three components must be considered when thinking about motor control of movement. MOTOR CONTROL FIGURE 3-1 Movement emerges from an interaction between the individual, the task, and the environment. (From Shumway-Cook A, Woollacott MH: Motor control: theory and practical applications, ed 4, Baltimore, 2012, Williams & Wilkins.) 68 Motor Control Time Frame Motor control happens not in the space of days or weeks, as is seen in motor development, but in fractions of seconds. Figure 3-2 illustrates a comparison of time frames associated with motor control, motor learning, and motor development. Motor control occurs because of physiologic processes that happen at cellular, tissue, and organ levels. Physiologic processes have to happen quickly to produce timely and efficient movement. What good does it do if you extend an outstretched arm after falling down? Extending your arm in a protective response has to be quick enough to be useful, that is, to break the fall. People with nervous system disease may exhibit the correct movement pattern, but they have impaired timing, producing the movement too slowly to be functional, or they have impaired sequencing of muscle activation, producing a muscle contraction at the wrong time. Both of these problems, impaired timing and impaired sequencing, are examples of deficits in motor control. Control Milliseconds Learning Hours, days, weeks Development Months, years, decades FIGURE 3-2 Time scales of interest from a motor control, motor learning, and motor development perspective. (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, St. Louis, 2012, Elsevier.) Role of Sensation in Motor Control Sensory information plays an important role in motor control. Initially, sensation cues reflexive movements in which few cognitive or perceptual abilities are needed. A sensory stimulus produces a reflexive motor response. Touching the lip of a newborn produces head turning, whereas stroking a newborn’s outstretched leg produces withdrawal. Sensation is an ever-present cue for motor behavior in the seemingly reflex-dominated infant. As voluntary movement emerges during motor development, sensation provides feedback accuracy for hand placement during reaching and later for creeping. Sensation from weight bearing reinforces maintenance of developmental postures such as the prone on elbows position and the hands and knees position. Sensory information is crucial to the mover when interacting with objects and maneuvering within an environment. Figure 3-3 depicts how sensation provides the necessary feedback for the body to know whether a task such as reaching or walking was performed and how well it was accomplished. Sensory experience contributes to development of postural control and motor skill acquisition. 69 a Touch Communication Aaa. Contact with support surface <(@)s 4 Sight %, Position in space Communication Fo, = Motor Movement response output pr Sound & g Corrmunication Balance Joints and muscles Position in space Weight bearing FIGURE 3-3 Sources of sensory feedback. Role of Feedback Feedback is a very crucial feature of motor control. Feedback is defined as sensory or perceptual information received as a result of movement. There is intrinsic feedback, or feedback produced by the movement. Sensory feedback can be used to detect errors in movement. Feedback and error signals are important for two reasons. First, feedback provides a means to understand the process of self-control. Reflexes are initiated and controlled by sensory stimuli from the environment surrounding the individual. Motor behavior generated from feedback is initiated as a result of an error signal produced by a process within the individual. The highest level of many motor hierarchies is a volitional, or self-control function, but there has been very little explanation of how it works. Second, feedback also provides the fundamental process for learning new motor skills. Intrinsic feedback comes from any sensory source from inside the body such as from proprioceptors or outside the body when the person sees that the target was not hit or the ball was hit out of bounds (Schmidt and Wrisberg, 2004). Extrinsic feedback is extra or augmented sensory information given to the mover by some external source (Schmidt and Wrisberg, 2004). A therapist or coach may provide enhanced feedback of the person’s motor performance. For this reason, feedback is a common element in motor control and motor-learning theories. Theories of Motor Control Early theories of motor control were first presented in the 1800s. Sherrington proposed a reflex model in which sequences of reflexes were chained together to produce movement. Reflexes were thought of as the building blocks of more complex movements. Other traditional theories were predicated on the hierarchical organization of the nervous system in which reflexes and reactions were assigned to different levels of the nervous system. More recent theories include the motor program and systems views. These will be briefly discussed. Reflex and Hierarchical Theories Many theories of motor control exist, but these two are the most traditional ones. A top-down perspective is characteristics of these theories. The cortex of the brain is seen as the highest level of control, with all subcortical structures taking orders from it. The cortex can and does direct movement. A person can generate an idea about moving in a certain way and the nervous system carries out the command. The ultimate level of motor control, voluntary movement, is achieved by maturation of the cortex. A relationship exists between the maturation of the developing brain and the emergence of motor 70 behaviors seen in infancy. One of the ways in which nervous system maturation has been routinely gauged is by the assessment of reflexes. The reflex is seen as the basic unit of movement in this motor control model. Movement is acquired from the chaining together of reflexes and reactions. A reflex is the pairing of a sensory stimulus with a motor response, as shown in Figure 3-4. Some reflexes are simple and others are complex. The simplest reflexes occur at the spinal cord level. An example of a spinal cord level reflex is the flexor withdrawal. A touch or noxious stimulus applied to the bottom of the foot produces lower extremity withdrawal. These reflexes are also referred to as primitive reflexes because they occur early in the life span of the infant. Another example is the palmar grasp. Primitive reflexes are listed in Table 3-1. Interneuron Sensory neuron Muscle Motor neuron FIGURE 3-4 Three-neuron nervous system. (Redrawn from Romero-Sierra C: Neuroanatomy: a conceptual approach, New York, 1986, Churchill Livingstone.) Table 3-1 Primitive Reflexes Reflex Age at Onset Integration Suck-swallow 28 weeks’ gestation] 2-5 months Rooting 28 weeks’ gestation] 3 months Flexor withdrawal 28 weeks’ gestation] 1-2 months Crossed extension 28 weeks’ gestation] 1-2 months Moro 28 weeks’ gestation] 4-6 months Plantar gras, 28 weeks’ gestation| 9 months Positive support 35 weeks’ gestation| 1-2 months Asymmetric tonic neck] Birth 4—6 months Palmar grasp Birth 9 months Symmetric tonic neck_| 4-6 months 8-12 months From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, St. Louis, 2012, Elsevier, p. 54. The next higher level of reflexes comprises the tonic reflexes, which are associated with the brain stem of the central nervous system. These reflexes produce changes in muscle tone and posture. Examples of tonic reflexes exhibited by infants are the tonic labyrinthine reflex and the asymmetric tonic neck reflex. In the latter, when the infant’s head is turned to the right, the infant’s right arm extends and the left arm flexes. The tonic labyrinthine reflex produces increased extensor tone when the infant is supine and increased flexor tone in the prone position. In this model, most infantile reflexes (sucking and rooting), primitive spinal cord reflexes, and tonic reflexes are integrated by 4 to 6 months. Exceptions do exist. Integration is the mechanism by which less mature responses are incorporated into voluntary movement. Nervous system maturation is seen as the ultimate determinant of the acquisition of postural control. As the infant develops motor control, brain structures above the spinal cord begin to control posture and movement until reactive balance reactions are developed. These are the righting, protective, and equilibrium reactions. Righting and equilibrium reactions are complex postural responses that continue to be present even in adulthood. These postural responses involve the head and trunk and provide the body with an automatic way to respond to movement of the center of gravity within and outside the body’s base of support. Extremity movements in response to quick displacements of the center of gravity out of the base of support are called protective reactions. These are also considered postural 71 reactions and serve as a back-up system should the righting or equilibrium reaction fail to compensate for a loss of balance. According to the hierarchic model of motor control, automatic postural responses are associated with the midbrain and cortex. The farther up one goes in the hierarchy, the more inhibition there is of lower nervous system structures and the movements they produce, that is, reflexes. Tonic reflexes inhibit spinal cord reflexes, and righting reactions inhibit tonic reflexes. Inhibition allows previously demonstrated stimulus-response patterns of movement to be integrated or modified into more volitional movements. A more complete description of these postural responses is given as part of the development of postural control from a hierarchic perspective. Development of Motor Control Development of motor control can be described by the relationship of mobility and stability of body postures (Sullivan et al., 1982) and by the acquisition of automatic postural responses (Cech and Martin, 2012). Initial random movements (mobility) are followed by maintenance of a posture (stability), movement within a posture (controlled mobility), and finally, movement from one posture to another posture (skill). The sequence of acquiring motor control is seen in key developmental postures in Figure 3-5. With acquisition of each new posture comes the development of control within that posture. For example, weight shifting in prone precedes rolling prone to supine; weight shifting on hands and knees precedes creeping; and cruising, or lateral weight shifting in standing precedes walking. The actual motor accomplishments of rolling, reaching, creeping, cruising, and walking are skills in which mobility is combined with stability, and the distal parts of the body —that is, the extremities— are free to move. The infant develops motor and postural control in the following order: mobility, stability, controlled mobility, and skill. MOBILITY STABILITY CONTROLLED MOBILITY SKILL Co-contraction ao fh oe =, L = Gb | Fa aes ‘ ? cee NOCK CO-COMPACION aul Hond ofented «uses Speech / eye control to vertical Supine flexion — ¥ ) i (> Weight shifting sss Unilateral reaching A ] Pd € Id a Ne Tame ex a i |_| ae ~ omni» Weight shifting » Unilateral reaching Prone on hands - ( h | \. ? t,~—~, * “4 TR \ - KL ea => Weight shifting & Crooping }/7 - ey All fours 4 —~ ey BZ ha a A \\ > Squat to stand >» Walking AW Semi-squat 7 ( \ be Ph ae > Weight shifting > Walking ,| & Stang FIGURE 3-5 Key postures and sequence of development. Stages of Motor Control Te Stage One Stage one is mobility, when movement is initiated. The infant exhibits random movements within an available range of motion for the first 3 months of development. Movements during this stage are erratic. They lack purpose and are often reflex-based. Random limb movements are made when the infant’s head and trunk are supported in the supine position. Mobility is present before stability. In adults, mobility refers to the availability of range of motion to assume a posture and the presence of sufficient motor unit activity to initiate a movement. Stage Two Stage two is stability, the ability to maintain a steady position in a weight-bearing, antigravity posture. It is also called static postural control. Developmentally, stability is further divided into tonic holding and cocontraction. Tonic holding occurs at the end of the shortened range of movement and usually involves isometric movements of antigravity postural extensors (Stengel et al., 1984). Tonic holding is most evident when the child maintains the pivot prone position (prone extension), as seen in Figure 3-5. Postural holding of the head begins asymmetrically in prone, followed by holding the head in midline, and progresses to holding the head up past 90 degrees from the support surface. In the supine position, the head is turned to one side or the other; then it is held in midline; and finally, it is held in midline with a chin tuck while the infant is being pulled to sit at 4 months (Figure 3-6). FIGURE 3-6 Chin tuck when pulled to sit. Cocontraction is the simultaneous static contraction of antagonistic muscles around a joint to provide stability in a midline position or in weight bearing. Various groups of muscles, especially those used for postural fixation, allow the developing infant to hold such postures as prone extension, prone on elbows and hands, all fours, and a semi-squat. Cocontraction patterns are shown in Figure 3-5. Once the initial relationship between mobility and stability is established in prone and later in all fours and standing, a change occurs to allow mobility to be superimposed on the already established stability. Stage Three Controlled mobility is mobility superimposed on previously developed postural stability by weight shifting within a posture. Proximal mobility is combined with distal stability. This controlled mobility is the third stage of motor control and occurs when the limbs are weight bearing and the body moves such as in weight shifting on all fours or in standing. The trunk performs controlled mobility when it is parallel to the support surface or when the line of gravity is perpendicular to the 73 trunk. In prone and all-fours positions, the limbs and the trunk are performing controlled mobility when shifting weight. The infant's first attempts at weight shifts in prone happen accidentally with little control. As the infant tries to reproduce the movement and practices various movement combinations, the movement becomes more controlled. Another example of controlled mobility is demonstrated by an infant in a prone on elbows position who sees a toy. If the infant attempts to reach for the toy with both hands, which she typically does before reaching with one hand, the infant is likely to fall on her face. If she perseveres and learns to shift weight onto one elbow, she has a better chance of obtaining the toy. Weight bearing, weight shifting, and cocontraction of muscles around the shoulder are crucial to the development of shoulder girdle stability. Proximal shoulder stability supports upper extremity function for skilled distal manipulation. If this stability is not present, distal performance may be impaired. Controlled mobility is also referred to as dynamic postural control. Stage Four Skill is the most mature type of movement and is usually mastered after controlled mobility within a posture. For example, after weight shifting within a posture such as in a hands-and-knees position, the infant frees the opposite arm and leg to creep reciprocally. Creeping is a skilled movement. Other skill patterns are also depicted in Figure 3-5. Skill patterns of movement occur when mobility is superimposed on stability in non—weight bearing; proximal segments stabilize while distal segments are free for movement. The trunk does skilled work when it is upright or parallel to the force of gravity. In standing, only the lower extremities are using controlled mobility when weight shifting occurs. If the swing leg moves, it performs skilled work while the stance limb performs controlled mobility. When an infant creeps or walks, the limbs that are in motion are using skill, and those in contact with the support surface are using controlled mobility. Creeping and walking are considered skilled movements. Skilled movements involve manipulation and exploration of the environment. Development of Postural Control Postural control develops in a cephalocaudal direction in keeping with Gesell’s developmental principles, which are discussed in Chapter 4. Postural control is demonstrated by the ability to maintain the alignment of the body —specifically, the alignment of body parts relative to each other and the external environment. The infant learns to use a group of automatic postural responses to attain and maintain an upright erect posture. These postural responses are continuously used when balance is lost in an effort to regain equilibrium. The sequence of development of postural reactions entails righting reactions, followed by protective reactions, and then equilibrium reactions. In the infant, head righting reactions develop first and are followed by the development of trunk righting reactions. Protective reactions of the extremities emerge next in an effort to safeguard balance in higher postures, such as sitting. Finally, equilibrium reactions develop in all postures beginning in prone. Traditionally, posture and movement develop together in a cephalocaudal direction, so balance is achieved in different positions relative to gravity. Head control is followed by trunk control; control of the head on the body and in space comes before sitting and standing balance. Righting Reactions Righting reactions are responsible for orienting the head in space and keeping the eyes and mouth horizontal. This normal alignment is maintained in an upright vertical position and when the body is tilted or rotated. Righting reactions involve head-and-trunk movements to maintain or regain orientation or alignment. Some righting reactions begin at birth, but most are evident between 4 and 6 months of age, as listed in Table 3-2. Gravity and change of head or body position provide cues for the most frequently used righting reactions. Vision cues an optical righting reaction, gravity cues the labyrinthine righting reaction, and touch of the support surface to the abdomen cues the body-on-the-head reaction. These three head righting reactions assist the infant in developing head control. Table 3-2 Righting and Equilibrium Reactions 74 75 Reaction Age at Onset Integration Head righting Neck (immature) 4-6 months Labyrinthine Persists Optical Persists Neck (mature) 5 years Trunk righting Body (immature) 4-6 mon Body (mature) 4—6 months 5 years Landau 1-2 years Protective 76 From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, St. Louis, 2012, Elsevier, p. 269. Head turning can produce neck-on-body righting, in which the body follows the head movement. If either the upper or lower trunk is turned, a body-on-body righting reaction is elicited. Either neck-on-body righting or body-on-body righting can produce log rolling or segmental rolling. Log rolling is the immature righting response seen in the first 3 months of life; the mature response emerges around 4 months of age. The purpose of righting reactions is to maintain the correct orientation of the head and body in relation to the ground. Head and trunk righting reactions occur when weight is shifted within a base of support; the amount of displacement determines the degree of response. For example, in the prone position, slow weight shifting to the right produces a lateral bend or righting of the head and trunk to the left. If the displacement is too fast, a different type of response may be seen; a protective response. Slower displacements are more likely to elicit head and trunk righting. These can occur in any posture and in response to anterior, posterior, or lateral weight shifts. Righting reactions have their maximum influence on posture and movement between 10 and 12 months of age, although they are said to continue to be present until the child is 5 years old. Righting reactions are no longer considered to be present if the child can come to standing from a supine position without using trunk rotation. The presence of trunk rotation indicates a righting of the body around the long axis. Another explanation for the change in motor behavior could be that the child of 5 years has sufficient abdominal strength to perform the sagittal plane movement of rising straight forward and attaining standing without using trunk rotation. Protective Reactions Protective reactions are extremity movements that occur in response to rapid displacement of the body by diagonal or horizontal forces. They have a predictable developmental sequence, which can be found in Table 3-2. By extending one or both extremities, the individual prepares for a fall or prepares to catch herself. A 4-month-old infant’s lower extremities extend and abduct when the infant is held upright in vertical and quickly lowered toward the supporting surface. At 6 months, the upper extremities show forward protective extension, followed by sideways extension at 7 to 8 months and backward extension at 9 months. Protective staggering of the lower extremities is evident by 15 to 17 months (Barnes et al., 1978). Protective reactions of the extremities should not be confused with the ability of the infant to prop on extended arms, a movement that can be self- initiated by pushing up from prone or by being placed in the position by a caregiver. Because an infant must be able to bear weight on extended arms to exhibit protective extension, training an infant to prop on extended arms or to push up from prone can be useful as treatment interventions. Equilibrium Reactions Equilibrium reactions are the most advanced postural reactions and are the last to develop. These reactions allow the body as a whole to adapt to slow changes in the relationship of the center of mass with the base of support. By incorporating the already learned head-and-trunk righting reactions, the equilibrium reactions add extremity responses to flexion, extension, or lateral head- and-trunk movements to regain equilibrium. In lateral weight shifts, the trunk may rotate in the opposite direction of the weight shift to further attempt to maintain the body’s center of mass within the base of support. The trunk rotation is evident only during lateral displacements. Equilibrium reactions can occur if the body moves relative to the support surface, as in leaning sideways, or if the support surface moves, as when one is ona tilt board. In the latter case, these movements are called tilt reactions. The three expected responses to a lateral displacement of the center of mass toward the periphery of the base of support in standing are as follows: (1) lateral head and trunk righting occurs away from the weight shift; (2) the arm and leg are opposite the direction of the weight shift abduct; and (3) trunk rotation away from the weight shift may occur. If the last response does not happen, the other two responses can provide only a brief postponement of the inevitable fall. At the point at which the center of gravity leaves the base of support, protective extension of the arms may occur, or a protective step or stagger may reestablish a stable base. Thus, the order in which the reactions are acquired developmentally is different from the order in which they are used for balance. Equilibrium reactions also have a set developmental sequence and timetable (see Table 3-2). Because prone is a position from which to learn to move against gravity, equilibrium reactions are seen first in prone at 6 months, then supine at 7 to 8 months, sitting at 7 to 8 months, on all fours at V7 9 to 12 months, and standing at 12 to 21 months. The infant is always working on more than one postural level at a time. For example, the 8-month-old infant is perfecting supine equilibrium reactions while learning to control weight shifts in sitting, freeing first one hand and then both hands. Sitting equilibrium reactions mature when the child is creeping. Standing and cruising are possible as equilibrium reactions are perfected on all fours. The toddler is able to increase walking speed as equilibrium reactions mature in standing. Motor Program Model of Motor Control As a result of a debate over the role of sensory information in motor actions, another concept of importance to current motor control and learning theories arose (Lashley, 1951). That concept is the motor program. A motor program is a memory structure that provides instructions for the control of actions. A program is a plan that has been stored for future use. The concept of a motor program is useful because it provides a means by which the nervous system can avoid having to create each action from scratch and thus can save time when initiating actions. There has been much debate over what is contained in a motor program. Different researchers have proposed a variety of programs. Motor program theory was developed to directly challenge the notion that all movements were generated through chaining or reflexes because even slow movements occur too fast for sensory input to influence them (Gordon, 1987). The implication is that for efficient movement to occur ina timely manner, an internal representation of movement actions must be available to the mover. “Motor programs are associated with a set of muscle commands specified at the time of action production, which do not require sensory input” (Wing et al., 1996). Schmidt (1988) expanded motor program theory to include the notion of a generalized motor program or an abstract neural representation of an action, distributed among different systems. Being able to mentally represent an action is part of developing motor control (Gabbard, 2009). The term motor program may also refer to a specific neural circuit called a central pattern generator (CPG), which is capable of producing a motor pattern, such as walking. CPGs exist in the human spinal cord. They are called stepping pattern generators (SPGs) located in each leg that control stepping movements at the hip and the knee (Yang et al., 2005). Postural control of the head and trunk and voluntary control of the ankle is also required for walking. Sensory feedback adjusts timing and reinforces muscle activation (Knikou, 2010). Systems Models of Motor Control A systems model of motor control is currently used to describe the relationship of various brain and spinal centers working together to control posture and movement. In a systems model, the neural control of posture and movement is distributed, that is, which areas of the nervous system that control posture or movement depend on the complexity of the task to be performed. Because the nervous system has the ability to self-organize, it is feasible that several parts of the nervous system are engaged in resolving movement problems; therefore, solutions are typically unique to the context and goal of the task at hand (Thelen, 1995). The advantage of a systems model is that it can account for the flexibility and adaptability of motor behavior in a variety of environmental conditions. A second characteristic of a systems model is that body systems other than the nervous system are involved in the control of movement. The most obvious other system to be involved is the musculoskeletal system. The body is a mechanical system. Muscles have viscoelastic properties. Physiologic maturation occurs in all body systems involved in movement production: muscular, skeletal, nervous, cardiovascular, and pulmonary. For example, if the contractile properties of muscle are not mature, certain types of movements may not be possible. If muscular strength of the legs is not sufficient, ambulation may be delayed. Muscle strength, posture, and perceptual abilities exhibit developmental trajectories, which can affect the rate of motor development by affecting the process of motor control. Feedback is a third fundamental characteristic of the systems models of motor control. To control movements, the individual needs to know whether the movement has been successful. In a closed- loop model of motor control, sensory information is used as feedback to the nervous system to provide assistance with the next action. A person engages in closed-loop feedback when playing a video game that requires guiding a figure across the screen. This type of feedback provides self- control of movement. A loop is formed from the sensory information that is generated as part of the 78 movement and is fed back to the brain. This sensory information influences future motor actions. Errors that can be corrected with practice are detected, and performance can be improved. This type of feedback is shown in Figure 3-7. initiated CLOSED LOOP Task completed Sensory feedback A Errors in movement detected OPEN LOOP Preprogrammed Error detection— response correction occurs is initiated after the response CNS generates motor commands FIGURE 3-7 A, B, Models of feedback. (Redrawn from Montgomery, PC, Connolly BH. Motor control and physical therapy: theoretical framework and practical application, Hixson, 1991, Chattanooga Group,) By contrast, in an open-loop model of motor control, movement is cued either by a central structure, such as a motor program, or by sensory information from the periphery. The movement is performed without feedback. When a baseball pitcher throws a favorite pitch, the movement is too quick to allow feedback. Errors are detected after the fact. An example of action spurred by external sensory information is what happens when a fire alarm sounds. The person hears the alarm and moves before thinking about moving. This type of feedback model is also depicted in Figure 3-7 and is thought to be the way in which fast movements are controlled. Another way to think of the difference between closed-loop and open-loop motor controls can be exemplified by someone who learns to play a piano piece. The piece is played slowly while the student is learning and receiving feedback, but once it is learned, the student can sit down and play it through quickly, from beginning to end. Components of the Postural Control System In the systems models, both posture and movement are considered systems that represent the interaction of other biologic and mechanical systems and movement components. The relationship between posture and movement is also called postural control. As such, posture implies a readiness to move, an ability not only to react to threats to balance but also to anticipate postural needs to support a motor plan. A motor plan or program is a plan to move, usually stored in memory. Seven components have been identified as part of a postural control system, as depicted in Figure 3-8. These are limits of stability, sensory organization, eye-head stabilization, the musculoskeletal system, motor coordination, predictive central set, and environmental adaptation. Postural control like motor control is a complex and ongoing process. 79 Limits of stability Sensory organization Environmental he adaptation Eye-head Postural stabilization Musculoskeletal control system system ™*\ Predictive Motor Pd coordination central set FIGURE 3-8 Components of normal postural control. (Redrawn from Duncan P, editor Balance: proceedings of the APTA forum, Alexandria, 1990, American Physical Therapy Association, with permission of the APTA.) Limits of Stability Limits of stability are the boundaries of the base of support (BOS) of any given posture. As long as the center of mass (COM) is within the base of support, the person is stable. An infant's base of support is constantly changing relative to the body’s size and amount of contact the body has with the supporting surface. Supine and prone are more stable postures by virtue of having so much of the body in contact with the support surface. However, in sitting or standing, the size of the base of support depends on the position of the lower extremities and on whether the upper extremities are in contact with the supporting surface. In standing, the area in which the person can move within the limits of stability or base of support is called the cone of stability, as shown in Figure 3-9. The central nervous system perceives the body’s limits of stability through various sensory cues. 80 FIGURE 3-9 Cone of stability. Keeping the body’s COM within the BOS constitutes balance. During quiet stance, as the body sways, the limits of stability depend on the interaction of the position and velocity of movement of the COM. We are more likely to lose balance if the velocity of the COM is high and at the limits of the BOS. The body perceives changes in the COM in a posture by detecting amplitude of center of pressure (COP) motion. The COP is the point of application of the ground reaction force. In standing, there would be a COP under each foot. You can feel how the COP changes as you shift weight forward and back while standing. Sensory Organization The visual, vestibular, and somatosensory systems provide the body with information about movement and cue postural responses. Maturation of the sensory systems and their relative contribution to balance have been extensively studied with some conflicting findings. Some of these conflicts may be related to the way balance is studied, whether static or dynamic balance is assessed, and to the maturation of sensorimotor control. Regardless of these differences, sensory input appears to be needed for the development of postural control. Vision is very important for the development of head control. Newborns are sensitive to the flow of visual information and can even make postural adjustments in response to this information (Jouen et al., 2000). Input from the visual system is mapped to neck movement initially and then to trunk movement as head and trunk control is established. The production of spatial maps of the position of various body parts appears to be linked to muscular action. The linking of posture at the neck to vision occurs before somatosensation is mapped to neck muscles (Shumway-Cook and Woollacott, 2012). Most people agree that vision is the dominant sensory system for the first 3 years of life and that infants rely on vision for postural control in the acquisition of walking. Vestibular information is also mapped to neck muscles at the same time as somatosensation is mapped. Eventually, mapping of combinations of sensory input such as visual-vestibular information is done (Jouen, 1984). This bimodal mapping allows for comparisons to be made 81 between previous and present postures. The mapping of sensory information from each individual sense proceeds from the neck to the trunk and on to the lower extremities (Shumway-Cook and Woollacott, 2012). Information from vision acts as feedback when the body moves and as an anticipatory cue in a feedforward manner before movement. As the child learns to make use of somatosensory information from the lower extremities, somatosensory input emerges as the primary sensory input on which postural response decisions are made. Somatosensation is the combined input from touch and proprioception. Adults use somatosensation as their primary source for postural response. When there is a sensory conflict, the vestibular system acts as a tiebreaker in making the postural response decision. If somatosensation says you are moving and vision says you are not, the vestibular input should be able to resolve the conflict to maintain balance. However, vestibular function relative to standing postural control does not reach adult levels even at the age of 15 according to Hirabayashi and Iwasaki (1995). Eye-Head Stabilization The head carries two of the most influential sensory receptors for posture and balance: the eyes and labyrinths. These two sensory systems provide ongoing sensory input about the movement of the surroundings and head, respectively. The eyes and labyrinths provide orientation of the head in space. The eyes must be able to maintain a stable visual image even when the head is moving, and the eyes have to be able to move with the head as the body moves. The labyrinths relay information about head movement to ocular nuclei and about position, allowing the mover to differentiate between egocentric (head relative to the body) and exocentric (head relative to objects in the environment) motion. Lateral flexion of the head is an egocentric motion. The movement of the head in space while walking or riding in an elevator is an example of exocentric motion. The head stabilization in space strategy (HSSS) involves an anticipatory stabilization of the head in space before body movement. A child first displays this strategy at 3 years of age while walking on level ground (Assaiante and Amblard, 1993). By maintaining the angular position of the head with regard to the spatial environment, vestibular inputs can be better interpreted. The HSSS appears to be mature in 7-year-olds (Assaiante and Amblard, 1995). Older adults have been shown to adopt this strategy when faced with distorted or incongruent somatosensory and visual information (DiFabio and Emasithi, 1997). Musculoskeletal System The body is a mechanically linked structure that supports posture and provides a postural response. The viscoelastic properties of the muscles, joints, tendons, and ligaments can act as inherent constraints to posture and movement. The flexibility of body segments, such as the neck, thorax, pelvis, hip, knee, and ankle, contribute to attaining and maintaining a posture or making a postural response. Each body segment has mass and grows at a different rate. Each way in which a joint can move represents a degree of freedom. Because the body has so many individual joints and muscles with many possible ways in which to move, certain muscles work together in synergies to control the degrees of freedom. Normal muscle tone is needed to sustain a posture and to support normal movement. Muscle tone has been defined as the resting tension in the muscle (Lundy-Ekman, 2013) and the stiffness in the muscle as it resists being lengthened (Basmajian and DeLuca, 1985). Muscle tone is determined by assessing the resistance felt during passive movement of a limb. Resistance is caused mainly by the viscoelastic properties of the muscle. On activating the stretch reflex, the muscle proprioceptors, the muscle spindles, and Golgi tendon organs contribute to muscle tone or stiffness. The background level of activity in antigravity muscles during stance is described as postural tone by Shumway- Cook and Woollacott (2012). Others also describe patterns of muscular tension in groups of muscles as postural tone. Together, the viscoelastic properties of muscle, the spindles, Golgi tendon organs, and descending motor tracts regulate muscle tone. Motor Coordination Motor coordination is the ability to coordinate muscle activation in a sequence that preserves posture. The use of muscle synergies in postural reactions and sway strategies in standing are examples of this coordination and are described in the upcoming section on neural control. Determination of the muscles to be used in a synergy is based on the task to be done and the environment in which the task takes place. 82 Strength and muscle tone are prerequisites for movement against gravity and motor coordination. Head-and-trunk control require sufficient strength to extend the head, neck, and trunk against gravity in prone; to flex the head, neck, and trunk against gravity in supine; and to laterally flex the head, neck, and trunk against gravity in side-lying. Predictive Central Set Predictive central set is that component of postural control that can best be described as postural readiness. Sensation and cognition are used as an anticipatory cue before movement as a means of establishing a state of postural readiness. This readiness or postural set must be present to support movement. Think of how difficult it is to move in the morning when waking up; the body is not posturally ready to move. Contrast this state of postural unpreparedness with an Olympic competitor who is so focused on the motor task at hand that every muscle has been put on alert, ready to act at a moment's notice. Predictive central set is critical to postural control. Mature motor control is characterized by the ability of the body, through the postural set, to anticipate what movement is to come, such as when you tense your arm muscles before picking up a heavy weight. Anticipatory preparation is an example of feedforward processing, in which sensory information is sent ahead to prepare for the movement to follow, in contrast to feedback, in which sensation from a movement is sent back to the nervous system for comparison and error detection. Many adult patients with neurologic deficits lack this anticipatory preparation, so postural preparedness is often a beginning point for treatment. Children with neurologic deficits may never have experienced using sensation in this manner. Environmental Adaptation Our posture and movement adapt to the environment in which the movement takes place in much the same way as we change our stance if riding on a moving bus and have nothing stable to grasp. Infants have to adapt to moving in a gravity-controlled environment after being in utero. The body’s sensory systems provide input that allows the generation of a movement pattern that dynamically adapts to current conditions. In a systems model, this movement pattern is not limited to the typical postural reactions. With development of postural networks, anticipatory postural control develops and is used to preserve posture. Adaptive postural control allows changes to be made to movement performance in response to internally or externally perceived needs. Nashner’s Model of Postural Control in Standing Nashner (1990) formulated a model for the control of standing balance over the course of some 20 years. His model describes three common sway strategies seen in quiet steady-state standing: the ankle strategy, the hip strategy, and the stepping strategy. An adult in a quiet standing position sways about the ankles. This strategy depends on having a solid surface in contact with the feet and intact visual, vestibular, and somatosensory systems. If the person sways backward, the anterior tibialis fires to bring the person forward; if the person sways forward, the gastrocnemius fires to bring the person back to midline. A second sway strategy, called the hip strategy, is usually activated when the base of support is narrow, as when standing crosswise on a balance beam. The ankle strategy is not effective in this situation because the entire foot is not in contact with the support surface. In the hip strategy, muscles are activated in a proximal-to-distal sequence, that is, muscles around the hip are activated to maintain balance before the muscles at the ankles. The last sway strategy is that of stepping. If the speed and strength of the balance disturbance are sufficient, the individual may take a step to prevent loss of balance or a fall. This stepping response is the same as a lower extremity protective reaction. The ankle and the hip strategies are shown in Figure 3-10. 83 ft FIGURE 3-10 Sway strategies. A, Postural sway about the ankle in quiet standing. B, Postural sway about the hip in standing on a balance beam. (Modified from Cech D, Martin S, editors: Functional movement development across the life span, ed 3, St. Louis, 2012, Elsevier, p. 271.) The visual, vestibular, and somatosensory systems previously discussed provide the body with information about movement and cue appropriate postural responses in standing. For the first 3 years of life, the visual system appears to be the dominant sensory system for posture and balance. Vision is used both as feedback as the body moves and as feedforward to anticipate that movement will occur. Children as young as 18 months demonstrate an ankle strategy when quiet standing balance is disturbed (Forssberg and Nashner, 1982). However, the time it takes for them to respond is longer than in adults. Results of studies of 4- to 6-year-old children’s responses to disturbances of standing balance were highly variable, almost as if balance was worse in this age group when compared to younger children. Sometimes the children demonstrated an ankle strategy, and sometimes they demonstrated a hip strategy (Shumway-Cook and Woollacott, 1985). It was originally postulated that children did not have adult-like responses until 10 years of age. Postural sway in standing on a moveable platform under normal vestibular and somatosensory conditions is greater for children 4 to 6 years of age than for children 7 to 10 years of age (Shumway-Cook and Woollacott, 1985). By 7 to 10 years of age, an adult sway strategy is demonstrated wherein the child is thought to depend primarily on somatosensory information. Vestibular information is also being used but the system is not yet mature. Interestingly, children with visual impairments are not able to minimize postural sway to the same extent as children who are not visually impaired (Portfors-Yeomans and Riach, 1995). This may be related to the child’s inability to fully use either somatosensory or vestibular information during this age period. Research supports that there is a transition period around 7 to 8 years that can be explained by the use of the HSSS (Rival et al., 2005). By 7 years of age, children are able to make effective use of HSSS that depends on dynamic vestibular cues (Assaiante and Amblard, 1995). However, the transition to adult postural responses in standing is not complete by 12 years of age. Children at 12 to 14 years of age are still not able to handle misleading visual information to make appropriate adult balance responses (Ferber-Viart et al., 2007). These researchers found that although the somatosensory inputs and scores in the 6- to 14-year-old subjects were as good as the young adults studied, their sensory organization was different. They concluded that children prefer visual input to vestibular input for determining balance responses and that vestibular information is the least effective for postural control. 84 85 Issues related to motor control Top Down or Distributed Control The issue of where the control of movement resides has always been at the heart of the discussion of motor control. Remember that motor control occurs in milliseconds as compared with the time it takes to learn a movement or to develop a new motor skill. The reflex hierarchical models are predicated on the cortex being the controller of movement. However, if there is no cortex, movement is still possible. The cortex can initiate movement but it is not the only neural structure able to do so. From studying pathology involving the basal ganglia, it is known that movement initiation is slowed in people with Parkinson disease. Other neural structures that can initiate or control movement include the basal ganglia, the cerebellum, and the spinal cord. The spinal cord can produce rudimentary reciprocal movement from activation of central pattern generators. The reflexive withdrawal and extension of the limbs has been modified to produce cyclical patterns of movement that help locomotion be automatic but is modifiable by higher centers of the brain. Lastly, the cerebellum is involved in movement coordination and timing of movements. The fact that more than one structure within the nervous system can affect and control movement lends credence for a distributed control of movement. There is no one location of control in the systems view of movement; the movement emerges from the combined need of the mover, the task, and the environment. The structures, pathways, and processes needed to most efficiently produce the movement are discovered as in finding the best way to get the task done. The structures, pathways, or processes that are continually used get better at the task and become the preferred way of performing that particular task. Developmentally, only certain structures, pathways, or processes are available early in development so that movements become refined and control improves with age. Movement control improves not only because of the changes in the central nervous system (CNS), but also because of the maturation of the musculoskeletal system. Because the musculoskeletal system carries out the movement, its maturation can also affect movement outcome. Degrees of Freedom The mechanical definition of degrees of freedom is “the number of planes of motion possible at a single joint” (Kelso, 1982). The degrees of freedom of a system have been defined as all of the independent movement elements of a control system and the number of ways each element can act (Schmidt and Wrisberg, 2004). There are multiple levels of redundancy within the CNS. Bernstein (1967) suggested that a key function of the CNS was to control this redundancy by minimizing the degrees of freedom or the number of independent movement elements that are used. For example, muscles can fire in different ways to control particular movement patterns or joint motions. In addition, many different kinematic or movement patterns can be executed to accomplish one specific outcome or action. During the early stages of learning novel tasks, the body may produce very simple movements, often “linking together two or more degrees of freedom” (Gordon, 1987), limiting the amount of joint motion by holding some joints stiffly via muscle cocontraction. As an action or task is learned, we first hold our joints stiffly through muscle coactivation and then, as we learn the task, we decrease coactivation and allow the joint to move freely. This increases the degrees of freedom around the joint (Vereijken et al., 1992). This concept is further discussed later in the chapter. Certainly, an increase in joint stiffness used to minimize degrees of freedom at the early stages of skill acquisition may not hold true for all types of tasks. In fact, different skills require different patterns of muscle activation. For example, Spencer and Thelen (1997) reported that muscle coactivity increases with the learning of a fast vertical reaching movement. They proposed that high-velocity movements actually result in the need for muscle coactivity to counteract unwanted rotational forces. However, during the execution of complex multijoint tasks, such as walking and rising from sitting to standing, muscle coactivation is clearly undesirable and may in fact negatively affect the smoothness and efficiency of the movements. The resolution of the degrees of freedom problem varies depending on the characteristics of the learner as well as on the components of the task and environment. Despite the various interpretations of Bernstein’s original hypothesis (1967), the resolution of the degrees of freedom problem continues to form the underlying basis for a 86 systems theory of motor control. Optimization Principles Optimization theory suggests that movements are specified to optimize a select cost function (Cruse et al., 1990; Nelson, 1983; Wolpert et al., 1995). Cost functions are those kinematic (spatial) or dynamic (force) factors that influence movement at an expense to the system. Motor skill development or relearning is aimed at achieving select objectives while minimizing cost to the system. Reducing such cost while meeting task demands and accommodating to task constraints theoretically solves the degrees of freedom problem and enhances movement efficiency. As children and adults struggle to achieve functional gains during development or during recovery from neural injury, they may appear to use inefficient movement strategies, at least from an outside view. In actuality, they may be expressing the most efficient movements available to them given their current resources. For example, a child with hemiplegic cerebral palsy may have the physical constraints of shoulder or wrist weakness and reduced finger fractionation (isolation). In an effort to reduce cost to the system while meeting tasks demands, she may use a “flexion synergy,” in which elbow flexion is used in combination with shoulder elevation and lateral trunk flexion to reach for objects placed at shoulder height. This flexion synergy is a strategy that seems to reduce the number of movement elements yet allows for successful attainment of the target object. Although this strategy may be useful in a specific situation, it may become habitual and may not be effective in performing a wide range of tasks. Researchers have found that children with hemiplegic cerebral palsy as a result of right hemisphere damage have deficits in using proprioceptive feedback to recognize arm position (Goble et al., 2005). Variability in postural control is seen during infancy. Variability is needed for the development of functional movement. Furthermore, being able to vary and adapt one’s posture makes exploration of the surrounding environment easier and affords opportunities for perception and action. An infant who lacks postural and movement variability is at risk for movement dysfunction. Dusing and Harbourne (2010) have suggested that lack of complex postural control may be an early indicator of developmental problems. Conversely, adding complexity to posture and movement variability may provide an impetus for functional changes in motor function. Age-Related Changes in Postural and Motor Control Infants learn to move by moving. Postural control supports movement and provides strategies upon which to scaffold motor actions, such as reaching, grasping, crawling, and walking. Early movements are characterized by large amounts of variability. Adaptation of movement is not evident initially but develops with experience (Hadders-Algra, 2010). Variability in postural control is seen in infancy. Infants scale the postural responses of their head to the surrounding visual information (Bertenthal et al., 1997). The ability to use visual information for postural responses improves from 5 to 9 months of age. Balance Strategies in Sitting Infants develop directionally specific postural responses before being able to sit (Hadders-Algra, 2008). These responses appear to be innate and are guided by an internal representation of the limits of stability such as orientation of the vertical axis and relationship of COM to BOS. This is consistent with the hypothesis of a central pattern generator being the source of initial postural responses (Hirschfeld and Forssberg, 1994). This circuitry determines the spatial characteristics of muscle activation that is triggered by afferent information. During this period of time, the infant demonstrates a large number of responses. With further development, the circuitry matures, and with experience, the initial variability is reduced. The temporal and spatial features of responses are fine-tuned to match task-specific demands. Multisensory afferent input is used to shape these adaptive responses. Most studies of the development of anticipatory postural control have been conducted in the sitting position using reaching as the task. Postural activity in the trunk was measured while an infant reached from a seated posture (Riach and Hayes, 1990). Trunk muscles were activated before muscles used for reaching. Researchers concluded that anticipatory postural control occurs before voluntary movements and is present in infants by 9 months of age (Hadders-Algra et al., 1996a). 87 Children appear to tolerate more imbalance as they grow up (Hay and Redon, 1999). Anticipatory control of posture increases from 3 to 8 years of age, with older children demonstrating more refined scaling of responses. In other words, children become better at matching the amount of postural preparation needed for a specific task. Less postural activation is needed when picking up a light object as compared to picking up a heavy object. Strategies in Standing Older adults have more spontaneous sway than younger individuals (Maki and Mcllroy, 1996; Sturnieks et al., 2008). The increase in sway is thought to be a compensation for the effects of gravity. However, the older adult may use increased sway to provide ongoing sensory information to postural control mechanisms in the CNS. Altering the sensory conditions provides a challenge to both young and older adults. With eyes closed, older adults stand more asymmetrically than younger adults. Older adults have been found to use a stiffening response of cocontracting muscles around the ankles joints rather than switching to using other sensory cues when vision is eliminated in quiet standing (Benjuya et al., 2004). Increased sway in a medial lateral direction is most predictive of falls in older adults (Maki et al., 1994). Stepping response may be more of a real-life response to external perturbations even if the position of the COM does not exceed the BOS (Rogers et al., 1996; Maki and Mcllroy, 1997). The model of motor control that best explains changes in posture and movement seen across the life span depend on the age and experience of the mover, the physical demands of the task to be carried out, and the environment in which the task is to be performed. The way in which a 2-year- old child may choose to solve the movement problem of how to reach the cookie jar in the middle of the kitchen table will be different from the solution devised by a 12-year-old child. The younger the child, the more homogeneous the movement solutions are. As the infant grows, the movement solutions become more varied, and that, in itself, may reflect the self-organizing properties of the systems of the body involved in posture and movement. Posture has a role in movement before, during, and after a movement. Posture should be thought of as preparation for movement. A person would not think of starting to learn to in-line skate from a seated position. The person would have to stand with the skates on and try to balance while standing before taking off on the skates. The person’s body tries to anticipate the posture that will be needed before the movement. Therefore, with patients who have movement dysfunction, the clinician must prepare them to move before movement is initiated. When learning in-line skating, the person continually tries to maintain an upright posture. Postural control maintains alignment while the person moves forward. If the person loses balance and falls, posture is reactive. When falling, an automatic postural response comes from the nervous system; arms are extended in protection. Stunt performers have learned to avoid injury by landing on slightly bent arms, then tucking and rolling. Through the use of prior experience and knowledge of present conditions, the end result is modified and a full-blown protective response is generated. In many instances, automatic postural responses must be unlearned to learn and perfect fundamental motor skills. Think of a broad jumper who is airborne and moving forward in a crouch position. To prevent falling backward, the jumper must keep his arms forward and counteract the natural tendency to reach back. 88 Motor learning Across the life span, individuals are faced with new motor challenges and must learn to perform new motor skills. An infant must learn how to hold up her head, roll over, sit, crawl, and eventually walk. Each skill takes time to master and occurs only after the infant has practiced each skill in several different ways. The young child then masters running, climbing on furniture, walking up stairs, jumping, and playing ball. The school-age child takes these tasks further to specifically kick a soccer ball into a net, throw a ball into a basketball hoop, ride a bike, or skateboard. As teens and adults learn new sports, they refine their skills, becoming more efficient at turning while on snow skis or pitching a baseball into the strike zone with more speed. Adults also learn to efficiently perform tasks related to their occupation. These tasks vary widely from one occupation to another and may include efficient computer keyboarding, climbing up a ladder, or lifting boxes. Older adults may need to modify their motor skill performance to accommodate for changes in strength and flexibility. For example, the older adult golfer may change her stance during a swing or learn to use a heavier golf club to maximize the distance of her drive. Often, injury or illness requires an individual to relearn how to sit up, walk, put on a shirt, or get into or out of a car. The method each individual uses to learn new movements demonstrates the process of motor learning. Motor learning examines how an individual learns or modifies a motor task. As discussed in the section on motor control, the characteristics of the task, the learner, and the environment will impact on the performance and learning of the skill. With motor learning, general principles apply to individuals of any age, but variations also have been found between the motor learning methods used by children, adults, and older adults. Definition and Time Frame Motor learning is defined as the process that brings about a permanent change in motor performance as a result of practice or experience (Schmidt and Wrisberg, 2004). The time frame of motor learning falls between the milliseconds involved in motor control and the years involved in motor development. Hours, days, and weeks of practice are part of motor development. It takes an infant the better part of a year to overcome gravity and learn to walk. The perfection of some skills takes years; ask anyone trying to improve a batting average or a soccer kick. Even though motor development, motor control, and motor learning take place within different time frames, these time frames do not exclude one or the other processes from taking place. In fact, it is possible that because these processes do have different time bases for action, they may be mutually compatible. 89 Theories of motor learning There are two theories of motor learning that have generated a great deal of study about how we control and acquire motor skills. Both theories use programs to explain how movements are controlled and learned; they are Adams’ closed-loop theory of motor learning (Adams, 1971) and Schmidt’s schema theory (Schmidt, 1975). The two theories differ in the amount of emphasis placed on open-loop processes that can occur without the benefit of ongoing feedback (Schmidt and Lee, 2005). Schmidt incorporated many of Adams’ original ideas when formulating his schema theory in an attempt to explain the acquisition of both slow and fast movements. Intrinsic and extrinsic feedbacks, as defined earlier in this chapter, are both important factors in these two theories. Adams’ Closed-Loop Theory The name of Adams’ theory emphasized the crucial role of feedback. The concept of a closed loop of motor control is one in which sensory information is funneled back to the central nervous system for processing and control of motor behavior. The sensory feedback is used to produce accurate movements. The basic premise of Adams’ theory is that movements are performed by comparing the ongoing movement with an internal reference of correctness that is developed during practice. This internal reference is termed as perceptual trace, which represents the feedback one would receive if the task were performed correctly. A perceptual trace is formed as the learner repeatedly performs an action. Through ongoing comparison of the feedback with the perceptual trace, a limb may be brought into the desired position. To learn the task, it would be necessary to practice the exact skill repeatedly to strengthen the correct perceptual trace. The quality of performance is directly related to the quality of the perceptual trace. The trace is made up of a set of intrinsic feedback signals that arise from the learner. Intrinsic feedback here means the sensory information that is generated through performance; for example, the kinesthetic feel of the movement. As a new movement is learned, correct outcomes reinforce development of the most effective, correct perceptual trace, although perceptual traces that lead to incorrect outcomes are discarded. The perceptual trace becomes stronger with repetition and more accurate in representing the correct performance as a result of feedback. With further study, limitations of the closed-loop theory of motor learning have been identified. One limitation is that the theory does not explain how movements can be explained when sensory information is not available. The theory also does not explain how individuals can often perform novel tasks successfully, without the benefit of repeated practice and perceptual trace. The ability of the brain to store individual perceptual traces for each possible movement has also been questioned, considering the memory storage capacity of the brain (Schmidt, 1975). Schmidt’s Schema Theory Schmidt’s schema theory was developed in direct response to Adams’ closed-loop theory and its limitations. Schema theory is concerned with how movements that can be carried out without feedback are learned, and it relies on an open-loop control element, the motor program, to foster learning. The motor program for a movement reflects the general rules to successfully complete the movement. These general rules, or schema, can then be used to produce the movement in a variety of conditions or settings. For example, the general rules for walking can be applied to walking on tile, on grass, on an icy sidewalk, or going up a hill. The motor program provides the spatial and temporal information about muscle activation needed to complete the movement (Schmidt and Lee, 2005). The motor program is the schema, or abstract memory, of rules related to skilled actions. According to schema theory, when a person produces a movement, four kinds of information are stored in short-term memory. 1. The initial conditions under which the performance took place (e.g., the position of the body, the kind of surface on which the individual carried out the action, or the shapes and weights of any objects that were used to carry out the task) 2. The parameters assigned to the motor program (e.g., the force or speed that was specified at the time of initiation of the program) 90 3. The outcome of the performance 4. The sensory consequences of the movement (e.g., how it felt to perform the movement, the sounds that were made as a result of the action, or the visual effect of the performance). These four kinds of information are analyzed to gain insight into the relationships among them and to form two types of schema: the recall schema and the recognition schema. The recall schema is used to select a method to complete a motor task. It is an abstract representation of the relationship among the initial conditions surrounding performance, parameters that were specified within the motor program, and the outcome of the performance. The learner, through the analysis of parameters that were specified in the motor program and the outcome, begins to understand the relationship between these two factors. For example, the learner may come to understand how far a wheelchair travels when varying amounts of force are generated to push the chair on a gravel pathway. The learner stores this schema and uses it the next time the wheelchair is moved on a gravel path. The recognition schema helps assess how well a motor behavior has been performed. It represents the relationship among the initial conditions, the outcome of the performance, and the sensory consequences that are perceived by the learner. Because it is formed in a manner similar to that of the recall schema, once it is established, the recognition schema is used to produce an estimate of the sensory consequences of the action that will be used to adjust and evaluate the motor performance of a given motor task. In motor learning, the motor behavior is assessed through use of the recognition schema. If errors are identified, they are used to refine the recall schema. Recall and recognition schemas are continually revised and updated as skilled movement is learned. Limitations of the schema theory have also been identified. One limitation is that the formation of general motor programs is not explained. Another question has arisen from inconsistent results in studies of effectiveness of variable practice on learning new motor skills, especially with adult subjects. zat Stages of motor learning It is generally possible to tell when a person is learning a new skill. The person’s performance lacks the graceful, efficient movement of someone who has perfected the skill. For example, when adults learn to snow ski, they typically hold their bodies stiffly, with knees straight and arms at their side. Over time, as they become more comfortable with skiing, they will bend and straighten their knees as they turn. Finally, when watching the experienced skier, the body fluidly rotates and flexes or extends as she maneuvers down a steep slope or completes a slalom race. The stages associated with mastery of a skill have been described and clearly differentiated between the early stages of motor learning and the later stages of motor learning. Two models of motor learning stages are described below and in Table 3-3. Table 3-3 Stages of Motor Learning Stage 3 Fitts’ stages of motor learning Cognitive stage Associative stage Autonomous stage Actively think about goal Refine performance Automatic performance Think about conditions Error correction Consistent, efficient performance “Neo-Bernsteinian” model of motor Novice stage Advanced stage Expert stage learning Decreased number of degrees of Release of some degrees of Uses all degrees of freedom for fluid, efficient freedom freedom movement General characteristics Stiff looking re fluid movement Automatic Inconsistent performance Fewer errors Fluid Errors Improved consistency Consistent Slow, nonfluid movement Improved efficiency Efficient Error correction From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, St. Louis, 2012, Elsevier, p. 77. In the early stages of motor learning, individuals have to think about the skill they are performing and may even “talk” their way through the skill. For example, when learning how to turn when snow skiing, the novice skier may tell herself to bend the knees upon initiating the turn, then straighten the knees through the turn, and then bend the knees again as the turn is completed. The skier might even be observed to say the words “bend, straighten, bend” or “down, up, down” as she turns. Early in the motor learning process, movements tend to be stiff and inefficient. The new learner may not always be able to successfully complete the skill or might hesitate, making the timing movements within the skill inaccurate. In the later stages of motor learning, the individual may not need to think about the skill. For example, the skier will automatically go through the appropriate motions with the appropriate timing as she makes a turn down a steep slope. Likewise, the baseball player steps up to the plate and does not think too much about how he will hit the ball. The batter will swing at a ball that comes into the strike zone automatically. If either the experienced skier or batter makes an error, they will self-assess their performance and try to correct the error next time. Fitts’ Stages In analyzing acquisition of new motor skills, Fitts (1964) described three stages of motor learning. The first stage is the cognitive phase, in which the learner has to consciously consider the goal of the task to be completed and recognize the features of the environment to which the movement must conform (Gentile, 1987). In a task such as walking across a crowded room, the surface of the floor and the location and size of the people within the room are considered regulatory features. If the floor is slippery, a person’s walking pattern is different than if the floor is carpeted. Background features, such as lighting or noise, may also affect task performance. During this initial cognitive phase of learning, an individual tries a variety of strategies to achieve the movement goal. Through this trial-and-error approach, effective strategies are built upon and ineffective strategies are discarded. At the next stage of learning, the associative phase, the learner has developed the general movement pattern necessary to perform the task and is ready to refine and improve the o2 performance of the skill. The learner makes subtle adjustments to adjust errors and to adapt the skill to varying environmental demands of the task. For example, a young baseball player may learn that he can more efficiently and consistently hit the ball if he chokes up on the bat. During this phase, the focus of the learner switches from “what to do” to “how to do the movement” (Schmidt, 1988). In the final stage of learning, the autonomous phase, the skill becomes more “automatic” because the learner does not need to focus all of her attention on the motor skill. She is able to attend to other components of the task, such as scanning for subtle environmental obstacles. At this phase, the learner is better able to adapt to changes in features in the environment. The young baseball player will be relatively successful at hitting the ball even when using different bats or if a cheering crowd is present. “Neo-Bernsteinian” Model This model of staging motor learning considers the learner’s ability to master multiple degrees of freedom as she learns a new skill (Bernstein, 1967; Vereijken, et al., 1992). Within this model, the initial stage of motor learning, the novice stage, is when the learner reduces the degrees of freedom that need to be controlled during the task. The learner will “fix” some joints so that motion does not take place and the degree of freedom is constrained at that joint. For example, think of the new snow skier who holds her knees stiffly extended while bending at the trunk to try to turn. The resultant movement is stiff-looking and not always effective. For example, if the slope of the hill is too steep, or if the skier tries to turn on an icy patch, the movement may not be effective. The second stage in this model, the advanced stage, is seen when the learner allows more joints to participate in the task, in essence releasing some of the degrees of freedom. Coordination is improved as agonist and antagonist muscles around the joint can work together to produce the movement, rather than cocontracting as they did to “fix” the joint in earlier movement attempts. The third stage of this model, the expert stage, is when all degrees of freedom necessary to perform a task in an efficient, coordinated manner are released. Within this stage, the learner can begin to adjust performance to improve the efficiency of the movement by adjusting the speed of the movement. Considering the skier, the expert may appreciate that by increasing the speed of descent, a turn may be easier to initiate. Open and Closed Tasks Movement results when an interaction exists among the mover, the task, and the environment. We have discussed the mover and the environment, but the task to be learned can be classified as either open or closed. Open skills are those done in environments that change over time, such as playing softball, walking on different uneven surfaces, and driving a car. Closed skills are skills that have set parameters and stay the same, such as walking on carpet, holding an object, or reaching for a target. These skills appear to be processed differently. Which type involves more perceptual information? Open skills require the mover to constantly update movements and to pay attention to incoming information about the softball, movement of traffic, or the support surface. Would a person have fewer motor problems with open or closed skills? Closed skills with set parameters pose fewer problems. Remember that open and closed skills are different from open-loop and closed-loop processing for motor control or motor learning. Effects of Practice Motor learning theorists have also studied the effects of practice on learning a motor task and whether different types of practice make initial learning easier. Practice is a key component of motor learning. Some types of practice make initial learning easier but make transferring that learning to another task more difficult. The more closely the practice environment resembles the actual environment where the task will take place, the better the transfer of learning will be. This is known as task-specific practice. Therefore, if you are going to teach a person to walk in the physical therapy gym, this learning may not transfer to walking at home, where the floor is carpeted. Many facilities use an Easy Street (a mock or mini home, work, and community environment) to help simulate actual conditions the patient may encounter at home. Of course, providing therapy in the home is an excellent opportunity for motor learning. fo, Massed versus Distributed Practice The difference between massed and distributed practice schedules is related to the proportion of rest time and practice time during the session. In massed practice, greater practice time than rest time occurs in the session. The amount of rest time between practice attempts is less than the amount of time spent practicing. In distributed practice conditions, the amount of rest time is longer than the time spent practicing. Constraint-induced therapy can be considered a modified form of massed practice in which learned nonuse is overcome by shaping or reinforcing (Taub et al., 1993). Shaping incorporates the motor learning concept of part practice as a task is learned in small steps, which are individually mastered. Successive approximation of the completed task is made until the individual is able to perform the whole task. In an individual with hemiplegia, the uninvolved arm or hand is constrained, thereby necessitating use of the involved (hemiplegic) upper extremity in functional tasks. Random versus Blocked Practice Another consideration in structuring a practice session is the order in which tasks are practiced. Blocked practice occurs when the same task is repeated several times in a row. One task is practiced several times before a second task is practiced. Random practice occurs when a variety of tasks is practiced in a random order, with any one skill rarely practiced two times in a row. Mixed practice sessions may also be useful in some situations in which episodes of both random and blocked practice are incorporated into the practice session. Constant practice occurs when an individual practices one variation of a movement skill several times in a row. An example would be repeatedly practicing standing up from a wheelchair or throwing a basketball into a hoop. Variable practice occurs when the learner practices several variations of a motor skill during a practice session. For example, a patient in rehabilitation may practice standing up from the wheelchair, standing up from the bed, standing up from the toilet, and standing up from the floor. A child might practice throwing a ball into a hoop, throwing a ball at a target on the wall, throwing a ball underhand, throwing a ball overhand, or throwing a ball to a partner all within the same session. Variable practice training is useful in helping the learner generalize a motor skill over a wide variety of environmental settings and conditions. Learning is thought to be enhanced by the variable practice because the strength of the general motor program rules, specific to the new task, would be increased. This mechanism is also considered as a way that an individual can attempt a novel task because the person can incorporate rules developed for previous motor tasks to solve the novel motor task. Whole versus Part Task Training A task can be practiced as a complete action (whole task practice) or broken up into its component parts (part practice). Continuous tasks such as walking, running, or stair climbing are more effectively learned as a whole task practice. It has been demonstrated that if walking is broken down into part practice of a component such as weight shifting forward over the foot, the learner demonstrates improvements in weight-shifting behavior but not generalize this improvement into the walking sequence (Winstein et al., 1989). Skills, which can be broken down into discrete parts, may be most effectively taught using part practice training. For example, a patient learning how to independently transfer out of a wheelchair might be first taught how to lock the brakes on the chair, then how to scoot forward in the chair. After these parts of the task are mastered, the patient might learn to properly place his feet, lean forward over the feet, and finally stand. Similarly, when learning a dressing task, a child might first be taught to pull a shirt over her head then push in each arm. Once these components are completed, the focus might be on learning how to fasten buttons or the zipper. Constraints to Motor Development, Motor Control, and Motor Learning Our movements are constrained or limited by the biomechanical properties of our bones, joints, and muscles. No matter how sophisticated the neural message is or how motivated the person is, if the part of the body involved in the movement is limited in strength or range, the movement may occur 94 incorrectly or not at all. If the control directions are misinterpreted, the intended movement may not occur. A person is only as good a mover as the weakest part. For some, that weakest part is a specific system, such as the muscular or nervous system, and for others, it is a function of a system, such as cognition. Development of motor control and the acquisition of motor abilities occur while both the muscular and skeletal systems are growing and the nervous system is maturing. Changes in all the body’s physical systems provide a constant challenge to the development of motor control. Thelen and Fisher (1982) showed that some changes in motor behavior, such as an infant's inability to step reflexively after a certain age, probably occur because the infant’s legs become too heavy to move, not because some reflex is no longer exhibited by the nervous system. We have already discussed that the difficulty an infant encounters in learning to control the head during infancy can be attributed to the head’s size being proportionately too big for the body. With growth, the body catches up to the head. As a linked system, the skeleton has to be controlled by the tension in the muscles and the amount of force generated by those muscles. Learning which muscles work well together and in what order is a monumental task. Adolescence is another time of rapidly changing body relationships. As children become adolescents, movement coordination can be disrupted because of rapid and uneven changes in body dimensions. The most coordinated 10- or 12-year-old can turn into a gawky, gangly, and uncoordinated 14- or 16-year-old. The teenager makes major adjustments in motor control during the adolescent growth spurt. Age-Related Changes in Motor Learning Children learn differently than adults. Children practice, practice, practice. For example, when learning to walk, an infant covers a distance equal to 29 football fields daily (Adolph et al., 2003). A typical 14-month-old takes more than 2,000 steps per hour (Adolph, 2008). These two examples lend support to using block practice to learn and retain a new skill. Infants demonstrate inherent variability in task performance. As young children are learning new gross motor tasks, blocked practice appears to lead to better transfer and perform the skill. Del Rey and colleagues (1983) had typically developing children (approximately 8 years old) practice a timing task at different speeds in either a blocked or random order and then tested them on a transfer test with the new coordination pattern. The researchers found that blocked practice led to better performance on the transfer task than did random practice. In Frisbee throwing experiments, accuracy in throwing the Frisbee at a target was improved by blocked practice in children, although adults improved accuracy the most with random practice (Pinto-Zipp and Gentile, 1995; Jarus and Goverover, 1999). The contextual interference provided by random practice schedules does not appear to help children learn new motor skills (Perez et al., 2005). Although most of the literature on children supports a blocked or mixed schedule for learning whole body tasks, some researchers have found that typically developing children may learn skilled or sport-specific skills if a variable practice schedule is used (Vera et al., 2008; Douvis, 2005; Granda and Montilla, 2003). This variable practice schedule combines blocked and random practice elements and allows the child to benefit from practicing the new skill with elements of contextual interference. Vera and associates (2008) found that 9-year-old children performed the skill of kicking a soccer ball best by following blocked or combined practice, but only children in a combined practice situation improved in dribbling the soccer ball. Similarly, Douvis (2005) examined the impact of variable practice on learning the tennis forehand drive in children and adolescents. Adolescents did better than children on the task, reflecting the influence of age and development, but both age groups did the best with variable practice. The variable practice sessions allowed the tennis players to use the forehand drive in a manner that more resembled the actual game of tennis, where a player may use a forehand drive, then a backhand drive. Older adults’ motor learning is affected by aging. In general older adults demonstrate deficits in sequential learning, learning new technology, and effortful bimanual coordination patterns. Some of these deficits may be related to age-related declines in force production, sensory capacity or speed of sensory processing, and issues with divided attention. The good news is that older adults can improve motor performance with practice. Older adults perform tasks they are learning more slowly and with greater errors when compared to younger adults but they do benefit equally, as compared to younger adults, from practice schedules conducive to motor learning. 95 Neural Plasticity Neural plasticity is the ability of the nervous system to change. Although it has always been hypothesized that the nervous system could adapt throughout life, there is now ample evidence that the adult brain maintains the ability for reorganization or plasticity (Butefisch, 2004; Doyon and Benali, 2005; Bruel-Jungerman et al., 2007). Traditionally, it was always thought that plasticity was limited to the developing nervous system. Critical periods are times when neurons compete for synaptic sites. Activity-dependent changes in neural circuitry usually occur during a restricted time in development or critical period, when the organism is sensitive to the effects of experience. The concept of plasticity includes the ability of the nervous system to make structural changes in response to internal and external demands. Learning and motor behavior appear to modulate neurogenesis throughout life. Experience is critical to development. Two types of neural plasticity have been described in the literature (Black, 1998). Unfortunately, the names given to them are confusing. One is experience- expectant, and the other is experience-dependent. In the course of typical prenatal and postnatal development, the infant is expected to be exposed to sufficient environmental stimuli at appropriate times. In fact, if the infant is not exposed to the proper quality and quantity of input, development will not proceed normally. This type of experience-expectant neural plasticity is exemplified in the sensory systems that are ready to function at birth but require experience with light and sound to complete maturation. Deprivation during critical time periods can result in the lack of expected development of vision and hearing. Experience-dependent neural plasticity allows the nervous system to incorporate other types of information from environmental experiences that are relatively unpredictable and idiosyncratic. These experiences are unique to the individual and depend on the context in which development occurs, such as the physical, social, and cultural environment. Lebeer (1998) refers to this as ecological plasticity, whereas Johnston uses the term activity-dependent plasticity. Climate, social expectations, and child-rearing practices can alter movement experiences. What each child learns depends on the unique physical challenges encountered. Motor learning as part of motor development is an example of experience-dependent neural plasticity. Experiences of infants in different cultures may result in alterations in the acquisition of motor abilities. Similarly, not every child experiences the exact same words, but every child does learn language. Activity-dependent plasticity is what drives changes in synapses or neuronal circuits as a result of experience or learning. Recovery following injury to the nervous system occurs in one of two ways. One is a result of spontaneous recovery and the other way is function induced. For a more in-depth discussion of injury-induced plasticity and recovery of function, see Shumway-Cook and Woollacott (2012). Function-induced recovery is also known as use-dependent cortical reorganization. Regardless of the terminology, change results from activity which produces cortical reorganization, just as early experience drives motor and sensory development. Experience can drive recovery of function. Kleim and Jones (2008) summarized the research to date on activity-dependent neural plasticity and recommended 10 principles for neurorehabilitation. These are listed in Table 3-4 and are congruent with the principles of motor learning involving repetition and task specificity. Table 3-4 Principles of Experience-Dependent Plasticity Principle Description Lack of activity of certain brain functions can lead to functional loss. Use it and improve it] Training a specific brain function can lead to improvement in that function. The training experience must be specific to the expected change. Repetition Active repetition is needed to induce change. Training must be of a sufficient intensity to induce change. Salience The stimulus used to produce a response must be appropriate. Plasticity is more likely to occur in the young brain versus the older brain. Time Timing of intervention may help or hinder recovery. Training on one task may positively affect another similar task. Interference Plasticity in response to one experience can interfere with the acquisition of other behaviors. (Adapted from Kleim, Jones: Principles of experience-dependent neural plasticity: Implications for rehabilitation after brain damage. J Speech Hear Res 51:S225-S239, 2008.) Interventions Based on Motor Control, Motor Learning, and 96 Neural Plasticity Principles Evidence-based practice is the integration of clinical expertise, the best available evidence, and patient characteristics (Sackett et al., 2000). Previously, interventions have been based on neurophysiologic approaches, which focus on the impairments seen in individuals with neurologic dysfunction. More recently emphasis is placed on the activity limitations and participation restrictions encountered by those with neurologic dysfunction. The adoption of the International Classification of Functioning, Disability, and Health (ICF) by the American Physical Therapy Association (APTA) necessitates a broader, more functionally based view of interventions and the impact of those interventions on the quality of life of the individual. Interventions must be relevant to the individual, whether a child or an adult. The therapist planning interventions has to make them interesting and engaging. The motor activities selected must be engaging and meaningful to the person. The therapist selects the task to be performed and the environment as well as determines the type of practice and when feedback is given. Active participation is required for motor learning. The physical therapist’s and physical therapist assistant’s view of motor control and motor learning influence the choice of approach to therapy with children and adults with neuromuscular problems. Given that the prevailing view of motor control and motor learning is a systems view, all body systems must be taken into consideration when planning an intervention. Size and level of maturity of the body systems involved in movement must be considered. The age appropriateness of tasks relative to the mover’s cognitive ability to understand the task should also be considered. Some interventions used in treating children with neurologic dysfunction focus only on developing reactive postural reactions. Although children need to be safe within any posture that they are placed in or attain on their own, children also need to learn adaptive postural responses. Adaptive responses are learned within the context of reaching and grasping, locomotion, and play activities. Movement experiences should be as close to reality as possible. Using a variety of movement sequences to assist the infant or child to change and maintain postures is of the utmost importance during therapy and at home. Setting up situations in which the child has to try out different moves to solve a movement problem is ideal and is often the best therapy. This activity-based approach can maximize physical function and foster social, emotional, and cognitive development. Principles of forced use of an extremity that might be ignored have been extremely effective in adults and children with hemiplegia (Taub et al., 1993; Charles et al., 2001, Charles et al., 2006). Constraint-induced movement therapy (CIMT) involves both constraint of the noninvolved upper extremity of an individual with hemiplegia and repetitive practice of skilled activities or functional tasks. Lin (2007) found that patients with chronic stroke had improved motor control strategies during goal-directed tasks after CIMT. The Hand-Arm Bimanual Intervention (HABIT) program is an example of an effective CIMT program for children with hemiplegic cerebral palsy (Charles and Gordon, 2006; Gordon et al., 2007). A recent systematic review by Huang and colleagues (2009) found that CIMT increases upper extremity use. More research needs to be done to establish the best dosage. The mass practice in CIMT is thought to induce cortical reorganization and mapping, which increases efficiency of task performance in the hemiplegic upper extremity (Taub et al., 2004; Nudo et al., 1996). These findings reflect the influence of CIMT on activity-dependent neural plasticity. Use of partial body weight support treadmill training (PBWTT) as a form of gait practice does not require the person to have postural control of the trunk before attempting to walk. Task-specific practice has been shown to positively affect outcomes in adults with hemiplegia, incomplete spinal cord injuries and children with Down syndrome and cerebral palsy. PBWTT has been studied extensively and has been found to be safe for patients poststroke (Moseley et al., 2005). In a recent Cochrane review, Mehrholz and associates (2014) found that PBWTT significantly increased gait velocity and walking velocity during rehabilitation. Those individuals who could walk before treadmill training were able to maintain endurance gains through the follow-up period. The authors concluded that treadmill training with or without body weight support may improve gait speed and endurance in patients after a stroke who could walk, but not in dependent walkers. Treadmill training is also used with patients who have incomplete spinal cord injuries. In this case, the lower extremities are maximally loaded for weight bearing while using a body weight support system and manual cues. Evidence shows an increase in endurance, gait speed, balance, and independence (Behrman and Harkema, 2000; Dobkin et al., 2006; Field-Fote and Roach, 2011; and Harkema et al., 2012). 97 Partial body-weight support treadmill training has been successfully used as an intervention for children with spinal cord injury (Behrman et al., 2014 CSM). Young children with Down syndrome who participated in treadmill training walked earlier than the control group (Ulrich et al., 2001). When comparing intensity of training, the higher intensity group walked earlier than the lower intensity group (Ulrich et al., 2008). Positive results are reported in children with cerebral palsy. In those with Gross Motor Function Classification Scale level III and IV, there was a significant increase in gait speed motor performance (Willoughly et al., 2010). How a therapy session is designed depends on the type of motor control theory espoused. Theories guide clinicians’ thinking about what may be the reason the patient has a problem moving and about what interventions may remediate the problem. Therapists who embrace a systems approach may have the patient perform a functional task in an appropriate setting, rather than just practice a component of the movement thought to be needed for that task. Rather than having the child practice weight shifting on a ball, the assistant has the child sit on a bench and shift weight to take off a shoe. Therapists who use a systems approach in treatment may be more concerned about the amount of practice and the schedule for when feedback is given than about the degree or normality of tone in the trunk or extremity used to perform the movement. Using a systems approach, an assistant would keep track of whether or not the task was accomplished (knowledge of results) as well as how well it was done (knowledge of performance). Knowledge of results is important for learning motor tasks. The goal of every therapeutic intervention, regardless of its theoretic basis, is to teach the patient how to produce functional movements in the clinic, at home, and in the community. Interventions must be developmentally appropriate regardless of the age of the person. Although it may not be appropriate to have an 80-year-old creeping on the floor or mat table, it would be an ideal activity for an infant. All of us learn movement skills better within the context of a functional activity. Play provides a perfect functional setting for an infant and child to learn how to move. The physical therapist assistant working with an extremely young child should strive for the most typical movement possible in this age group although realizing that the amount and extent of the neurologic damage incurred will set the boundaries for what movement patterns are possible. Remember that it is also during play that a child learns valuable cause-and-effect lessons when observing how her actions result in moving herself or moving an object. Movement through the environment is an important part of learning spatial concepts. Motor learning must always occur within the context of function. It would not be an appropriate context for learning about walking to teach a child to walk on a movable surface, for example, because this task is typically performed on a non-movable surface. The way a task is first learned is usually the way it is remembered best. When stressed or in an unsafe situation, we often revert to this way of moving. For example, on many occasions a daughter of a friend is observed to go up and down the long staircase in her parents’ home, foot over foot without using a railing. When her motor skills were filmed in a studio in which the only stairs available were ones that had no back, the same child reverted to stepping up with one foot and bringing the other foot up to the same step (marking time) to ascend and descend. She perceived the stairs to be less safe and chose a less risky way to move. Infants and young children should be given every opportunity to learn to move correctly from the start. This is one of the major reasons for intervening early when an infant exhibits motor dysfunction. Motor learning requires practice and feedback. Remember what had to be done to learn to ride a bicycle without training wheels. Many times, through trial and error, you tried to get to the end of the block. After falls and scrapes, you finally mastered the task, and even though you may not have ridden a bike in a while, you still remember how. That memory of the movement is the result of motor learning. Assessing functional movement status is a routine part of the physical therapist’s examination and evaluation. Functional status may provide cues for planning interventions within the context of the functional task to be achieved. Therapeutic outcomes must be documented based on the changing functional abilities of the patient. When the physical therapist reexamines and reevaluates a patient with movement dysfunction, the physical therapist assistant can participate by gathering objective data about the number of times the person can perform an activity, what types of cues (verbal, tactile, pressure) result in better or worse performance, and whether the task can be successfully performed in more than one setting, such as the physical therapy gym or the patient's dining room. Additionally, the physical therapist assistant may comment on the consistency of the patient’s motor behavior. For instance, does the infant roll consistently from prone to supine or roll only occasionally when something or someone extremely interesting is enticing the infant to engage 98 in the activity? Chapter summary Motor control is ever-present. It directs posture and movement. Without motor control, no motor development or motor learning could occur. Motor learning provides a mechanism for the body to attain new skills regardless of the age of the individual. Motor learning requires feedback in the form of sensory information about whether the movement occurred and how successful it was. Practice and experience play major roles in motor learning. Motor development is the age-related process of change in motor behavior. Motor development is also the tasks acquired and learned during the process of moving. Neural plasticity is the ability of the nervous system to adapt to experience whether during the developmental process or as part of relearning actions limited by a neurologic insult. A neurologic deficit can affect an individual’s ability to engage in age- appropriate motor tasks (motor development), to learn or relearn motor skills (motor learning), or to perform the required movements with sufficient quality and efficiency to be effective (motor control). Purposeful movement requires that all three processes be used continually and contingently across the life span. Review questions 1. Define motor control, motor learning, and neural plasticity. 2. How do sensation, perception, and sensory organization contribute to motor control and motor learning? 3. How does posture influence motor development, motor control, and motor learning? 4. How is a postural response determined when visual and somatosensory input conflict? 5. When in the life span, can “adult” sway strategies be consistently demonstrated? 6. How much attention to a task is needed in the various phases of motor learning? 7. Give an example of an open task and of a closed task. 8. 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Describe the acquisition and refinement of fundamental movement patterns during childhood. 6. Describe age-related changes in functional movement patterns across the life span. 7. Describe how age-related systems changes affect posture, balance, and gait in older adults. 105 Introduction The Life Span Concept Normal developmental change is typically presumed to occur in a positive direction; that is, abilities are gained with the passage of time. For the infant and child, aging means being able to do more. The older infant can sit alone, and the older child can run. With increasing age, a teenager can jump higher and throw farther than a school-age child. Developmental change can also occur in a negative direction. Speed and accuracy of movement decline after maturity. When one looks at the ages of the gold medal winners in the last Olympics, it is apparent that motor performance peaks in early adolescence and early adulthood. Older adults perform motor activities more slowly and take longer to learn new motor skills. Traditional views of motor development are based on the positive changes that lead to maturity and the negative changes that occur after maturity. A true life span perspective of motor development includes all motor changes occurring as part of the continuous process of life. This continuous process is not a linear one but rather is a circular process. Some even describe motor development as a spiral process. Motor development does not occur in isolation of other developmental domains such as the psychological domain or the sociocultural domain. Figure 4-1 depicts the relationship of an individual’s mind and body developing within the sociocultural environment. Movement develops within three domains: physical, psychological, and sociocultural. FIGURE 4-1 Depiction of the relationship of an individual’s psychological (mind) and physical (body) self within the sociocultural environment. (From Cech D, Martin S: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders, p. 17.) A Life Span Approach The concept of life-span development is not new. Baltes (1987) originally identified five characteristics to use when assessing a theory for its life-span perspective. The following list reflects the original four criteria and the new fifth one used to view development from a lifelong perspective: = Lifelong a Multidimensional = Plastic a Embedded in history = Multicausal 106 Recently, Baltes et al. (2006) revisited the theoretical underpinnings of life span theory. They reinforced the idea that development is NOT complete at maturity. The multidimensional quality of life span theory provides a complete framework for ontogenesis (development). Culture and the knowledge gained from all domains make a significant impact on a person's life course. Biological plasticity is accompanied by cultural competence so that there is a gain/loss dynamic that occurs during development. There are no gains without losses and no loss without gains. In essence, this is the adaptive capacity of the person. Context, the original fifth criteria has been replaced by multicausal meaning that one can arrive at the same destination by different means or by a combination of means. Life span development is not constrained to travel a single course or developmental trajectory. There is variability. No one period of life can be understood without looking at its relationship to what came before and what lies ahead. History affects development in three ways as seen in Figure 4-2. The normative age-graded influence is seen in those developmental tasks described by Havinghurst (1972) for each period of development. Age-graded physical, psychological, and social milestones would fall into this category. Walking at 12 months and obtaining a driver's license at 16 years of age are examples of physical age-graded tasks. Understanding simple concepts such as round objects always roll and getting along with same age peers in adolescence are examples from the psychological and social domains. Moreover, normative history-graded influences come from the effect of when a person is born. Each of us is part of a birth cohort or group. Some of us are Baby Boomers and others are Millennials. All people in an age cohort share the same history of events, such as World War II, the Challenger disaster, the terrorist attack of 9/11, the Boston Marathon bombing, and the polar vortex. When you were born makes a difference in expectations and behaviors, these historical events shape the life of the cohort. The last history-related influence comes from things that happen to a person that have no norms or no expectations, such as winning the lottery, losing a parent, or having a child with a developmental disability. These are part of your own unique personal history. Life-span development provides a holistic framework in which aging is a lifelong process of growing up and growing old. Development within the biophysical, psychological, and sociocultural domains is enriched when viewed through a life-span perspective. Ontogenetic time Normative Normative Nor normative age history graded graded FIGURE 4-2 Three major biocultural influences on life span development. (From Cech D, Martin S: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders, p. 17.) Life-Span View of Motor Development The concept of motor development has been broadened to encompass any change in movement abilities that occurs across the span of life, so changes in the way a person moves after childhood are included. Motor development continues to elicit change, from conception to death. Think of the classic riddle of the pharaohs: what creeps in the morning and walks on two legs in the afternoon and on three in the evening? The answer is a human in various stages, as an infant who creeps, a toddler who walks alone throughout adulthood, and an older adult who walks with a cane at the end of life. 107 108 Developmental time periods Age is the most useful way to measure change in development because it is a universally recognized marker of biologic, psychological, and social progression. Infants become children, then adolescents, and finally adults at certain ages. Aging is a developmental phenomenon. Stages of cognitive development are associated with age, as are societal expectations regarding the ability of an individual to accept certain roles and functions. Defining these time periods gives everyone a common language when talking about motor development and allows comparison across developmental domains (physical, psychological, and social). Everyone knows that a 3-year-old child is not an adult, but when does childhood stop and adolescence begin? When does an adult become an older adult? A list of commonly defined time periods that are used throughout the text is found in Table 4-1. Table 4-1 Developmental Time Periods (Changes to Older Adulthood) Period Time Span Childhood 2-10 years (females ae 2-12 years (males) Adolescence 10-18 years (females) 12-20 years (males) Early adulthood _| 18/2040 years Middle adulthood} 40-70 years Older adulthood _| 70 years to death Infancy Infancy is the first period of development and spans the initial 2 years of life following birth. During this time, the infant establishes trust with caregivers and learns to be autonomous. The world is full of sensory experiences that can be sampled and used to learn about actions and the infant’s own movement system. The infant uses sensory information to cue movement and uses movement to explore and learn about the environment. Therefore, a home must be baby-proofed to protect an extremely curious and mobile infant or toddler. Childhood Childhood begins at 2 years and continues until adolescence. Childhood fosters initiative to plan and execute movement strategies and to solve daily problems. The child is extremely aware of the surrounding environment, at least one dimension at a time. During this time, she begins to use symbols, such as language, or uses objects to represent things that can be thought of but are not physically present. The blanket draped over a table becomes a fort, or pillows become chairs for a tea party. Thinking is preoperational, with reasoning centered on the self. Self-regulation is learned with help from parents regarding appropriate play behavior and toileting. Self-image begins to be established during this time. By 3 to 5 years of age, the preschooler has mastered many tasks such as sharing, taking turns, and repeating the plot of a story. The school-age child continues to work industriously for recognition on school projects or a special school fund-raising assignment. Now the child is able to classify objects according to certain characteristics, such as round, square, color, and texture. This furtherance of thinking abilities is called concrete operations. The student can experiment with which container holds more water (the tall, thin one or the short, fat one) or which string is longer. Confidence in one’s abilities strengthens an already established positive self-image. Adolescence Adolescence covers the period right before, during, and after puberty, encompassing different age spans for boys and girls because of the time difference in the onset of puberty. Puberty and, therefore, adolescence begins at age 10 for girls and age 12 for boys. Adolescence is 8 years in length regardless of when it begins. Because of the age difference in the onset of adolescence, girls may exhibit more advanced social emotional behavior than their male counterparts. In a classroom of 13- 109 year-olds, many girls are completing puberty, whereas most boys are just entering it. Adolescence is a time of change. The identity of the individual is forged, and the values by which the person will live life are embraced. Physical and social-emotional changes abound. The end result of a successful adolescence is the ability to know who one is, where one is going, and how one is going to get there. The pursuit of a career or vocation assists the teenager in moving away from the egocentrism of childhood (Erikson, 1968). Cognitively, the teenager has moved into the formal operations stage in which abstract problems can be solved by inductive and deductive reasoning. These cognitive abilities help one to weather the adolescent identity crisis. Practicing logical decision making during this period of life prepares the adolescent for the rigors of adulthood, in which decisions become more and more complex. Adulthood As a concept, adulthood is a twentieth-century phenomenon. Adulthood is the longest time period of human life and the one about which the least is known. Adulthood is achieved by 20 years of age biologically, but psychologically it may be marked by as much as a 5-year transition period from late adolescence (17 years) to early adulthood (22 years). Levinson (1986) called this period the early adulthood transition because it takes time for the adolescent to mature into an adult. Research supports the existence of this and other transition periods. Although most of adulthood has been considered one long period of development, some researchers, such as Levinson, identify age- related stages. Middle adulthood begins at 40 years, with a 5-year transition from early adulthood, and it ends with a 5-year transition into older adulthood (age 60). Arnett (2000, 2004, 2007) proposed a theory of emerging adulthood. The period between adolescence and the beginning of adulthood is seen as beginning at age 18 and ending at age 25. The characteristics seen during this time are: (1) a feeling of being in-between, (2) instability, (3) identity exploration, (4) self-focus, and (5) possibility. Arnett suggests that the forging of the person’s identity occurs during this time period as opposed to adolescence as espoused by Erikson. There is some data to support the prolongation of adolescence into the early college years and the delay of taking on adult roles until after graduation. George Valliant (2002), a psychiatrist and director of the Harvard study of adult development, inserted two new stages into Erikson’s (1968) original eight stages: career consolidation and keeper of the meaning. Career consolidation comes between Erikson’s stages of intimacy and generativity. In career consolidation stage, a person chooses a career. It begins between 20 and 40 years of age when young adults become focused on assuming a social identity within the work world. This is an extension of the person’s personal identity forged in earlier stages. Valliant (2002) identified four criteria that transform a “job” or “hobby” into a “career.” They are competence, commitment, contentment, and compensation. The other stage will be discussed later in this section. What makes a person an adult? Is there a magic age or task to be attained that indicates when a person is an adult? Legally, you are an adult at 18. However, there are many 18-year-olds who would more than likely consider themselves as emerging adults. Regardless of the socioeconomic group a person belongs to, four criteria for adulthood continue to resound in the literature (Arnett, 2007). To be an adult, one must accept responsibility for your actions, make independent decisions, be more considerate of others, and be financially independent. “Maturity requires the acceptance of responsibility and empathy for others” (Purtilo and Haddad, 2007, p. 272). Keeper of meaning is the additional stage Vaillant (2002) interjected between Erikson's generativity and integrity stages. It comes near the end of generativity so the person is in late middle adulthood. The role of the keeper of meaning is to preserve one's culture rather than care for successive generations. The focus is on conservation as well as preservation of society's institutions. The person in this stage guides groups and preserves traditions. Think of the interest older adults often have in geneology as an example of this stage in development. Family Systems The concept of family is very broad with families having many different structures and life styles. Single-parent families have increased tremendously over the past decades. Regardless of structure, family function is affected by each member of the family. This can be thought of as family dynamics or in Bronfenbrenner's model as a system of interacting elements. Each parent affects the other, the child or children, and in turn, the child or children affect the parent. The family as a system is 110 embedded in larger social systems such as the extended family, neighborhood, and school and religious organizations. All of these systems can influence the family. Recognizing the dynamics within a family is very important when establishing a therapeutic relationship. Family-centered intervention is a life-span approach (Chiarello, 2013). Families have a life cycle in which stages and transitions have been identified. However, the reader is referred to Carter and McGoldrick (2005) for an expanded and updated discussion of family. Older Adulthood Gerontologists, those researchers who study aging, use age 70 as the beginning of old age (Atchley and Barusch, 2004). We are aging from the moment we are born. Much is known about aging. The major theory of aging is the free radical theory. It is also known as the oxidative damage hypothesis. Oxidative damage accumulates in the large molecules of our body, such as DNA, RNA, protein, carbohydrates, and lipids. The nervous and muscular systems are particularly prone to oxidative damage caused by the tissues’ high metabolic rate. Age-related systems decline that can in some ways be offset by good nutrition, hydration, and exercise. Successful aging is possible if the older adult stays engaged and active and does not disengage from the world. Rowe and Kahn (1997) identified three components of successful aging based on longitudinal studies by the MacArthur Foundation. The number one component is avoiding disease and disability; number two is having a high cognitive and physical functional capacity; and number three is active engagement with life. Unlike the activity theorist, Rowe and Kahn (1997) defined activity as something that holds societal value. The activity does not have to be remunerated for it to be considered as productive. 111 Influence of cognition and motivation The three processes of motor development, motor control, and motor learning are influenced to varying degrees by a person’s intellectual ability. Impairments in cognitive ability can affect an individual's ability to learn to move. A child with intellectual disability may not have the ability to learn movement skills at the same rate as a child of normal intelligence. The rate of developmental change in a child with an intellectual disability is decreased in all domains: physical, psychological, and social. Thus, acquisition of motor skills is often as delayed as the acquisition of other knowledge. Just as cognition can affect motor development, the motor system can affect cognition. Diamond (2000), Piek et al. (2008), and Pitcher et al. (2011) linked motor development and subsequent cognitive ability. The close interrelation of the prefrontal cortex and the cerebellum parallels the protracted development of the motor system. Motor development of children between birth and 4 years predicted cognitive performance at school age (Piek et al., 2008). The two most negative outcomes of being born prematurely and having a low birth weight are impaired motor and cognitive development (Hack and Fanaroff, 2000). Grounded cognition is a concept in which cognition is embedded in the environment and the body (Barsalou, 2010). The child makes use of perceptual motor experiences to develop cognition in a learning to learn paradigm. Researchers have called for therapists to recognize object interaction, sitting, and locomotion as models for grounded cognition (Lobo et al., 2012). As a recommendation, add pretend play to the model for grounded cognition because it provides support for language development as well as motor development. Pretend play is a natural progression from object interaction to mental representation of objects not in view. See Chapter 5 for additional information regarding play. Motivation to move comes from intellectual curiosity. Typically developing children are innately curious about the movement potential of their bodies. Infants become visually aware of their own movement. This optically produced awareness is called visual proprioception (Gibson, 1966; Gibson, 1979). Locomotion affords toddlers more exploration of the environment which supports psychological development (Anderson et al., 2014). Children move to be involved in some sports- related activities, such as tee-ball or soccer. Adolescents often define themselves by their level of performance on the playing field, so a large part of their identity is connected to their athletic prowess. Adults may routinely participate in sports-related activities as part of their leisure time. One hopes that activity is part of a commitment to fitness developed early in life. Motor control is needed for motor learning, for the execution of motor programs, and for progression through the developmental sequence. The areas of the brain involved in idea formation can be active in triggering movement. Movement is affected by the ability of the mind to understand the rules of moving. Children around the age of 5 begin to develop the ability to imagine motion or mentally represent action (Gabbard, 2009). This is termed motor imagery. There is a positive association between motor abilities in children and their motor imagery (Gabbard et al., 2012). Children continue to show improvements in this ability even into adolescence (Molina et al., 2008; Choudhury et al., 2007). Movement is also a way of exerting control over the environment. Remember the old sayings: “mind over matter” and “I think I can.” Learning to control the environment begins with controlling one’s own body. To interact with objects and people within the environment, the child must be oriented within space. We learn spatial relationships by first orienting to our own bodies, then using ourselves as a reference point to map our movements within the environment. Physical educators and coaches have used the ability of the athlete to know where he or she is on the playing field or the court to better anticipate the athlete’s own or the ball’s movement. The role of visualizing movement as a way to improve motor performance is documented in the literature (Wang and Morgan, 1992). Sports psychologists have extensively studied cognitive behavioral strategies, including motivation, and recognize how powerful these strategies can be in improving motor performance (Meyers et al., 1996). We have all had experience with trying to learn a motor skill that we were interested in as opposed to one in which we had no interest. Think of the look on an infant’s face as she attempts that first step; one little distraction and down she goes. Think also of how hard you may have to concentrate to master in-line skating; would you dare to think of other things while careening down a sidewalk for the first time? Because development takes place in more than one dimension, not just in the motor area, the following psychological 112 theories, with which you may already be familiar, are used to demonstrate what a life-span perspective is and is not. These psychological theories can also reflect the role movement may play in the development of intelligence, personality, and perception. Piaget Piaget (1952) developed a theory of intelligence based on the behavioral responses of his children. He designated the first 2 years of life the sensorimotor stage of intelligence. During this stage, the infant learns to understand the world by associating sensory experiences with physical actions. Piaget called these associations schemas. The infant develops schemas for looking, eating, and reaching, to name just a few. From 2 to 7 years is the preoperational stage of intelligence during which the child is able to represent the world by symbols, such as words and objects. The increased use of language is the beginning of symbolic thought. During the next stage, concrete operations, logical thought occurs. Between 7 and 11 years of age, children can mentally reverse information. For example, if they learned that 6 plus 4 equals 10, then 4 plus 6 would also equal 10. The last stage is that of formal operations, which Piaget thought began at 12 years of age. Although research has not completely supported the specific chronologic years to which Piaget attributed these stages, the stages do occur in this order. The stage of formal operations begins in adolescence, which, according to our time periods, begins at 10 years in girls and at 12 years in boys. Piaget’s stages are related to developmental age in Table 4-2. Table 4-2 Piaget’s Stages of Cognitive Development Life Span Period Stage Characteristics Infancy Sensorimotor Pairing of sensory and motor reflexes leads to purposeful activit Preschool Preoperational Unidimensional awareness of environment Begins use of symbols School age Concrete operational] Solves problems with real objects Classification, conservation Pubescence Formal operational | Solves abstract problems Induction, deduction Data from Piaget J: Origins of intelligence, New York, 1952, International University Press. Piaget studied the development of intelligence up to adolescence, when abstract thought becomes possible. Because abstract thought is the highest level of cognition, he did not continue to look at what happened to intelligence after maturity. Because Piaget’s theory does not cover the entire life span, it does not represent a life-span approach to intellectual development. However, Piaget does offer useful information about how an infant can and should interact with the environment during the first 2 years of life. These first 2 years are critical to the development of intelligence. Regardless of the age of the child, the cognitive level must always be taken into account when one plans therapeutic intervention. Maslow and Erikson In contrast, Maslow (1954) and Erikson (1968) looked at the entire spectrum of development from beginning to end. Maslow identified the needs of the individual and how those needs change in relation to a person’s social and psychological development. Rather than describing stages, Maslow developed a hierarchy in which each higher level depends on mastering the one before. The last level mastered is not forgotten or lost but is built on by the next. Maslow stressed that an individual must first meet basic physiological needs to survive, and then and only then can the individual meet the needs of others. The individual fulfills physiological needs, safety needs, needs for loving and belonging, needs for esteem, and finally self-actualization. Maslow’s theory is visually depicted in Figure 4-3. A self-actualized person is self-assured, autonomous, and independent; is oriented to solving problems; and is not self-absorbed. Although Maslow’s theory may not appear to be embedded in history, it tends to transcend any one particular time in history by being universally applicable. 113 Self- actualization Esteem Love, Belongingness, Affection Physiologic/Survival Needs (Food, Water, Elimination) FIGURE 4-3 Maslow’s hierarchy. (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) Erikson described stages that a person goes through to establish personality. These stages are linked to ages in the person’s life, with each stage representing a struggle between two opposing traits. For example, the struggle in infancy is between trust and mistrust. The struggle in adolescence is ego identity. Erikson’s theory as shown in Table 4-3 is an excellent example of a life- span approach to development. Table 4-3 Erikson’s Eight Stages of Development Life Span Period Stage Characteristics Late infanc’ Autonomy versus shame or doubt} Independence, self-control Childhood (pre-school) Initiation of own activity School age Industry versus inferiorit Working on projects for recognition Sense of self: physically, socially, sexually] Early adulthood Intimacy versus isolation Relationship with significant other Late adulthood Ego integrity versus despair Sense of wholeness, vitality, wisdom Adapted from Erikson E: IDENTITY: youth and crisis. © 1968 W.W. Norton & Company. Used by permission of W.W. Norton & Company. Although all three of these psychologists present important information that will be helpful to you when you work with people of different ages, it is beyond the scope of this text to go into further detail. The reader is urged to pursue more information on any of these theorists to add to an understanding of people of different ages and at different stages of psychological development. A life-span perspective can assist in an understanding of motor development by acknowledging and taking into consideration the level of intellectual development the person has attained or is likely to attain. 114 Theories of Motor Development The two prevailing theories of motor development are the dynamic systems theory and the neuronal group selection theory. These theories reflect the state of our current knowledge. Thelen and Smith (1994) proposed a functional view of the process of motor development that they called a dynamical systems theory (DST). In this theory, movement emerges from the interaction of multiple body systems. DST incorporates the developmental biomechanical aspects of the mover, along with the developmental status of the mover’s nervous system, the environmental context in which the movement occurs and the task to be accomplished by the movement. The acquisition of postural control and balance are driven by the requirement of the specific task demands and the demands of gravity. Movement abilities associated with the developmental sequence are the result of motor control, which organizes movements into efficient patterns. DST is both a theory of motor control and of motor development. The brain and the neuromotor systems must interact to meet the developmental demands of the mover. Growth, maturation, and adaptation of all body systems contribute to the acquisition of movement not just the nervous system. Movement emerges from the interaction of all body systems, the task at hand, and the environment in which it takes place. To acquire motor skills, the mover has to control the number of planes of motion possible at a single joint and then multiple joints. This is the degrees of freedom problem discussed in Chapter 3. Bernstein thought that the new or novice mover minimized the number of independent movement elements used until control was developed. The new walker is a great example of controlling degrees of freedom. The upper trunk is kept in extension by placing the arms in high guard while the lower trunk is kept stable by anteriorly tilting the pelvis. The infant is left with only having to pick up each leg at a time as if stepping in place. A little forward momentum is used to propel the new walker. Neuronal group selection (Andreatta, 2006) proposes that motor skills result from the interaction of developing body dynamics and the structure or functions of the brain. The brain’s structures are changed by how the body is used (moved). The brain’s growing neural networks are sculpted to match efficient movement solutions. Three requirements must be met for neuronal selection to be effective in a motor system. First, a basic repertoire of movement must be present. Second, sensory information has to be available to identify and select adaptive forms of movement, and third, there must be a way to strengthen the preferred movement responses. The infant is genetically endowed with spontaneously generated motor behaviors. Figure 4-4 illustrates rudimentary neural networks that produce initial motor behaviors. This example involves activation of postural muscles in sitting infants. As the infant’s multiple sensory systems provide perception, the strength of synaptic connections between brain circuits is varied with selection of some networks that predispose one action over another. Environmental and task demands become part of the neural ensemble for producing movements. Spatial maps are formed and mature neural networks emerge as a product of use and sensory feedback. The maps that develop via the process of neuronal selection are preferred pathways. They become preferred because they are the ones that are used more often. These pathways connect large amounts of the nervous system and provide an interconnected organization of perception, cognition, emotion, and movement (Campbell, 2000). Prestructured Selected motor commands motor commands Experience- .-s, 200 dependent sos aN selection ae — sae a Dorsal Ria Dorsal FIGURE 4-4 A developmental process according to the neuronal group selection theory is exemplified by the development of postural muscle activation patterns in sitting infants. Before independent sitting, the infant exhibits a large variation of muscle activation patterns in response to external perturbations, including a backward body sway. Various postural muscles on the ventral side of the body are contracted in different combinations, sometimes together with inhibition of the dorsal muscles. Among the large repertoire of response patterns are the patterns 115 later used by adults. With increasing age, the variability decreases and fewer patterns are elicited. Finally, only the complete adult muscle activation patterns remain. If balance is trained during the process, the selection is accelerated. (Redrawn from Forssberg H: Neural control of human motor development. Curr Opin Neurobiol 9:676-682, 1999.) The theory of neuronal group selection supports a dynamic systems theory of motor control/motor development. According to neuronal group selection, the brain and nervous system are guided during development by a genetic blueprint and initial activity, which establishes rudimentary neuronal circuits. These early neuronal circuits are examples of self-organization. The use of certain circuits over others reinforces synaptic efficacy and strengthens those circuits. This is the selectivity that comes from exploring different ways of moving. Lastly, maps are developed that provide the organization of patterns of spontaneous movement in response to mover and task demands. The linking of these early perception-action categories is the cornerstone of development (Edelman, 1987). Other body systems, such as the skeletal, muscular, cardiovascular, and pulmonary systems develop and interact with the nervous system so that the most efficient movement pattern is chosen for the mover. According to this theory, there are no motor programs. The brain is not thought of as a computer and movement is not hardwired. This theory supports the idea that neural plasticity may be a constant feature across the life span. Neural plasticity is the ability to adapt structures in the nervous system to support desired functions. Neurons that fire together, wire together. Movement variability has always been considered a hallmark of normal movement. This integration of multiple systems allows for a variety of movement strategies to be used to perform a functional task. In other words, think of how many different ways a person can reach for an object or how many different ways it is possible for a person to move across a room. 116 Developmental concepts Many concepts apply to human motor development. These are not laws of development but merely guiding thoughts about how to organize information on motor development. The concepts are related to the direction of change in the pattern of skill acquisition and concepts related to the types of movement displayed during different stages of development. The one overriding concept about which all developmentalists continue to agree is that development is sequential (Gesell et al., 1974). The developmental sequence is still recognized by most developmental authorities. Areas of disagreement involve the composition of the sequence. Which specific skills are always part of the sequence is debated, and whether one skill in the sequence is a prerequisite for the next skill in the sequence has been questioned. Epigenesis Motor development is epigenetic. Epigenesis is a theory of development that states that a human being grows and develops from a simple organism to a more complex one through progressive differentiation. An example from the plant world is the description of how a simple, round seed becomes a beautiful marigold. Motor development generally occurs in an orderly sequence, based on what has come before; not like a tower of blocks, built one on top of the other, but like a pyramid, with a foundation on which the next layer overlaps the preceding one. This pyramid allows for growth and change to occur in more than one direction at the same time (Figure 4-5). The developmental sequence is generally recognized to consist of the development of head control, rolling, sitting, creeping, and walking. The sequence of actions are known as motor milestones. The rate of change in acquiring each skill may vary from child to child within a family, among families, and among families of different cultures. Sequences may overlap as the child works on several levels of skills at the same time. For example, a child can be perfecting rolling while learning to balance in sitting. The lower-level skill does not need to be perfect before the child goes on to try something new. Some children even bypass a stage, such as creeping, and go on to another higher- level skill, such as walking without doing any harm developmentally. FIGURE 4-5 Epigenetic development. 117 Directional Concepts of Motor Development Postural development tends to proceed from cephalic to caudal and proximal to distal. Cephalic to Caudal Cephalocaudal development is seen in the postnatal development of posture. Head control in infants begins with neck movements and is followed by development of trunk control. Postnatal postural development mirrors what happens in the embryo when the primitive spinal cord closes. Closure occurs first in the cervical area and then progresses in two directions at once, toward the head and the tail of the embryo (Martin, 1989). The infant develops head and neck and then trunk control. Overlap exists between the development of head-and-trunk control; think of a spiral beginning around the mouth and spreading outward in all directions encompassing more and more of the body (Figure 4-6). Development of postural control of the head and neck can be a rate- limiting factor in early motor development. If control of the head and neck is not mastered, subsequent motor development will be delayed. *5 at) FIGURE 4-6 Infant and spiral development. Proximal to Distal As a linked structure, the axis or midline of the body must provide a stable base for head, eye, and extremity movements to occur with any degree of control. The trunk is the stable base for head movement above and for limb movements distally. Imagine what would happen if you could not maintain an erect sitting posture without the use of your arms and you tried to use your arms to catch a ball thrown to you. You would have to use your arms for support, and if you tried to catch the ball, you would probably fall. Or imagine not being able to hold your head up. What chance 118 would you have of being able to follow a moving object with your eyes? Early in development, the infant works to establish midline neck control by lifting the head from the prone position, then establishes midline trunk control by extending the spine against gravity, followed by establishing proximal shoulder and pelvic girdle stability through weight bearing. In some positions, the infant uses the external environment to support the head and trunk to move the arms and legs. Reaching with the upper extremities is possible early in development but only with external trunk support, as when placed in an infant seat in which the trunk is supported. Once again, the infant first controls the midline of the neck, then the trunk, followed by the shoulders and pelvis before she controls the arms, legs, hands, and feet. General Concepts of Development Dissociation A general concept is that development proceeds from mass movements to specific movements or from simple movements to complex movements. This concept can be interpreted in several different ways. Mass can refer to the whole body, and specific can refer to smaller parts of the body. For example, when an infant moves, the entire body moves; movement is not isolated to a specific body part. Infant movement is characterized by the mass movements of the trunk and limbs. The infant learns to move the body as one unit, as in log rolling, before she is able to move separate parts. The ability to separate movement in one body part from movement in another body part is called dissociation. Mature movements are characterized by dissociation, and typical motor development provides many examples. When an infant learns to turn her head in all directions without trunk movement, the head can be said to be dissociated from the trunk. Reaching with one arm from a prone on elbows position is an example of limb dissociation from the trunk. While the infant creeps on hands and knees, her limb movements are dissociated from trunk movement. Additionally, when the upper trunk rotates in one direction and the lower trunk rotates in the opposite direction during creeping (counter-rotation), the upper trunk is dissociated from the lower trunk and vice versa. Reciprocal Interweaving Periods of stability and instability of motor patterns have been observed by many developmentalists. Gesell et al. (1974) presented the concept of reciprocal interweaving to describe the cyclic changes they observed in the motor control of children over the course of early development. Periods of equilibrium were balanced by periods of disequilibrium. Head control, which appears to be fairly good at one age, may seem to lessen at an older age, only to recover as the infant develops further. At each stage of development, abilities emerge, merge, regress, or are replaced. During periods of disequilibrium, movement patterns regress to what was present at an earlier time, but after a while, new patterns emerge with newfound control. At other times, motor abilities learned in one context, such as control of the head in the prone position, may need to be relearned when the postural context is changed; for example, when the child is placed in sitting. Some patterns of movement appear at different periods, depending on need. The reappearance of certain patterns of movement at different times during development can also be referred to as reciprocal interweaving. One of the better examples of this reappearance of a pattern of movement is seen with the use of scapular adduction. Initially, this pattern of movement is used by the infant to reinforce upper trunk extension in the prone position. Later in development, the toddler uses the pattern again to maintain upper trunk extension as she begins to walk. This use in walking is described as a high-guard position of the arms. Reciprocal interweaving represents a spiral pattern of development. Variation and Variability Motor development can be described as occurring in two phases of variability. During the initial phase of variability, motor patterns are extremely variable as the mover explores all kinds of possible movement combinations. The sensory information generated by these movements continues to shape the nervous system’s development. There is mounting evidence that self- produced sensorimotor experience plays a pivotal role in motor development (Hadders-Algra, 2010). 119 The second phase of variability begins when the nervous system is able to make sense of the sensory information produced by movement to be able to select the most appropriate motor response for the situation. The mechanism for the switch from primary to secondary variability is unknown. The age at which adaptive responses occur can vary, depending on the function involved. For example, sucking behavior exhibits secondary variability before term (Eishima, 1991). The mechanics of sucking are well worked out and coordinated by birth. Postural adjustments are seen in the trunk at 3 months of age (Hedburg et al., 2005). All basic motor functions are thought to reach a beginning stage of secondary variability around 18 months of age. These basic motor functions include posture and locomotion as well as reaching and grasping. Variation and variability have always been considered hallmarks of typical motor development. Children who move in stereotypical ways or appear stuck in one pattern of movement have been deemed to be at risk. Assessment of variability in postural control during infancy may hold promise for early identification of motor problems (Dusing and Harbourne, 2010). Biomechanical Considerations in Motor Development Physiologic Flexion to Antigravity Extension to Antigravity Flexion The next concepts to be discussed are related to changes in the types of movement displayed during different stages of development. Some movements are easier to perform at certain times during development. Factors affecting movement include the biomechanics of the situation, muscle strength, and level of neuromuscular maturation and control. Full-term babies are born with predominant flexor muscle tone (physiologic flexion). The limbs and trunk naturally assume a flexed position (Figure 4-7). If you try to straighten or uncoil any extremity, it will return to its original position easily. It is only with the influence of gravity, the infant’s body weight, and probably some of the early reflexes that the infant begins to extend and lose the predisposition toward flexion. As development progresses, active movement toward extension occurs. Antigravity extension is easiest to achieve early on because the extensors are in lengthened position from the effect of the newborn’s physiologic flexed posture. The extensors are ready to begin functioning before the shortened flexors. The infant progresses from being curled up in a fetal position, dominated by gravity, to exhibiting the ability to extend against gravity actively. Antigravity flexion is exhibited from the supine position and occurs later than antigravity extension. FIGURE 4-7 Physiologic flexion in a newborn. Babies have a C-shaped spine at birth. Exposure to head lifting in prone develops the secondary cervical curve. Without exposure to the prone position in the form of tummy time, the ability of the infant to lift and turn the head is diminished. The risk of plagiocephaly or a misshapen head is increased, because in supine, the infant tends to assume an asymmetrical head posture. The neck muscles are not strong enough to maintain the head in midline. Tummy time is essential to encourage lifting and turning of the head to strengthen the neck muscles bilaterally. 120 Developmental processes Motor development is a result of three processes: growth, maturation, and adaptation. Growth Growth is any increase in dimension or proportion. Examples of ways that growth is typically measured include size, height, weight, and head circumference. Infants’ and children’s growth is routinely tracked at the pediatrician’s office by use of growth charts (Figure 4-8). Growth is an important parameter of change during development because some changes in motor performance can be linked to changes in body size. Typically, the taller a child grows, the farther she can throw a ball. Strength gains with age have been linked to increases in a child’s height and weight (Malina et al., 2004). Failure to grow or discrepancies between two growth measures can be an early indicator of a developmental problem. IR FIGURE 4-8 Growth chart. (Used with permission of Ross Products Division, Abbott Laboratories Inc., Columbus, OH 43216. From NCHS Growth Charts © 1982 Ross Products Division, Abbott Laboratories Inc.). Maturation Maturation is the result of physical changes that are caused by preprogrammed internal body processes. Maturational changes are those that are genetically guided, such as myelination of nerve fibers, the appearance of primary and secondary bone growth centers (ossification centers), increasing complexity of internal organs, and the appearance of secondary sexual characteristics. 121 Some growth changes, such as those that occur at the ends of long bones (epiphyses), occur as a result of maturation; when the bone growth centers (under genetic control) are active, length increases. After these centers close, growth is stopped, and no more change in length is possible. Adaptation Adaptation is the process by which environmental influences guide growth and development. Adaptation occurs when physical changes are the result of external stimulation. An infant adapts to being exposed to a contagion, such as chickenpox, by developing antibodies. The skeleton is remodeled during development in response to weight bearing and muscular forces (Wolfe’s law) exerted on it during functional activities. As muscles pull on bone, the skeleton adapts to maintain the appropriate musculotendinous relationships with the bony skeleton for efficient movement. This same adaptability can cause skeletal problems if musculotendinous forces are abnormal (unbalanced) or misaligned and may thus produce a deformity. 122 Motor milestones The motor milestones and the ages at which these skills can be expected to occur can be found in Tables 4-4 and 4-5. Remember there are wide variations in time frames during which milestones are typically achieved. Table 4-4 Infant Motor Milestones e Roll segmentally supine to prone 6-8 months Sit alone steadily Creep reciprocally, pulls to stand 8-9 months Walk alone 12 months Table 4-5 Reach, Grasp, and Release Milestones Milestone Ag Action Age Visual regard of objects 0-2 months Swipes at objects 1-3 months Visually directed reaching 3.5-4.5 months} Reaching from prone on elbow 6 months Retains objects placed in hand 4 months Palmar grasp 6 months Radial-palmar grasp 7 months Scissors gras; 8 months Radial-digital grasp 9 months Inferior pincer 10-12 months Superior pincer 12 months Three-jaw chuck 12 months Involuntary release 14 months Transfers at midline 4 months Transfers across body 7 months Voluntary release 7-10 months Release a block into small container| 12 months Release pellet into small container_| 15 months Head Control An infant should exhibit good head control by 4 months of age. The infant should be able to keep the head in line with the body (ear in line with the acromion) when he or she is pulled to sit from the supine position (Figure 4-9). When the infant is held upright in a vertical position and is tilted in any direction, the head should tilt in the opposite direction. A 4-month-old infant, when placed in a prone position, should be able to lift the head up against gravity past 45 degrees (Figure 4-10). The infant acquires an additional component of antigravity head control, the ability to flex the head from supine position, at 5 months. 123 FIGURE 4-10 Head lifting in prone. A 4-month-old infant lifts and maintains head past 45 degrees in prone. (From Wong DL: Whaley and Wong’s essentials of pediatric nursing, ed 5, St. Louis, 1997, Mosby.) Segmental Rolling Rolling is the next milestone. Infants log roll (at 4 to 6 months) before they are able to demonstrate segmental rotation (at 6 to 8 months). When log rolling, the head and trunk move as one unit without any trunk rotation. Segmental rolling or rolling with separate upper and lower trunk rotation should be accomplished by 6 to 8 months of age. Rolling from prone to supine precedes rolling from supine to prone, because extensor control typically precedes flexorcontrol. The prone position provides some mechanical advantage because the infant’s arms are under the body and can push against the support surface. If the head, the heaviest part of the infant, moves laterally, gravity will assist in bringing it toward the support surface and will cause a change of position. 124 Sitting This next milestone represents a change in functional orientation for the infant. The previous norm for achieving independent sitting was 8 months of age (Figure 4-11). However, according to the World Health Organization (WHO) (2006) the mean age at which infants around the world now sit, is 6.1 months (SD of 1.1). Sitting independently is defined as sitting alone when placed. The back should be straight, without any kyphosis. No hand support is needed. The infant does not have to assume a sitting position but does have to exhibit trunk rotation in the position. The ability to turn the head and trunk is important for interacting with the environment and for dynamic balance. FIGURE 4-11 Sitting independently. Creeping and Cruising Babies may first crawl on their tummy, but according to WHO (2006), infants reciprocally creep on all fours at 8.5 months (SD 1.7) (see Figure 4-13). Reciprocal means that the opposite arm and leg move together and leave the other opposite pair of limbs to support the weight of the body. By 10 to 11 months of age, most infants are pulling up to stand and are cruising around furniture. Cruising is walking sideways while being supported by hands or tummy on a surface (Figure 4-12). The coffee table and couch are perfect for this activity because they are usually the correct height to provide sufficient support to the infant (Figure 4-13). Some infants skip crawling on the belly and go into creeping on hands and knees. Other infants skip both forms of prone movement and pull to stand and begin to walk. 125 FIGURE 4-12 A and B, Cruising around furniture. FIGURE 4-13 Reciprocal creeping. Walking The last major gross motor milestone is walking (Figure 4-14). The new walker assumes a wide base of support, with legs abducted and externally rotated; exhibits lumbar lordosis; and holds the arms in high guard with scapular adduction. The traditional age range for this skill has been 12 to 18 months; however, an infant as young as 7 months may demonstrate this ability. Children demonstrate great variability in achieving this milestone. The most important milestones are probably head control and sitting, because if an infant is unable to achieve control of the head and trunk, control of extremity movements will be difficult if not impossible. WHO (2006) gives an average age of 12.1 months (SD 1.8) for children to accomplish independent movement in upright. There are ethnic differences in the typical age of walking. African-American children have been found to walk earlier (10.9 months) (Capute et al., 1985), while some Caucasian children walk as late as 15.5 months (Bayley, 2005). It is acceptable for a child to be ahead of typical developmental guidelines; however, delays in achieving these milestones are cause for concern. 126 Wy : cy KY FIGURE 4-14 A and B, Early walking: wide stance, pronated feet, arms in high guard, “potbelly,” and lordotic back. Reach, Grasp, and Release Reaching patterns influence the ability of the hand to grasp objects. Reaching patterns depend on the position of the shoulder. Take a moment to try the following reaching pattern. Elevate your scapula and internally rotate your shoulder before reaching for the pencil on your desk. Do not compensate with forearm supination, but allow your forearm to move naturally into pronation. Although it is possible for you to obtain the pencil using this reaching pattern, it would be much easier to reach with the scapula depressed and the shoulder externally rotated. Reaching is an upper arm phenomenon. The position of the shoulder can dictate which side of the hand is visible. Prehension is the act of grasping. To prehend or grasp an object, one must reach for it. Development of reach, grasp, and release is presented in Table 4-5. Hand Regard The infant first recognizes the hands at 2 months of age, when they enter the field of vision (Figure 4-15). The asymmetric tonic neck reflex, triggered by head turning, allows the arm on the face side of the infant to extend and therefore is in a perfect place to be seen or regarded. Because of the predominance of physiologic flexor tone in the newborn, the hands are initially loosely fisted. The infant can visually regard other objects, especially if presented to the peripheral vision. 127 FIGURE 4-15 Hand regard aided by an asymmetric tonic neck reflex. Reflexive and Palmar Grasp The first type of grasp seen in the infant is reflexive, meaning it happens in response to a stimulus, in this case, touch. In a newborn, touch to the palm of the hand once it opens, especially on the ulnar side, produces a reflexive palmar grasp. Reflexive grasp is replaced by a voluntary palmar grasp by 6 months of age. The infant is no longer compelled by the touch of an object to grasp but may grasp voluntarily. Palmar grasp involves just the fingers coming into the palm of the hand; the thumb does not participate. Evolution of Voluntary Grasp Once grasp is voluntary at 6 months, a progressive change occurs in the form of the grasp. At 7 months, the thumb begins to adduct, and this allows for a radial-palmar grasp. The radial side of the hand is used along with the thumb to pick up small objects, such as 1-inch cubes. Radial palmar grasp is replaced by radial-digital grasp as the thumbs begin to oppose (Figures 4-16 and 4-17). Objects can then be grasped by the ends of the fingers, rather than having to be brought into the palm of the hand. The next two types of grasp involve the thumb and index finger only and are called pincer grasps. In the inferior pincer grasp, the thumb is on the lateral side of the index finger, as if you were to pinch someone (Figure 4-18). In the superior pincer grasp, the thumb and index finger are tip to tip, as in picking up a raisin or a piece of lint (Figure 4-19). An inferior pincer grasp is seen between 9 and 12 months of age, and a superior pincer grasp is evident by 1 year. Another type of grasp that may be seen in a 1-year-old infant is called a three-jaw chuck grasp (Figure 4-20). The wrist is extended, and the middle and index fingers and the thumb are used to grasp blocks and containers. FIGURE 4-16 Age 7 months: radial palmar grasp (thumb adduction begins); mouthing of objects. (From Cech D, 128 Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) FIGURE 4-17 Age 9 months: radial digital grasp (beginning opposition). (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) FIGURE 4-18 Age 9 to 12 months: inferior pincer grasp (isolated index pointing). (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) FIGURE 4-19 Age 1 year: superior pincer grasp (tip to tip). (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) 129 FIGURE 4-20 Age 1 year: three-jaw chuck grasp (wrist extended with ulnar deviation); maturing release. (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) Release As voluntary control of the wrist, finger, and thumb extensors develops, the infant is able to demonstrate the ability to release a grasped object (Duff, 2012). Transferring objects from hand to hand is possible at 5 to 6 months because one hand can be stabilized by the other. True voluntary release is seen around 7 to 9 months and is usually assisted by the infant’s being externally stabilized by another person’s hand or by the tray of a highchair. Mature control is exhibited by the infant’s release of an object into a container without any external support (12 months) or by putting a pellet into a bottle (15 months). Release continues to be refined and accuracy improved with ball throwing in childhood. 130 Typical motor development The important stages of motor development in the first year of life are those associated with even months 4, 6, 8, 10, and 12 (Table 4-6). Typical motor behavior of a 4-month-old infant is characterized by head control, support on arms and hands, and midline orientation. Symmetric extension and abduction of the limbs against gravity and the ability to extend the trunk against gravity characterize the 6-month-old infant. An infant 6 to 8 months old demonstrates controlled rotation around the long axis of the trunk that allows for segmental rolling, counterrotation of the trunk in crawling, and creeping. The 6-month-old may sit alone and play with an object. This milestone is being reached earlier than previously reported. Arm support may be needed until the child shows more dynamic control of the trunk and can make postural adjustments to lifting the limbs. A 10-month-old balances in standing, and a 12-month-old walks independently. Although the even months are important because they mark the attainment of these skills, the other months are crucial because they prepare the infant for the achievement of the control necessary to attain these milestones. Table 4-6 Important Stages of Development Age Stage 1 Internal body processes stabilize Basic biologic rhythms are established Spontaneous grasp and release are established 3-4months | Forearm support develops Head control is established Midline orientation is present 4-5months | Antigravity control of extensors and flexors begins Bottom lifting is present 6 months Strong extension-abduction of limbs is present Complete trunk extension is present Pivots on tummy Sits alone Spontaneous trunk rotation begins Trunk control develops along with sitting balance 8-10 months | Movement progression is seen in crawling, creeping, pulling to stand, and cruising 11-12 months} Independent ambulation occurs May move in and out of full squat 16-17 months} Carries or pulls an object while walking Walks sideways and backward 20-22 months} Easily squats and recovers toy 24 months Arm swing is present during ambulation Heel strike is present during ambulation Infant Birth to Three Months Newborns assume a flexed posture regardless of their position because physiologic flexor tone dominates at birth. Initially, the newborn is unable to lift the head from a prone position. The newborn’s legs are flexed under the pelvis and prevent contact of the pelvis with the supporting surface. If you put yourself into that position and try to lift your head, even as an adult, you will immediately recognize that the biomechanics of the situation are against you. With your hips in the air, your weight is shifted forward, thus making it more difficult to lift your head even though you have more muscular strength and control than a newborn. Although you are strong enough to overcome this mechanical disadvantage, the infant is not. The infant must wait for gravity to help lower the pelvis to the support surface and for the neck muscles to strengthen to be able to lift the head when in the prone position. The infant will be able to lift the head first unilaterally (Figure 4- 21), then bilaterally. 131 FIGURE 4-21 Unilateral head lifting in a newborn. (From Cech D, Martin S, editors: Functional movement development across the life span, ed 3, Philadelphia, 2012, WB Saunders.) Over the next several months, neck and spinal extension develop and allow the infant to lift the head to one side, to lift and turn the head, and then to lift and hold the head in the midline. As the pelvis lowers to the support surface, neck and trunk extensors become stronger. Extension proceeds from the neck down the back in a cephalocaudal direction, so the infant is able to raise the head up higher and higher in the prone position. By 3 months of age, the infant can lift the head to 45 degrees from the supporting surface. Spinal extension also allows the infant to bring the arms from under the body into a position to support herself on the forearms (Figure 4-22). This position also makes it easier to extend the trunk. Weight bearing through the arms and shoulders provides greater sensory awareness to those structures and allows the infant to view the hands while in a prone position. ~ FIGURE 4-22 Prone on elbows. When in the supine position, the infant exhibits random arm and leg movements. The limbs remain flexed, and they never extend completely. In supine, the head is kept to one side or the other because the neck muscles are not yet strong enough to maintain a midline position. If you wish to make eye contact, approach the infant from the side because asymmetry is present. An asymmetric tonic neck reflex may be seen when the baby turns the head to one side (Figure 4-23). The arm on the side to which the head is turned may extend and may allow the infant to see the hand while the other arm, closer to the skull, is flexed. This “fencing” position does not dominate the infant’s posture, but it may provide the beginning of the functional connection between the eyes and the hand that is necessary for visually guided reaching. Initially, the baby’s hands are normally fisted, but in the first month, they open. By 2 to 3 months, eyes and hands are sufficiently linked to allow for reaching, grasping, and shaking a rattle. As the eyes begin to track ever-widening distances, the infant will watch the hands explore the body. 132 FIGURE 4-23 Asymmetric tonic neck reflex in an infant. When an infant is pulled to sit from a supine position before the age of 4 months, the head lags behind the body. Postural control of the head has not been established. The baby lacks sufficient strength in the neck muscles to overcome the force of gravity. Primitive rolling may be seen as the infant turns the head strongly to one side. The body may rotate as a unit in the same direction as the head moves. The baby can turn to the side or may turn all the way over from supine to prone or from prone to supine (Figure 4-24). This turning as a unit is the result of a primitive neck righting reflex. A complete discussion of reflexes and reactions is presented following this section. In this stage of primitive rolling, separation of upper and lower trunk segments around the long axis of the body is missing. FIGURE 4-24 Primitive rolling without rotation. Four Months Four months is a critical time in motor development because posture and movement change from asymmetric to more symmetric. The infant is now able to lift the head in midline past 90 degrees in the prone position. When the infant is pulled to sit from a supine position, the head is in line with the body. Midline orientation of the head is present when the infant is at rest in the supine position (Figure 4-25). The infant is able to bring her hands together in the midline and to watch them. In fact, the first time the baby gets both hands to the midline and realizes that her hands, to this point only viewed wiggling in the periphery, are part of her body, a real “aha” occurs. Initially, this discovery may result in hours of midline hand play. The infant can now bring objects to the mouth 133 with both hands. Bimanual hand play is seen in all possible developmental positions. The hallmark motor behaviors of the 4-month-old infant are head control and midline orientation. “a FIGURE 4-25 Midline head position in supine. Head control in the 4-month-old infant is characterized by being able to lift the head past 90 degrees in the prone position, to keep the head in line with the body when the infant is pulled to sit (see Figure 4-9), to maintain the head in midline with the trunk when the infant is held upright in the vertical position and is tilted in any direction (Figure 4-26). Midline orientation refers to the infant’s ability to bring the limbs to the midline of the body, as well as to maintain a symmetric posture regardless of position. When held in supported sitting, the infant attempts to assist in trunk control. The positions in which the infant can independently move are still limited to supine and prone at this age. Lower extremity movements begin to produce pelvic movements. Pelvic mobility begins in the supine position when, from a hook-lying position, the infant produces anterior pelvic tilts by pushing on her legs and increasing hip extension, as in bridging (Bly, 1983). Active hip flexion in supine produces posterior tilting. Random pushing of the lower extremities against the support surface provides further practice of pelvic mobility that will be used later in development, especially in gait. 134 FIGURE 4-26 A and B, Head control while held upright in vertical and tilted. The head either remains in midline or tilts as a compensation. Five Months Even though head control as defined earlier is considered to be achieved by 4 months of age, lifting the head against gravity from a supine position (antigravity neck flexion) is not achieved until 5 months of age. Antigravity neck flexion may first be noted by the caregiver when putting the child down in the crib for a nap. The infant works to keep the head from falling backward as she is lowered toward the supporting surface. This is also the time when infants look as though they are trying to climb out of their car or infant seat by straining to bring the head forward. When the infant is pulled to sit from a supine position, the head now leads the movement with a chin tuck. The head is in front of the body. In fact, the infant often uses forward trunk flexion to reinforce neck flexion and to lift the legs to counterbalance the pulling force (Figure 4-27). Amz FIGURE 4-27 A, Use of trunk flexion to reinforce neck flexion as the head leads during a pull-to-sit maneuver. B, Use of leg elevation to counterbalance neck flexion during a pull-to-sit maneuver. From a froglike position, the infant is able to lift her bottom off the support surface and to bring her feet into her visual field. This “bottom lifting” allows her to play with her feet and even to put them into her mouth for sensory awareness (Figure 4-28). This play provides lengthening for the 135 hamstrings and prepares the baby for long sitting. The lower abdominals also have a chance to work while the trunk is supported. Reciprocal kicking is also seen at this time. FIGURE 4-28 Bottom lifting. As extension develops in the prone position, the infant may occasionally demonstrate a “swimming” posture (Figure 4-29). In this position, most of the weight is on the tummy, and the arms and legs are able to be stretched out and held up off the floor or mattress. This posture is a further manifestation of extensor control against gravity. The infant plays between this swimming posture and a prone on elbows or prone on extended arms posture (Figure 4-30). The infant makes subtle weight shifts while in the prone on elbows position and may attempt reaching. Movements at this stage show dissociation of head and limbs. FIGURE 4-29 “Swimming” posture, antigravity extension of the body. 136 FIGURE 4-30 Prone on extended arms. _ A 5-month-old infant cannot sit alone but may be supported at the low back. The typically developing infant can sit in the corner of a couch or on the floor if propped on extended arms. 5- month-old infants placed in sitting demonstrate directionally appropriate activation of postural muscles in response to movement of the support surface (Hadders-Algra et al., 1996). Six Months A 6-month-old infant becomes mobile in the prone position by pivoting in a circle (Figure 4-31). The infant is also able to shift weight onto one extended arm and to reach forward with the other hand to grasp an object. The reaching movement is counterbalanced by a lateral weight shift of the trunk that produces lateral head and trunk bending away from the side of the weight shift (Figure 4-32). This lateral bending in response to a weight shift is called a righting reaction. Righting reactions of the head and trunk are more thoroughly discussed in the next section. Maximum extension of the head and trunk is possible in the prone position along with extension and abduction of the limbs away from the body. This extended posture is called the Landau reflex and represents total body righting against gravity. It is mature when the infant can demonstrate hip extension when held away from the support surface, supported only under the tummy. The infant appears to be flying (Figure 4-33). This final stage in the development of extension can occur only if the hips are relatively adducted. Too much hip abduction puts the gluteus maximus at a biomechanical disadvantage and makes it more difficult to execute hip extension. Excessive abduction is often seen in children with low muscle tone and increased range of motion, such as in Down syndrome. These children have difficulty performing antigravity hip extension. 137 FIGURE 4-31 Pivoting in prone. FIGURE 4-32 Lateral righting reaction. 138 « FIGURE 4-33 A, Eliciting a Landau reflex. B, Spontaneous Landau reflex. Segmental rolling is now present and becomes the preferred mobility pattern when rolling, first from prone to supine, which is less challenging, and then from supine to prone. Antigravity flexion control is needed to roll from supine to prone. The movement usually begins with flexion of some body part, depending on the infant and the circumstances. Regardless of the body part used, segmental rotation is essential for developing transitional control (Figure 4-34). Transitional movements are those that allow change of position, such as moving from prone to sitting, from the four-point position to kneeling, and from sitting to standing. Only a few movement transitions take place without segmental trunk rotation, such as moving from the four-point position to kneeling and from sitting to standing. Individuals with movement dysfunction often have problems making the transition smoothly and efficiently from one position to another. The quality of movement affects the individual's ability to perform transitional movements. FIGURE 4-34 Ato C, Segmental rolling from supine to prone. The 6-month-old infant can sit up if placed in sitting. The typically developing infant can sit in the corner of a couch or on the floor if propped on extended arms. A 6-month-old cannot purposefully move into sitting from a prone position but may incidentally push herself backward along the floor. Coincidentally, while pushing, her abdomen may be lifted off the support surface, allowing the pelvis to move over the hips, with the end result of sitting between the feet. Sitting between the feet is called W sitting and should be avoided in infants with developmental movement problems, because it can make it difficult to learn to use trunk muscles for balance. The posture provides positional stability, but it does not require active use of the trunk muscles. Concern also exists about the abnormal stress this position places on growing joints. In typically developing children, there is less concern because these children move in and out of the position more easily, rather than remaining in it for long periods of time. Having developed trunk extension in the prone position, the infant can sit with a relatively straight back with the exception of the lumbar spine (Figure 4-35). The upper and middle parts of the trunk are not rounded as in previous months, but the lumbar area may still demonstrate forward flexion. Although the infant’s arms are initially needed for support, with improving trunk control, first one hand and then both hands will be freed from providing postural support to explore objects and to engage in more sophisticated play. When balance is lost during sitting, the 139 infant extends the arms for protection while falling forward. In successive months, this same upper extremity protective response will be seen in additional directions, such as laterally and backward. FIGURE 4-35 Early sitting with a relatively straight back except for forward flexion in the lumbar spine. The pull-to-sit maneuver with a 6-month-old often causes the infant to pull all the way up to standing (Figure 4-36). The infant will most likely reach forward for the caregiver’s hands as part of the task. A 6-month-old likes to bear weight on the feet and will bounce in this position if she is held. Back-and-forth rocking and bouncing in a position seem to be prerequisites for achieving postural control in a new posture (Thelen, 1979). Repetition of rhythmic upper extremity activities is also seen in the banging and shaking of objects during this period. Reaching becomes less dependent on visual cues as the infant uses other senses to become more aware of body relationships. The infant may hear a noise and may reach unilaterally toward the toy that made the sound (Duff, 2012). 140 FIGURE 4-36 A and B, Pull-to-sit maneuver becomes pull-to-stand. Although complete elbow extension is lacking, the 6-month-old’s arm movements are maturing such that a mid—pronation-supination reaching pattern is seen. A position halfway between supination and pronation is considered neutral. Pronated reaching is the least mature reaching pattern and is seen early in development. Supinated reaching is the most mature pattern because it allows the hand to be visually oriented toward the thumb side, thereby increasing grasp precision (Figure 4-37). Reaching patterns originate from the shoulder because early in upper extremity development, the arm functions as a whole unit. Reaching patterns are different from grasping patterns, which involve movements of the fingers. FIGURE 4-37 Supinated reaching. Seven Months Trunk control improves in sitting and allows the infant to free one or both hands for playing with objects. The infant can narrow her base of support in sitting by adducting the lower extremities as the trunk begins to be able to compensate for small losses of balance. Dynamic stability develops 141 from muscular work of the trunk. An active trunk supports dynamic balance and complements the positional stability derived from the configuration of the base of support. The different types of sitting postures, such as ring sitting, wide abducted sitting, and long sitting, provide the infant with different amounts of support. Figure 4-38 shows examples of sitting postures in typically developing infants with and without hand support. Lateral protective reactions begin to emerge in sitting at this time (Figure 4-39). Unilateral reach is displayed by the 7-month-old infant (Figure 4- 40), as is an ability to transfer objects from hand to hand. 142 C FIGURE 4-38 Sitting postures. A, Ring sitting propped forward on hands. B, Half-long sitting. C, Long sitting. FIGURE 4-39 Lateral upper extremity protective reaction in response to loss of sitting balance. 143 FIGURE 4-40 Unilateral reach. Sitting is a functional and favorite position of the infant. Because the infant’s back is straight, the hands are free to play with objects or extend and abduct to catch the infant if a loss of balance occurs, as happens less frequently at this age. Upper trunk rotation is demonstrated during play in sitting as the child reaches in all directions for toys (see Figure 4-38, C). If a toy is out of reach, the infant can prop on one arm and reach across the body to extend the reach using trunk rotation and reverse the rotation to return to upright sitting. With increased control of trunk rotation, the body moves more segmentally and less as a whole. This trend of dissociating upper trunk rotation from lower trunk movement began at 6 months with the beginning of segmental rotation. Dissociation of the arms from the trunk is seen as the arms move across the midline of the body. More external rotation is evident at the shoulder (turning the entire arm from palm down, to neutral, to palm up) and allows supinated reaching to be achieved. By 8 to 10 months, the infant’s two hands are able to perform different functions such as holding a bottle in one hand while reaching for a toy with the other (Duff, 2002). Eight Months Now the infant can move into and out of sitting by deliberately pushing up from sidelying position. The child may bear weight on her hands and feet and may attempt to “walk” in this position (bear walking) after pushing herself backward while belly crawling. Some type of prewalking progression, such as belly crawling (Figure 4-41), creeping on hands and knees (see Figure 4-13), or sitting and hitching, is usually present by 8 months. Hitching in a sitting position is an alternative way for some children to move across the floor. The infant scoots on her bottom with or without hand support. We have already noted how pushing up on extended arms can be continued into pushing into sitting. Pushing can also be used for locomotion. Because pushing is easier than pulling, the first type of straight plane locomotion achieved by the infant in a prone position may be backward propulsion. Pulling is seen as strength increases in the upper back and shoulders. All this upper extremity work in a prone position is accompanied by random leg movements. These random leg movements may accidentally cause the legs to be pushed into extension with the toes flexed and may thus provide an extra boost forward. In trying to reproduce the accident, the infant begins to learn to belly crawl or creep forward. 144 FIGURE 4-41 Belly crawling. Nine Months A 9-month-old is constantly changing positions, moving in and out of sitting (including side sitting) (Figure 4-42) and into the four-point position. As the infant experiments more and more with the four-point position, she rhythmically rocks back and forth and alternately puts her weight on her arms and legs. In this endeavor, the infant is aided by a new capacity for hip extension and flexion, other examples of the ability to dissociate movements of the pelvis from movements of the trunk. The hands-and-knees position, or quadruped position, is a less supported position requiring greater balance and trunk control. As trunk stability increases, simultaneous movement of an opposite arm and leg is possible while the infant maintains weight on the remaining two extremities. This form of reciprocal locomotion is called creeping. Creeping is often the primary means of locomotion for several months, even after the infant starts pulling to stand and cruising around furniture. Creeping provides fast and stable travel for the infant and allows for exploration of the environment. A small percentage (4.3%) of infants never creep on hands and knees according to the World Health Organization (2006). 145 OG FIGURE 4-42 Side sitting. Reciprocal movements used in creeping require counterrotation of trunk segments; the shoulders rotate in one direction while the pelvis rotates in the opposite direction. Counterrotation is an important element of erect forward progression (walking), which comes later. Other major components needed for successful creeping are extension of the head, neck, back, and arms, and dissociation of arm and leg movements from the trunk. Extremity dissociation depends on the stability of the shoulder and pelvic girdles, respectively, and on their ability to control rotation in opposite directions. Children practice creeping about 5 hours a day and can cover the distance of two football fields (Adolph, 2003). When playing in the quadruped position, the infant may reach out to the crib rail or furniture and may pull up to a kneeling position. Balance is maintained by holding on with the arms rather than by fully bearing the weight through the hips. The infant at this age does not have the control necessary to balance in a kneeling or half-kneeling (one foot forward) position. Even though kneeling and half-kneeling are used as transitions to pull to stand, only after learning to walk is such control possible for the toddler. Pulling to stand is a rapid movement transition with little time spent in either true knee standing or half-kneeling. Early standing consists of leaning against a support surface, such as the coffee table or couch, so the hands can be free to play. Legs tend to be abducted for a wider base of support, much like the struts of a tower. Knee position may vary between flexion and extension, and toes alternately claw the floor and flare upward in an attempt to assist balance. These foot responses are considered equilibrium reactions of the feet (Figure 4-43). 146 FIGURE 4-43 Equilibrium reactions of the feet. Baby learns balance in standing by delicate movements of the feet: “fanning” and “clawing.” (Redrawn by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities. New York, © 1978 Teachers College, Columbia University, p. 117. All rights reserved.) Once the infant has achieved an upright posture at furniture, she practices weight shifting by moving from side to side. While in upright standing and before cruising begins in earnest, the infant practices dissociating arm and leg movements from the trunk by reaching out or backward with an arm while the leg is swung in the opposite direction. When side-to-side weight shift progresses to actual movement sideways, the baby is cruising. Cruising is done around furniture and between close pieces of furniture. This sideways “walking” is done with arm support and may be a means of working the hip abductors to ensure a level pelvis when forward ambulation is attempted. These maneuvers always make us think of a ballet dancer warming up at the barre before dancing. In this case, the infant is warming up, practicing counterrotation in a newly acquired posture, upright, before attempting to walk (Figure 4-44). Over the next several months, the infant will develop better pelvic-and-hip control to perfect upright standing before attempting independent ambulation. 147 FIGURE 4-44 Cruising maneuvers. A, Cruising sideways, reaching out. B, Standing, rotating upper trunk backward. C, Standing, reaching out backward, elaborating with swinging movements of the same-side leg, thus producing counterrotation. (Redrawn by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities. New York, © 1978 Teachers College, Columbia University, p. 121. All rights reserved.) Toddler Twelve Months The infant becomes a toddler at 1 year. Most infants attempt forward locomotion by this age. The caregiver has probably already been holding the infant’s hands and encouraging walking, if not placing the infant in a walker. Use of walkers continues to raise safety issues from pediatricians. The American Academy of Pediatrics (AAP) recently reaffirmed their policy statement on injuries associated with walker use (AAP, 2012). Also, too early use of walkers does not allow the infant to sufficiently develop upper body and trunk strength needed for the progression of skills seen in the prone position. Typical first attempts at walking are lateral weight shifts from one widely abducted leg to the other (Figure 4-45). Arms are held in high guard (arms held high with the scapula adducted, shoulders in external rotation and abducted, elbows flexed, and wrist and fingers extended). This position results in strong extension of the upper back that makes up for the lack of hip extension. As an upright trunk is more easily maintained against gravity, the arms are lowered to midguard (hands at waist level, shoulders still externally rotated), to low guard (shoulders more neutral, elbows extended), and finally to no guard. = == A — 8 FIGURE 4-45 A and B, Independent walking. The beginning walker keeps her hips and knees slightly flexed to bring the center of mass closer to the ground. Weight shifts are from side to side as the toddler moves forward by total lower 148 extremity flexion, with the hip joints remaining externally rotated during the gait cycle. Ankle movements are minimal, with the foot pronated as the whole foot contacts the ground. Toddlers take many small steps and walk slowly. The instability of their gait is seen in the short amount of time they spend in single-limb stance (Martin, 1989). As trunk stability improves, the legs come farther under the pelvis. As the hips and knees become more extended, the feet develop the plantar flexion needed for the push-off phase of the gait cycle. Sixteen to Eighteen Months By 16 to 17 months, the toddler is so much at ease with walking that a toy can be carried or pulled at the same time. With help, the toddler goes up and down the stairs, one step at a time. Without help, the toddler creeps up the stairs and may creep or scoot down on her buttocks. Most children will be able to walk sideways and backward at this age if they started walking at 12 months or earlier. The typically developing toddler comes to stand from a supine position by rolling to prone, pushing up on hands and knees or hands and feet, assuming a squat, and rising to standing (Figure 4-46). FIGURE 4-46 Progression of rising to standing from supine. A, Supine. B, Rolling. C, Four-point position. D, Plantigrade. E, Squat. F, Semi-squat. G, Standing. Most toddlers exhibit a reciprocal arm swing and heel strike by 18 months of age, with other adult gait characteristics manifested later. They walk well and demonstrate a “running-like” walk. Although the toddler may still occasionally fall or trip over objects in her path because eye-foot coordination is not completely developed, the decline in falls appears to be the result of improved balance reactions in standing and the ability to monitor trunk and lower extremity movements kinesthetically and visually. The first signs of jumping appear as a stepping off “jump” from a low object, such as the bottom step of a set of stairs. Children are ready for this first step-down jump after being able to walk down a step while they hold the hand of an adult (Wickstrom, 1983). Momentary balance on one foot is also possible. 149 Two Years The 2-year-old’s gait becomes faster, arms swing reciprocally, steps are bigger, and time spent in single-limb stance increases. Many additional motor skills emerge during this year. A 2-year-old can go up and down stairs one step at a time, jump off a step with a two-foot take-off, kick a large ball, and throw a small one. Stair climbing and kicking indicate improved stability during shifting of body weight from one leg to the other. Stepping over low objects is also part of the child’s movement capabilities within the environment. True running, characterized by a “flight” phase when both feet are off the ground, emerges at the same time. Quickly starting to run and stopping from a run are still difficult, and directional changes by making a turn require a large area. As the child first attempts to jump off the ground, one foot leaves the ground, followed by the other foot, as if the child were stepping in air. Fundamental Movement Patterns (Three to Six Years) Three Years Fundamental motor patterns such as hopping, galloping, and skipping develop from 3 to 6 years of age. Wickstrom (1983) also includes running, jumping, throwing, catching, and striking in this category. Other reciprocal actions mastered by age 3 are pedaling a tricycle and climbing a jungle gym or ladder. Locomotion can be started and stopped based on the demands from the environment or from a task such as playing dodge ball on a crowded playground. A 3-year-old child can make sharp turns while running and can balance on toes and heels in standing. Standing with one foot in front of the other, known as tandem standing, is possible, as is standing on one foot for at least 3 seconds. A reciprocal gait is now used to ascend stairs with the child placing one foot on each step in alternating fashion but marking time (one step at a time) when descending. Jumping begins with a step-down jump at 18 months and progresses to jumping up off the floor with two feet at the same time at age 2. Jumps can start with a one-foot or two-foot take-off. The two-foot take-off and land is more mature. Jumps can involve running then jumping as in a running broad jump or jumping from standing still, as in a standing broad jump. Jumping has many forms and is part of play or game activities. Jumping ability increases with age. Hopping on one foot is a special type of jump requiring balance on one foot and the ability to push off the loaded foot. It does not require a maximum effort. “Repeated vertical jumps from 2 feet can be done before true hopping can occur” (Wickstrom, 1983) (see Figure 4-47). Neither type of jump is seen at an early age. Hopping one or two times on the preferred foot may also be accomplished by 3% years when there is the ability to stand on one foot and balance long enough to push off on the loaded foot. A 4-year-old child should be able to hop on one foot four to six times. Improved hopping ability is seen when the child learns to use the nonstance leg to help propel the body forward. Before that time, all the work is done by pushing off with the support foot. A similar pattern is seen in arm use; at first, the arms are inactive; later, they are used opposite the action of the moving leg. Gender differences for hopping are documented in the literature, with girls performing better than boys (Wickstrom, 1983). This may be related to the fact that girls appear to have better balance than boys in childhood. 150 FIGURE 4-47 Vertical jump. Immature form in the vertical jump showing “winging” arm action, incomplete extension, quick flexion of the legs, and slight forward jump. (From Wickstrom RL: Fundamental motor patterns, ed 3, Philadelphia, 1983, Lea & Febiger.) Four Years Rhythmic relaxed galloping is possible for a 4-year-old child. Galloping consists of a walk on the lead leg followed by a running step on the rear leg. Galloping is an asymmetrical gait. A good way to visualize galloping is to think of a child riding a stick horse. Toddlers have been documented to gallop as early as 20 months after learning to walk (Whitall, 1989), but the movement is stiff with arms held in high guard as in beginning walking. A 4-year-old has better static and dynamic balance as evidenced by the ability to stand on either foot for a longer period of time (4 to 6 seconds) than a 3-year-old. Now she can descend stairs with alternating feet. Four-year-olds can catch a small ball with outstretched arms if it is thrown to them, and they can throw a ball overhand from some distance. Throwing begins with an accidental letting go of an object at about 18 months of age. From 2 to 4 years of age, throwing is extremely variable, with underhand and overhand throwing observed. Gender differences are seen. A child of 2% years can throw a large or small ball 5 feet (Figure 4-48 and Table 4-7) (Wellman, 1967). The ball is not thrown more than 10 feet until the child is more than 4 years of age. The distance a child is able to propel an object has been related to a child’s height, as seen in Figure 4-49 (Cratty, 1979). Development of more mature throwing is related to using the force of the body and combination of leg and shoulder movements to improve performance. BALL-CATCHING ACHIEVEMENTS OF PRESCHOOL CHILDREN 70 oO Large ball (16.25 inches) 60 > Small ball (9.5 inches) Age (months) y o 40 Method 1: Arms held straight in front of body Method 2: Elbows positioned in front of body Method 3: Elbows positioned at side of body 0 1 2 3 Method BALL-THROWING ACHIEVEMENTS OF PRESCHOOL CHILDREN 80 70 _ 60 e = S e 50 2 oa = 40 O-— Small ball (9.5 inches) 30 (©) Large ball (16.25 inches) 0 5 10 15 20 Distance (feet) FIGURE 4-48 Wellman graphs. A, Ball-catching skill is attained at a certain level of performance with the large ball before the same level of skill is achieved with the small ball. B, At 30 months, a small or large ball can be thrown 5 feet. It will take 10 more months for the child to be able to throw the large ball the same distance as the small ball. (Redrawn from Espanschade AS, Eckert HM: Motor development, Columbus, OH, 1967, Charles E. Merrill.) 151 Table 4-7 Ball-Throwing Achievements of Preschool Children Motor Age in Months Distance of Throw (feet) Small Ball (912 inch) Large Ball (16% inch) CC ea nei From Wellman BL: Motor achievements of preschool children. Child Educ 13:311—316, 1937. Reprinted by permission of the Association for Childhood Education International, 3615 Wisconsin Avenue, NW, Washington, DC. 4 1/2 yr. old 31/2 yr.old 21/2 yr. old 4 ft. 6 ft FIGURE 4-49 Throwing distances increase with increasing age. (From Cratty BJ: Perceptual and Motor Development in Infants and Children, ed 2. © 1979 Prentice Hall. Reprinted by permission of Pearson Education, Inc., Upper Saddle River, New Jersey.) “Although throwing and catching have a close functional relationship, throwing is learned a lot more quickly than catching” (Wickstrom, 1977). Catching ability depends on many variables, the 152 least of which is ball size, speed, arm position of the catcher, skill of the thrower, and age-related sensory and perceptual factors. Some of these perceptual factors involve the use of visual cues, depth perception, eye-hand coordination, and the amount of experience the catcher has had with playing with balls. Closing the eyes when an object is thrown toward one is a fear response common in children (Wickstrom, 1977) and has to be overcome to learn to catch or strike an object. Precatching requires the child to interact with a rolling ball. Such interaction typically occurs while the child sits with legs outstretched and tries to trap the ball with legs or hands. Children learn about time and spatial relationships of moving objects first from a seated position and later in standing when chasing after a rolling or bouncing ball. The child tries to stop, intercept, and otherwise control her movements and to anticipate the movement of the object in space. Next, the child attempts to “catch” an object moving through the air. Before reaching age 3, most children must have their arms prepositioned to have any chance of catching a ball thrown to them. Most of the time, the thrower, who is an adult, bounces the ball to the child, so the burden is on the thrower to calculate where the ball must bounce to land in the child’s outstretched arms. Figures 4-50 and 4- 51 show two immature catchers, one 33 months old and the other 48 months old. As catching matures, the hands are used more, with less dependence on the arms and body. The 4-year-old still has maturing to do in perfecting the skill of catching. aia FIGURE 4-50 Immature catching. A 33-month-old boy extends his arms before the ball is tossed. He waits for the ball without moving, responds after the ball has touched his hands, and then gently traps the ball against his chest. It is essentially a robot-like performance. (From Wickstrom RL: Fundamental motor patterns, ed 3, Philadelphia, 1983, Lea & Febiger.) FIGURE 4-51 A 4-year-old girl waits for the ball with arms straight and hands spread. Her initial response to the ball is a clapping motion. When one hands contacts the ball, she grasps at it and gains control by clutching it against her chest. (From Wickstrom RL: Fundamental motor patterns, ed 3, Philadelphia, 1983, Lea & Febiger.) Striking is the act of swinging and hitting an object. Developmentally, the earliest form of striking is for the child to use arm extension to hit something with her hand. When a child holds an 153 implement, such as a stick or a bat, she continues to use this form of movement, which results in striking down the object. 2- to 4-year-olds demonstrate this immature striking behavior. Common patterns of striking are overhand, sidearm, and underhand. Without any special help, the child will progress slowly to striking more horizontally. Mature form of striking is usually not demonstrated until at least 6 years of age (Malina et al., 2004). As the child progresses from striking down to a more horizontal striking (sidearm), more and more trunk rotation is seen as the child’s swing matures (Roberton and Halverson, 1977). A mature pattern of striking consists of taking a step, turning away, and then swinging (step-turn-swing) (Wickstrom, 1983). Kicking is a special type of striking and one in which the arms play no direct role. Children most frequently kick a ball in spontaneous play and in organized games. A 2-year-old is able to kick a ball on the ground. A child of 5 years is expected to kick a ball rolled toward her 12 feet in the air, and a child of 6 years is expected to run and kick a rolling ball up to 4 feet (Folio and Fewell, 2000). Gesell (1940) expected a 5-year-old to kick a soccer ball up to 8 to 11% feet and a 6-year-old to be able to kick a ball up to 10 to 18 feet. Measuring performance in kicking is difficult before the age of 4 years. Annual improvements begin to be seen at the age of 5 years (Gesell, 1940). Kicking requires good static balance on the stance foot and counterbalancing the force of the kick with arm positioning. Five Years At 5 years of age, a child can stand on either foot for 8 to 10 seconds, walk forward on a balance beam, hop 8 to 10 times on one foot, make a 2- to 3-foot standing broad jump, and skip on alternating feet. Skipping requires bilateral coordination. At this age, the child can change directions and stop quickly while running. She can ride a bike, roller-skate, and hit a target with a ball from 5 feet away. Six Years A 6-year-old child is well-coordinated and can stand on one foot for more than 10 seconds, with eyes open or eyes closed. This ability is important to note because it indicates that vision can be ignored and balance can be maintained. A 6-year-old can throw and catch a small ball from 10 feet away. A first grader can walk on a balance beam on the floor, forward, backwards, and sideways without stepping off. She continues to enjoy and use alternate forms of locomotion, such as riding a bicycle or roller-skating. Patterns of movement learned in game-playing form the basis for later sports skills. Throughout the process of changing motor activities and skills, the nervous, muscular, and skeletal systems are maturing, and the body is growing in height and weight. Power develops slowly in children because strength and speed within a specific movement pattern are required (Bernhardt-Bainbridge, 2006). Fundamental motor skills demonstrate changes in form over time. Between 6 and 10 years of age, a child masters the adult forms of running, throwing, and catching. Figure 4-52 depicts when 60% of children were able to demonstrate a certain developmental level for the listed fundamental motor skills. Stage 1 is an immature form of the movement, and stage 4 or 5 represents the mature form of the same movement. A marked gender difference is apparent in overhand throwing. It is not uncommon to see young children demonstrate a mature pattern of movement at one age and a less mature pattern at a later age. Regression of patterns is possible when the child is attempting to combine skills. For example, a child who can throw overhand while standing may revert to underhand throwing when running. Alterations between mature and immature movement is in line with Gesell’s concept of reciprocal interweaving. Individual variation in motor development is considerable during childhood. Even though 60% of children have achieved the fundamental motor skills as listed in Figure 4-52, 40% of the children have not achieved them by the ages given. 154 Stages of Fundamental Motor Skills { 234 5 === Girls T 2 3 a Kicking —— ee ee ee ee ee ee ee 12 3 4 Running fetasesae 1 2 3 4 sick 1 2 3 4 1 2 3s 4 5 Catching —— oe ee oe oe oe t2 3 4 1 2 3 4 pping F Nabe j ee: Skipping ans 24 36 48 60 72 84 96 108 120 Age, months FIGURE 4-52 Ages at which 60% of boys and girls were able to perform at specific developmental levels for several fundamental motor skills. Stage 1 is immature; stage 4 or 5 is mature. (Reprinted by permission from Seefledt V, Haubenstricker J: Patterns, phases, or stages: An analytical model for the study of developmental movement. In Kelso JAS, Clark JE, editors: The development of movement control and coordination, 1982, p. 314.) Gait The majority of children begin walking at the end of the first year of life but it takes years for the child to exhibit mature gait characteristics. Factors associated with the achievement of upright gait are sufficient extensor muscle strength, dynamic balance, and postural control of the head within the limits of stability of the base of support. A new walker’s movement is judged by how long she has been walking, not by the age at the onset of the skill. After about 5 months of walking practice, the infant is able to exhibit an inverted pendulum mechanism that makes walking more efficient (Ivanenko et al., 2007). With practice, the duration of single limb support increases and the period of double limb support declines. Arm swing and heel strike are present by 2 years of age (Sutherland et al., 1988). Out-toeing has been reduced and pelvic rotation and a double knee-lock pattern are present. This pattern refers to the two periods of knee extension in gait, one just before heel strike and another as the body moves over the foot during stance phase. In between, at the moment of heel strike, the knee is flexed to help absorb the impact of the body’s weight. Cadence decreases as stride length increases. Gait velocity almost doubles between 1 and 7 years, and the pelvic span to ankle spread span ratio increases. The latter gait lab measurement indicates that the base of support narrows over time. Rapid changes in temporal and spatial gait parameters occur during the first 4 years of life with slower changes continuing until 7 years when gait is considered mature by motion standards (Stout, 2001). Experience and practice play a significant role in gait development. Age-Related Differences in Movement Patterns beyond Childhood 155 Many developmentalists have chosen to look only at the earliest ages of life when motor abilities and skills are being acquired. The belief that mature motor behavior is achieved by childhood led researchers to overlook the possibility that movement could change as a result of factors other than nervous system maturation. Although the nervous system is generally thought to be mature by the age of 10 years, changes in movement patterns do occur in adolescence and adulthood. Research shows a developmental order of movement patterns across childhood and adolescence with trends toward increasing symmetry with increasing age (Sabourin, 1989; VanSant, 1988a). VanSant (1988b) identified three common ways in which adults came to stand. These are shown in Figure 4-53. The most common pattern was to use upper extremity reach, symmetrical push, forward head, neck and trunk flexion, and a symmetrical squat (see Figure 4-53, A). The second most common way was identical to the first pattern up to an asymmetrical squat (see Figure 4-53, B). The next most common way involved an asymmetrical push and reach, followed by a half-kneel (see Figure 4-53, C). In a separate study of adults in their 20s through 40s, there was a trend toward increasing asymmetry with age (Ford-Smith and VanSant, 1993). Adults in their 40s were more likely to demonstrate the asymmetric patterns of movement seen in young children (VanSant, 1991). The asymmetry of movement in the older adult may reflect less trunk rotation resulting from stiffening of joints or lessening of muscle strength, factors that make it more difficult to come straight forward to sitting from a supine position. A. Most common R fF) x . fas + (a a os eo aN Hey 7} —- 4 ¥ care Pad . All Urias Pressure Splints wS JSeature a zipper closure % SN for easy donning. A FIGURE 5-6 A and B, Use of pediatric air splints for knee control in standing and elbow control in prone reaching. (Courtesy Arden Medical, Ltd.) Handling Tips The following should be considered when you physically handle a child with neurologic deficit. 183 1. Allow the child to do as much of the movement as possible. You will need to pace yourself and will probably have to go more slowly than you may think. For example, when bringing a child into a sitting position from supine, roll the child slowly to one side and give the child time to push up onto her hand, even if she can only do this part of the way, such as up to an elbow. In addition, try to entice the child to roll to the side before attempting to have her come to sit. Using a toy to encourage reaching to roll can also be used. The effects of gravity can be reduced by using an elevated surface, such as a wedge, under the head and upper trunk to make it easier to move into side-lying before coming to sit. 2. When carrying a child, encourage as much head and trunk control as the child can demonstrate. Carry the child in such a way that head and trunk muscles are used to maintain the head and trunk upright against gravity while you are moving. This allows the child to look around and see where you are going. 3. When trying to move the limbs of a child with spasticity, do not pull against the tightness. Do move slowly and rhythmically, starting proximally at the child’s shoulders and pelvis. The position of the proximal joints can influence the position of the entire extremity. Changing the position of the proximal joint may also reduce spasticity throughout the extremity. 4. Many children with severe involvement and those with athetosis show an increased sensitivity to touch, sound, and light. These children startle easily and may withdraw from contact to their hands, feet, and mouth. Encourage the child to keep her head in the midline of the body and the hands in sight. Weight bearing on hands and feet is an important activity for these children. 5. Children with low postural tone should be handled more vigorously, but they tire more easily and require more frequent rest periods. Avoid placing children in a supine position to play because they need to work against gravity in the prone position to develop their extensor muscles. Their extensors are so weak that the extremities assume a “frog” position of abduction when these children are supine. Strengthening of abdominal muscles can be done with the child in a semireclined supine position. Encourage arm use and visual learning. By engaging visual tracking, the child may learn to use the eyes to encourage head and trunk movement. Infant seats are appropriate for the young child with low tone who needs head support, but an adapted corner chair is better for the older child. 6. When encouraging movements from proximal joints, remember that wherever your hands are, the child will not be in control. If you control the shoulders, the child has to control the head and trunk, that is, above and below where you are handling. Keep this in mind anytime you are guiding movement. If you want the child to control a body part or joint, you should not be holding on to that area. 7. Ultimately, the goal is for the child to initiate and guide her own movements. Handling should be decreased as the child gains more control. If the child exhibits movement of satisfactory quality only while you are guiding the movement but is not able to assist in making the same movements on her own, you must question whether motor learning is actually taking place. The child must actively participate in movement to learn to move. For movement to have meaning, it must have a goal such as object exploration or locomotion. Use of Sensory Input to Promote Positioning and Handling Touch An infant begins to define the edges of her own body by touch. Touch is also the first way in which an infant finds food and experiences self-calming when upset. Infant massage is a way to help parents feel comfortable about touching their infant. The infant can be guided to touch the body as a prelude to self-calming (Intervention 5-5). Positioning the infant in side-lying often makes it easier for her to touch her body and to see her hands and feet (an important factor). Awareness of the body’s midline is an essential perceptual ability. If asymmetry in movement or sensation exists, then every effort must be made to equalize the child’s awareness of both sides of the body when the child is being moved or positioned. Additional tactile input can be given to that side of the body in the form of touch or weight bearing. The presence of asymmetry in sensation and movement can contribute to arm and leg length differences. Shortening of trunk muscles can occur because of lack of equal weight bearing through the pelvis in sitting or as compensation for unilateral muscular paralysis. Trunk muscle imbalance can also lead to scoliosis. 184 Intervention 5-5 Teaching Self-Calming Using touch to self-calm in supported supine and side-lying positions. A. The infant can be guided to touch the body as a prelude to self-calming. B. Positioning the child in side-lying often makes it easier for him to touch his body and to see her hands and feet—important points of reference. Touch and movement play important roles in developing body and movement awareness and balance. Children with hypersensitivity to touch may need to be desensitized. Usually, gentle but firm pressure is better tolerated than light touch when a child is overly sensitive. Light touch produces withdrawal of an extremity or turning away of the face in children who exhibit tactile defensiveness (Lane, 2002). Most typically developing children like soft textures before rough ones, but children who appear to misperceive tactile input may actually tolerate coarse textures, such as terry cloth, better than soft textures. General guidelines for use of tactile stimulation with children with tactile defensiveness have been outlined by Koomar and Bundy (2002). These include the following: (1) having the child administer the stimulation; (2) using firm pressure but realizing that light touch can be used if the child is indeed perceiving light touch as deep pressure; (3) applying touch to the arms and legs before the face; (4) applying the stimulation in the direction of hair growth; (5) providing a quiet, enclosed area for the stimulation to take place; (6) substituting proprioception for tactile stimulation or combining deep pressure with proprioception. Textured mitts, paintbrushes, sponges, and vibrators provide different types of tactile stimulation. Theoretically, deep touch or pressure to the extremities has a central inhibitory effect that is more general, even though this touch is applied to a specific body part (Ayres, 1972). The expected outcome is that the child will have an increased tolerance to touch, be able to concentrate better, and exhibit better organized behavior. If handling the child is to be an effective part of intervention, the infant or child must be able to tolerate touch. A child who is defensive about touch to the face usually also has increased sensitivity to touch inside the mouth. Such children may have difficulty in eating textured foods. Oral motor therapy is a specialized area of practice that requires additional education. A physical, occupational, or speech therapist may be trained to provide this type of care. The physical therapist assistant may be taught specific interventions by the therapist, which are applicable to a particular child in a specific setting. However, these interventions are beyond the scope of this book and are only referred to in general terms. Vestibular System The three semicircular canals of the vestibular system are fluid-filled. Each set of canals responds to movement in different planes. Cartwheels, somersaults, and spinning produce movement in different canals. Linear movement (movement in line with the body orientation) can improve head lifting when the child is in prone or supine position. Swinging a child in a hammock in a prone or supine position produces such linear movement and encourages head lifting (Figure 5-7). Movement stimulation often works to alert a child affected by lethargy or one with low muscle tone because the vestibular system has a strong influence on postural tone and balance. The vestibular 185 system causes a response when the flow of fluid in the semicircular canals changes direction. However, constant movement results in the child’s habituation or becoming used to the movement and does not produce a response. Rapid, quick movement, as in sitting on a movable surface, can alert the child. Fast, jerky movement facilitates an increase in tone if the child’s resting tone is low. Slow, rhythmic movement decreases high tone. FIGURE 5-7 Child in a hammock. Approximation Application of compression through joints in weight bearing is approximation. Rocking on hands and knees and bouncing on a ball in sitting are examples of activities that provide approximation. Additional compression can be given manually through the body parts into the weight-bearing surface. Joints may also be approximated by manually applying constant pressure through the long axis of aligned body parts. Intermittent compression can also be used. Both constant pressure and intermittent pressure provide proprioceptive cues to alert postural muscles to support the body, as in sitting and bouncing on a trampoline. The speed of the compressive force and the give of the support surface provide differing amounts of joint approximation. The direction of movement can be varied while the child is rocking on hands and knees. Compression through the length of the spine is achieved from just sitting, as a result of gravity, but this compression can be increased by bouncing. Axial compression or pressure through the head and neck must be used cautiously in children with Down syndrome because of the 15% incidence of atlantoaxial instability in this population (Tassone and Duey-Holtz, 2008). External compression can also be given through the shoulders into the spine while the child is sitting, or through the shoulders or hips when the child is in a four-point position (Intervention 5-6). The child’s body parts must always be aligned prior to receiving manual compression, with compression graded to the tolerance of the child. Less compression is better in most instances. Use of approximation is illustrated in the following example involving a young girl with athetoid cerebral palsy. When the clinician placed a hand lightly but firmly on the girl’s head as she was attempting to maintain a standing position, the child was more stable within the posture. She was then asked to assume various ballet positions with her feet, to help her learn to adjust to different-sized bases of support and still maintain her balance. During the next treatment session, the girl initiated the stabilization by placing the therapist’s hand on her head. Gradually, external stabilization from the therapist’s hand was able to be withdrawn. 186 Intervention 5-6 Compression of Proximal Joints A. Manual approximation through the shoulders in sitting. B. Manual approximation through the shoulders in the four-point position. Intermittent or sustained pressure can also be used to prepare a limb or the trunk to accept weight prior to loading the limb as in gait or laterally shifting weight onto the trunk. Prior to weight bearing on a limb, such as in propped sitting, the arm can be prepared to accept the weight by applying pressure from the heel of the hand into the shoulder with the elbow straight but not locked (Intervention 5-7). This is best done with the arm in about 45 degrees of external rotation. Think of the typical position of the arm when it is extended as if to catch yourself. The technique of using sustained pressure for the trunk is done by applying firm pressure along the side of the trunk on which the weight will be shifted (Intervention 5-8). The pressure is applied along one side of the trunk from the middle of the trunk out toward the hip and shoulder prior to assisting the child to turn onto that side. This intervention can be used as preparation for rolling or coming to sit through side-lying. A modification of this intervention is used prior to or as you initiate a lateral weight shift to assist trunk elongation. Intervention 5-7 Preparation for Upper Extremity Weight Bearing 187 Application of pressure through the heel of the hand to approximate the joints of the upper extremity. Intervention 5-8 Preparation for Weight Acceptance Firm stroking of the trunk in preparation for weight acceptance. A. Beginning hand position. B. Ending hand position. Vision Visual images entice a child to explore the environment. Vision also provides important information for the development of head control and balance. Visual fixation is the ability to look with both eyes for a sustained time. To encourage looking, find out whether the child prefers faces or objects. In infants, begin with black and white objects or a stylized picture of a face and then add colors such as red and yellow to try to attract the child’s attention. You will have the best success if you approach the infant from the periphery because the child’s head will most likely be turned to 188 the side. Next, encourage tracking of objects to the midline and then past the midline. Before infants can maintain the head in the midline, they can track from the periphery toward the midline, then through ever-widening arcs. Directional tracking ability then progresses horizontally, vertically, diagonally, and rotationally (clockwise and counterclockwise). If the child has difficulty using both eyes together or if the eyes cross or turn out, alert the supervising physical therapist, who may suggest that the child see an optometrist or an ophthalmologist. Children who have eye problems corrected early in life may find it easier to develop head control and the ability to reach for objects. Children with permanent visual impairments must rely on auditory signals within the environment to entice them to move. Just as you would use a toy to help a child track visually, use a rattle or other noisemaker to encourage head turning, reaching, and rolling toward the sound. The child has to be able to localize or determine where the sound is coming from before these types of activities are appropriate. Children with visual impairments generally achieve motor milestones later than typically developing children. Hearing Although hearing does not specifically play a role in the development of posture and movement, if the acoustic nerve responsible for hearing is damaged, then the vestibular nerve that accompanies it may also be impaired. Impairment of the vestibular nerve or any part of the vestibular system may cause balance deficits because information from head movement is not translated into cues for postural responses. In addition, the close coordination of eye and head movements may be compromised. When working with preschoolers with hearing impairment, clinicians have often found that these children have balance problems. Studies have shown that both static and dynamic balance are impaired in this population and produce motor deficits (de Sousa et al., 2012; Livingstone and McPhillips, 2011). Auditory cues can be used to encourage movement and, in the visually impaired, may provide an alternative way to direct or guide movement. 189 Preparation for movement Postural Readiness Postural readiness is the usual preparation for movement. It is defined as the ability of the muscles to exhibit sufficient resting tone to support movement. Sufficient resting tone is evident by the child’s ability to sustain appropriate postural alignment of the body before, during, and after performing a movement task. In children with neurologic deficit, some positions can be advantageous for movement, whereas others may promote abnormally strong tonic reflexes (Table 5-2). A child in the supine position may be dominated by the effect of the tonic labyrinthine reflex, which causes increased extensor tone, and thus decreases the possibility that the child will be able to roll to prone or come to sit easily. If the tone is too high or too low, or if the body is not appropriately aligned, movement will be more difficult, less efficient, and less likely to be successful. Table 5-2 Advantages and Disadvantages of Different Positions Advantages Disadvantages Position Can begin early weight bearing through the lower extremities when the knees are bent and feet are flat on the support surface. Positioning of the head and upper trunk in forward flexion can decrease the effect of the STLR. Can facilitate use of the upper extremity in play or object exploration. Lower extremities can be positioned in flexion over a roll, ball, or bolster. Effect of STLR can be strong and not easily overcome. Supine can be disorienting because it is associated with sleeping. The level of arousal] is lowest in this position, so it may be more difficult to engage the child in meaningful activity. Excellent for dampening the effect of most tonic reflexes because of the neutral position of the head; achieving protraction of the shoulder and pelvis; separating the upper and lower trunk; achieving trunk elongation on the down side; separating the right and left sides of the body; and promoting trunk stability by dissociating the upper and lower trunk. Excellent position to promote functional movements, such as rolling and coming to sit or as a transition from sitting to supine or prone. Promotes weight bearing through the upper extremities (prone on elbows or extended arms); stretches the hip and knee flexors and facilitates the development of active extension of the neck and upper trunk. In young or very developmentally disabled children, it may facilitate development of head control and may promote eye-hand relationships. With the addition of a movable surface, upper extremity protective reactions may be elicited. Prone It may be more difficult to maintain the position without external support or a special device, such as a side lyer. Shortening of the upper trunk muscles may occur if the child is always positioned on the same side. Flexor posturing may increase because of the influence of the PTLR. Breathing may be more difficult for some children secondary to inhibition of the diaphragm, although ventilation may be better. Prone is not recommended for young children as a sleeping posture because of its relationship with an increased incidence of sudden infant death syndrome. Sitting Promotes active head and trunk control; can provide weight bearing through the upper and lower extremities; frees the arms for play; and may help normalize visual and vestibular input as well as aid in feeding. The extended trunk is dissociated from flexed lower extremities. Excellent position to facilitate head and trunk righting reactions, trunk equilibrium reactions, and upper extremity protective extension. One or both upper extremities can be dissociated from the trunk. Side sitting romotes trunk elongation and rotation. Weight bearing through all four extremities with the trunk working against gravity. Provides an excellent opportunity for dissociation and reciprocal movements of the extremities and as a transition to side sitting if trunk rotation is possible. Quadruped| Sitting is a flexed posture. A child may be unable to maintain trunk extension because of a lack of strength or too much flexor tone. Optimal seating at 90-90-90 may be difficult to achieve and may require external support. Some floor-sitting postures, such as cross- sitting and W sitting, promote muscle tightness and may predispose to lower extremity contractures. The flexed posture is difficult to maintain because of the influence of the STNR, which can encourage bunny hopping as a form of locomotion. When trunk rotation is lacking, children often end up W sitting. Kneeling | Kneeling is a dissociated posture; the trunk and hips are extended while the knees are flexed. Provides a stretch to the hip flexors. Hip and pelvic control can be developed in this position, which can be a transition posture to and from side sitting or to half-kneeling and standing. Provides weight bearing through the lower extremities and a stretch to the hip and knee flexors and ankle plantar flexors; can promote active head and trunk control and may normalize visual input. Standing Kneeling can be difficult to control, and children often demonstrate anj inability to extend at the hips completely because of the influence of the STNR. A significant amount of external support may be required; may not be a long-term option for the child. Adapted from Lemkuhl LD, Krawczyk L: Physical therapy management of the minimally-responsive patient following traumatic brain injury: coma stimulation. Neurol Rep 17:10-17, 1993. PTLR, Prone tonic labyrinthine reflex; STLR, supine tonic labyrinthine reflex; STNR, symmetric tonic neck reflex. Postural Alignment Alignment of the trunk is required prior to trying to elicit movement. When you slump in your chair before trying to come to stand, your posture is not prepared to support efficient movement. When the pelvis is either too anteriorly or too posteriorly tilted, the trunk is not positioned to respond with appropriate righting reactions to any weight shift. Recognizing that the patient is lying or sitting asymmetrically should cue repositioning in appropriate alignment. To promote weight bearing on the hands or feet, one must pay attention to how limbs are positioned. Excessive rotation of a limb may provide mechanical locking into a posture, rather than afford the child’s muscles an opportunity to maintain the position. Examples of excessive rotation can be seen in the elbows of a child with low tone who attempts to maintain a hands-and-knees position or whose knees are hyperextended in standing. Advantages and disadvantages of different positions are discussed in Chapter 6 as they relate to the effects of exaggerated tonic reflexes, which are most often evident in children with cerebral palsy. Manual Contacts Manual contacts at proximal joints are used to guide movement or to reinforce a posture. The 190 shoulders and hips are most commonly used either separately or together to guide movement from one posture to another. Choosing manual contacts is part of movement preparation. The more proximal the manual contacts, the more you control the child’s movements. Moving contacts more distally to the elbow or knee or to the hands and feet requires that the child take more control. A description of the use of these manual contacts is given in the section of this chapter on positioning and handling. Rotation Slow, rhythmic movement of the trunk and extremities is often helpful in decreasing muscle stiffness (Intervention 5-9). Some children are unable to attempt any change in position without this preparation. When using slow, rhythmic movements, one should begin at proximal joints. For example, if tightness in the upper extremities is evident, then slow, alternating pressure can be applied to the anterior chest wall, followed by manual protraction of the scapula and depression of the shoulder, which is usually elevated. The child’s extremity is slowly and rhythmically externally rotated as the arm is abducted away from the body and elevated. The abduction and elevation of the arm allow for some trunk lengthening, which can be helpful prior to rolling or shifting weight in sitting or standing. Always starting at proximal joints provides a better chance for success. Various hand grasps can be used when moving the upper extremity. A handshake grasp is commonly used, as is grasping the thumb and thenar eminence (Figure 5-8). Extending the carpometacarpal joint of the thumb also decreases tone in the extremity. Be careful to avoid pressure in the palm of the hand if the child still has a palmar grasp reflex. Do not attempt to free a thumb that is trapped in a closed hand without first trying to alter the position of the entire upper extremity. Intervention 5-9 Trunk Rotation Slow, rhythmic rotation of the trunk in side-lying to decrease muscle tone and to improve respiration. 191 FIGURE 5-8 Handshake grasp. When a child has increased tone in the lower extremity muscles, begin with alternating pressure on the pelvis (anterior superior iliac spine), first on one side and then the other (Intervention 5-10). As you continue to rock the child’s pelvis slowly and gently, externally rotate the hip at the proximal thigh. As the tone decreases, lift the child’s legs into flexion as bending the hips and knees can significantly reduce the bias toward extension. With the child’s knees bent, continue slow, rhythmic rotation of one or both legs and place the legs into hook lying. Pressure can be given from the knees into the hips and into the feet to reinforce this flexed position. The more the hips and knees are flexed, the less extension is possible, so in cases of extreme increased tone, the knees can be brought to the chest with continued slow rotation of the bent knees across the trunk. By positioning the child’s head and upper body into more flexion in the supine position, you may also flex the child’s lower extremities more easily. A wedge, bolster, or pillows can be used to support the child’s upper body in the supine position. The caregiver should avoid positioning the child supine without ensuring that the child has a flexed head and upper body, because the legs may be too stiff in extension as a result of the supine tonic labyrinthine reflex. Lower trunk rotation initiated with one or both of the child’s lower extremities can also be used as a preparatory activity prior to changing position, such as rolling from supine to prone (Intervention 5-11). If the child’s hips and knees are too severely flexed and adducted, gently rocking the child’s pelvis by moving the legs into abduction by means of some outward pressure on the inside of the knees and downward pressure from the knees into the hips may allow you to slowly extend and abduct the child’s legs (Intervention 5-12). When generalized increased tone exists, as in a child with quadriplegic cerebral palsy, slow rocking while the child is prone over a ball may sufficiently reduce tone to allow initiation of movement transitions, such as rolling to the side or head lifting in prone (Intervention 5-13). Intervention 5-10 Alternating Pelvic Pressure 192 Alternating pressure with manual contact on the pelvis can be used to decrease muscle tone and to facilitate pelvic and lower extremity motion. Intervention 5-11 Lower trunk rotation initiated by flexing one leg over the other and facilitating rolling from supine to prone. Intervention 5-12 193 Lower Trunk Rotation and Pelvic Rocking Lower trunk rotation and pelvic rocking to aid in abducting the lower extremities in the presence of increased adductor muscle tone. Intervention 5-13 Use of the Ball for Tone Reduction and Head Lifting A 8 c A, B. Slow rocking on a ball can promote a reduction in muscle tone. C. Head lifting. 194 195 Interventions to foster head and trunk control The following positioning and handling interventions can be applied to children with a variety of disorders. They are arranged developmentally, because children need to acquire some degree of head control before they are able to control the trunk in an upright posture. Both head and trunk control are necessary components for sitting and standing. Head Control Several different ways of encouraging head control through positioning in prone, in supine, and while being held upright in supported sitting are presented here. The interventions can be used to promote development of head control in children who do not exhibit appropriate control. Many interventions can be used during therapy or as part of ahome program. The decision about which interventions to use should be based on a thorough examination by the physical therapist and the therapeutic goals outlined in the child’s plan of care. Positioning to Encourage Head Control Prone over a Bolster, Wedge, or Half-Roll Prone is usually the first position in which the newborn experiences head lifting; therefore, it is one of the first positions used to encourage development of head control. When an infant is placed over a small roll or bolster, the child’s chest is lifted off the support surface, and this maneuver takes some weight off the head. In this position, the infant’s forearms can be positioned in front of the roll, to add further biomechanical advantage to lifting the head. The child’s elbows should be positioned under the shoulders to provide weight-bearing input for a support response from the shoulder girdle muscles. A visual and auditory stimulus, such as a mirror, brightly colored toy, or noisemaker, can be used to encourage the child to lift the head. Lifting is followed by holding the head up for a few seconds first in any position, then in the midline. A wedge may also be used to support the infant’s entire body and to keep the arms forward. The advantage of a half-roll is that because the roll does not move, the child is less likely to “roll” off it. It may be easier to obtain forearm support when the child is positioned over a half-roll or a wedge of the same height as the length of the child’s upper arm (Intervention 5-14, A). Intervention 5-14 Positions to Encourage Head Control 196 A. Positioning the child prone over a half-roll encourages head lifting and weight bearing on the elbows and forearms. B. Positioning the child supine on a wedge in preparation for anterior head lifting. C. A feeder seat/floor sitter that allows for different degrees of inclination. Supine on a Wedge or Half-Roll Antigravity flexion of the neck is necessary for balanced control of the head. Although most children exhibit this ability at around 5 months of age, children with disabilities may find development of antigravity flexion more of a challenge than cervical extension, especially children with underlying extensor tone. Preparatory positioning in a supine position on a wedge or half-roll puts the child in a less difficult position against gravity to attempt head lifting (Intervention 5-14, B). The child should be encouraged to keep the head in the midline while he is positioned in supine. A midline position can be encouraged by using a rolled towel arch or by providing a visual focus. Toys or objects can be attached to a rod or frame, as in a mobile, and placed in front of the child to encourage reaching with the arms. If a child cannot demonstrate any forward head movement, increasing the degree of incline so the child is closer to upright than to supine may be beneficial. This can also be accomplished by using an infant seat or a feeder seat with a Velcro base that allows for different degrees of inclination (Intervention 5-14, C). Interventions to Encourage Head Control Modified Pull-to-Sit Maneuver The beginning position is supine. The hardest part of the range for the child’s head to move through in the pull-to-sit maneuver is the initial part in which the force of gravity is directly perpendicular to the head (Figure 5-9). The infant or child has to have enough strength to initiate the movement. Children with disabilities may have extreme head lag during the pull-to-sit transition. Therefore, 197 the maneuver is modified to make it easier for the child to succeed. The assistant provides support at the child’s shoulders and rotates the child toward herself and begins to move the child toward sitting on a diagonal (Intervention 5-15). The assistant may need to wait for the child to bring the head and upper body forward into sitting. The child may be able to help with only the last part of the maneuver as the vertical position is approached. If the child tries to reinforce the movement with shoulder elevation, the assistant’s index fingers can depress the child’s shoulders and thus can avoid this substitution. Improvement in head control can be measured by the child’s ability to maintain the head in midline in various postures, by exhibiting neck-righting reactions or by assisting in the maneuver earlier during the range. As the child’s head control improves, less trunk rotation is used to encourage the neck muscles to work against gravity as much as possible. More distal contacts such as the elbows and finally the hands can be used to initiate the pull-to-sit maneuver (see Intervention 5-2). These distal manual contacts are not recommended if the child has too much joint laxity. GRAVITY GRAVITY FIGURE 5-9 Relationship of gravity with the head in supported supine and supported sitting positions. Intervention 5-15 Modified Pull-to-Sit Maneuver 198 A. Position the child on an inclined surface supine in preparation for anterior head lifting. B. Provide support at the child’s shoulder, rotate the child toward yourself, and begin to move the child toward sitting on a diagonal. Upright in Supported Sitting In the child’s relation to gravity, support in the upright sitting position (Box 5-1) is probably an easier position in which to maintain head control, because the orientation of the head is in line with the force of gravity. The head position and the force of gravity are parallel (see Figure 5-9), whereas when a child is in supine or prone position, the force of gravity is perpendicular to the position of the head at the beginning of head lifting. This relationship makes it more difficult to lift the head from either supine or prone position than to maintain the head when either held upright in vertical or held upright in supported sitting. This is why a newborn has total head lag as one tries to pull the baby to sit, but once the infant is sitting, the head appears to sit more stably on the shoulders. A child who is in supine or prone position uses only neck flexors or extensors to lift the head. In the upright position, a balance of flexors and extensors is needed to maintain the head position. The only difference between being held upright in the vertical position and being held upright in supported sitting is that the trunk is supported in the latter position and thus provides some proprioceptive input by approximation of the spine and pelvis. Manual contacts under or around the shoulders are used to support the head (Figure 5-10). Establishing eye contact with the child also assists head stability because it provides a stable visual input to orient the child to the upright position. To encourage head control further, the child can be placed in supported sitting in an infant seat or a feeder seat as a static position, but care should be taken to ensure the infant’s safety in such a seat. Never leave a child unattended in an infant seat or other seating device without a seat belt and/or shoulder harness to keep the child from falling forward, and never place such a device on a table unless the child is constantly supervised. Box 5-1 Progression of Supported Sitting 1. Sitting in the corner of a sofa. 2. Sitting in a corner chair or a beanbag. 3. Side sitting with one arm propped over a bolster or half-roll. 4, Sitting with arms forward and supported on an object, such as a pillow or a ball. 5. Sitting in a high chair. 199 FIGURE 5-10 Early head control in supported sitting. Weight Shifting from Supported Upright Sitting The beginning position is with the child seated on the lap of the assistant or caregiver and supported under the arms or around the shoulders. Support should be firm to provide some upper trunk stability without causing any discomfort to the child. Because the child’s head is inherently stable in this position, small weight shifts from the midline challenge the infant to maintain the head in the midline. If possible, just visually engaging the child may be enough to assist the child in maintaining head position or righting the head as weight is shifted. As the child becomes able to accept challenges, larger displacements may be given. Carrying in Prone The child’s beginning position is prone. Because prone is the position from which head lifting is the easiest, when a child is in the prone position with support along the midline of the trunk, this positioning may encourage head lifting, as shown in Intervention 5-4, F. The movement produced by the person who is carrying the child may also stimulate head lifting because of the vestibular system’s effect on postural muscles. Another prone position for carrying can be used in the case of a child with flexor spasticity (Intervention 5-16, A). One of the caregiver’s forearms is placed under the child’s shoulders to keep the arms forward, while the other forearm is placed between the child’s thighs to keep one hip straight. Some lower trunk rotation is achieved as the pelvis is turned from the weight of the dangling leg. Intervention 5-16 Carrying Positions to Encourage Head Control 200 A. In the case of a child with flexor spasticity, the caregiver can place one forearm under the child’s shoulders to keep his arms forward and place the other forearm between his thigh, while keeping one hip straight. B. When the child is carried in the upright position, the back of the child’s head is supported against the caregiver’s chest. Carrying in Upright The beginning position is upright. To encourage use of the neck muscles in the development of head control, the child can be carried while in an upright position. The back of the child’s head and trunk can be supported against the caregiver's chest (Intervention 5-16, B). The child can be carried, facing forward, in a snuggler or a backpack. For those children with slightly less head control, the caregiver can support around the back of the child’s shoulders and head in the crook of an elevated elbow, as shown in Intervention 5-4, A. An older child needs to be in a more upright posture than is pictured, with the head supported. Prone in a Hammock or on a Suspended Platform Swing The beginning position is prone. Movement stimulation using a hammock or a suspended swing can give vestibular input to facilitate head control when the child is in a prone position. When using a mesh hammock, you should place pillows in the hammock and put the child on top of the pillows. The child’s head should be supported when the child is not able to lift it from the midline (see Figure 5-7). As head control improves, support can gradually be withdrawn from the head. When vestibular stimulation is used, the change in direction of movement is detected, not the continuous rhythm, so be sure to vary the amount and intensity of the stimulation. Always watch for signs of overstimulation, such as flushing of the face, sweating, nausea, or vomiting. Vestibular stimulation may be used with children who are prone to seizures. However, you must be careful to avoid visual stimulation if the child’s seizures are brought on by visual input. The child can be blindfolded or wear a baseball cap pulled down over the eyes to avoid visual stimulation. Trunk Control Positioning for Independent Sitting As stated previously, sitting is the position of function for the upper extremities, because self-care activities, such as feeding, dressing, and bathing, require use of upper extremity, as does playing with objects. Positioning for independent sitting may be more crucial to the child’s overall level of function than standing, especially if the child’s ambulation potential is questionable. Independent sitting can be attained in many ways. Propped sitting can be independent, but it will not be functional unless one or both hands can be freed to perform meaningful activities. Progression of sitting based on degree of difficulty is found in Box 5-2. 201 Box 5-2 Progression of Sitting Postures Based on Degree of Difficulty 1. Sitting propped forward on both arms. 2. Sitting propped forward on one arm. 3. Sitting propped laterally on both arms. 4, Sitting propped laterally on one arm. 5. Sitting without hand support. 6. Side sitting with hand support. 7. Side sitting with no hand support. Sitting Propped Forward on Both Arms The beginning position is sitting, with the child bearing weight on extended arms. Various sitting postures can be used, such as abducted long sitting, ring sitting, or tailor sitting. The child must be able to sustain some weight on the arms. Preparatory activities can include forward protective extension or pushing up from prone on elbows. Gentle approximation through the shoulders into the hands can reinforce the posture. Weight bearing encourages a supporting response from the muscles of the shoulder girdle and the upper extremities to maintain the position. Sitting Propped Forward on One Arm The beginning position is sitting, as described in the previous paragraph. When bilateral propping is possible, weight shifting in the position can encourage unloading one extremity for reaching or pointing and can allow for propping on one arm. Sitting Propped Laterally on One Arm If the child cannot support all her weight on one arm laterally, then part of the child’s weight can be borne by a bolster placed between the child’s side and the supporting arm (Figure 5-11). Greater weight acceptance can be practiced by having the child reach with the other hand in the direction of the supporting hand. When the location of the object to be reached is varied, weight is shifted and the child may even attempt to change sitting postures. FIGURE 5-11 Sitting propped laterally on one arm over a bolster. Sitting without Hand Support 202 Progressing from support on one hand to no hand support can be encouraged by having the child shift weight away from the propped hand and then have her attempt to reach with the propped hand. A progression of propping on objects and eventually on the child’s body can be used to center the weight over the sitting base. Engaging the child in clapping hands or batting a balloon may also afford opportunities to free the propping hand. Short sitting with the feet supported can also be used as a way to progress from sitting with hand support to using one hand to using no hands for support. Side Sitting Propped on One Arm Side sitting is a more difficult sitting posture in which to play because trunk rotation is required to maintain the posture to have both hands free for play. Some children are able to attain and maintain the posture only if they prop on one arm, a position that allows only one hand free for play and so negates any bimanual or two-handed activities. Again, the use of a bolster can make it easier to maintain the propped side-sitting posture. Asymmetric side sitting can be used to promote weight bearing on a hip on which the child may avoid bearing weight, as in hemiplegia. The lower extremities are asymmetrically positioned. The lower leg is externally rotated and abducted while the upper leg is internally rotated and adducted. Side Sitting with No Hand Support Achievement of independent side sitting can be encouraged in much the same way as described in the previous paragraph. Movement Transitions that Encourage Trunk Rotation and Trunk Control Once a child is relatively stable within a posture, the child needs to begin work on developing dynamic control. One of the first things to work on is shifting weight within postures in all directions, especially those directions used in making the transition or moving from one posture to another. The following are general descriptions of movement transitions commonly used in functional activities. These transitions can be used during therapy and can also be an important part of any home program. Rolling from Supine to Prone Using the Lower Extremity The beginning position is supine. Intervention 5-17 shows this transition. Using your right hand, grasp the child’s right lower leg above the ankle and gently bring the child’s knee toward the chest. Continue to move the child’s leg over the body to initiate a rolling motion until the child is side- lying or prone. Alternate the side toward which you turn the child. Initially, infants roll as a log or as one complete unit. As they mature, they rotate or roll segmentally. If the lower extremity is used as the initiation point of the movement, the pelvis and lower trunk will rotate before the upper trunk and shoulders. As the child does more of the movement, you will need to do less and less until, eventually, the child can be enticed to roll using a sound or visual cue or by reaching with an arm. Intervention 5-17 Rolling from Supine to Prone 203 Movement sequence of rolling supine to prone. A. With the right hand, grasp the child’s left lower leg above the ankle and gently bring her knee toward the chest. B and C. Continue to move the child’s leg over the body to initiate a rolling motion until the child is in the side-lying or prone position. Coming to Sit from Supine The beginning position is supine. Position yourself to one side of the child. Reach across the child’s body and grasp the hand farthest away from you. Bring the child’s arm across the body so the child has turned to the side and is pushing up with the other arm. Stabilize the child’s lower extremities so the rotation occurs in the trunk and is separate from leg rotation. Coming to Sit from Prone The beginning position is prone. Elongate the side toward which you are going to roll the child. Facilitate the roll to side-lying and proceed as follows in coming to sit from side-lying as described in the next paragraph. Coming to Sit from Side-Lying The beginning position is with the child lying on one side, facing away from you with the head to the right. The child’s lower extremities should be flexed. If lower extremity separation is desirable, the child’s lower leg should be flexed and the top leg allowed to remain straight. Apply gentle pressure on the uppermost part of the child’s shoulder in a downward and lateral direction. The child’s head should right laterally, and the child should prop on the downside elbow. If the child experiences difficulty in moving to propping on one elbow, use one hand to assist the downward arm into the correct position. Your upper hand can now move to the child’s top hip to direct the weight shift diagonally back over the flexed hip while your lower hand assists the child to push up on the downward arm. Part of this movement progression is shown in Intervention 5-2. The child’s movements can be halted anywhere during the progression to improve control within a specific range or to encourage a particular component of the movement. The child ends up sitting with or without hand support, or the support arm can be placed over a bolster or half-roll if more support is needed to maintain the end position. The child’s sitting position can range from long abducted sitting, propping forward on one or both extended arms, to half-ring sitting with or without propping. These positions can be maintained without propping if the child is able to maintain them. Sitting to Prone 204 This transition is used to return to the floor after playing in sitting. It can be viewed as the reverse of coming to sit from side-lying. In other words, the child laterally shifts weight to one side, first onto an extended arm and then to an elbow. Finally, the child turns over the arm and into the prone position. Some children with Down syndrome widely abduct their legs to lower themselves to prone. They lean forward onto outstretched arms as they continue to swing their legs farther out and behind their bodies. Children with hemiplegic involvement tend to move or to make the transition from sitting to prone position by moving over the noninvolved side of the body. They need to be encouraged to shift weight toward and move over the involved side and to put as much weight as possible on the involved upper extremity. Children with bilateral involvement need to practice moving to both sides. Prone to Four-Point The beginning position is prone. The easiest way to facilitate movement from prone to four-point is to use a combination of cues at the shoulders then the hips, as shown in Intervention 5-18. First, reach over the upper back of the child and lift gently. The child’s arms should be flexed beside the upper body at the beginning of the movement. By lifting the shoulders, the child may bring the forearms under the body in a prone on elbows or puppy position. Continue to lift until the child is able to push up on extended arms. Weight bearing on extended arms is a prerequisite for assuming a hands-and-knees position. If the child requires assistance to maintain arms extended, a caregiver can support the child at the elbows, or pediatric air splints can be used. Next, lift the hips up and bring them back toward the feet, just far enough to achieve a four-point position. If the child needs extra support under the abdomen, a bolster, a small stool, or pillows can be used to help sustain the posture. Remember, four-point may just be a transitional position used by the child to go into kneeling or sitting. Not all developmentally normal children learn to creep on hands and knees. Depending on the predominant type of muscle tone, creeping may be too difficult to achieve for some children who demonstrate mostly flexor tone in the prone position. Children with developmental delays and minimal abnormal postural tone can be taught to creep. Intervention 5-18 Promoting Progression from Prone to Kneeling 205 Facilitating the progression of movement from prone to prone on elbows to quadruped position using the shoulders and hips as key points of control. A. Before beginning, the child’s arms should be flexed beside the upper body. Reach over the upper back of the child and lift her shoulders gently. B. As her shoulders are lifted, the child may bring her forearms under the body in a prone on elbows or puppy position. Continue to lift until the child is able to push up on extended arms. C, D. Next, lift the child’s hips up and bring them back toward her feet, just far enough to achieve a four-point position. E. Promoting movement from quadruped to kneeling using the shoulders. The child extends her head before her hips. Use of the hips as a key point may allow for more complete extension of the hips before the head is extended. Four-Point to Side Sitting The beginning position is four-point. Once the child can maintain a hands-and-knees position, start work on moving to side sitting to either side. This transition works on control of trunk lowering while the child is in a rotated position. Dissociation of lower trunk movements from upper trunk 206 movements can also be practiced. A prerequisite is for the child to be able to control or tolerate diagonal weight shifts without falling. So many times, children can shift weight anteriorly and posteriorly, but not diagonally. If diagonal weight shifting is not possible, the child will often end up sitting on the heels or between the feet. The latter position can have a significant effect on the development of lower extremity bones and joints. The degree to which the child performs side sitting can be determined by whether the child is directed to go all the way from four-point to side sitting on the support surface, or by whether the movement is shortened to end with the child side sitting on pillows or a low stool. If movement to one side is more difficult, movement toward the other side should be practiced first. Four-Point to Kneeling The beginning position is four-point. Kneeling is accomplished from a four-point position by a backward weight shift followed by hip extension with the rest of the child’s body extending over the hips (see Intervention 5-18, E). Some children with cerebral palsy try to initiate this movement by using head extension. The extension should begin at the hips and should progress cephalad (toward the head). A child can be assisted in achieving an upright or tall-kneeling position by placement of extended arms on benches of increasing height to aid in shifting weight toward the hips. In this way, the child can practice hip extension in smaller ranges before having to move through the entire range. Kneeling to Side Sitting The beginning position is kneeling. Kneeling is an extended position because the child’s back must be kept erect with the hips extended. Kneeling is also a dissociated posture because while the hips are extended, the knees are flexed and the ankles are passively plantar flexed to extend the base of support and to provide a longer lever arm. Lowering from kneeling requires eccentric control of the quadriceps. If this lowering occurs downward ina straight plane, the child will end up sitting on his feet. If the trunk rotates, the lowering can proceed to allow the child to achieve a side-sitting position. Kneeling to Half-Kneeling The beginning position is kneeling. The transition to half-kneeling is one of the most difficult to accomplish. Typically developing children often use upper limb support to attain this position. To move from kneeling to half-kneeling, the child must unweight one lower extremity. This is usually done by performing a lateral weight shift. The trunk on the side of the weight shift should lengthen or elongate while the opposite side of the trunk shortens in a righting reaction. The trunk must rotate away from the side of the body toward which the weight is shifted to assist the unweighted lower extremity’s movement (Intervention 5-19). The unweighted leg is brought forward, and the foot is placed on the support surface. The resulting position is a dissociated one in which the forward leg is flexed at all joints, while the loaded limb is flexed at the knee and is extended at the hip and ankle (plantar flexed). Intervention 5-19 Kneeling to Half-Kneeling 207 A. Kneel behind the child and place your hands on the child’s hips. B. Shift the child’s weight laterally, but do not let the child fall to the opposite side, as is depicted. The child’s trunk should elongate on the weight-bearing side, and with some trunk rotation, the child may be able to bring the opposite leg forward. C. If the child is unable to bring the opposite leg forward, assist as depicted. Health Sciences Company. Reproduced by permission. All rights reserved.) Coming to Stand The beginning position is sitting. Coming to stand is probably one of the most functional movement transitions. Clinicians spend a great deal of time working with people of all ages on this movement transition. Children initially have to roll over to prone, move into a hands-and-knees position, creep over to a person or object, and pull up to stand through half-kneeling. The next progression in the developmental sequence adds moving into a squat from hands-and-knees and pulling the rest of the way up on someone or something. Finally, the 18-month-old can usually come to stand from a squat without assistance (Figure 5-12). As the abdominal muscles become stronger, the child in supine turns partially to the side, pushes with one arm to sitting, then goes to a squat and on up to standing. The most mature pattern is to come straight up from supine, to sitting with no trunk rotation, to assuming a squat, and then coming to stand. From prone, the most mature progression is to push up to four-point, to kneeling and half-kneeling, and then to standing. Independent half- kneeling is a difficult position because of the configuration of the base of support and the number of body parts that are dissociated from each other. 208 FIGURE 5-12 A to C, Coming to stand from a squat requires good lower extremity strength and balance. 209 Adaptive equipment for positioning and mobility Decisions regarding adaptive equipment for positioning and mobility should be made based on input from the team working with the infant or child. Adaptive equipment can include bolsters, wedges, walkers, and wheeled mobility devices. The decision about what equipment to use, however, is ultimately up to the parents. Barriers to the use of adaptive equipment may include, but are not limited to, architectural, financial, cosmetic, and behavioral constraints. Sometimes, children do not like the equipment the therapist thinks is most therapeutic. Any piece of equipment should be used on a trial basis before being purchased. Regarding wheelchair selection, a team approach is advocated. Members of the assistive technology team may include the physical therapist, the occupational therapist, the speech therapist, the classroom teacher, the rehabilitation engineer, and the vendor of durable medical equipment. The child and family are also part of the team because they are the ones who will use the equipment. The physical therapist assistant may assist the physical therapist in gathering information regarding the need for a wheelchair or piece of adaptive equipment, as well as providing feedback on how well the child is able to use the device. For more information on assistive technology, refer to O’Shea and Bonfiglio (2012) or Jones and Puddefoot (2014). The 90-90-90 rule for sitting alignment should be observed. In other words, the feet, knees, and hips should be flexed to approximately 90 degrees. This degree of flexion allows weight to be taken on the back of the thighs, as well as the ischial tuberosities of the pelvis. If the person cannot maintain the normal spinal curves while in sitting, thought should be given to providing lumbar support. The depth of the seat should be sufficient to support no more than % of the thigh (Wilson, 2001). Supporting more than % of the thigh leads to excessive pressure on the structures behind the knee, whereas less support may require the child to compensate by developing a kyphosis. Other potential problems, such as neck extension, scapular retraction, and lordosis of the lumbar spine, can occur if the child is not able to keep the trunk extended for long periods of time. In such cases, the child may feel as though he is falling forward. Lateral trunk supports are indicated to control asymmetries in the trunk that may lead to scoliosis. Goals for Adaptive Equipment Goals for adaptive equipment are listed in Box 5-3. Many of these goals reflect what is expected from positioning because adaptive equipment is used to reinforce appropriate positions. For example, positioning should give a child a postural base by providing postural alignment needed for normal movement. Changing the alignment of the trunk can have a positive effect on the child’s ability to reach. Supported sitting may counteract the deforming forces of gravity, especially in a child with poor trunk control who cannot maintain an erect trunk posture. Simply supporting the child’s feet takes much of the strain off trying to keep weight on the pelvis in a chair that is too high. When at all possible, the child’s sitting posture with adaptive equipment should approximate that of a developmentally normal child’s by maintaining all spinal curves. Box 5-3 Anticipated Goals for Use of Adaptive Equipment = Gain or reinforce typical movement. m Achieve proper postural alignment. = Prevent contractures and deformities. m Increase opportunities for social and educational interactions. = Provide mobility and encourage exploration. = Increase independence in activities of daily living and self-help skills. = Assist in improving physiologic functions. = Increase comfort. (Data from Wilson J: Selection and use of adaptive equipment. In Connolly BH, Montgomery PC, editors: Therapeutic Exercise in Developmental Disabilities, ed 2. Thorofare, NJ, 2001, Slack, pp. 167-182.) What follows is a general discussion of considerations for positioning in supine and prone, 210 sitting, side-lying, and standing. Supine and Prone Posture Positioning Positioning the child prone over a half-roll, bolster, or wedge is often used to encourage head lifting, as well as weight bearing on forearms, elbows, and even extended arms. These positions are seen in Intervention 5-20. Supine positioning can be used to encourage symmetry of the child’s head position and reaching forward in space. Wedges and half-rolls can be used to support the child’s head and upper trunk in more flexion. Rolls can be placed under the knees, also to encourage flexion. Intervention 5-20 Encouraging Head Lifting and Upper Extremity Weight Bearing Using Prone Supports A. Positioning the child prone over a half-roll encourages head lifting and weight bearing on elbows and forearms. B. Positioning the child prone over a bolster encourages head lifting and shoulder control. C. Positioning the child prone over a wedge promotes upper extremity weight bearing and function. (B, Courtesy Kaye Products, Hillsborough, NC.) Sitting Posture Positioning Many sitting postures are available for the typically developing child who moves and changes position easily. However, the child with a disability may have fewer positions from which to choose, depending on the amount of joint range, muscle extensibility, and head and trunk control required in each position. Children normally experiment with many different sitting postures, although some of these positions are more difficult to attain and maintain. Sitting on the floor with the legs extended is called long sitting. Long sitting requires adequate hamstring length (Figure 5- 13, A) and is often difficult for children with cerebral palsy, who tend to sit on the sacrum with the 211 pelvis posteriorly tilted (Figure 5-14). During ring sitting on the floor, the soles of the feet are touching, the knees are abducted, and the hips are externally rotated such that the legs form a ring. Ring sitting is a comfortable sitting alternative because it provides a wider base of support; however, the hamstrings can and do shorten if this sitting posture is used exclusively (see Figure 5- 13, B). Tailor sitting, or cross-legged floor sitting, also takes some strain off the hamstrings and allows some children to sit on their ischial tuberosities for the first time (see Figure 5-13, C). Again, the hamstrings will shorten if this sitting posture is the only one used by the child. The use of tailor sitting must be carefully evaluated in the presence of increased lower extremity muscle tone, especially in the hamstring and gastrocnemius-soleus muscles. In addition, in many of these sitting positions, the child’s feet are passively allowed to plantar flex and invert, thereby encouraging tightening of the heel cords. If independent sitting is not possible, then adaptive seating should be considered. FIGURE 5-13 Sitting postures. A, Long sitting. B, Ring sitting. C, Tailor sitting. 212 FIGURE 5-14 Sacral sitting. (From Burns YR, MacDonald J: Physiotherapy and child, London, WB Saunders Company Ltd., 1996.) the growing The most difficult position to move into and out of appears to be side sitting. Side sitting is a rotated posture and requires internal rotation of one lower extremity and external rotation of the other lower extremity (Figure 5-15, A). Because of the flexed lower extremities, the lower trunk is rotated in one direction—a maneuver necessitating that the upper trunk be rotated in the opposite direction. A child may have to prop on one arm to maintain side sitting if trunk rotation is insufficient (Figure 5-15, B). Some children can side sit to one side but not to the other because of lower extremity range-of-motion limitations. In side sitting, the trunk on the weight-bearing side lengthens to keep the center of gravity within the base of support. Children with hemiplegia may not be able to side sit on the involved side because of an inability to elongate or rotate the trunk. They may be able to side sit only if they are propped on the involved arm, a maneuver that is often impossible. Because weight bearing on the involved side is a general goal with any person with hemiplegia, side sitting is a good position to work toward with these children (Intervention 5-21). Actively working into side sitting from a four-point or tall-kneeling position can be therapeutically beneficial because so many movement transitions involve controlled trunk rotation. Advantages of using the four-point position to practice this transition are that some of the weight is taken by the arms and less control is demanded of the lower extremities. As trunk control improves, you can assist the child in moving from tall kneeling on the knees to heel sitting and finally from tall kneeling to side sitting to either side. From tall kneeling, the base of support is still larger than in standing, and the arms can be used for support, if needed. 213 A B FIGURE 5-15 _ Side sitting. A, Without propping. B, With propping on one arm for support. Intervention 5-21 Encouraging Weight Bearing on the Hemiplegic Hip 214 Place the child in side sitting on the hemiplegic side. Elevation of the hemiplegic arm promotes trunk and external rotation elongation. Children with disabilities often have one preferred way to sit, and that sitting position can be detrimental to lower extremity development and the acquisition of trunk control. For example, W sitting puts the hips into extreme internal rotation and anteriorly tilts the pelvis, thereby causing the spine to be extended (see Figure 5-4, A). In this position, the tibias are subjected to torsional factors that, if sustained, can produce permanent structural changes. Children with low postural tone may accidentally discover this position by pushing themselves back between their knees. Once these children “discover” that they no longer need to use their hands for support, it becomes difficult to prevent them from using this posture. Children with increased tone in the hip adductor group also use this position frequently because they lack sufficient trunk rotation to move into side sitting from prone. Behavior modification has been typically used to attempt to change a child’s habit of W sitting. Some children respond to verbal requests of “sit pretty,” but often the parent is worn out from constantly trying to have the child correct the posture. As with most habits, if the child can be prevented from ever discovering W sitting, that is optimal. Otherwise, substitute another sitting alternative for the potentially deforming position. For example, if the only way the child can independently sit on the floor is by W sitting, place the child in a corner chair or other positioning device that requires a different lower extremity position. 215 Adaptive Seating Many positions can be used to facilitate movement, but the best position for activities of daily living is upright sitting. How that posture is maintained may necessitate caregiver assistance or adaptive equipment for positioning. In sitting, the child can more easily view the world and can become more interested in interacting with people and objects within the environment. Ideally, the position should allow the child as much independence as possible while maintaining safety. Adaptive seating may be required to meet both these criteria. Some examples of seating devices are shown in Figure 5-16. The easier it is to use a piece of adaptive equipment, the more likely the caregiver will be to use it with the child. A ttl a SS i FIGURE 5-16 Adaptive seating devices. A, Posture chair. B, Bolster chair. A, (Courtesy TherAdapt Products, Inc., Bensenville IL. B, Courtesy Kaye Products, Inc., Hillsborough, NC.) Children without good head control often do not have sufficient trunk control for sitting. Stabilizing the trunk alone may improve the child’s ability to maintain the head in midline. Additionally, the child’s arms can be brought forward and supported on a lap tray. If the child has poor head control, then some means to support the head will have to be incorporated into the seating device (see Figure 5-5). When sitting a child with poor head and trunk control, the child’s back must be protected from the forces of gravity, which accentuate a forward-flexed spine. Although children need to be exposed to gravity while they are in an upright sitting position to develop trunk control, postural deviation can quickly occur if muscular control is not sufficient. Children with low tone often demonstrate flared ribs (Figure 5-17) as a result of an absence of sufficient trunk muscle development to anchor the rib cage for breath support. Children with trunk muscle paralysis secondary to myelodysplasia may require an orthotic device to support the trunk during sitting. Although the orthosis can assist in preventing the development of scoliosis, it may not totally prevent its development because of the inherent muscle imbalance. The orthosis may or may not be initially attached to lower extremity bracing. 216 FIGURE 5-17 Rib flare. (From Moerchen VA: Respiration and motor development: A systems perspective. Neurol Rep 18:9, 1994. Reprinted from the Neurology Report with the permission of the Neurology Section, APTA.) Adaptive seating is widely used for children with disabilities despite the fact that there is limited research supporting its effectiveness. In the most recent systematic review of effectiveness of adaptive seating for children with cerebral palsy, the authors concluded there was limited high quality research available (Chung et al., 2008). Despite that finding, some positive effects on participation, play, and family life have been documented (Rigby et al., 2009; Ryan et al., 2009). A bolster chair is depicted in Figure 5-15, B. Sitting on a chair with an anteriorly inclined seat, such as seen in Figure 5-15, A, was found to improve trunk extension (Miedaner, 1990; Sochaniwskyz et al., 1991). Others (Dilger and Ling, 1986) found that sitting a child with cerebral palsy on a posteriorly inclined wedge decreased her kyphosis (Intervention 5-22). The evidence is not conclusive for whether seat bases should be anteriorly or posteriorly inclined (Chung et al., 2008). Seating requirements must be individually assessed, depending on the therapeutic goals. A child may benefit from several different types of seating, depending on the positioning requirements of the task being performed. Intervention 5-22 Facilitating Trunk Extension Awd Sitting on a posteriorly inclined wedge may facilitate trunk extension. Adjustable-height benches are excellent therapeutic tools because they can easily grow with the child throughout the preschool years. They can be used in assisting children with making the transition from sitting to standing, as well as in providing a stable sitting base for dressing and playing. The height of the bench is important to consider, relative to the amount of trunk control demanded from the child. Depending on the child’s need for pelvic support, a bench allows the child to use trunk muscles to maintain an upright trunk posture during play or to practice head and trunk postural responses when weight shifts occur during dressing or playing. Additional pelvic support can be added to some therapeutic benches, as seen in Figure 5-2. The bench can be used to pull up on and to encourage cruising. Side-Lying Position Side-lying is frequently used to orient a child’s body around the midline, particularly in cases of severe involvement or when the child’s posture is asymmetric when the child is placed either prone or supine. In a child with less severe involvement, side-lying can be used to assist the child to develop control of flexors and extensors on the same side of the body. Side-lying is often a good sleeping posture because the caregiver can alternate the side the child sleeps on every night. For sleeping, a long body pillow can be placed along the child’s back to maintain side-lying, with one end of the pillow brought between the legs to separate them and the other end under the neck or head to maintain midline orientation. Lower extremities should be flexed if the child tends to be ina more extended posture. For classroom use, a commercial side lyer or a rolled-up blanket (Intervention 5-23) may be used to promote hand regard, midline play, or orientation. Intervention 5-23 Using a Side-Lyer 218 Use of a side lyer ensures that a child experiences a side-lying position and may promote hand regard, midline play, or orientation. Positioning in side lying is excellent for dampening the effects of most tonic reflexes. Positioning in Standing Positioning in standing is often indicated for its positive physiologic benefits, including growth of the long bones of the lower extremities. Standing can also encourage alerting behavior, peer interaction, and upper extremity usage for play and self-care. The upper extremities can be weight bearing or free to move because they are no longer needed to support the child’s posture. The upright orientation can afford the child perceptual opportunities. Many devices can be used to promote an upright standing posture, including prone and supine standers, vertical standers, standing frames, and standing boxes. Standing programs can have beneficial effects on bone mineral density, hip development, range of motion and spasticity (Paleg et al., 2014). A standing device is indicated for children who are nonambulatory, minimally ambulatory, or who are not active in standing, as long as there are no contraindications. For hip health, standing should be introduced to children between 9 and 10 months of age. A posture management program should include a passive component using a prone/supine or vertical standing device and a dynamic component in which the stander moves, vibrates, changes from sit to stand, or is propelled by the user (Paleg et al., 2013) (Figure 5-18). 219 FIGURE 5-18 Prone stander with table attachment. (Courtesy Rifton Equipment, Rifton, NY.) Prone standers support the anterior chest, hips, and anterior surface of the lower extremities. The angle of the stander determines how much weight is borne by the lower extremities and feet. When the angle is slightly less than 90 degrees, weight is optimal through the lower extremities and feet (Aubert, 2008). If the child exhibits neck hyperextension or a high-guard position of the arms when in the prone stander, its continued use needs to be reevaluated by the supervising physical therapist. Use of a prone stander is indicated if the goal is physiologic weight bearing or hands-free standing. Supine standers are an alternative to prone standers for some children. A supine stander is similar to a tilt table, so the degree of tilt determines the amount of weight borne by the lower extremities and feet. For children who exhibit too much extension in response to placement in a prone stander, a supine stander may be a good alternative. However, postural compensations develop in some children with the use of a supine stander. These compensations include kyphosis from trying to overcome the posterior tilt of the body. Asymmetric neck postures or a Moro response may be accentuated, because the supine stander perpetuates supine positioning. Use of a supine stander in these situations may be contraindicated. Vertical standers support the child’s lower extremities in hip and knee extension and allow for complete weight bearing. The child’s hands are free for upper extremity tasks, such as writing at a blackboard (Intervention 5-24). The child controls the trunk. The need to function within different environments must be considered when one chooses adaptive equipment for standing. Ina classroom, the use of a stander is often an alternative to sitting, and because the device is adjustable, more than one child may be able to benefit from its use. Continual monitoring of a child’s response to any type of stander should be part of the physical therapist’s periodic reexamination of the child. The physical therapist assistant should note changes in posture and abilities of any child using any piece of adaptive equipment. 220 Intervention 5-24 Vertical Standers Vertical standers support the child’s lower extremities in hip and knee extension and allow for varying amounts of weight bearing depending on the degree of inclination. The child’s hands are free for upper extremity tasks, such as writing at a blackboard, playing with toys (A), or working in the kitchen (B). (Courtesy Kaye Products, Hillsborough, NC.) Dosage for standing programs has recently been presented by Paleg et al. (2013, 2014) and are in Table 5-3. Table 5-3 Recommended Optimal Dosages for Pediatric Supported Standing Programs Outcome Dosage Level of Evidence Hip biomechanics 60 minutes/day in 30°-60° of total bilateral hip abduction} Levels 2-5 Spastici 30-45 minutes/da’ Level 2 Source: Paleg, Smith and Glickman, 2014. Positioning in upright standing is important for mobility, specifically ambulation. Orthotic support devices and walkers are routinely used with young children with myelodysplasia. Ambulation aids can also be important to children with cerebral palsy who do not initially have the balance to walk independently. Two different types of walkers are most frequently used in children with motor deficit. The standard walker is used in front of the child, and the reverse posture control walker is used behind the child. These walkers can have two wheels in the front. The traditional walker is then called a rollator. Difficulties with the standard walker include a forward trunk lean. The child’s line of gravity ends up being anterior to the feet, with the hips in flexion. When the child pushes a reverse walker forward, the bar of the walker contacts the child’s gluteal muscles and gives a cue to extend the hips. Because the walker is behind the child, the walker cannot move too far ahead of the child. The reverse walker can have two or four wheels. In studies conducted in children with cerebral palsy, use of the reverse walker (Figure 5-19) resulted in positive changes in gait and upright posture (Levangie et al., 1989). Each child needs to be evaluated on an individual basis by the physical therapist to determine the appropriate assistive device for ambulation. The device should provide stability, safety, and an energy-efficient gait pattern. 221 FIGURE 5-19 Reverse posture walker. (Courtesy Kaye Products, Inc., Hillsborough, NC.) Dad Functional movement in the context of the child’s world Any movement that is guided by the clinician should have functional meaning. This meaning could be derived as part of a sequence of movement, as a transition from one posture to another, or as part of achieving a task such as touching a toy or exploring an object. Play is a child’s occupation and the way in which the child most frequently learns the rules of moving. Physical therapy incorporates play as a means to achieve therapeutic goals. Structuring the environment in which the treatment session occurs and planning which toys you want the child to play with are all part of therapy. Setting up a situation that challenges the child to move in new ways is motivating to most children. Some suggestions from Linder (2008) and Ratliffe (1998) for toys and strategies to use with children of different ages can be found in Table 5-4. Table 5-4 Appropriate Toys and Intervention Strategies for Working with Children Intervention Strategies Rattles, plastic keys Stuffed animals Smiling, cooing, tickling while face to face Present interesting toys Mobiles Play peek-a-boo; play “So big!” Busy box Dangle toys that make noise when contacted Blocks Push, poke, pull, turn Mirror Encourage reaching, changing positions by moving toys; demonstrate banging objects together, progress to Push toys, ride-on toys knocking down Plastic cups, dishes Tummy time Demonstrate making things “go” Pretend to drink and eat; take tums Toddlers Stackable or nesting toys, blocks | Demonstrate stacking; use different size containers to put things in Farm set, toy animals Grocery cart, pretend food Dolls Dump truck Water toys Pop-up toys Push toys, ride-on toys Books Preschoolers | Balls, plastic bats, blocks Pillows, blankets, cardboard boxes Obstacle course Play dough, clay Sand box Books Puzzles, peg board, string beads Building toys, such as blocks Dress-up clothes, costumes Musical toys, instruments Playground equipment Playground equipment Bicycles Balls, nets, bats, goals Dolls and action figures Beads to string Blocks Magic sets Board games Roller skates, ice skates Building sets Computer games Set up enticing environments and stories Pretend to pour and feed the baby doll Encourage the child to include the doll in multistep routines like going to bed Pretend to fill and empty a dump truck Include in bath time Making things “go” Demonstrate making things “go” Read and describe, turn pages Gross motor play, rough housing Build a fort, play house Seek and find objects, spatial concepts of over, under, around, and through Manipulate shapes Encourage digging, pouring, finding buried objects Encourage the child to tell the story Encourage and assist as needed Construct real or imaginary things Create scenarios for child or encourage the child to create scripts and then follow her lead Incorporate music and dance into play with instruments and costumes Kickball or “duck, duck, goose” Imaginative games (pirates, ballet dancers, gymnastics) Ride around neighborhood, go on a treasure hunt Encourage peer play and sports Develop scripts as a basis for play Start with large and move to smaller beads Copy design Create illusions Give child sense of success Physical play, endurance Constructive play Use adaptive switches if needed From Linder T: Transdisciplinary play-based intervention, ed 2. Baltimore, 2008, Brooks; Ratliffe KT: Clinical pediatrics physical therapy: a guide for the physical therapy team. St Louis, 1998, CV Mosby, pp. 65-66. Play can and should be a therapy goal for any young child with a motor deficit. Play fosters language and cognition in young children in addition to providing motivation to move. Parents need to be coached to play with their child in a meaningful way. Play encourages self-generated sensorimotor experiences that will support a child’s development in all domains. A developmental hierarchy of play is found in Table 5-5. Play gets more complex with age. Initially, play is sensorimotor in nature, a term Piaget used to describe the first stage of intellectual development. 223 The child explores the sensory and motor aspects of his or her world while establishing a social bond with the caregivers. At the end of the first year, sensorimotor play evolves into functional play. The infant begins to understand the functional use of objects. The child plays functionally with realistic toys; for example, combing her hair or drinking from a cup. This is the beginning of pretend play although some categorize it as functional play with pretense. As the child gets older, objects are used to represent other objects not present, for example, a banana is used as a telephone or a stick becomes a magic wand. Pretend play is one of the most important forms of play, because in order to demonstrate pretend play, the child has to have a mental representation of the object in mind. Table 5-5 Play Development Age Type of Pla’ Purpose/Child Actions 0-6 months | Sensorimotor play: social and exploratory play] Establish attachment with caregivers 6-12 months | Sensorimotor play—functional play Explore the world Learn cause and effect 12-24 months} Functional/relational play Learn functional use of objects and to orient play toward peers 18-24 months} Pretend play emerges Play functionally with realistic toys Pretend one object can symbolically represent another object Pretend play Pretend dolls and animals are real Constructive play Develop scripts as a basis for play Physical play Draw and do puzzles Engage in rough and tumble play, jumping, chasing, swinging, sliding] Games with rules Problem solving, think abstractly Negotiate rules Play with friends Pretend play becomes more and more imaginative during preschool years and can be described as sociodramatic play. Children who demonstrate pretend play are considered socially competent (Howes and Matheson, 1992). Increasing the complexity of play in children with neurologic deficits should be a goal in any physical therapy plan of care. Additionally, two other forms of play are seen during the preschool years—constructive and physical play. Constructive play involves drawing, doing puzzles, and constructing things out of blocks, cardboard boxes, or any other material at hand. Physical play is very important during this time as physical play develops fundamental motor skills that are prerequisites for games and sports. The last stage of play is games with rules. Physical play is to be encouraged to provide a foundation for a lifetime of fitness as well as fun. Linder identified six principles for supporting appropriate complexity of play that can be used with children at all levels (Box 5-4). Box 5-4 Principles to Support Play Complexity 1. Provide opportunities for many kinds of play — Take into consideration cultural differences regarding floor play or messy play. — Example: Locate areas of the home (inside or outside) that would support the child’s play. — Example: Demonstrate how to play with common everyday objects. 2. Increase the play level — The parent or caregiver can demonstrate a higher level of play by modeling. — Plan play dates with a child who plays at a higher level of play, the child will provide the modeling. — Example: Change the child’s activity of putting blocks into a cup to pretending to pour something from the cup or drinking from the cup. The parent could pretend to take a bite of the block as if it were a piece of cake. 3. Add materials — Add a new object once a child is repeating actions in order to expand the child’s routine. — Example: Give a cloth to a child playing with a doll to entice the child to cover the doll with the cloth, or to use the cloth as a burp cloth. 4, Add language — Add sounds, words, and/or rhythms to the play to enrich the context and encourage attention. — Describing what is happening increases the child’s vocabulary. — Example: The child is moving a toy bus across the floor and the parent makes appropriate sounds or asks what sounds the bus would make. Sing the wheels on the bus. 5. Add actions 224 — Add an action once a child repeats an action in order to expand the child’s routine. — Example: The child pretends to put on a hat; expand that action to then pretending to go fora walk in the park or ask what would the child need to put on or take with her if it were raining? 6. Add ideas — Present novel ideas to the child that build on what the child is already thinking. — Example: Suggest making a card for the teacher and providing the child with paper, markers, and/or glitters to combine on her own. — Example: Provide the child with various hats or a dress up box that might trigger scenarios like being a fireman, postman, cowboy, or a chef. (Modified from Linder T: Transdisciplinary Play-Based Intervention, ed 2. Baltimore, 2008 Brooks.) Chapter summary Children with neurologic impairments, regardless of the cause of the deficits, need to move and play. Part of any parent’s role is to foster the child’s movement exploration of the world. To be a good explorer, the child has to come in contact with objects and people of the world. By teaching the family how to assist the child to move and play, the clinician can encourage full participation in life. By supporting areas of the child’s body that the child cannot support, functional movement of other body parts, such as eyes, hands, and feet, can be engaged in object exploration. The adage that if the individual cannot get to the world, the world should be brought to the individual, is true. The greatest challenge for physical therapists and physical therapist assistants who work with children with neurologic deficits may be to determine how to bring the world to a child with limited head or trunk control or limited mobility. Therapists need to foster function, family, fun, friends, and fitness as measures of participation in life (Rosenbaum and Gorter, 2011). There is never just one answer but rather there are many possibilities to the problems presented by these children. The typical developmental sequence has always been a good source of ideas for positioning and handling. Additional ideas can come from the child’s play interests and curiosity and the imagination of the therapist and the family. Review questions 1. What two activities should always be part of any therapeutic intervention? 2. What are the purposes of positioning? 3. What sensory inputs help to develop body and movement awareness? 4. Identify two of the most important handling tips. 5. How can play complexity be expanded in therapy? 6. Give three reasons to use adaptive equipment. 7. What are the two most functional postures (positions to move from)? 8. What are the disadvantages of using a quadruped position? 9. Why is side sitting a difficult posture? 10. Why is standing such an important activity? Case studies Reviewing Positioning and Handling Care: Josh, Angie, and Kelly For each of the case studies listed here, identify appropriate ways to pick up, carry, feed, or dress the child. Identify any adaptive equipment that could assist in positioning the child for a functional activity. Give an example of how the parent could play with the child. Case 1 Josh is a 6-month-old with little head control who has been diagnosed as a floppy infant. He does not like the prone position. However, when he is prone, he is able to lift his head and turn it from 225 side to side, but he does not bear weight on his elbows. He eats slowly and well but tires easily. Case 2 Angie is a 9-month-old who exhibits good head control and fair trunk control. She has low tone in her trunk and increased tone in her lower extremities (hamstrings, adductors, and gastrocnemius- soleus complex). When her mother picks her up under the arms, Angie crosses her legs and points her toes. When Angie is in her walker, she pushes herself backward. Her mother reports that Angie slides out of her high chair, which makes it difficult for her to finger feed. Case 3 Kelly is a 3-year-old who has difficulty in maintaining any posture against gravity. Head control and trunk control are inconsistent. She can bear weight on her arms if they are placed for her. She can sit on the floor for a short time when she is placed in tailor sitting. When startled, she throws her arms up in the air (Moro reflex) and falls. She wants to help get herself dressed and undressed. Possible suggestions Case 1 Picking up/Carrying: Use maximum head and trunk support, facilitate rolling to the side, and gather him in a flexed position before picking him up. You could carry him prone to increase tolerance for the position and for the movement experience. Feeding: Use an infant seat. Positioning for Functional Activity: Position him prone over a half-roll with toys at eye level. Positioning for Play: Position him on your tummy while you are lying on the floor, make eye contact and noises to encourage head lifting and pushing up on arms. Engage child in vocal play and mouth games (tickling and making bubbles). The caregiver should be face to face on the floor while encouraging and assisting in pushing up in prone as seen in Figure 5-20. FIGURE 5-20 Caregiver encouraging the infant to push up from prone. Case 2 Picking up/Carrying: From sitting, pick her up, ensuring lower extremity flexion and separation if possible. Carry her astride your hip, with her trunk and arms rotated away from you. Feeding: Attach a seatbelt to the high chair. Support her feet so the knees are higher than the hips. Towel rolls can be used to keep the knees abducted. A small towel roll can be used at the low back to encourage a neutral pelvis. Mobility: Consult with the supervising therapist about the use of a walker for this child. Positioning for Functional Activity: Sit her astride a bolster to play at a table. A bolster chair with a tray can also be used. A bolster or the caregiver’s leg can be used to work on undressing and dressing. Reaching down for clothing and returning to upright sitting can work the trunk muscles. Positioning for Play: Sit her on a bench and put objects such as blocks on a low table in front of her. Practice coming to stand with her feet sufficiently under her to keep her heels on the ground. Help her come to stand and play with the toys or objects on the low table. She could also sit astride 226 a bolster and come to stand to play. Getting on and off the bolster would be fun, as well as picking the objects to reach for. Consider partially hiding objects under a cloth to have the child retrieve a hidden object. Introduce toys that can be pushed or pulled while in a standing position. Pretend to have tea parties with the use of plastic plates and cups. Case 3 Picking up/Carrying: Assist her to move into sitting using upper extremity weight bearing for stability. Pick her up in a flexed posture and place her in a corner seat on casters to transport or in a stroller. Dressing: Position her in ring sitting on the floor, with the caregiver ring sitting around her for stability. Stabilize one of her upper extremities and guide her free arm to assist with dressing. Another option could include sitting on a low dressing bench with her back against the wall and being manually guided to assist with dressing. Positioning for Functional Activity: Use a corner floor sitter to give a maximum base of support. She could sit in a chair with arms, her feet supported, the table at chest height, and one arm holding on to the edge of the table while the other arm manipulates toys or objects. Positioning for Play: Seated in a chair with arms and feet on the floor, she can push a large, weighted ball to the parent. Play in tall kneeling with one arm extended for support on a bench while placing puzzle pieces. Engage her in a story related to the theme of the puzzle. Ask her to dramatize an event in her life. Incorporate songs and books into activities requiring static holding and controlling movement transitions. 227 References Aubert EK. Adaptive equipment and environmental aids for children with disabilities. In: Tecklin JS, ed. Pediatric physical therapy. ed 4 Philadelphia: JB Lippincott; 2008:389-414. Ayres AJ. Sensory integration and learning disorders. Los Angeles: Western Psychological Services; 1972. Charman T, Baron-Cohen S. Brief report: prompted pretend play in autism. J] Autism Dev Disord. 1997;27:325-332. Chung J, Evans J, Lee C, et al. Effectiveness of adaptive seating on sitting posture and postural control in children with cerebral palsy. Pediatr Phys Ther. 2008;20:303-317. de Sousa AM, de Franca Barros J, de Sousa Neto BM. Postural control in children with typical development and children with profound hearing loss. Int J Gen Med. 2012;5:433-439. Dilger NJ, Ling W. The influence of inclined wedge sitting on infantile postural kyphosis. Dev Med Child Neurol. 1986;28:23. 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Lexington, Kentucky: University of Kentucky; 2014. Miedaner JA. The effects of sitting positions on trunk extension for children with motor impairment. Pediatr Phys Ther. 1990;2:11-14. O’Shea RK, Bonfiglio BS. Assistive technology. In: Campbell SK, Palisano RJ, Orlin MN, eds. Physical therapy for children. ed 4 St Louis: Saunders; 2012. Paleg G, Smith B, Glickman L. Systematic review and evidence-based clinical recommendations for dosing of pediatric-supported standing programs. Pediatr Phys Ther. 2013;25:232-247. Paleg G, Smith B, Glickman L: Evidence-based clinical recommendations for dosing of pediatric supported standing programs. Presented at the APTA Combined Sections Meeting, Feb 4, 2014, Las Vegas, NV. Pfeifer LI, Pacciulio AM, dos Santos CA, dos Santos JL, Stagnitti KE. Pretend play of children with cerebral palsy. Am J Occup Ther. 2011;31:390-402. Ratliffe KT. Clinical pediatric physical therapy. St Louis: CV Mosby; 1998. Rigby PJ, Ryan SE, Campbell KA. Effect of adaptive seating devices on the activity performance of children with cerebral palsy. Arch Phys Med Rehabil. 2009;90:1389-1395. 228 Rosenbaum P, Gorter JW. The ‘F-words’ in childhood disability: I swear this is how we should think!. Child Care Health Dev. 2011;38(4):457—-463. Rutherford MD, Young GS, Hepburn S, Rogers SJ. A longitudinalstudy of pretend play in autism. J Autism Dev Disord. 2007;1024—1039. Ryan SE, Campbell KA, Rigby PJ, et al. The impact of adaptive seating devices on the lives of young children with cerebral palsy and their families. Arch Phys Med Rehabil. 2009;90:27-33. Sochaniwskyz A, Koheil R, Bablich K, et al. Dynamic monitoring of sitting posture for children with spastic cerebral palsy. Clin Biomech. 1991;6:161-167. Tassone JC, Duey-Holtz A. Spine concerns in the Special Olympian with Down syndrome. Sports Med Arthrosc. 2008;16(1):55-60. Wilson JM. Selection and use of adaptive equipment. In: Connolly BH, Montgomery PC, eds. Therapeutic exercise in developmental disabilities. ed 2 Thorofare, NJ: Slack; 2001:167-182. World Health Organization. Motor development study: windows of achievement for six gross motor milestones. Acta Paediatr Suppl. 2006;450:86-95. 229 CHAPTER 6 230 Cerebral Palsy Objectives After reading this chapter, the student will be able to: 1. Describe the incidence, etiology, and classification of cerebral palsy (CP). 2. Describe the clinical manifestations and associated deficits seen in children with CP throughout the life span. 3. Discuss the physical therapy management of children with CP throughout the life span. 4, Discuss the medical and surgical management of children with CP. 5. Describe the role of the physical therapist assistant in the treatment of children with CP. 6. Discuss the importance of activity and participation throughout the life span of a child with CP. 231 Introduction Cerebral palsy (CP) is a group of disorders of posture and movement that occur secondary to damage to the developing fetal or infant brain. The damage is static and may be called a static encephalopathy because it represents a problem with brain structure or function. Once an area of the brain is damaged, the damage does not spread to other areas of the brain, as occurs in a progressive neurologic disorder, such as brain tumor or spinal muscle atrophy. However, because the brain is connected to many different areas of the nervous system, the lack of function of the originally damaged areas may interfere with the ability of these other areas to function properly. Despite the static nature of the brain damage in CP, the clinical manifestations of the disorder may appear to change as the child grows older. Although movement demands increase with age, the child’s motor abilities may not be able to change quickly enough to meet these demands. In addition to the motor deficits, impairments in communication, cognition, sensation, perception, and behavior may be evident. CP is characterized by decreased function, activity limitations, delayed motor development, and impaired muscle tone and movement patterns. How the damage to the central nervous system manifests depends on the developmental age of the child at the time of the brain injury and on the severity and extent of that injury. In CP, the brain is damaged early in the developmental process, and this injury results in disruption of voluntary movement. When damage occurs before birth or during the birth process, it is considered congenital cerebral palsy. Up to 80% of the cases of CP are due to prenatal factors (Longo and Hankins, 2009). The earlier in prenatal development that a system of the body is damaged, the more likely it is that the damage will be severe. The infant’s nervous system is extremely vulnerable during the first trimester of intrauterine development. Brain damage early in gestation is more likely to produce moderate to severe motor involvement of the entire body (quadriplegia), whereas damage later in gestation may result in primarily lower extremity motor involvement (diplegia). If the brain is damaged after birth, the CP is considered to be acquired. Acquired cases of CP account for approximately 20% of the cases (Longo and Hankins, 2009). 232 Incidence The reported incidence of CP in the general population is about 2.1 cases per 1000 live births (Oskoui et al., 2013). The prevalence of CP in the United States, or the number of individuals within a population who have the disorder, has remained relatively the same since 1996 and is reported to range from 3.1 to 3.6 per 1000 children (Christensen et al., 2014). In fact, with increased survival rates in extremely low birth weight and very preterm infants, there has been an increased prevalence of cerebral palsy (Vincer et al., 2006; Wilson-Costello et al., 2005). Smaller preterm infants are more likely to demonstrate moderately severe CP, because the risk of CP is greater with increasing prematurity and lower birth weights (Hintz et al., 2011). 233 Etiology CP can have multiple causes, some of which can be linked to a specific time period. Not all causes of CP are well understood. Typical causes of CP and the relationship of these causes with prenatal, perinatal, or postnatal occurrences are listed in Table 6-1. Any condition that produces anoxia, hemorrhage, or damage to the brain can result in cerebral palsy, but it is not usually one event but many that cause the end result. Vulnerability to cerebral palsy changes relative to gestational age and the subtype of cerebral palsy (Nelson, 2008). Prematurity and intrauterine growth restriction are consistently identified as risk factors for cerebral palsy. Table 6-1 Risk Factors Associated with Cerebral Palsy Prenatal Factors Perinatal Factors Postnatal Factors Maternal infec tions Prematurity Neonatal infection «Rubella Obstetric complications Intraventricular hemorrhage « Herpessimplex Birth trauma * Toxoplasmosis « Twins or multiple births —oos Low birth weight Placental abnormalities ee a A a ST Modified from Glanzman A: Cerebral palsy. In Goodman C, Fuller KS, editors: Pathology: implications for the physical therapist, ed 3. Philadelphia, 2009, WB Saunders, p. 1518. Prenatal Causes When the cause of CP is known, it is most often related to problems experienced during intrauterine development. A fetus exposed to maternal infections, such as rubella, herpes simplex, cytomegalovirus, or toxoplasmosis, early in gestation can incur damage to the motor centers of the fetus’s brain. If the placenta, which provides nutrition and oxygen from the mother, does not remain attached to the uterine wall throughout the pregnancy, the fetus can be deprived of oxygen and other vital nutrients. The placenta can become inflamed or develop thrombi, either of which can impair fetal growth. The reader is referred to Nelson (2008) for a review of causative factors in cerebral palsy. Forty-four percent of children with spastic CP were found to have growth disturbances at birth (Blair and Stanley, 1992). A recent study associated CP with both high and low birth length and head circumference as well as with low birth weight and ponderal index (Dahlseng et al., 2014). The ponderal index is the ratio of height to the cube root of weight; it is an indicator of body mass or chubbiness in infants. Rh factor is found in the red blood cells of 85% of the population. When blood is typed for transfusion or crossmatching, both ABO classification and Rh status are determined. Rh incompatibility occurs when a mother who is Rh-negative delivers a baby who is Rh-positive. The mother becomes sensitive to the baby’s blood and begins to make antibodies if she is not given the drug RhoGAM (Rh immune globulin). The development of maternal antibodies predisposes subsequent Rh-positive babies to kernicterus, a syndrome characterized by CP, high-frequency 234 hearing loss, visual problems, and discoloration of the teeth. When the antibody injection of RhoGAM is given after the mother’s first delivery, the development of kernicterus in subsequent infants can be prevented. Additional maternal problems that can place an infant at risk for neurologic injury include diabetes and toxemia during pregnancy. In diabetes, the mother’s metabolic deficits can cause stunted growth of the fetus and delayed tissue maturation. Toxemia of pregnancy causes the mother’s blood pressure to become so high that the baby is in danger of not receiving sufficient blood flow and, therefore, oxygen. Maldevelopment of the brain and other organ systems is commonly seen in children with CP (Himmelmann and Uvebrant, 2011). Genetic disorders and exposure to teratogens can produce brain malformations. A teratogen is any agent or condition that causes a defect in the fetus; these include radiation, drugs, infections, and chronic illness. Antibiotic use and genitourinary infections have been associated with an increased risk of CP (Miller et al., 2013). The greater the exposure to a teratogen, the more significant the malformation. Central nervous system malformations can contribute to brain hemorrhages and anoxic lesions (Horstmann and Bleck, 2007). Perinatal Causes An infant may experience asphyxiation resulting from anoxia (a lack of oxygen) during labor and delivery. Prolonged or difficult labor because of a breech presentation (bottom first) or the presence of a prolapsed umbilical cord also contributes to asphyxia. The brain may be compressed, or blood vessels in the brain may rupture during the birth process. Although asphyxia has generally been accepted as a significant cause of CP, only a small percentage of cases of CP are due to asphyxia around the time of birth (Nelson, 2008). Fortunately, these conditions are not common. Perinatal ischemic stroke is now recognized as a major cause of cerebral palsy with the advent of imaging. Hemiplegic cerebral palsy is the most common type in term-born infants. Stroke can occur before birth as well as around the time of birth. Risk factors can be related to disorders of the mother, infant, and placenta. Inflammation and infection can trigger thrombosis, which can lead to cerebral infarct. In very preterm infants, there is a risk of developing periventricular leukomalacia (PVL), a necrosis of the white matter in the arterial watershed areas around the ventricles. The fibers of the corticospinal tract to the lower extremities are particularly vulnerable. Decreased blood flow to this area (Figure 6-1) may result in spastic diplegic cerebral palsy. The incidence of PVL is inversely related to gestational age. Preterm infants between 23 and 32 weeks of gestation are at particular risk for this problem due to autoregulation of blood flow of the central nervous system (CNS) (Glanzman, 2009). 235 Leg Trunk Arm Medulla (: S > ' SS. Pyramid FIGURE 6-1 Schematic diagram of corticospinal tract fibers from the motor cortex through the periventricular region into the pyramid of the medulla. The fibers from the lower extremities are most vulnerable to periventricular leukomalacia, which may result in spastic diplegic cerebral palsy. (Modified from Volpe JJ: Hypoxic ischemic encephalopathy: Neuropathology and pathogenesis. In Vope JJ: Neurology of the neonate, Philadelphia, 1995, WB Saunders.) The two biggest risk factors for CP continue to be prematurity and low birth weight. One-fourth of children with cerebral palsy were born prematurely and weighed less than 1500 g (3.3 lbs), while about half of children with cerebral palsy were born premature and weighed less than 2500 g (5.5 lbs). A gestational age less than 37 weeks and small size for gestational age are compounding risk factors for neurologic deficits. However, a birth weight of less than 1500 g, regardless of gestational age, is also a strong risk factor for CP. Thus, any full-term infant weighing less than 1500 g may be at risk for CP. Although CP is more likely to be associated with premature birth, 25% to 40% of cases have no known cause (Russman and Gage, 1989). Neuroimaging is very helpful as 70% to 90% of children with CP will demonstrate significant diagnostic findings (Accardo et al., 2004; Ancel et al., 2006). Postnatal Causes An infant or toddler may acquire brain damage secondary to cerebral hemorrhage, trauma, infection, or anoxia. These conditions can be related to motor vehicle accidents, child abuse in the form of shaken baby syndrome, near-drowning, or lead exposure. Meningitis and encephalitis (inflammatory disorders of the brain) account for 60% of cases of acquired CP (Horstmann and Bleck, 2007). 236 237 Classification The designation “cerebral palsy” does not convey much specific information about the type or severity of movement dysfunction a child exhibits. CP can be classified at least three different ways: (1) by distribution of involvement; (2) by type of abnormal muscle tone and movement; and (3) by severity which is best described according to the Gross Motor Function Classification System (GMFCS) (Palisano et al., 2008) rather than using the terms mild, moderate, or severe. Distribution of Involvement The term plegia is used along with a prefix to designate whether four limbs, two limbs, one limb, or half the body is affected by paralysis or weakness. Children with quadriplegic CP have involvement of the entire body, with the upper extremities usually more severely affected than the lower extremities (Figure 6-2, A). These children have difficulty in developing head and trunk control, and they may or may not be able to ambulate. If they do learn to walk, it may not be until middle childhood. Children with quadriplegia and diplegia have bilateral brain damage. Children with diplegia have primarily lower extremity involvement, although the trunk is almost always affected to some degree (Figure 6-2, B). Some definitions of diplegia state that all four limbs are involved, with the lower extremities more severely involved than the upper ones. Diplegia is often related to premature birth, especially if the child is born at around 32 weeks of gestation or 2 months premature. For this reason, spastic diplegia has been labeled the CP of prematurity. A SPASTIC QUADRIPLEGIA B SPASTIC DIPLEGIA C RIGHT SPASTIC HEMIPLEGIA 1 Dominant extension Dominant flexio 2c on FIGURE 6-2 A-C, Distribution of involvement in cerebral palsy. Children with hemiplegic CP have one side of the body involved, as is seen in adults after a stroke (Figure 6-2, C). Children with hemiplegia have incurred unilateral brain damage. Although these designations seem to focus on the number of limbs or the side of the body involved, the limbs are connected to the trunk. The trunk is always affected to some degree when a child has CP. The trunk is primarily affected by abnormal tone in hemiplegia and quadriplegia, or it is secondarily affected, as in diplegia, when the trunk compensates for lack of controlled movement in the involved lower limbs. Abnormal Muscle Tone and Movement CP is routinely classified by the type and severity of abnormal muscle tone exhibited by the child. Tone abnormalities run the gamut from almost no tone to high tone. Children with the atonic type of CP present as floppy infants (Figure 6-3). In reality, the postural tone is hypotonic or below normal. Uncertainty exists regarding the ultimate impairment of tone when an infant presents with hypotonia because tone can change over time as the infant attempts to move against gravity. The tone may remain low, may increase to normal, may increase beyond normal to hypertonia, or may 238 fluctuate from high to low to normal. Continual low tone in an infant impedes the development of head and trunk control, and it interferes with the development of mature breathing patterns. Tonal fluctuations are characteristically seen in the child with a dyskinetic or athetoid type of CP. Although abnormal tone is easily recognized, the relationship between abnormal tone and abnormalities in movement is less than clear. FIGURE 6-3 Hypotonic infant. The abnormal tone manifested in children with CP may be the nervous system’s response to the initial brain damage, rather than a direct result of the damage. The nervous system may be trying to compensate for a lack of feedback from the involved parts of the body. The distribution of abnormal muscle tone may change when the child’s body position changes relative to gravity. A child whose posture is characterized by an extended trunk and limbs when supine may be totally flexed (head and trunk) when sitting because the child’s relationship with gravity has changed (Figure 6-4). Tonal differences may be apparent even within different parts of the body. A child with spastic diplegia may exhibit some hypertonic muscles in the lower extremities and may display hypotonic trunk muscles. The pattern of tone may be consistent in all body positions, or it may change with each new relationship with gravity. The degree or amount of abnormal tone is judged relative to the degree of resistance encountered with passive movement. Rudimentary assessments can be made based on the ability of the child to initiate movement against gravity. In general, the greater the resistance to passive movement, the greater the difficulty is seen in the child’s attempts to move. 299 B FIGURE 6-4 A, Child in extension in the supine position. B, The same child demonstrating a flexed sitting posture. Spasticity By far the most common type of abnormal tone seen in children with CP is spasticity. Spasticity is a velocity-dependent increase in muscle tone. Hypertonus is increased resistance to passive motion that may not be affected by the speed of movement. Clinically, these two terms are often used interchangeably. Classification and differentiation of the amount of tone above normal are subjective and are represented by a continuum from mild to moderate to severe. The mild and moderate designations usually describe a person who has the ability to move actively through at least part of the available range of motion. Severe hypertonus and spasticity indicate extreme difficulty in moving, with an inability to complete the full range of motion. In the latter instance, the child may have difficulty even initiating movement without use of some type of inhibitory technique. Prolonged increased tone predisposes the individual to contractures and deformities because, in most situations, an antagonist muscle cannot adequately oppose the pull of a spastic muscle. Hypertonus tends to be found in antigravity muscles, specifically the flexors in the upper extremity and the flexors and extensors in the lower extremity. The most severely involved muscles in the upper extremity tend to be the scapular retractors and the elbow, forearm, wrist, and finger flexors. The same lower extremity muscles that are involved in children with diplegia are seen in quadriplegia and hemiplegia: hip flexors and adductors; knee flexors, especially medial hamstrings; and ankle plantar flexors. The degree of involvement among these muscles may vary, and additional muscles may also be affected. Trunk musculature may exhibit increased tone as well. Increased trunk tone may impair breath control for speech by hampering the normal excursion of the diaphragm and chest wall during inspiration and expiration. As stated earlier, spasticity may not be present initially at birth, but it can gradually replace low muscle tone as the child attempts to move against gravity. Spasticity in CP is of cerebral origin; that is, it results from damage to the central nervous system by a precipitating event, such as an intraventricular hemorrhage. Spastic paralysis results from a classic upper motor neuron lesion. The 240 muscles affected depend on the type of CP—quadriplegia, diplegia, or hemiplegia. Figure 6-2 depicts typical involvement in these types of spastic CP. Transient Dystonia This condition is a temporary one seen in as many as 60% of all preterm infants who have a low birth weight and even in some term infants. While the characteristics seen during the first year life may be transient, they have been linked to behavior deficits later in life in some studies. The characteristics are troubling to a physical therapist because it is often impossible to distinguish these from clinical signs of early cerebral palsy. The characteristics include: increased tone in neck extensor muscles, hypotonia, irritability, and lethargy during the neonatal period; increased tone in extremity muscles, low tone in the trunk muscles, shoulder retraction, and scapular adduction with a persistent asymmetric tonic neck reflex (ATNR) and persistent + support reflex at age 4 months; and immature postural reactions with minimal trunk rotation, continued trunk hypotonia, and extremity hypertonicity at 6 to 8 months. Rigidity Rigidity is an uncommon type of tone seen in children with CP. It indicates severe damage to deeper areas of the brain, rather than to the cortex. Muscle tone is increased to the point that postures are held rigidly, and movement in any direction is impeded. Dyskinesia Dyskinesia means disordered movement. Athetosis, the most common dyskinetic syndrome, is characterized by disordered movement of the extremities, especially within their respective midranges. Movements in the midrange are especially difficult because of the lack of postural stability on which to superimpose movement. As the limb moves farther away from the body, motor control diminishes. Involuntary movements result from attempts by the child to control posture and movement. These involuntary movements can be observed in the child’s entire extremity, distally in the hands and feet, or proximally in the mouth and face. The child with athetosis must depend on external support to improve movement accuracy and efficiency. Difficulty in feeding and in speech can be expected if the oral muscles are involved. Speech usually develops, but the child may not be easily understood. Athetoid CP is characterized by decreased static and dynamic postural stability. Children with dyskinesia lack the postural stability necessary to allow purposeful movements to be controlled for the completion of functional tasks (Figure 6-5). Muscle tone often fluctuates from low to high to normal to high such that the child has difficulty in maintaining postural alignment in all but the most firmly supported positions and exhibits slow, repetitive involuntary movements. 241 FIGURE 6-5 Standing posture in a child with athetoid cerebral palsy. Ataxia Ataxia is classically defined as a loss of coordination resulting from damage to the cerebellum. Children with ataxic CP exhibit loss of coordination and low postural tone. They usually demonstrate a diplegic distribution, with the trunk and lower extremities most severely affected. This pattern of low tone makes it difficult for the child to maintain midline stability of the head and trunk in any posture. Ataxic movements are jerky and irregular. Children with ataxic CP ultimately achieve upright standing, but to maintain this position, they must stand with a wide base of support as a compensation for a lack of static postural control (Figure 6-6). Postural reactions are slow to develop in all postures, with the most significant balance impairment demonstrated during gait. 242 FIGURE 6-6 Ataxic cerebral palsy. Children with ataxia walk with large lateral displacements of the trunk in an effort to maintain balance. Their gait is often described as “staggering” because of these wide displacements, which are a natural consequence of the lack of stability and poor timing of postural corrections. Together, these impairments may seem to spell imminent disaster for balance, but these children are able, with practice, to adjust to the wide displacements in their center of gravity and to walk without falling. Wide displacements and slow balance reactions are counteracted by the wide base of support. Arm movements are typically used as a compensatory strategy to counteract excessive truncal weight shifts. The biggest challenge for the clinician is to allow the child to ambulate independently using what looks like a precarious gait. Proper safety precautions should always be taken, and some children may need to wear a helmet for personal safety. Assistive devices do not appear to be helpful during ambulation unless they can be adequately weighted, and even then, these devices may be more of a deterrent than a help. 243 Functional classification In keeping with the World Health Organization’s International Classification of Functioning Disability and Health (ICF) the best way to classify a disorder like CP is to look at the impact on function. The GMFCS (Palisano et al., 2008) is the preferred way to classify mobility in children with CP. The Manual Ability Classifications System (MACS) (Eliasson et al., 2006) is the preferred way to classify how children with CP use their hands when engaged in activities of daily living. There is also the Communication Function Classification System (CFCS) (Hidecker et al., 2011) for children with CP. Interprofessional communication will be enhanced by utilizing these tools which provide standardized terminology and stratification of levels of function. Use of the classification systems should also enhance communication among parents and professionals when discussing a child’s level of function and long-term outcomes. Use of all three classification systems can provide a functional profile of the child (Effgen et al., 2014). See Table 6-2 for a general description of the five levels of each of the classification systems. Only the GMFCS will be discussed in more detail here. Table 6-2 Classification Systems for Cerebral Palsy Mobilit Gross Motor Classification System (GMFCS) Level I: Walks without limitations Level II: Walks with limitations Level III: Walks using a hand-held mobility device Level IV: Self-mobility with limitations, may use power mobilit Level V: Transported in a manual wheelchair Hand use Manual Ability Classification System (MACS, Level I: Handles objects easily and successfully Level II: Handles most objects but with somewhat reduced quality or speed of achievement Level III: Handles objects with difficulty, needs help to prepare or modify activities Level IV: Handles a limited selection of easily managed objects in adapted situations Level V: Does not handle objects and has severely limited ability to perform simple actions Communication| Communication Function Classification System (CFCS: Level I: Effective sender and receiver with unfamiliar and familiar partners Level II: Effective but slower-paced sender or receiver with unfamiliar and familiar partners Level Ill: Effective sender and receiver with familiar partners Level IV: Sometimes effective sender or receiver with familiar partners Level V: Seldom effective sender and receiver even with familiar partners Sources: Data from Eliasson et al., 2006; Hidecker et al., 2011; Palisano et al., 2008. The GMFCS (Palisano et al., 2008) is a five-level scale that determines a motor level for a child with a motor disability. Level I is walks without limitations; Level II is walks with limitations; Level III is walks using a hand-held mobility device; Level IV is limited self-mobility, may use power mobility; and Level V represents the most serious limitation, being transported in a manual wheelchair. More detailed descriptions of these levels, based on age bands, are used for children before their 2nd birthday, between the 2nd and 4th birthdays, between the 4th and 6th birthdays, between 6th and 12th birthdays, and between the 12th and 18th birthdays. The GMFCS is based on usual performance, what the child does rather than what she is known to be able to do at her best, which is capability. The older age bands reflect the potential impact of the environment on function and the personal preference of the child/youth in regard to mobility. A summary of the expectations for the older age bands can be found in Figure 6-7. A description of all levels can be found on the CanChild website: www.canchild.ca. 244 GMFCS E & R descriptors and illustrations for children between their 6th and 12th birthdays: GMFCS Level! Children walk at home, school, outdoors and in the community. They can climb stairs without the use of a railing. Children perform gross motor skills such as running and jumping, but speed, balance and coordination are limited. Children walk in most settings and climb stairs holding on to a railing. They may experience difficulty walking long distances and balancing on uneven terrain, inclines, in crowded areas or confined spaces. Children may walk with physical assistance, a hand-held mobility device, or use wheeled mobility over long distances. Children have only minimal ability to perform gross motor skills such as running and jumping. GMFCS Level Ill Children walk using a hand-held mobility device in most settings. They may climb stairs holding on to a railing with supervision or assistance. Children use wheeled mobility when traveling long distances and may self-propel for shorter distances. GMFCS Level IV Children use methods of mobility that require physical assistance of powered mobility in most settings. They may walk for short distances at home with physical assistance or use powered mobility or a body support walker when positioned. At school, outdoors and in the community children are transported in a manual wheelchair or use powered mobility. Children are transported in a manual wheelchair in all settings. Children are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements. 245 GMFCS E & R descriptors and illustrations for children between their 12th and 18th birthdays GMFCS Level! Youth walk at home, school, outdoors and in community. Youth are able to climb stairs without physical assistance or a railing. They perform gross motor skills such as running and jumping but speed, balance and coordination are limited. GMFCS Level Il Youth walk in most settings but environmental factors and personal choice influence mobility choices, At school or work they may require a hand-held mobility device for safety and climb stairs holding on to a railing. Outdoors and in the community youth may use wheeled mobility when traveling long distances. GMFCS Level Ill Youth are capable of walking using a hand-held mobility device. Youth may climb stairs holding on to a railing with supervision or assistance. At school they may self-propel a manual wheelchair of use powered mobility. Outdoors and in the community youth are transported in a wheelchair or use powered mobility. Youth use wheeled mobility in most settings. Physical assistance of one to two people is required for transfers, Indoors, youth may walk short distances with physical assistance, use wheeled mobility or a body support walker when positioned. They may operate a powered Chair, otherwise are transported in a manual wheelchair. Youth are transported in a manual wheelchair in all settings. Youth are limited in their ability to maintain antigravity head and trunk postures and control leg and arm movements. Self-mobility is severely limited, even with the use of assistive technology. FIGURE 6-7 Gross Motor Function Classification System. 246 Diagnosis Many children are not formally diagnosed as having CP until after 6 months of age. In children with a severely damaged nervous system, as in the case of quadriplegic involvement, early diagnosis may not be difficult. However, children with hemiplegia or diplegia with mild involvement may not be identified as having a problem until they have difficulty in pulling to stand at around 9 months of age. Lack of early detection may deprive these children of beneficial early intervention. Hypotonia in infancy may be a precursor to athetosis and may be observed as the child works to move against gravity (Senesac, 2013). Many years of research have been devoted to developing sensitive assessment tools that will allow pediatricians and pediatric physical therapists to identify infants with CP as early as 4 to 6 months of age. Observation of a child’s movements in certain antigravity postures may be more revealing than testing reflexes or assessing developmental milestones (Pathways Awareness Foundation, 1992). 247 Pathophysiology Spastic diplegia, quadriplegia, and hemiplegia can be caused by varying degrees of intraventricular hemorrhage (Table 6-3). Depending on which fibers of the corticospinal tract are involved and whether the damage is bilateral or unilateral, the resultant neurologic deficit manifests as quadriplegia, diplegia, or hemiplegia. Spastic quadriplegia is most often associated with Grade III intraventricular hemorrhage in premature infants. What used to be classified as a Grade IV hemorrhage is now called periventricular hemorrhagic infarction (PHI). Preterm infants with low birth weights and PHI are at a substantially higher risk for neurologic problems. Premature infants born at 32 weeks of gestation are especially vulnerable to white matter damage around the ventricles from hypoxia and ischemia. PVL is the most common cause of spastic diplegia, because the fibers of the corticospinal tract that go to the lower extremities are most exposed. Spastic hemiplegia, the most common type of CP, can result from unilateral brain damage secondary to PHI in the preterm infant. In the term infant, a more likely cause is cerebral malformations, such as an arteriovenous malformation, intracerebral hemorrhage, or cerebral infarct (Fenichel, 2009). Athetosis involves damage to the basal ganglia and has been associated with erythroblastosis fetalis, anoxia, and respiratory distress. Erythroblastosis, a destruction of red blood cells, occurs in the newborn when Rh incompatibility of maternal-fetal blood groups exists. Ataxia is related to damage to the cerebellum. Table 6-3 Pathophysiology of Cerebral Palsy Cause Deficit S S Periventricular leukomalacia Intrauterine disease pastic quadriplegia Hypoxic-ischemic injury Spastic quadriplegia Periventricular hemorrhage (preterm infants: Spastic hemiplegia Cerebral malformations, cerebral infarcts, intracerebral hemorrhage (term infants) Spastic hemiplegia Selective neuronal necrosis of the cerebellum Ataxia Status marmoratus (hypermyelination in basal ganglia) Athetosis ypastic diplegia From Fenichel GM: Clinical pediatric neurology: a signs and symptoms approach, ed 6. Philadelphia, 2009, Saunders; Goodman CG, Fuller KS: Pathology: implications for the physical therapist, ed 3. Philadelphia, 2009, Saunders; Umphred DA, editor: Neurological rehabilitation, ed 6. St Louis, 2013, Mosby. 248 Associated deficits The deficits associated with CP are presented in the order in which they may become apparent in the infant with CP (Box 6-1). Early signs of motor dysfunction in an infant often present as problems with feeding and breathing. Box 6-1 Deficits Associated with Cerebral Palsy Feeding and speech impairments Breathing inefficiency Visual impairments Hearing impairments Intellectual disability Seizures Feeding and Speech Impairments Poor suck-swallow reflexes and uncoordinated sucking and breathing may be evidence of CNS dysfunction in a newborn. Persistence of infantile oral reflexes, such as rooting or suck-swallow, or exaggerations of normally occurring reflexes, such as a tonic bite or tongue thrust, can indicate abnormal oral motor development. A hyperactive or hypoactive response to touch around and in the mouth is also possible. Hypersensitivity may be seen in the child with spastic hemiplegia or quadriplegia, whereas hyposensitivity may be evident in the child with low-tone CP. Feeding is considered a precursor to speech, so the child who has feeding problems may well have difficulty in producing intelligible sounds. Lip closure around the nipple is needed to prevent loss of liquids during sucking. Lip closure is also needed in speech to produce “p,” “b,” and “m” sounds. If the infant cannot bring the lips together because of tonal problems, feeding and sound production will be hindered. The tongue moves in various ways within the mouth during sucking and swallowing and later in chewing; these patterns change with oral motor development. These changes in tongue movements are crucial not only for taking in food and swallowing, but also for the production of various sounds requiring specific tongue placement within the oral cavity. Breathing Inefficiency Breathing inefficiency may compound feeding and speech problems. Typically developing infants are belly breathers and only over time do they develop the ability to use the rib cage effectively to increase the volume of inspired air. Gravity promotes developmental changes in the configuration of the rib cage that place the diaphragm in a more advantageous position for efficient inspiration. This developmental change is hampered in children who are delayed in experiencing being in an upright posture because of lack of attainment of age-appropriate motor abilities, such as head and trunk control. Lack of development in the upright posture can result in structural deformities of the ribs, such as rib flaring, and functional limitations, such as poor breath control and shorter breath length that is inadequate for sound production. Abnormally increased tone in the trunk musculature may allow only short bursts of air to be expelled and produce staccato speech. Low muscle tone can predispose children to rib flaring because of lack of abdominal muscle development. Intellectual disability, hearing impairment, or central language processing impairment may further impede the ability of the child with CP to develop effective oral communication skills. Intellectual Disability Children with CP have many other problems associated with damage to the nervous system that also relate to and affect normal development. The most common of these are vision and hearing impairments, feeding and speech difficulties, seizures, and intellectual disability. The classification of intellectual disability is given in Chapter 8, and thus not found in this chapter. Although no 249 direct correlation exists between the severity of motor involvement and the degree of intellectual disability, the percentage of children with CP with intellectual disability has been estimated at between 25% and 45% (Fenichel, 2009; Yin Foo et al., 2013). Intelligence tests require a verbal or motor response, either of which may be impaired in these children. Mean cognitive scores in children with cerebral palsy are related to gestational age and birth weight (Accardo, 2008). The risk for intellectual disability increases 1.4-fold when an infant is born between 32 and 36 weeks and 7- fold if born before 32 weeks of gestation. It is further suggested that children of normal intelligence who have CP may be at risk of having learning disabilities or other cognitive or neurobehavioral impairments. In general, children with spastic hemiplegia or diplegia, athetosis, or ataxia are more likely to have normal or higher than normal intelligence, whereas children with more severe types of CP, such as spastic quadriplegia, rigidity, or a mixed type, are more likely to exhibit intellectual disability (Hoon and Tolley, 2013). However, as with any generalizations, exceptions always exist. Yin Foo et al. (2013) proposed using a clinical reasoning tool to select appropriate IQ assessments for children with CP. It is extremely important to not make judgments about a child’s intellectual status based solely on the severity of the motor involvement. Seizures The site of brain damage in CP may become the focal point of abnormal electrical activity, which can cause seizures. Epilepsy is a disease characterized by recurrent seizures. Approximately 40% of children with CP experience seizures that must be managed by medication (Nordmark et al., 2001). A smaller percentage may have a single seizure episode related to high fever or increased intracranial pressure. Children with CP or intellectual disability are more likely to develop seizures than are typically developing children. Seizures are classified as generalized, focal, or unclassified and are listed in Table 6-4. Generalized seizures are named for the type of motor activity the person exhibits. Focal seizures used to be called partial seizures, which were simple or complex, depending on whether the child experiences a loss of consciousness. Focal seizures can have either sensory or motor manifestations or both. Unclassified seizures do not fit in any other category. Epilepsy syndromes have common signs and symptoms, EEG features, characteristics, and the same genetic origin or pathogenesis. Table 6-4 Classification of Seizures International Classification of 4 Manifestation of Seizures Seizures Generalized seizures Seizures that are generalized to the entire body; always involve a loss of consciousness Tonic-clonic seizure Begin with a tonic contraction (stiffening) of the body, then change to clonic movements (jerking) of the bod Tonic seizure Stiffening of the entire bod Clonic seizure Myoclonic jerks start and stop abruptly Atonic seizure Sudden lack of muscle tone Absence seizure Nonconvulsive seizure with a loss of consciousness; blinking, staring, or minor movements lasting a few seconds Myoclonic seizure Irregular, involuntary contraction of a muscle or group of muscles Focal seizures Seizures not generalized to the entire body; a variety of sensory or motor symptoms may accompany this type of seizure; the distinction between partial seizures has been eliminated (Berg et al., 2010’ Syndromes See Berg et al., 2010 Unclassified seizure Seizures that do not fit into the above categories Adapted from Ratliffe KT: Clinical pediatric physical therapy, St Louis, 1998, Mosby, p. 410; and Berg et al., 2010. Children with CP and mild intellectual disability tend to exhibit focal seizures as do children in all spastic CP types (Carlsson et al., 2003). Children with CP caused by CNS infections, CNS malformations, and gray-matter damage are more likely to demonstrate seizures than children whose CP is caused by white-matter damage or an unknown event (Carlsson et al., 2003). The age of onset of the seizure activity appears to be related to the type of cerebral palsy. Children with quadriplegia demonstrate an earlier onset than those with hemiplegia. Early onset of seizures in hemiplegia has significant impact on cognition. Fifty percent of children with hemiplegic CP have epilepsy (Fenichel, 2009). When working with children, the clinician should question parents and caregivers about the children’s history of seizure activity. The physical therapist assistant should always document any seizure activity observed in a child, including time of occurrence, duration, loss of consciousness, motor and sensory manifestations, and status of the child after the seizure. Visual Impairments Vision is extremely important for the development of balance during the first 3 years of life 250 (Shumway-Cook and Woollacott, 2012). Any visual difficulty may exacerbate the inherent neuromotor problems that typically accompany a diagnosis of CP. Eye muscle control can be negatively affected by abnormal tone and can lead to either turning in (esotropia) or turning out (exotropia) of one or both eyes. Strabismus is the general term for an abnormal ocular condition in which the eyes are crossed. In paralytic strabismus, the eye muscles are impaired. Strabismus is present in many children with CP (Batshaw et al., 2013), with the highest incidence in children with quadriplegia and diplegia (Styer-Acevedo, 1999). Nystagmus is most often seen in children with ataxia. In nystagmus, the eyes move back and forth rapidly in a horizontal, vertical, or rotary direction. Normally, nystagmus is produced in response to vestibular stimulation and indicates the close relationship between head movement and vision. The presence of nystagmus may complicate the task of balancing the head or trunk. Some children compensate for nystagmus by tilting their heads into extension, a move that can be mistaken for neck retraction and abnormal extensor tone. The posteriorly tilted head position gives the child the most stable visual input. Although neck retraction is generally to be avoided, if it is a compensation for nystagmus, the extended neck posture may not be avoidable. Visual deficits are common in children with hemiplegic CP (Ashwal et al., 2004). These deficits may include homonymous hemianopia, or loss of vision in half the visual field. Every child with hemiplegia should have a detailed assessment of vision. Children with visual impairments may have more difficulty in developing head and trunk control and in exploring their immediate surroundings. Visual function should be assessed in any infant or child who is exhibiting difficulty in developing head control or in reaching for objects. Clinically, the child may not follow a familiar face or turn to examine a new face. If you suspect that a child has a visual problem, report your suspicions to the supervising physical therapist. Hearing, Speech, and Language Impairments Almost one-third of children with CP have hearing, speech, and language problems. As already mentioned, some speech problems can be secondary to poor motor control of oral muscles or respiratory impairment. Language difficulties in the form of expressive or receptive aphasia can result when the initial damage that caused the CP also affects the brain areas responsible for understanding speech or producing language. For most of the right-handed population, speech centers are located in the dominant left hemisphere. Clinically, the child may not turn toward sound or be able to localize a familiar voice. Hearing loss may be present in any type of CP, but it occurs in a higher percentage of children with quadriplegia. These children should be evaluated by an audiologist to ascertain whether amplification is warranted. 251 Physical therapy examination The physical therapist conducts a thorough examination and evaluation of the child with CP that includes a history, observation, and administration of specific standardized tests of development. Test selection is based on the reason for the evaluation: screening, information gathering, treatment planning, eligibility determination, or outcomes measurement. A discussion of developmental assessment is beyond the scope of this text; refer to Effgen (2013) for information on specific developmental assessment tools. However, the most commonly used measure of gross motor function in children with CP is the Gross Motor Function Measure (GMFM) (Russell et al., 2002). The physical therapist assistant needs to have an understanding of the purpose of the examination and awareness of the tools commonly administered and of the process used within a particular treatment setting. For example, an arena assessment may be used when evaluating a young child or a play-based assessment, while a one-on-one evaluation may be used in the school system. The physical therapist assistant should be familiar with the information reported by the physical therapist in the child’s examination: social and medical history; range of motion; muscle tone, strength, and bulk; reflexes and postural reactions; mobility skills; transfers; activities of daily living (ADLs), recreation, play, and leisure; and adaptive equipment. The assistant needs to be aware of the basis on which the physical therapist makes decisions about the child’s plan of care. The physical therapist’s responsibility is to make sure that the goals of therapy and the strategies to be used to implement the treatment plan are thoroughly understood by the physical therapist assistant. Neuromuscular Impairments, Activity Limitations, and Participation Restrictions The physical therapy examination should identify the neuromuscular impairments and the present or anticipated functional limitations of the child with CP. Many physical impairments, such as too much or too little range of motion or muscle extensibility, are related to the type of tone exhibited, its distribution, and its severity. Impairments in muscle activation and motor control can affect the ability to perform daily activities. Activity limitations such as sitting, standing up, or use of the extremities result from these impairments. Activity limitations lead to restrictions in participation. In the spastic type of CP, the impairments are often related to lack of range, movement, muscle stiffness, and increased muscle tone. Children with athetoid or ataxic CP may have some of the same functional limitations, but their impairments are related to too much mobility and too little stability. The impairments and activity limitations of the child with hypotonic CP are similar to those of children with Down syndrome; therefore, refer to Chapter 8 for a discussion of intervention strategies. The Child with Spastic Cerebral Palsy The child with spasticity often moves slowly and with difficulty. When movement is produced, it occurs in predictable, stereotypical patterns that occur the same way every time with little variability. The child with spasticity can have activity limitations in head and trunk control, performance of movement transitions, ambulation, use of the extremities for balance and reaching, and ADLs (Table 6-5). Table 6-5 Impairments, Activity Limitations, Participation Restrictions, and Focus of Treatment in Children with Spasticity 202 Body Structure/Function Activity Limitation Participation Restriction Focus of Treatment Muscle tone/extensibility | Delayed gross and fine motor skills } Social engagement Educate family about CP Selective motor control Increase parents’ handling skills = Motor recruitment Change positions against gravity = Cocontraction Activate postural muscles Practice movement transitions Optimize sensorimotor experiences Increase play complexity Sit to stand/ walking Strength training Head Control The child with spasticity can have difficulty in developing head control because of increased tone, persistent primitive reflexes, exaggerated tonic reflexes, or absent or impaired sensory input. Because the child often has difficulty in generating enough muscle force to maintain a posture or to move, substitutions and compensatory movements are common. For example, an infant who cannot control the head when held upright or supported in sitting may elevate the shoulders to provide some neck stability. Trunk Control Lack of trunk rotation and a predominance of extensor or flexor tone can impair the child’s ability to roll. Inadequate trunk control prevents independent sitting. In a child with predominantly lower extremity problems, the lack of extensibility at the hips may prevent the attainment of an aligned sitting position. The child compensates by rounding the upper back to allow for sitting (see Figure 6-4, B). Trunk rotation can be absent or impaired secondary to a lack of balanced development of the trunk extensors and flexors. Without this balance, controlled lateral flexion is not possible, nor is rotation. Absent trunk rotation makes transitional movements (moving from one posture to another) extremely difficult. The child with spasticity may discover that it is possible to achieve a sitting position by pushing the body backward over passively flexed and adducted legs, to end up in a W-sitting position (Figure 6-8). This posture should be avoided because its use can impede further development of trunk control and lower extremity dissociation. 253 FIGURE 6-8 W sitting. Influence of Tonic Reflexes Tonic reflexes are often obligatory in children with spastic CP. When a reflex is obligatory, it dominates the child’s posture. Obligatory tonic reflexes produce increased tone and postures that can interfere with adaptive movement. When they occur during the course of typical development, they do not interfere with the infant’s ability to move. The retention of these reflexes and their exaggerated expression appear to impair the acquisition of postural responses such as head and neck righting reactions and use of the extremities for protective extension. The retention of these tonic reflexes occurs because of the lack of normal development of motor control associated with CP. Tonic reflexes consist of the tonic labyrinthine reflex (TLR), the asymmetric tonic neck reflex (ATNR), and the symmetric tonic neck reflex (STNR), all of which are depicted in Figure 6-8. The TLR affects tone relative to the head’s relationship with gravity. When the child is supine, the TLR causes an increase in extensor tone, whereas when the child is prone, it causes an increase in flexor tone (Figure 6-9, A, B). Typically, the reflex is present at birth and then is integrated by 6 months. It is thought to afford some unfolding of the flexed infant to counter the predominance of physiologic flexor tone at birth. If this reflex persists, it can impair the infant's ability to develop antigravity motion (to flex against gravity in supine and to extend against gravity in prone). An exaggerated TLR affects the entire body and can prevent the child from reaching with the arms in the supine position or from pushing with the arms in the prone position to assist in coming to sit. The TLR can affect the child’s posture in sitting because the reflex is stimulated by the head’s relationship with gravity. If the child loses head control posteriorly during sitting, the labyrinths sense the body as being supine, and the extensor tone produced may cause the child to fall backward and to slide out of the chair. Children who slump into flexion when the head is flexed may be demonstrating the influence of a prone TLR. 254 A Supine tonic labyrinthine resex B Prone tonic labyrinthine reflex Asymmetric tonic neck refiex D Symmetric tonic neck reflex FIGURE 6-9 Tonic reflexes. The ATNR causes associated upper extremity extension on the face side and flexion of the upper extremity on the skull side (see Figure 6-8, C). For example, turning the head to the right causes the right arm to extend and the left arm to bend. This reflex is usually apparent only in the upper extremities in a typically developing child; however, in the child with CP, the lower extremities may also be affected by the reflex. The ATNR is typically present from birth to 4 to 6 months. If this reflex persists and is obligatory, the child will be prevented from rolling or bringing the extended arm to her mouth. The asymmetry can affect the trunk and can predispose the child to scoliosis. In extreme cases, the dominant ATNR can produce hip dislocation on the flexed side. The STNR causes the arms and legs to flex or extend, depending on the head position (see Figure 6-9, D). If the child’s head is flexed, the arms flex and the legs extend; if the head is extended, vice versa. This reflex has the potential to assist the typically developing infant in attaining a four-point or hands-and-knees position. However, its persistence prevents reciprocal creeping and allows the child only to “bunny hop” as a means of mobility in the four-point position. When the STNR is obligatory, the arms and legs imitate or contradict the head movement. The child either sits back on the heels or thrusts forward. Maintaining a four-point position is difficult, as are any dissociated movements of the extremities needed for creeping. The exaggeration of tonic reflexes and the way in which they may interfere with functional movement by producing impairments are found in Table 6-6. Table 6-6 Influence of Tonic Reflexes on Functional Movement Tonic Reflex Impairment Functional Movement Limitation TLR in supine] Contractures Rolling from supine to prone Abnormal vestibular input Reaching in supine Limited visual field Coming to sit Sitting TLR in prone | Contractures Rolling from prone to supine Abnormal vestibular input Coming to sit Limited visual field Sitting ATNR Contractures Segmental rolling Hip dislocation Reaching Trunk asymmetry Bringing hand to mouth Scoliosis Sitting STNR Contractures Creeping Lack of upper and lower extremity dissociation] Kneeling Lack of trunk rotation Walking 200 ATNR, Asymmetrical tonic neck reflex; STNR, symmetrical tonic neck reflex; TLR, tonic labyrinthine reflex. Movement Transitions The child with spasticity often lacks the ability to control or to respond appropriately to shifts in the center of gravity that should typically result in righting, equilibrium, or protective reactions. These children are fearful and often do not feel safe because they have such precarious static and dynamic balance. In addition, the child’s awareness of poor postural stability may lead to an expectation of falling based on prior experience. The inability to generate sufficient muscle activity in postural muscles for static balance is further compounded by the difficulty in anticipating postural changes in response to body movement; these features make performance of movement transitions, such as prone to sitting or the reverse, sitting to prone, more difficult. Mobility and Ambulation Impaired lower extremity separation hinders reciprocal leg movements for creeping and walking; therefore, some children learn to move forward across the floor on their hands and knees by using a “bunny hopping” pattern that pulls both legs together. Other ways that the child with spasticity may attempt to move is by “commando crawling,” forcefully pulling the arms under the chest and simultaneously dragging stiff legs along the floor. The additional effort by the arms increases lower extremity muscle tone in extensor muscle groups and may also interfere when the child tries to pull to stand and to cruise around furniture. The child may attain a standing position only on tiptoes and with legs crossed (Figure 6-10). Cruising may not be possible because of a lack of lower extremity separation in a lateral direction. Walking is also limited by an absence of separation in the sagittal plane. Adequate trunk control may be lacking to provide a stable base for the stance leg, and inadequate force production may prevent controlled movement of the swing leg. Because of absent trunk rotation, arm movements are often used to initiate weight shifts in the lower extremities or to substitute for a lack of lower extremity movement. The arms may remain in a high- guard position to reinforce weak trunk muscles by sustaining an extended posture and thus delay the onset of arm swing. 256 FIGURE 6-10 Tiptoe standing. Extremity Usage Reaching in any position may be limited by an inability to bear weight on an extremity or to shift weight onto an extremity and produce the appropriate balance response. Weight bearing on the upper extremities is necessary for propped sitting and for protective extension when other balance responses fail. Lower extremity weight bearing is crucial to independent ambulation. The child with spasticity is at risk of contractures and deformities secondary to muscle and joint stiffness and to muscle imbalances from increased tone. Spasticity may be present only in extremity muscles, whereas the trunk may demonstrate low muscle tone. In an effort to overcome gravity, the child may try to use the abdominal muscles to attain sitting from a supine position. Excessive exertion can increase overall tone and can result in lower extremity extension and possible scissoring (hip adduction) of the legs through associated reactions. The Child with Athetosis or Ataxia The most severe impairments and activity limitations in children with athetosis or ataxia are related to the lack of postural stability. These are listed in Table 6-7. The inability to maintain a posture is evident in the lack of consistent head and trunk control. The child exhibits large, uncompensated movements around the long axis of the body or extremities. In contrast to children with spasticity who lack movement, children with athetosis or ataxia lack postural stability. Because of this instability, the child with athetosis or ataxia may use abnormal movements, such as an asymmetric tonic neck posture, to provide additional stability for functional movements, such as using a pointer or pushing a joystick. Overuse of this posture can predispose the child with CP to scoliosis or hip subluxation. Table 6-7 257 Impairments, Activity Limitations, Participation Restrictions, and Focus of Treatment in Children with Athetosis Body Structure/Function Activity Limitation Participation Restriction Focus of Treatment Muscle tone Delayed gross and fine Seltfeeding Educate parents motor skills Selective motor Delayed oral motor skills Increased time to carry out activities of daily living Focus parents’ handling on stability control Slow gait and other tasks * Lack of stability * Lack of cocontraction * Poor coordination Slow postural Postural instability Increase midline holding in postures responses Balance Lack of graded. Decreased play Weight bearing through arms for safer movement transitions movement Decreased leisure Control and direct movement with resistance; resist reciprocal movements 258 Physical therapy intervention Children with CP demonstrate impairments, functional limitations, and movement dysfunction throughout their lifetime. Four stages of care are used to describe the continuum of physical therapy management of the child with CP from infancy to adulthood. Physical therapy goals and treatment are presented within the framework of these four stages: early intervention, preschool, school age and adolescence, and adulthood. Because the brain damage occurs in a developing motor system, the primary emphasis of physical therapy intervention is to foster motor development and to learn functional motor skills. When a child learns to move for the first time, the infant’s own movements provide sensory feedback for the learning process to occur. If the feedback is incorrect or is incorrectly perceived, the movement may be learned incorrectly. Children with CP tend to develop stereotypical patterns of movement because they have difficulty in controlling movement against gravity. These stereotypical patterns interfere with developing functional motor skills. Inaccurate motor learning appears to occur in CP. The child (1) moves incorrectly; (2) learns to move incorrectly; and (3) continues to move incorrectly, thereby setting up a cycle for more and more abnormal movement. By assisting the child to experience more functional and normal movement, the clinician promotes functional movement and allows the child more independence within his or her environment. The acquisition of motor milestones and of subsequent skills has to be viewed as the promotion of the child’s highest possible independent level of function. Although the developmental sequence can act as a guide for formulating treatment goals and as a source of treatment activities, it should not be adhered to exclusively. Just because one skill comes before another in the typical developmental sequence, it does not mean that it is a prerequisite for the next skill. A good example of this concept is demonstrated by looking at the skill of creeping. Creeping is not a necessary prerequisite for walking. In fact, learning to creep may be more difficult for the child because creeping requires weight shifting and coordination of all four extremities. Little is to be gained by blindly following the developmental sequence. In fact, doing so may make it more difficult for the child to progress to upright standing. The physical therapist is responsible for formulating and directing the plan of care. The physical therapist assistant implements interventions designed to assist the child to achieve the goals as outlined in the plan of care. Therapeutic interventions may include positioning, developmental activities, and practicing postural control within cognitively and socially appropriate functional tasks. The physical therapist assistant can foster motor development through play and use play to expand the child’s ability to self-generate perceptual motor experiences. The physical therapist assistant can model positive social interactions for the caregiver and provide family education. General Treatment Ideas Child with Spasticity Treatment for the child with spasticity focuses on mobility in all possible postures and transitions between these postures. The tendency to develop contractures needs to be counteracted by range of motion, positioning, and development of active movement. Areas that are prone to tightness may include shoulder adductors and elbow, wrist, and finger flexors in children with quadriplegic involvement, whereas hip flexors and adductors, knee flexors, and ankle plantar flexors are more likely to be involved in children with diplegic involvement. Children with quadriplegia can show lower extremity tightness as well. These same joints may be involved unilaterally in hemiplegia. Useful techniques to inhibit spasticity include weight bearing; weight shifting; slow, rhythmic rocking; and rhythmic rotation of the trunk and body segments. Active trunk rotation, dissociation of body segments, and isolated joint movements should be included in the treatment activities and home program. Appropriate handling can increase the likelihood that the child will receive more accurate sensory feedback for motor learning. Advantages and Disadvantages of Different Positions The influence of tonic reflexes on functional movement is presented in the earlier section of this chapter. The advantages of using different positions in treatment are now discussed. Both advantages and disadvantages can be found in the previous chapter in Table 5-2. The reader is also 209 referred to Chapter 5 for descriptions of facilitating movement transitions between positions. Supine Early weight bearing can be performed when the child is supine, with the knees bent and the feet flat on the support surface. To counteract the total extension influence of the TLR, the child’s body can be flexed by placing the upper trunk on a wedge and the legs over a bolster. Flexion of the head and upper trunk can decrease the effect of the supine TLR. Dangling or presenting objects at the child’s eye level can facilitate the use of the arms for play or object exploration. Side Lying This position is best to dampen the effect of most of the tonic reflexes because of the neutral position of the head. Be careful not to allow lateral flexion with too thick a support under the head. It is also relatively easy to achieve protraction of the shoulders and pelvis, as well as trunk rotation, in preparation for rolling and coming to sit. The side the child is lying on is weight bearing and should be elongated. This maneuver can be done passively before the child is placed into the side-lying position (see Intervention 5-8), or it may occur as a result of a lateral weight shift as the child’s position is changed. Prone The prone position promotes weight bearing through the upper extremities, as well as providing some stretch to the hip and knee flexors. Head and trunk control can be facilitated by the development of active extension as well as promoting eye-head relationships. Movement while the child is prone, prone on elbows or prone on extended arms, can promote upper extremity loading and weight shift. Sitting Almost no better functional position exists than sitting. Weight bearing can be accomplished through the extremities while active head and trunk control is promoted. An extended trunk is dissociated from flexed lower extremities. Righting and equilibrium reactions can be facilitated from this position. ADLs such as feeding, dressing, bathing, and movement transitions can all be encouraged while the child is sitting. Quadruped The main advantage of the four-point or quadruped position is that the extremities are all weight bearing, and the trunk must work directly against gravity. The position provides a great opportunity for dissociated movements of limbs from the trunk and the upper trunk from the lower trunk. Kneeling As a dissociated posture, kneeling affords the child the opportunity to practice keeping the trunk and hips extended while flexed at the knees. The hip flexors can be stretched, and balance responses can be practiced without having to control all lower extremity joints. Playing in kneeling is developmentally appropriate, and with support, the child can also practice moving into half- kneeling. Standing The advantages of standing are obvious from a musculoskeletal standpoint. Weight bearing through the lower extremities is of great importance for long bone growth. Weight bearing can produce a prolonged stretch on heel cords and knee flexors while promoting active head and trunk control. Upright standing also provides appropriate visual input for social interaction with peers. Child with Athetosis or Ataxia Treatment for the child with athetosis focuses on stability in weight bearing and the use of developmental postures that provide trunk or extremity support. Useful techniques include approximation, weight bearing, and moving within small ranges of motion with resistance as tolerated. The assistant can use sensory cues that provide the child with information about joint and postural alignment, such as mirrors, weight vests, and heavier toys that provide some resistance but 260 do not inhibit movement. Grading movement within the midrange, where instability is typically the greatest, is the most difficult for the child. Activities that may be beneficial include playing “statues,” holding ballet positions, and holding any other fixed posture, such as stork standing. Use of hand support in sitting, kneeling, and standing can improve the child’s stability. Visually fixing on a target may also be helpful. As the child grows older, the assistant should help the child to develop safe movement strategies during customary ADLs. If possible, the child should be actively involved in discovering ways to overcome his or her own particular obstacles. Valued Life Outcomes Giangreco et al. (2011) identified five life outcomes that should be highly valued for all children, even those with severe disabilities: 1. Being safe and healthy both physically and emotionally 2. Having a safe, stable home in which to live now and in the future 3. Having meaningful personal relationships 4. Having control and choice based on age and culture 5. Engaging in meaningful activities in a variety of places within a community These outcomes can be used to guide goal setting for children with disabilities across the life span. Giangreco et al. (2011) continue to support linking educational curriculum to individually determined life outcomes. They provide a guide to education planning which is collaborative and family-centered for young children and life outcome based for the school-aged child. School-based interventions must be focused on education needs of the child (Effgen, 2013). Perhaps by having a vision of what life should be like for these children, we can be more future-oriented in planning and giving support to these children and their families. This approach is certainly in keeping with the ICF focus on activities and participation of children with disabilities. We must always remember that children with disabilities grow up to be adults with disabilities. First Stage of Physical Therapy Intervention: Early Intervention (Birth to 3 Years) Theoretically, early therapy can have a positive impact on nervous system development and recovery from injury. The ability of the nervous system to be flexible in its response to injury and development is termed plasticity. Infants at risk for neurologic problems may be candidates for early physical therapy intervention to take advantage of the nervous system’s plasticity. The decision to initiate physical therapy intervention and at what level (frequency and duration) is based on the infant’s neuromotor performance during the physical therapy examination and the family’s concerns. Several assessment tools designed by physical therapists are used in the clinic to try to identify infants with CP as early as possible. Pediatric physical therapists need to update their knowledge of such tools continually. As previously stated, a discussion of these tools is beyond the scope of this text because physical therapist assistants do not evaluate children’s motor status. However, a familiarity with tools used by physical therapists can be gained by reading the text by Effgen (2013) or Campbell et al. (2012). Typical problems often identified during a physical therapy examination at this time include lack of head control, inability to track visually, dislike of the prone position, fussiness, asymmetric postures secondary to exaggerated tonic reflexes, tonal abnormalities, and feeding or breathing difficulty. Early intervention usually spans the first 3 years of life. During this time, typically developing infants are establishing trust in their caregivers and are learning how to move about safely within their environments. Parents develop a sense of competence through taking care of their infant and guiding them in safe exploration of the world. Having a child with a disability is stressful for a family. By educating the family about the child’s disability and by teaching the family ways to position, carry, feed, and dress the child, the therapist and the therapist assistant practice family- centered intervention. The therapy team must recognize the needs of the family in relation to the child, rather than focusing on the child’s needs alone. Federal funding to states provides for the screening and intervention from birth to 3 years of age of children who have or are at risk for having disabilities and their families. Periodic assessment by a pediatric physical therapist who comes into the home may be sufficient to monitor an infant’s development and to provide parent education. Hospitals that provide intensive care for newborns often have follow-up clinics in which children are examined at regular 261 intervals. Instruction in home management, including specific handling and positioning techniques, is done by the therapist assigned to that clinic. Infants can be seen for ongoing early intervention services in the home. Physical therapy provides activity-based interventions that are embedded into daily routines and meet the goals of the family as outlined in an individualized family service plan (IFSP). At 3 years of age, the child may likely transition into an early childhood program in a public school to continue to receive services. Role of the Family The family is an important component in the early management of the infant with CP. Family- centered care is best practiced in pediatric physical therapy (Chiarello, 2013). Bamm and Rosenbaum (2008) reviewed the genesis, development, and implementation of family-centered care, which was introduced more than 40 years ago. The most frequently delineated concepts of family- centered care in child health literature are: 1. Recognizing the family as a constant in the child’s life and the primary source of strength and support for the child. 2. Acknowledging the diversity and uniqueness of children and families. 3. Acknowledging that parents bring expertise. 4. Recognizing that family-centered care fosters competency. 5. Encouraging collaboration and partnership between families and health-care providers. 6. Facilitating family-to-family support and networking (McKean et al., 2005). Families and professionals prioritize important issues differently. Families identify communication, availability, and accessibility as the most important issues in contrast to professionals who identify education, information, and counseling as most important. Bamm and Rosenbaum (2008) identified the four barriers and supports to implementing family-centered care. They are attitudinal, conceptual, financial, and political factors which can be viewed negatively or positively in affecting the implementation of family-centered care. Regardless of these factors, family-centered care is the preferred service delivery philosophy for physical therapy in any setting and can be utilized across the life span (Chiarello, 2013). Role of the Physical Therapist Assistant The physical therapist assistant’s role in providing ongoing therapy to infants is determined by the supervising physical therapist. The neonatal intensive care unit is not an appropriate practice setting for a physical therapist assistant or an inexperienced physical therapist because of the acuity and instability of very ill infants. Specific competencies must be met to practice safely within this specialized environment, and meeting these competencies usually requires additional coursework and supervised work experience. These competencies have been identified and are available from the Section on Pediatrics of the American Physical Therapy Association. The role of the physical therapist assistant in working with the child with CP is as a member of the health-care team. The makeup of the team varies depending on the age of the child. During infancy, the team may be small and may consist only of the infant, parents, physician, and therapist. By the time the child is 3 years old, the rehabilitation team may have enlarged to include additional physicians involved in the child’s medical management and other professionals such as an audiologist, an occupational therapist, a speech pathologist, a teacher, and a teacher’s aide. The physical therapist assistant is expected to bring certain skills to the team and to the child, including knowledge of positioning and handling techniques, use of adaptive equipment, management of impaired tone, and developmental activities that foster motor abilities and movement transitions within a functional context. Because the physical therapist assistant may be providing services to the child in the home or at school, the assistant may be the first to observe additional problems or be told of a parental concern. These concerns should be communicated to the supervising therapist in a timely manner. 1. General goals of physical therapy in early intervention are to: 2. Promote infant-parent interaction. 3. Encourage development of functional skills and play. 4. Promote sensorimotor development. 5. Establish head and trunk control. 6. Attain and maintain upright orientation. 262 Handling and Positioning Handling and positioning in the supine or “en face” (face-to-face) posture should promote orientation of the head in the midline and symmetry of the extremities. A flexed position is preferred so the shoulders are forward and the hands can easily come to the midline. Reaching is encouraged by making sure that objects are within the infant’s grasp. The infant can be encouraged to initiate reaching when in the supine position by being presented with visually interesting toys. Positioning with the infant prone is also important because this is the position from which the infant first moves into extension. Active head lifting when in prone can be encouraged by using toys that are brightly colored or make noise. Some infants do not like being in prone, and the caregiver has to be encouraged to continue to put the infant in this position for longer periods. Carrying the infant in prone can increase the child’s tolerance for the position. The infant should not sleep in prone, however, because of the increased incidence of sudden infant death syndrome in infants who sleep in this position (American Academy of Pediatrics, 1992). Carrying positions should accentuate the strengths of the infant and should avoid as much abnormal posturing as possible. The infant should be allowed to control as much of her body as possible for as long as possible before external support is given. Figure 6-11 shows a way to hold the child to increase tolerance to prone and to provide gentle movement; refer to Chapter 5 for other carrying positions. Additionally, Figure 6-11 depicts a way to engage a child in moving and playing. FIGURE 6-11 Holding, moving, and playing as a way to control the head and body against gravity. (Redrawn from Shepherd RB: Cerebral palsy in infancy, Elsevier, 2014, p. 247.) Most handling and positioning techniques represent use of the developmental sequence in the management of the child with CP popularized by the Bobaths. Although their neurodevelopmental approach is used in this population, research evidence of its effectiveness over other, more activity- based approaches is minimal. As the reader is aware, neurologic development occurs at the same time at which the child’s musculoskeletal and cognitive systems are maturing. Motor learning must take place if any permanent change in motor behavior is to occur. Affording the infant opportunities to self-generate sensorimotor experiences is an excellent way to promote motor exploration and social play. Remember that movement variability is the hallmark of an adaptable neuromuscular system. Feeding and Respiration A flexed posture facilitates feeding and social interaction between the child and the caregiver. The more upright the child is, the easier it is to promote a flexed posture of the head and neck. Although it is not appropriate for a physical therapist assistant to provide oral motor therapy for an infant with severe feeding difficulties, the physical therapist assistant could assist in positioning the infant during a therapist-directed feeding session. One example of a position for feeding is shown in Intervention 6-1, A. The face-to-face position can be used for a child who needs trunk support. Be careful that the roll does not slip behind the child’s neck and encourage extension. Other examples 263 of proper body positioning for improved oral motor and respiratory functioning during mealtime are depicted in Intervention 6-1, B. Deeper respirations can also be encouraged prior to feeding or at other times by applying slight pressure to the child’s thorax and abdominal area prior to inspiration. This maneuver can be done when the child is in the side-lying position, as shown in Intervention 6-2, or with bilateral hand placements when the child is supine. The tilt of the wedge makes it easier for the child to use the diaphragm for deeper inspiration, as well as expanding the chest wall. Intervention 6-1 Positioning for Feeding A. The face-to-face position can be used for a child who needs trunk support. Be careful that the roll does not slip behind the child’s neck, and encourage extension. B. A young child is positioned for feeding in a car seat with adaptations using towel rolls. C. A young child positioned on a prone stander is standing for mealtime. D. A child is positioned in a high chair with adaptations for greater hip stability and symmetry during feeding. E. A child is positioned in his wheelchair with an adapted seat insert, a tray, and hip stabilizing straps for mealtime. (A, Reprinted by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities, New York, 1978, Teachers College Press, p. 201. ©1978 Teachers College, Columbia University. All rights reserved; B to E, From Connolly BH, Montgomery PC: Therapeutic exercise in developmental disabilities, ed 2. Thorofare, NJ, 2001, Slack.) Intervention 6-2 Facilitating Deeper Inspiration 264 In side lying, slight pressure is applied to the lateral thorax to facilitate deeper inspiration. (Reprinted by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities, New York, 1978, Teachers College Press, p. 199. © 1978 Teachers College, Columbia University. All rights reserved.) Therapeutic Exercise Gentle range-of-motion exercises may be indicated if the infant has difficulty reaching to the midline, has difficulty separating the lower extremities for diapering, or has tight heel cords. Infants do not have complete range of motion in the lower extremities normally, so the hips should never be forced into what would be considered full range of adduction or extension for an adult. Parents can be taught to incorporate range of motion into the daily routines of diapering, bathing, and dressing. The reader is referred to the instruction sheets by Jaeger (1987) as a good source of home program examples to use for maintenance of range of motion. Motor Skill Acquisition The skills needed for age-appropriate play vary. Babies look around and reach first from the supine position and then from the prone position, before they start moving through the environment. Adequate time playing on the floor is needed to encourage movement of the body against gravity. Gravity must be conquered to attain upright sitting and standing postures. Body movement during play is crucial to body awareness. Movement within the environment is necessary for spatial orientation to the external world. Although floor time is important and is critical for learning to move against gravity, time spent in supine and prone positions must be balanced with the benefits of being in an upright orientation. All children need to be held upright, on the parent’s lap, and over the shoulder to experience as many different postures as are feasible. Refer to Chapter 5 for specific techniques that may be used to encourage head and trunk control, upper extremity usage, and transitional movements. Constraint-Induced Movement Therapy (CIMT) Young children with cerebral palsy from 18 months to 3 years who have unilateral upper extremity 265 involvement are good candidates for CIMT. A short arm cast is applied to the noninvolved arm to prevent the child with hemiplegia from using the unaffected extremity which forces use of the affected arm. Children from ages 3 to 6 may also be treated in the clinic or at home with this intervention, although as the child transitions to school, it may be harder to ensure the child’s cooperation. CIMT is the most researched intervention used for children with hemiplegic CP (Case- Smith, 2014; Charles et al., 2006; DeLuca et al., 2003, 2012). A full description of the intervention is beyond the scope of this text. Physical therapy and occupational therapy are typically part of the protocols with the focus on intensive repetition for motor learning. Results have been very positive, with improvements in arm function (DeLuca et al., 2003; Eliasson et al., 2005) and gait (Coker et al., 2010). Functional Postures The two most functional positions for a person are sitting and standing, because upright orientation can be achieved with either position. Some children with CP cannot become functional in standing because of the severity of their motor involvement, but almost every child has the potential to be upright in sitting. Function in sitting can be augmented by appropriate seating devices, inserts, and supports. For example, the child with spastic diplegia, as in Figure 6-12, has difficulty sitting on the floor and playing because of hamstring stiffness, which prevents her from flexing her hips. By having the child sit on a stool with feet on the floor, as in Figure 6-12, B, the child exhibits better arm use in play and a more upright sitting posture. In Figure 6-12, C, having the child sit on a low stool allows her to practice moving her body away from the midline to reach for a toy. This movement was blocked while sitting on the floor by her wide abducted sitting posture. = = FIGURE 6-12 Function in sitting. A, An infant with diplegia has difficulty playing because tight hamstrings prevent adequate hip flexion for sitting squarely on the floor. B, A child is able to play while sitting on a stool with feet on the floor. C, A wide abducted floor sitting posture prevents lateral movement away from the midline, limiting her reach. Sitting on a stool with her feet on the floor enables her to balance as she shifts her body laterally. (From Shepherd RB: Cerebral palsy in infancy, Elsevier, 2014, p. 249.) When motor control is insufficient to allow independent standing, a standing program can be implemented. Upright standing can be achieved by using a supine or prone stander, along with orthoses for distal control. Standers provide lower extremity weight bearing while they support the child’s trunk. The child is free to work on head control in a prone stander and to bear weight on the upper extremities or engage in play. In a supine stander, the child’s head is supported while the hands are free for reaching and manipulation. The trunk and legs should be in correct anatomic alignment. Standing programs were typically begun when the child is around 12 to 16 months of age. Stuberg (1992) recommended standing for at least 60 minutes, four or five times per week, as a general guideline. It is now recommended that supported standing begin early at 9 to 10 months (Paleg et al., 2013). The goals are to improve bone density and hip development and to manage contractures. Paleg et al. (2013) recommend 60 to 90 minutes per day for 5 days to positively affect bone mineral density. For hip health, 60 minutes a day with the lower extremities in 30 to 60 degrees of bilateral hip abduction while in a supported stander is recommended. Forty-five to sixty minutes is recommended to affect range of motion of the lower extremity and to affect spasticity. 266 Independent Mobility Mobility can be achieved in many ways. Rolling is a form of independent mobility but may not be practical, except in certain surroundings. Sitting and hitching (bottom scooting with or without extremity assistance) are other means of mobility and may be appropriate for a younger child. Creeping on hands and knees can be functional, but upright ambulation is still seen as the most acceptable way for a child to get around because it provides the customary and expected orientation to the world. The use of body-weight support devices has increased as part of gait training of children with CP. Some early interventions that may be useful for the infant with CP have been suggested by Shepherd (2014). She stresses ways that a typical infant uses her legs during infancy such as when kicking, moving the body up and down on fixed feet as in a squat or crouch, moving from sit to stand to sit, and stepping up and down and walking. Intervention 6-3is crouching to standing or squatting and crouching. Intervention 6-4 is moving from sit to stand and stand to sit. Weight bearing through the feet from an early age can assist in keeping the gastrocnemius and soleus muscles lengthened since they tend to stiffen over time and develop a contracture that might require surgery. Intervention 6-5 is stepping up and down. These interventions can be continued throughout this stage of physical therapy management. Intervention 6-3 Squatting and Crouching 267 Exercises and games to train lower limb control. Children are squatting to pick up toys or to take a toy out of the box. Intervention 6-4 Sitting to Stand and Stand to Sit 268 Sit-stand-sit exercise. A, The therapist steadies the infant as he does not yet have the ability to balance throughout the action. B, The therapist moves the infant’s knee (and body mass) forward to show him what he must do. C, This little boy needs assistance to initiate knee flexion for sitting. Intervention 6-5 Stepping up and Down A and B, With manual contacts at the pelvis, encourage the infant to place a foot on a small flat 269 object and bring weight forward, repeat with the other leg. Child may support herself on rails or a table while stepping. Gradually increase the height of the object to increase activation of the leg muscles. Assist the infant in stepping forward and up but do not take all of the infant’s weight. C, Practice stepping sideways as in cruising. Place an object to either side and encourage stepping up laterally. (From Shepherd RB: Physiotherapy in Paediatrics, ed 3, Oxford, 1995, Butterworth-Heinemann.) Ambulation Predictors A prediction of ambulation potential can be made on the basis of the type and distribution of disordered movements, as well as by achievement of motor milestones (Table 6-8). The less of the body is involved, the greater the potential for ambulation. Children with spastic quadriplegia show the largest variability in their potential to walk. Children who display independent sitting or the ability to scoot along the floor on the buttocks by the age of 2 years have a good chance of ambulating (Watt et al., 1989). Table 6-8 Predictors of Ambulation for Cerebral Palsy Predictor Ambulation Potential By diagnosis: Monoplegia Hemiplegia Ataxia Diplegia Spastic quadriplegia By motor function: Sits independent by 2 years Sits independent by 34 years Presence of primitive reactions beyond 2 years Absence of postural reactions beyond 2 years Independently crawled symmetrically or reciprocally by 214-3 years} 100% Source: Glanzman A: Cerebral palsy. In Goodman C, Fuller KS, editors: Pathology: implications for the physical therapist, St. Louis, Saunders, 2015, p. 1524. “From Pallas Alonso CR, de la Cruz B, Lopez MC, et al: Cerebral palsy and age of sitting and walking in very low birth weight infants. An Esp Pediatr 53:48-52, 2000. t From da Paz Junior, Burnett SM, Braga LW: Walking prognosis in cerebral palsy: A 22-year retrospective analysis. Dev Med Child Neurol 36:130—134, 1994. A child with CP may achieve independent ambulation with or without an assistive device. Children with spastic hemiplegia are more likely to ambulate at the high end of the normal range, which is 18 months. Some researchers report a range of up to 21 months (Horstmann and Bleck, 2007). Typical ages for ambulation have been reported in children with spastic diplegia, with most walking at 24 to 36 months. Those that do not walk until 48 months require some types of assistive device, such as crutches, canes, or a walker. Other investigators have reported that if ambulation is possible for a child with any level of involvement, it usually takes place by the time the child is age 8 (Glanzman, 2009). Most children do not require extra encouragement to attempt ambulation, but they do need assistance and practice in bearing weight equally on their lower extremities, in initiating reciprocal limb movement, and in balancing. Postural reactions involving the trunk are usually delayed, as are extremity protective responses. Impairments in transitional movements from sitting to standing can impede independence. In children with hemiplegic CP, movements initiated with the involved side of the body may be avoided, with all the work of standing and walking actually accomplished by the uninvolved side. Body Weight-Supported Treadmill Training (BWSTT) Use of BSWTT has become an acceptable rehabilitation strategy for improving the walking performance of children with CP. A harness can be used to support an infant as she learns to walk, to keep the child safe for walking practice, as seen in Figure 6-13, or while engaged in another activity. Data on using a harness apparatus to partially support a child’s body weight while training ambulation on a treadmill has shown that children at GMFCS levels III and IV significantly increased gross motor performance and walking speed (Willoughby et al., 2009). Early task-specific practice is beneficial for acquiring the ability to ambulate. Richards et al. (1997) studied the use of 270 such a system in four children with CP and concluded that it would be possible to train children as young as 19 months of age. In a study of older children, there were positive changes in motor test scores and in the ability to transfer of some children (Schind] et al., 2000). A twelve-week program performed two days a week resulted in improved walking performance in children with CP (Kurz et al., 2011). The changes in stepping kinematics were strongly correlated with changes in step length, walking speed, and GMFM score. Additional studies have shown that BWSTT improves gait in children with CP (Cherng et al., 2007; Dodd and Foley, 2007; Mattern-Baxter et al., 2009). FIGURE 6-13 Body-Weight Support Treadmill Use. (Treadmill with harness, with permission from LiteGait, Mobility Research, Tempe, AZ; From Shepherd RB: Cerebral palsy in infancy, Elsevier, 2014, p. 7.) The research is equivocal when comparing the effect of treadmill training and overground walking. Willoughby et al. (2010) found no difference between the two groups in walking speed or in walking in the school environment. However, Grecco et al. (2013) found that their treadmill- training group demonstrated greater improvement than the overground-walking group. The difference was significant after treatment and on follow-up. It should be noted that in the study of Willoughby et al. partial weight support was used while on the treadmill and the participants were GMECS levels III or IV, whereas in the study of Grecco et al. the treadmill was used without partial weight support and the participants were GMFCS levels I to III. Use of a treadmill with or without partial body weight support needs to continue to be researched to develop appropriate protocols for children at different GMFCS levels. Power Mobility Mobility within the environment is too important for the development of spatial concepts to be delayed until the child can move independently. Power mobility should be considered a viable option even for a young child. As young as 17 to 20 months, some children with disabilities have 271 learned to maneuver a motorized wheelchair (Butler, 1986, 1991). Just because a child is taught to use power mobility does not preclude working concurrently on independent ambulation. This point needs to be stressed to the family. Early use of power mobility has been shown to have positive effects on young children who are unable to move independently (Guerette et al., 2013). Refer to the first international consensus on power mobility recently published by Livingstone and Paleg (2014). Clinical practice suggestions are made for using power mobility in children with different abilities, needs, and ages. Children with CP who are not mobile but have the cognitive skills of a 12-month- old should be evaluated for power mobility. The mismatch of motor and cognition has the potential to produce negative developmental outcomes (Anderson et al., 2014). Other mobility alternatives include devices such as prone scooters, adapted tricycles, battery-powered riding toys, and manual wheelchairs. The independence of moving on one’s own teaches young children that they can control the environment around them, rather than being controlled. Second Stage of Physical Therapy Intervention: Preschool Period The major emphasis during the preschool period is to promote mobility and functional independence in the child with CP. Depending on the distribution and degree of involvement, the child with CP may or may not have achieved an upright orientation to gravity in sitting or standing during the first 3 years of life. By the preschool period, most children’s social sphere has broadened to include day-care attendants, babysitters, preschool personnel, and playmates, so mobility is not merely important for self-control and object interaction; it is a social necessity. All aspects of the child’s being —mental, motor, and social-emotional—are developing concurrently during the preschool period in an effort to achieve functional independence. Physical therapy goals during the preschool period are: 1. Establish a means of independent mobility 2. Promote functional movement 3. Improve performance of ADLs such as grooming and dressing 4. Promote social interaction with peers The physical therapist assistant is more likely to work with a preschool-age child than with a child in an infant intervention program. Within a preschool setting, the physical therapist assistant implements certain aspects of the treatment plan formulated by the physical therapist. Activities may include promoting postural reactions to improve head and trunk control, teaching transitions such as moving from sitting to standing, stretching to maintain adequate muscle length for function, strengthening and endurance exercises for promoting function and health, and practice of self-care skills as part of the child’s daily home or classroom schedule. Independent Mobility If the child with CP did not achieve upright orientation and mobility in some fashion during the early intervention period, now is the time to make a concerted effort to assist the child to do so. For children who are ambulatory with or without assistive devices and orthoses, it may be a period of monitoring and reexamining the continued need for either the assistive or orthotic device. Some children who may not have previously required any type of assistance may benefit from one now because of their changing musculoskeletal status, body weight, seizure status, or safety concerns. Their previous degree of motor control may have been sufficient for a small body, but with growth, control may be lost. Any time the physical therapist assistant observes that a child is having difficulty with a task previously performed without problems, the supervising therapist should be alerted. Although the physical therapist performs periodic reexaminations, the physical therapist assistant working with the child should request a reexamination any time negative changes in the child’s motor performance occur. Positive changes should, of course, be thoroughly documented and reported because these, too, may necessitate updating the plan of care. Gait Ambulation may be possible in children with spastic quadriplegia if motor involvement is not too severe. The attainment of the task takes longer, and gait may never be functional because the child requires assistance and supervision for part or all of the components of the activity. Therefore, ambulation may be considered only therapeutic, that is, another form of exercise done during therapy. 272 Specific gait difficulties seen in children with spastic diplegia include lack of lower extremity dissociation, decreased single-limb and increased double-limb support time, and limited postural reactions during weight shifting. Children with spastic diplegia have problems dissociating one leg from the other and dissociating leg movements from the trunk. They often fix (stabilize) with the hip adductors to substitute for the lack of trunk stability in upright necessary for initiation of lower limb motion. Practicing coming to stand over a bolster can provide a deterrent to lower extremity adduction while the child works on muscular strengthening and weight bearing (Intervention 6-6, A). If the child cannot support all the body’s weight in standing or during a sit-to-stand transition, have part of the child’s body weight on extended arms while the child practices coming to stand, standing, or shifting weight in standing (Intervention 6-6, B). Intervention 6-6 Coming to Stand over a Bolster — 273 B A. Practicing coming to stand over a bolster can provide a deterrent to lower extremity adduction and can work on lower extremity strengthening and weight bearing. B. If the child cannot support all the body’s weight in standing or during a sit-to-stand transition, part of the child’s body weight can be borne on extended arms while the child practices coming to stand, standing, or weight shifting in standing. (A, From Campbell SK, editor: Physical therapy for children, ed 4. St. Louis, 2012, WB Saunders.; B, Reprinted by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities, New York, 1978, Teachers College Press, p. 163. © 1978 Teachers College, Columbia University. All rights reserved.) Practicing lateral trunk postural reactions may automatically result in lower extremity separation as the lower extremity opposite the weight shift is automatically abducted (Intervention 6-7). The addition of trunk rotation to the lateral righting may even produce external rotation of the opposite leg. Pushing a toy and shifting weight in step-stance are also useful activities to practice lower extremity separation. As the child decreases the time in double-limb support by taking a step of appropriate length, she can progress to stepping over an object or to stepping up and down off a step. Single-limb balance can be challenged by using a floor ladder or taller steps. Having the child hold on to vertical poles decreases the amount of support and facilitates upper trunk extension (Figure 6-14). The walkable LiteGait could be used to transition someone from treadmill walking to overground walking (Figure 6-15). Many children can benefit from using a type of assistive device, such as a rolling reverse walker, during gait training (Figure 6-16). Orthoses may also be needed to enhance ambulation. Intervention 6-7 Balance Reaction on a Bolster 274 Practicing lateral trunk postural reactions may automatically result in lower extremity separation as the lower extremity opposite the weight shift is automatically abducted. FIGURE 6-14 Standing with poles. 279 FIGURE 6-15 Walkable LiteGait (With permission from LiteGait, Mobility Research, Tempe, AZ; From Shepherd RB: Cerebral palsy in infancy, Elsevier, 2014, p. 7.) 276 FIGURE 6-16 Walker (rolling reverse). Orthoses The most frequently used orthosis in children with CP who are ambulatory is a type of ankle-foot orthosis (AFO). The standard AFO is a single piece of molded polypropylene. The orthosis extends 10 to 15 mm distal to the head of the fibula. The orthosis should not pinch the child behind the knee at any time. All AFOs and foot orthoses (FOs) should support the foot and should maintain the subtalar joint in a neutral position. Hinged AFOs have been shown to allow a more normal and efficient gait pattern (Middleton et al., 1988). In a review by Morris (2002), prevention of plantar flexion was found to improve gait efficiency. Ground reaction AFOs have been recommended by some clinicians to decrease the knee flexion seen in the crouch gait of children with spastic CP (Figure 6-17). Other clinicians state that this type of orthotic device does not work well if the crouch results from high tone in a child with spastic diplegia (Ratliffe, 1998). Knutson and Clark (1991) found that foot orthoses could be helpful in controlling pronation in children who do not need ankle stabilization. Dynamic AFOs have a custom-contoured soleplate that provides forefoot and hindfoot alignment. There is substantial evidence that use of AFOs in children with CP at GMFCS levels I to III controls the ankle and foot during both phases of gait improves gait efficiency (Morris et al., 2011). 277 FIGURE 6-17 Ground reaction ankle-foot orthoses. (From Campbell SK, editor: Physical therapy for children, ed 4. St. Louis, 2012, WB Saunders.) An AFO may be indicated, following surgery or casting to maintain musculotendinous length gains. The orthosis may be worn during both the day and at night. Proper precautions should always be taken to inspect the skin regularly for any signs of skin breakdown or excessive pressure. The physical therapist should establish a wearing schedule for the child. Areas of redness lasting more than 20 minutes after brace removal should be reported to the supervising physical therapist. A child with unstable ankles who needs medial lateral stability may benefit from a supramalleolar orthosis (SMO). This orthotic device allows the child to move freely into dorsiflexion and plantar flexion while restricting mediolateral movement. An SMO or an FO may be indicated for a child with mild hypertonia or foot pronation (Knutson and Clark, 1991; Buccieri, 2003; George and Elchert, 2007). In the child with hypotonia or athetoid CP, the SMO or FO may provide sufficient stability within a tennis shoe to allow ambulation. General guidelines for orthotic use can be found in Table 6-9. Table 6-9 General Foot and Ankle Splinting Guidelines Splints Status Application Solid AFO neutral to + 3° DF Nonambulators, beginning standers | 1. Less than 3° of DF 2. Genu recurvatum associated with decreased ankle DF or weakness 3. Need for medial-lateral stability 4. Nighttime/positional stretching AFO with 90° posterior stop and free DF (hinged | Clients with some, but limited, Application of 1-4 above, but need more passive DF during movement, such as ambulation, functional mobilit squatting, steps, and sit to stand Floor reaction AFO (set DF depending on weight | Crouch gait For clients with decreased ability to maintain knee extension during ambulation line in standing) Full passive knee extension in standing SMO Standers/ambulators with pronation | 1. Need medial-lateral ankle stability at the ankles 2. Would like opportunity to use active plantar flexion 3. Decreased DF not a problem during gait From Glanzman A: Cerebral palsy. In Goodman CC, Fuller K, editors: Pathology: implications for the physical therapist, ed 3. St. Louis, Saunders, 2015, p. 1529. 278 AFO, Ankle-foot orthosis; DF, dorsiflexion; SMO, supramalleolar orthosis. Assistive Devices Some assistive devices should be avoided in this population. For example, walkers that do not require the child to control the head and trunk as much as possible are passive and may be of little long-term benefit. When the use of a walker results in increased lower extremity extension and toe walking, a more appropriate means of encouraging ambulation should be sought. Exercise saucers can be as dangerous as walkers. Jumpers should be avoided in children with increased lower extremity muscle tone. If a child has not achieved independent functional ambulation before the age of 3 years, some alternative type of mobility should be considered at this time. An adapted tricycle, a manual wheelchair, a mobile stander, a battery-powered scooter, and a power wheelchair are all viable options. Power options are being explored earlier and earlier for children. Use of power mobility does not necessarily mean that the child does not have the potential to be an overground walker. Power Mobility Children with more severe involvement, as in quadriplegia, do not have sufficient head or trunk control, let alone adequate upper extremity function, to ambulate independently even with an assistive device. For them, some form of power mobility, such as a wheelchair or other motorized device, may be a solution. For others, a more controlling apparatus such as a gait trainer may provide enough trunk support to allow training of the reciprocal lower extremity movements to propel the device (Figure 6-18). M.O.V.E. (Mobility Opportunity Via Education, 1300 17th Street, City Centre, Bakersfield, CA 93301-4533) is a program developed by a special education teacher to foster independent mobility in children who experience difficulty with standing and walking, especially severely physically disabled children. Early work with equipment has been expanded to include a curriculum and an international organization that promotes mobility for all children. Much of the equipment is available at Rifton Equipment (P.O. Box 901, Rifton, NY 12471-1901). FIGURE 6-18 Rifton gait trainer. (Courtesy Rifton Equipment, Rifton, NY.) For children already using power mobility, studies have shown that the most consistent use of the wheelchair is at school. When parents and caregivers of children who use power mobility were interviewed, two overriding issues were of greatest concern—accessibility and independence. 279 Although the wheelchair was viewed as a way to foster independence in an otherwise dependent child, most caregivers stated that they had some difficulty with accessibility, either in the home or in other local environments. To increase the benefit derived from a power wheelchair, the environment it is to be used in must be accessible, the needs of the caregiver must be considered, and the child must be adequately trained to develop skill in driving the wheelchair (Berry et al., 1996). Livingstone and Paleg (2014) note that power mobility is appropriate even for children who never become competent drivers. Medical Management This section presents the medical and surgical management of children with CP, because during this period of life, they are most likely to require either form of intervention for spasticity or musculoskeletal deficits. Medications The most common oral medications used to manage spasticity include the benzodiazepines, diazepam (Valium), clonazepam, (Klonopin), alpha, agonists, tizanidine (Zanaflex), baclofen (Lioresal), and dantrolene (Dantrium) (Accardo, 2008; Tilton, 2009). The mechanism of action and potential adverse effects are found in Table 6-10. Sedation, fatigue, and generalized weakness are common side effects which can negatively impact the child’s function. Increased drooling has been reported to interfere with feeding and speech (Erkin et al., 2010; Batshaw et al, 2013). Usefulness of oral medications can be limited due to their various side effects. The use of a pump to deliver baclofen directly to the spinal cord has been promoted because it takes less medication to achieve a greater effect. The youngest age at which a child would be considered for this approach is 3 years. It takes up to 6 months to see functional gains. The procedure is expensive, and the benefits are being studied. Because implantation of the pump is a neurosurgical procedure, further discussion is found under that heading. Table 6-10 Oral Medications for Spasticity Medication Mechanism of Action Side Effects Benzodiazepine Inhibits release of excitatory neurotransmitters Sedation, ataxia, physical dependence, impaired memory] Valium), (Klonopin’ Alpha-2 adrenergic agonist] Decreased release of excitatory neurotransmitters Sedation, hypotension, nausea, vomiting, hepatitis (Zanaflex) Dantrolene Inhibits release of calcium at sarcoplasmic reticulum Weakness, nauseas, vomiting, hepatitis Dantrium Baclofen Inhibits release of excitatory neurotransmitters in the spinal cord] Sedation, ataxia, weakness, hypotension Adapted from Theroux MC, DiCindio S: Major surgical procedures in children with cerebral palsy. Anesthesiology Clin 32:63-81, 2014. Botulinum Toxin Traditionally, spasticity has also been treated in the adult population with injections of chemical agents, such as alcohol or phenol, to block nerve transmission to a spastic muscle. Although this procedure is not routinely done in children with spasticity because of pain and discomfort, a new alternative is being used. Botulinum bacterium produces a powerful toxin that can inhibit a spastic muscle. If a small amount is injected into a spastic muscle group, weakness and decline of spasticity can be achieved for up to 3 to 6 months. These effects can make it easier to position a child, to fit an orthosis, to improve function, or to provide information about the appropriateness of muscle lengthening. More than one muscle group can be injected. The lack of discomfort and ease of administration are definite advantages over motor point blocks using alcohol or phenol (Gormley, 2001). Surgical Management Orthopedic surgery is an often-inevitable occurrence in the life of a child with CP. Indications for surgery may be to (1) decrease pain; (2) correct or prevent deformity; and (3) improve function. The decision to undergo an operation should be a mutual one among the physician, the family, the child, and the medical and educational teams. Children with CP have dynamic problems, and surgical treatment may provide only static solutions, so all areas of the child’s function should be 280 considered. The therapist should modify the child’s treatment plan according to the type of surgical procedure, postoperative casting, and the expected length of time of immobilization. A plan should be developed to address the child’s seating and mobility needs and to instruct everyone how to move and position the child safely at home and school. Surgical procedures to lengthen soft tissues are most commonly performed in children with CP and include tendon lengthening and release of spastic muscle groups. Surgical procedures to lengthen tight adductors or hamstrings may be recommended for the child to continue the best postural alignment or to maintain ambulatory status. In a tenotomy, the tendon is completely severed. A partial tendon release can include severing part of the tendon or muscle fibers or moving the attachment of the tendon. A neurectomy involves severing the nerve to a spastic muscle and thereby producing denervation. The child is usually placed in a spica cast or bilateral long leg casts for 6 to 8 weeks to immobilize the area. A 3-week period of casting has been found to be useful in lengthening the triceps surae (Tardieu et al., 1982, 1988). A child with tight heel cords who has not responded to traditional stretching or to plaster casting may require surgical treatment to achieve a flat (plantigrade) foot. Surgical lengthening of the heel cord is done to improve walking (Figure 6-19). The results of surgical treatment are more ankle dorsiflexion range and weaker plantar flexors. Davids et al. (2011) found increased ankle dosiflexion during swing phase in children with CP after surgical lengthening of the heel cord. Overlengthening can occur, resulting in a calcaneal gait or too much dorsiflexion during stance. This condition may predispose the child to a crouched posture and the development of hamstring and hip flexion contractures (Horstmann and Bleck, 2007). Rattey et al. (1993) reported that children who underwent heel-cord lengthening at 6 years of age or older did not have a recurrence of tightness. Davids et al. (2011) further stated that surgical lengthening should only be considered for the correction of fixed muscle contractures that did not respond to nonoperative treatments, such as manual stretching, serial casting, and strength training (Damiano et al., 1995a, b; Damiano et al., 1999). Tight heel cord before operation Lengthened heel cord after operation 281 FIGURE 6-19 Heel cord lengthening. Single-event multilevel surgery (SEML) has become the norm for children with CP. SEML is defined as “two or more soft-tissue or bony surgical procedures at two or more anatomical levels during one operative procedure, requiring only one hospital admission and one period of rehabilitation” (McGinley et al., 2012 p. 117). More complex orthopedic surgical procedures may be indicated in the presence of hip subluxation or dislocation. The hip may subluxate secondary to muscle imbalances from an obligatory ATNR. The skull side leg is pulled into flexion and adduction. Conservative treatment typically includes appropriate positioning to decrease the influence of the ATNR, passive stretching of tight muscle groups, and an abduction splint at night (Styer-Acevedo, 2008). If the hip becomes dislocated and produces pain and asymmetry, surgical treatment is indicated. The problem can be dealt with surgically in many ways, depending on its severity and acuity. The most minimal level of intervention involves soft tissue releases of the adductors, iliopsoas muscles, or proximal hamstrings. The next level requires an osteotomy of the femur in which the angle of the femur is changed by severing the bone, derotating the femur, and providing internal fixation. By changing the angle, the head of the femur is put back into the acetabulum. Sometimes, the acetabulum has to be reshaped in addition to the osteotomy. A hip replacement or arthrodesis could even be an option. Bony surgical procedures are much more complex and require more lengthy immobilization and rehabilitation. Gait analysis in a gait laboratory can provide a clearer picture on which to base surgical decisions than visual assessment of gait. Quantifiable information about gait deviations in a child with CP is gained by observing the child walk from all angles and collecting data on muscle output and limb range of motion during the gait cycle. Video analysis and surface electromyography provide additional invaluable information for the orthopedic surgeon. This information can be augmented by temporary nerve blocks or botulinum-toxin injections to ascertain the effects of possible surgical interventions. A recent study by Marconi et al. (2014) assessed the effect of SEMLs on gait parameters in children with CP. Participants were between the ages of 9 and 16 years with GMFCS levels between I to III. The energy cost of walking was significantly reduced and thought to be due to a reduction in energy cost of muscular work used to maintain the posture rather than to an improvement in mechanical efficiency. According to the systematic review of McGinley et al. (2012), there is a trend toward positive outcomes in gait as a result of SEMLs. Neurosurgery Selective posterior or dorsal rhizotomy (SDR) has become an accepted treatment for spasticity in certain children with CP. Peacock et al. (1987) began advocating the use of this procedure in which dorsal roots in the spinal cord are identified by electromyographic response (Figure 6-20). Dorsal roots are selectively cut to decrease synaptic, afferent activity within the spinal cord which decreases spasticity. Through careful selection, touch and proprioception remain intact. Ideal candidates for this procedure are children with spastic diplegia or hemiplegia with moderate motor control and an IQ of 70 or above (Cole et al., 2007; Gormley, 2001). Following rhizotomy, a child requires intense physical therapy for several months postoperatively to maximize strength, range of motion, and functional skills (Gormley, 2001). Physical therapy can be decreased to 1 to 2 times a week within a year. Once the spasticity is gone, weakness and incoordination are prevalent. Any orthopedic surgical procedures that are still needed should not be performed until after this period of rehabilitation. If the child is to undergo neurosurgery, it should be completed 6 to 12 months before any orthopedic surgery (Styer-Acevedo, 1999). Cole et al. (2007) excluded any child who had had any multilevel surgery. Hurvitz et al. (2010) surveyed adults who had an SDR as children. The majority reported an improved quality of life with only 10% reporting a decrease. 282 Brain Corticospinal tract Sensory (afferent) fibers \ : Spinal cord Muscle \ N i spindle \W re Se %' Motor (efferent) . fibers ee Stretch reflex arc FIGURE 6-20 Selective dorsal rhizotomy (SDR). (From Batshaw ML: Children with developmental disabilities, ed 4. Baltimore, 1997, Paul H. Brookes.) Implantation of a baclofen pump is a neurosurgical procedure. The pump, which is the size of a hockey puck, is placed beneath the skin of the abdomen, and a catheter is threaded below the skin around to the back, where it is inserted through the lumbar spine into the intrathecal space. This placement allows the direct delivery of the medication into the spinal fluid. The medication is stored inside the disk and can be refilled by injection through the skin. It is continuously given, with the dosage adjustable and controlled by a computer (Figure 6-21). According to Brochard et al. (2009), the greatest advantage is the adjustable dosages, with a resulting real decrease in spasticity and the reversibility of the procedure unlike the permanence of SDR. Lower amounts of medication can be given, because the drug is delivered to the site of action, with fewer systemic complications. Intrathecal Baclofen (ITB) therapy is used mostly with children with quadriplegia. Brochard et al. (2009) studied the effects of ITB therapy on gait of children with CP and found that spasticity was decreased and gait capacity measured by the Gillette Functional Assessment Questionnaire significantly increased. 283 PU ; Baclofen pump. FIGURE 6-21 (Courtesy Medtronic, Inc.) Functional Movement Strength and endurance are incorporated into functional movements against gravity and can be repeated continuously over the course of a typical day. Kicking balls, carrying objects of varying weights, reaching overhead for dressing or undressing, pulling pants down and up for toileting, and climbing or walking up and down stairs and ramps can be used to promote strength, endurance, and coordination. Endurance can be promoted by having a child who can ambulate use a treadmill (Figure 6-22) or dance or play tag during recess. Preschool is a great time to foster an appreciation of physical activity that will become a lifetime habit. 284 FIGURE 6-22 Treadmill. Use of positioning can provide a prolonged static stretch. Manual stretching of the muscles most likely to develop contractures should be incorporated into the child’s functional tasks. Positions used while dressing, eating, and sleeping should be reviewed periodically by a member of the therapy team with the child’s parents. Stretching may need to be part of a therapy program in addition to part of the home program conducted by the parents. The evidence suggests that 6 hours of elongation is needed to produce a change in muscle length (Tardieu et al., 1988). The most important positions for a preschooler are standing, lying, and sitting on a chair or on the floor to play. Teachers should be made aware of the importance of varying the child’s position during the day. If a preschooler cannot stand independently, a standing program should be incorporated into the child’s daily routine in the classroom and at home. Such a standing program may well be carried over from a program started when the child was younger. Standing devices are pictured in Chapter 5. Activities of Daily Living and Peer Interaction While the child is in preschool, the ability to perform ADLs may not seem to be an important issue; however, if it takes a child with CP twice as long to toilet than her classmates, what she misses is the social interaction during snack time and when on the playground. Social-emotional development depends on interactions among peers, such as sharing secrets, pretend play, and learning game playing. Making these opportunities available to the child with CP may be one of the most important things we can do in physical therapy because these interactions help form the child’s self-image and social competence. Immobility and slow motor performance can create social isolation. Always take the child’s level of cognitive ability into consideration when selecting a game or activity to incorporate into therapy. If therapy takes place in an outpatient setting, the clinician should plan an activity that will keep the child’s interest and will also accomplish predetermined movement goals. When therapy is incorporated into the classroom, the activity to be carried out by 285 the child may have already been selected by the teacher and will need to address an educational need. The assistant may need to be creative by using an alternative position to assist the child to improve performance within the context of a classroom activity. Some classroom periods such as free play or story time may be more easily adapted for therapeutic intervention. Physical therapy services provided in the school setting must be educationally relevant and address goals on the student’s individual education plan. Young children with CP and limited mobility have a lower frequency of participation in home, school, and community activities (Chiarello et al., 2012). The lower frequency of participation was explained by the child’s physical ability and adaptive behavior; the latter being the biggest determinant. This finding is in keeping with other researches supporting the importance of person- environment interaction as being crucial for children’s participation (Majnemer et al., 2008; Palisano et al., 2011). A list of activities that young children with CP participate in can be found in Table 6-11. Chiarello et al. (2014) confirmed that age and gross motor ability contributed to the frequency and enjoyment of participation by children with CP from age 18 to 60 months. Table 6-11 Activities Participated in by the Highest and Lowest Percentage of Young Children with CP Activit Sample of Activities Percentage Play activities Playing with toys Watching TV or a video Skill development Listening to stories Drawing and coloring Reading or looking at books Taking swimming lessons Participating in community organizations Learning to dance Doing gymnastics Taking music lessons Active physical recreation] Doing team sports Social activities Listening to music Adapted from Chiarello et al: Understanding participation of preschool-age children with cerebral palsy. J Early Intervention 34(1):3-19, 2012. Function in sitting can be augmented by the use of assistive technology such as communication devices and environmental controls. The child can use eye, head, or hand pointing to communicate or to activate other electronic devices. Children with neuromotor dysfunction should also achieve upright orientation to facilitate social interaction. McEwen (1992) studied interactions between students with disabilities and teachers and found that when students with disabilities were in a more upright position, such as sitting on a chair rather than on the floor, the level of interaction increased. Third Stage of Physical Therapy Intervention: School Age and Adolescence During the next two major periods of development, the focus of physical therapy intervention is to safeguard all previous gains. This may be easier said than done because the school-age child may be understandably and appropriately more interested in the school environment and in friends than in physical therapy. Rosenbaum and Gorter (2011) address the need for professionals working with children with CP to recognize the five F’s—function, family, fun, fitness, and friends. School-age children need to experience play, have fun, get fit, have friends, engage in family routines, and plan for the future. By focusing on activities that the school-age child wants to engage in and modifying the task or the environment to allow the child to actively participate, function and fitness can be promoted. Self-Responsibility and Motivation The school-age child should also be taking some degree of responsibility for the therapy program. An exercise record in the form of a calendar may be a way to motivate the younger child to perform exercises on a routine basis. A walking program may be used to work on increasing endurance and cardiovascular fitness. Finding an activity that motivates the student to improve performance may be as simple as timing an obstacle course, increasing the time spent on a treadmill, or improving the number of repetitions. Everyone loves a contest. Find out what important motor task the student wants to accomplish. Can the child carry a tray in the cafeteria (Figure 6-23)? Does she want to be 286 able to dribble a basketball or pedal a bicycle? Be sure it is something the child wants to do. FIGURE 6-23 Carrying a tray. Adolescents are notorious for ignoring adults’ directions, so lack of interest in therapy can be especially trying during this period. However, adolescence can work in favor of compliance with physical therapy goals if the student becomes so concerned about appearances that he or she is willing to work harder to modify a gait deviation or to decrease a potential contracture. Some teenagers may find it more difficult to ambulate the longer distances required in middle school, or they may find that they do not have the physical stamina to carry books and make multiple trips to and from their lockers and still have energy to focus attention in the classroom. Poor endurance in performing routine self-care and personal hygiene functions can cause difficulty as the teen demands more privacy and seeks personal independence while still requiring physical assistance. By being creative, the therapist can help the teen locate recreational opportunities within the community and tailor goals to meet the individual’s needs. Circuit training (Blundell et al., 2003) used with young children with CP found improvements in gait velocity and strength that were maintained after the training ceased. A circuit-training program in the Netherlands (Gorter et al., 2009) demonstrated improved aerobic endurance in children (GMECS level I or II) 8 to 13 years of age after 9 weeks of twice-a-week training, with every session lasting 30 minutes. An interactive video home-based intervention (Bilde et al., 2011) resulted in positive changes in children in sit to stand and step ups in the frontal and sagittal planes as well as endurance. No change in balance, tested using the Romberg, was seen, but visual perceptual abilities significantly increased. The children (GMFCS level I or II) were 6 to 13 years of age and trained about 30 minutes a day with a novel system delivered via the internet. In the first published study using the Wii gaming system, Deutsch et al. (2008) reported that using this system was feasible with an 11-year-old with spastic diplegia at GMFCS level III. Positive changes were 287 documented in postural control, functional mobility, and visual-perceptual processing. The program was carried out in a summer school setting. Physiologic Changes Other great potential hazards to continued independent motor performance are the physical and physiologic changes brought on by adolescence. Greater growth of the lower extremities in relation to the trunk and upper body can produce a less stable gait. Growth spurts in which muscle length does not keep up with changes in bone length can cause problems with static balance and dynamic balance. During periods of rapid growth, bone length may outstrip the ability to elongate of the attached muscles, with resulting potential contracture formation. The development of such contractures may contribute to a loss of independent mobility or to a loss in movement efficiency. In other words, the student may have to work harder to move. Some teens may fall with increasing frequency. Others may limit distances walked in an effort to preserve function or to save energy for school-related tasks and learning. Any change in functional ambulation ability should be reported to the supervising physical therapist so the therapist can evaluate the need for a change in the student’s treatment plan. The student may benefit from a change in either assistive device or orthosis. In some instances, the loss of functional upright ambulation is a real possibility, and a wheelchair evaluation may be warranted. Another difficulty that can arise during this period is related to body mass changes secondary to the adolescent’s growth. Increasing body weight compared with a disproportionately smaller muscle mass in the adolescent with CP can represent a serious threat to continued functional independence. Physical therapy goals during the school years and through adolescence are to: 1. Continue independent mobility. 2. Develop independent ADL and instrumental ADL skills. 3. Foster fitness and development of a positive self-image. 4. Foster community integration. 5. Develop a vocational plan. 6. Foster social interaction with peers. Independence Strength Studies have shown that adolescents with CP can increase strength when they are engaged in a program of isokinetic resistance exercises (MacPhail, 1995). Strengthening has been shown to improve gait and motor skills in adolescents and school-age children with CP (Van den Berg-Emons et al., 1998; Dodd et al., 2002). The programs vary in the frequency of the interventions and overall duration. Gains were shown after a short program (4 weeks) consisting of twice-a-week circuit training in 4- to 8-year-olds (Blundell et al., 2003). Dodd et al. (2003) conducted a randomized clinical trial that showed that 6 weeks of training increased knee extensor and ankle plantar flexor strength. Even better, the results were maintained for 3 months. They suggested that the strength gains were reflected in stair climbing as well as running, jumping, and walking. The use of traditional electrical stimulation or functional electric stimulation (FES) has also been reported in the literature with positive results (Carmick, 1995, 1997; van der Linden, 2008). While therapeutic electrical stimulation has been promoted to improve muscle mass in children with CP, a study by Sommerfelt et al. (2001) concluded that it had no significant effect on gait or motor function in children with spastic diplegic CP. van der Linden (2008) found an increase in dorsiflexion that significantly affected gait kinematics. Strengthening should be a component of a physical therapy program for children with CP. Children with CP are known to have poor muscle endurance as well as poor strength (Damiano, 2003). Fitness Students with physical disabilities, such as CP, are often unable to participate fully in physical education. If the physical education teacher is knowledgeable about adapting routines for students with disabilities, the student may experience some cardiovascular benefits. The neuromuscular deficits affect the ability of a student with CP to perform exercises. Students with CP have higher 288 energy costs for routine activities. Studies done in Canada and Scandinavia have shown improvements in walking speed and other motor skills when students were involved in exercise programs (Bar-Or, 1990). Dresen et al. (1985) showed a reduction in the oxygen cost of submaximal activities after a 10-week training program. More recently, Provost et al. (2007) reported that a statistically significant improvement in walking speed and energy consumption was found in children with CP after an intensive treadmill training using partial body-weight support. These were children already ambulatory as compared with many previous studies done with children who were not ambulatory (Bodkin et al., 2003; Richards et al., 1997). Damiano (2003) recommended that FES-cycling machines be used to promote muscular endurance in children and adolescents with CP. Kurz et al. (2012) reported that a twice-a-week program of BWSTT improved stepping in children with CP but did not improve endurance based on results of a 6-minute walk test. Fitness in all students with disabilities needs to be fostered as part of physical therapy to improve overall health and quality of life. Availability of recreation and leisure activities that are appropriate and accessible are easier to come by than in the past. It is no less important for the individual with a disability to remain physically active and to achieve some degree of health-related fitness than it is for a person without disabilities. In fact, it may be more important for the person with CP to work on aerobic fitness as a way to prevent a decline in ambulation in adulthood. Recreational and leisure activities, sports- related or not, should be part of every adolescent's free time. Swim programs at the YMCA, local fitness club, or elsewhere provide wonderful opportunities to socialize, develop and improve cardiovascular fitness, control weight, and maintain joint and muscle integrity. Recent attention has been given to encouraging children and adolescents with CP to participate in aquatic and martial arts programs to improve movement, balance, and self-esteem. Wheelchair athletics are a good option for school-age children or adolescents in places with junior wheelchair sports programs. Community Integration Accessibility is an important issue in transportation and in providing students with disabilities easy entrance to and exit from community buildings. Accessibility is often a challenge to a teenager who may not be able to drive because of CP. Every effort should be made to support the teenager’s ability to drive a motor vehicle, because the freedom this type of mobility provides is important for social interaction and vocational pursuits. Fourth Stage of Physical Therapy Intervention: Adulthood Physical therapy goals during adulthood are to foster: 1. Independence in mobility and ADLs 2. Healthy lifestyle 3. Community participation 4. Independent living 5. A vocation Even though five separate goals are identified for this stage of rehabilitation, they are all part of the role in life of an adult. Society expects adults to live on their own and to participate within the community where they live and work. This can be the ultimate challenge to a person with CP or any lifelong disability. Living facilities that offer varied levels of assisted living are available in some communities. Adults with CP may live on their own, in group homes, in institutions, or in nursing homes. Some continue to live at home with aging parents or with older siblings. Employment figures from the National Longitudinal Transition Study (Wagner et al., 2006) found that only 40% of young adults with childhood onset disabilities were employed 2 years out of high school, 20% less than same-age peers without disabilities. Despite the focus on transition services for the adolescent with CP, employment has not been a major goal for the adult with CP. Factors that determine the ability of an adult with CP to live and work independently are cognitive status, degree of functional limitations, and adequacy of social and financial support. Family and educators play a significant role in providing the child and adolescent with CP with expectation to participate in work. Clinicians must help the adolescent with CP to transition to adulthood by being aware of and working with vocational rehabilitation services (Huang et al., 2013). Specific services provided by vocational rehabilitation institutes predicted employment outcomes as: (1) use of rehabilitation assistive technology; (2) on-the-job support; (3) job placement assistance; (4) on-the- 289 job training; and (5) support services for basic living. Early prior planning between therapist and vocational counselor can provide a foundation for later employment (Vogtle, 2013.) Future Directions Two studies have used functional magnetic resonance imaging (fMRI) to document changes in the brain related to treadmill training. Kurz et al. (2012) used magnetoencephalography (MEG) to study if BWSTT would alter the neuromagnetic activity in the sensorimotor cortices that represent the foot in children with CP. They found that the neuromagnetic responses representing the foot were weakened after 6 weeks of BSWTT. Theirs was only the second study to look at how exercise altered the activation of the sensorimotor cortices. Phillips et al. (2007) demonstrated a change in ankle dorsiflexion after intensive treadmill training. Sensorimotor experiences have been theorized to drive motor behavior through reorganization of the brain (Anderson et al., 2014). Activity-focused interventions have the potential to produce changes in children with CP that go beyond preventing musculoskeletal impairments and maximizing physical function. Activity can affect neural structures and pathways (Damiano, 2006). Chapter summary The child with CP presents the physical therapist and the physical therapist assistant with a lifetime of opportunities to assist in attaining meaningful functional goals. These goals revolve around the child’s achievement of some type of mobility and mastery of the environment, including the ability to manipulate objects, to communicate, and to demonstrate as much independence as possible in physical, cognitive, and social functions. The needs of the child with CP and her family change in relation to the child’s maturation and reflect the family’s priorities at any given time. Physical therapy may be one of many therapies the child receives. Physical therapists and physical therapist assistants are part of the team working to provide the best possible care for the child within the context of the family, school, and community. Regardless of the stage of physical therapy management, families need to be empowered to be an integral part of informed decision-making. Goals need to be meaningful and based on what the child needs to learn to do in order to participate meaningfully in life. Activities that promote fitness must be part of physical therapy interventions for adolescents and adults with CP. The long-term goal must always be to optimize movement, promote the parent—infant and parent-child relationship, and expand sensorimotor and perceptual experiences to support cognition and plan to fully engage in all aspects of adult life. Every child with CP deserves an optimal quality of life. Review questions 1. Why may the clinical manifestations of CP appear to worsen with age even though the pathologic features are static? 2. Name the two greatest risk factors for CP. 3. What is the most common type of abnormal tone seen in children with CP? 4. How may abnormal tonic reflexes interfere with acquisition of movement in a child with CP? 5. Compare and contrast the focus of physical therapy intervention in a child with spastic CP and in a child with athetoid CP. 6. What is the role of the physical therapist assistant when working with a preschool-age child with Cr? 7. What type of orthosis is most commonly used by children with CP who ambulate? 8. At what age should a child with CP begin to take some responsibility for the therapy program? 9. What medications are used to manage spasticity in children with CP? 10. What are the expected life outcomes that should be used as a guide for goal setting with children with disabilities? 290 Case Studies Rehabilitation Unit Initial Examination and Evaluation: JC History Chart Review JC is a 6-year-old girl with moderate spastic diplegic CP (GMFCS Level III). She was born at 28 weeks of gestation, required mechanical ventilation, and sustained a left intraventricular hemorrhage. She received physical therapy as part of an infant intervention program. She sat at 18 months of age. At 3 years of age, she made the transition into a school-based preschool program. She had two surgical procedures for heel cord tendon transfers and adductor releases of the hips. She is now making the transition into a regular first grade. JC has a younger sister. Both parents work. Her father brings her to weekly outpatient therapy. JC goes to day care or to her grandparents’ home after school. Subjective JC’s parents are concerned about her independence in the school setting. Objective Systems Review Communication/Cognition: JC communicates easily and appropriately. Her intelligence is within the normal range. Cardiovascular/Pulmonary: Normal values for age. Integumentary: Intact Musculoskeletal: AROM and strength intact in the upper extremities but impaired in the trunk and lower extremities. Neuromuscular: Coordination within functional limits in the upper extremity, but impaired in the lower extremities. Tests and Measures Anthropometrics: Height 46 inches, Weight 45 lbs, BMI 15 (20-24 is normal). Motor Function: JC can roll to either direction and can achieve sitting by pushing up from side lying. She can get into a quadruped position from prone and can pull herself into kneeling. She attains standing by moving into half-kneeling with upper extremity support. She can come to stand from sitting in a straight chair without hand support but adducts her knees to stabilize her legs. Neurodevelopmental Status: Peabody Developmental Motor Scales (PDMS) Developmental Motor Quotient (DMQ) = 69, with an age equivalent of 12 months. Fine-motor development is average for her age (PDMS DMQ = 90). 291 Range of Motion R L R L a a 0 bes! 0 0 ° ee a Reflex Integrity: Patellar 3 +, Achilles 3 +, Babinski present bilaterally. Moderately increased tone is present in the hamstrings, adductors, and plantar flexors bilaterally. Posture: JC demonstrates a functional scoliosis with the convexity to the right. The right shoulder and pelvis are elevated. JC lacks complete thoracic extension in standing. The pelvis is rotated to the left in standing. Leg length is 23.5 inches bilaterally, measured from ASIS to medial malleolus. Muscle Performance: Upper extremity strength appears to be WFL because JC can move her arms against gravity and take moderate resistance. Lower extremity strength is difficult to determine in the presence of increased tone but is generally less than fair with the left side appearing to be stronger than the right. Gait, Locomotion, and Balance: JC ambulates independently 15 feet using a reverse-facing 292 walker while wearing solid polypropylene AFOs. She can take five steps independently without a device before requiring external support for balance. She goes up and down stairs, alternating feet using a handrail. She can maneuver her walker up and down a ramp and a curb with stand by assist. JC requires stand-by assistance to move about with her walker in the classroom and when getting up and down from her desk. Incomplete trunk righting is present with any displacement in sitting. No trunk rotation present with lateral displacements in sitting. Upper extremity protective reactions are present in all directions in sitting. JC stands alone for 3 to 4 minutes every trial. She exhibits no protective stepping when she loses her balance in standing. Sensory Integrity: Intact. Self-care: JC is independent in eating and in toileting with grab bars. She requires moderate assistance with dressing secondary to balance. Play: JC enjoys reading Junie B. Jones books and playing with dolls. Assessment/evaluation JC is a 6-year-old girl with moderately severe spastic diplegic CP. She is independently ambulatory with a reverse-facing walker and AFOs for short distances on level ground. She is at GMFCS level III. She attends a regular first grade class. She is seen for outpatient physical therapy once a week for 45 minutes. Problem List 1. Dependent in ambulation without an assistive device 2. Impaired strength and endurance to perform age-appropriate motor activities 3. Impaired dynamic sitting and standing balance 4. Dependent in dressing Diagnosis JC exhibits impaired motor function associated with nonprogressive disorders of the CNS— congenital origin, which is guide pattern 5C. This pattern includes CP. Prognosis JC will improve her functional independence and functional skills in the school setting. Her rehabilitation potential for the following goals is good. Short-Term Goals (actions to be achieved by midyear review) 1. JC will ambulate independently within her classroom. 2. JC will perform weight shifts in standing while throwing and catching a ball. 3. JC will walk on a treadmill with arm support for 10 consecutive minutes. 4. JC will ambulate 25 feet without an assistive device three times a day. 5. JC will don and doff AFOs, shoes, and socks, independently. Long-Term Goals (end of first grade) 1. JC will ambulate independently without an assistive device on level surfaces. 2. JC will be able to go up and down a set of three stairs, step over step, without holding on to a railing. 3. JC will walk continuously for 20 minutes without resting. 4. JC will dress herself for school in 15 minutes. Plan Coordination, Communication, and Documentation The physical therapist and physical therapist assistant will be in frequent communication with JC’s family and teacher regarding her physical therapy program. Outcomes of interventions will be documented on a weekly basis. Patient/Client Instruction JC and her parents will be given suggestions to assist her in becoming more independent at home, such as getting clothes out the night before and getting up early enough to complete the dressing tasks before leaving for school. JC and her family will be instructed in a home exercise program consisting of stretching and strengthening. A reminder calendar will assist her in remembering to perform her exercises four times a week. Procedural Interventions Increase dynamic trunk postural reactions by using a movable surface to shift her weight and to facilitate responses in all directions. 1. Practice coming to stand while sitting astride a bolster. One end of the bolster can be placed ona 293 stool of varying height to decrease the distance needed for her to move from sitting to standing. Begin with allowing her to use hand support and then gradually withdraw it. 2. Practice stepping over low objects, first with upper extremity support followed by gradual withdrawal of support; next practice stepping up and down one step without the railing while giving manual support at the hips. 3. Walk at a slow speed on a treadmill using hand support for 5 minutes. Gradually increase the time. Once she can tolerate 15 minutes, begin to increase speed. 4. Time her ability to maneuver an obstacle course involving walking, stepping over objects, moving around objects, going up and down stairs, and throwing a ball and beanbags. Monitor and track her personal best time. Vary the complexity of the tasks involved, according to how efficient she is at completing them. Follow-up JC is now 12 years old. Secondary to rapid growth, especially in her lower extremities and extensive hip and knee flexion contractures, she is once again ambulating with a reverse-facing wheeled walker. She is able to stand independently for 5 seconds and to take 13 steps before falling or requiring external support. She has been evaluated for surgical releases, but the gait studies indicate significant lower extremity weakness and increased cocontraction of these muscles during gait. The orthopedist believes that she would not have sufficient strength to ambulate following surgery. Physical therapy goals are to increase hip and knee range of motion, gluteus maximus, quadriceps, and ankle musculature strength and to regain the ability to ambulate independently without an assistive device. What treatment interventions could be used to attain these functional goals? Questions to think about = What interventions could be part of JC’s home exercise program? = How can fitness be incorporated into her physical therapy program? 294 References Accardo PJ, editor: Capute & Accardo's neurodevelopmental disabilities in infancy and childhood, vol 1, ed 3, Baltimore, 2008, Paul H. Brookes. 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A systematic review of the effectiveness of treadmill training for children with cerebral palsy. Disabil Rehabil. 2009;31(24):1971-1979. Willoughby KL, Dodd KJ, Shields N, Foley S. Efficacy of partial body weight-supported treadmill training compared with overground walking practice for children with cerebral palsy: a randomized clinical trial. Arch Phys Med Rehabil. 2010;91:333-339. Wilson-Costello DE, Friedman H, Minich N, Fanaroff AA, Hack M. Improved survival rates with increased neurodevelopmental disability for extremely low birth weight infants in the 1990s. Pediatrics. 2005;115(4):997-1003. Yin Foo R, Guppy M, Johnston LM. Intelligence assessments for children with cerebral palsy: a systematic review. Dev Med Child Neurol. 2013;55(10):911-918. 299 CHAPTER 7 300 Myelomeningocele Objectives After reading this chapter, the student will be able to: 1. Describe the incidence, prevalence, etiology, and clinical manifestations of myelomeningocele. 2. Describe common complications seen in children with myelomeningocele. 3. Discuss the medical and surgical management of children with myelomeningocele. 4. Articulate the role of the physical therapist assistant in the treatment of children with myelomeningocele. 5. Describe appropriate interventions for children with myelomeningocele. 6. Recognize the importance of functional training throughout the life span of a child with myelomeningocele. 301 Introduction Myelomeningocele (MMC) is a complex congenital anomaly. Although it primarily affects the nervous system, it secondarily involves the musculoskeletal and urologic systems. MMC is a specific form of myelodysplasia that is the result of faulty embryologic development of the spinal cord, especially the lower segments. The caudal end of the neural tube or primitive spinal cord fails to close before the 28th day of gestation (Figure 7-1, A). Definitions of basic myelodysplastic defects can be found in Table 7-1. Accompanying the spinal cord dysplasia (abnormal tissue growth) is a bony defect known as spina bifida, which occurs when the posterior vertebral arches fail to close in the midline to form a spinous process (Figure 7-1, C to E). The normal spine at birth is seen in Figure 7-1, B. The term spina bifida is often used to mean both the bony defect and the various forms of myelodysplasia. When the bifid spine occurs in isolation, with no involvement of the spinal cord or meninges, it is called spina bifida occulta (see Figure 7-1, C). Usually, no neurologic impairment occurs in persons with spina bifida occulta. The area of skin over the defect may be marked by a dimple or tuft of hair and can go unnoticed. In spina bifida cystica, patients have a visible cyst protruding from the opening caused by the bony defect. The cyst may be covered with skin or meninges. This condition is also called spina bifida aperta, meaning open or visible. If the cyst contains only cerebrospinal fluid (CSF) and meninges, it is referred to as a meningocele because the “cele” (cyst) is covered by the meninges (see Figure 7-1, D). When the malformed spinal cord is present within the cyst, the lesion is referred to as a myelomeningocele (see Figure 7-1, E). In MMC, the cyst may be covered with only meninges or with skin. Motor paralysis and sensory loss are present below the level of the MMC. The most common location for MMC is in the lumbar region. NORMAL EMBRYONIC DEVELOPMENT Neural plate Neural told Neural groove Neural tube closed 8 a ay e A e NORMAL SPINE AT BIRTH SPINA BIFIDA OCCULTA Tuft of hair Incompiete vertebra SPINA BIFIDA CYSTICA SPINA BIFIDA CYSTICA Myolomoningocete FIGURE 7-1 Types of spina bifida. A, Normal formation of the neural tube during the first month of gestation. B, Complete closure with normal development in cross-section on the left and in longitudinal section on the right. C, Incomplete vertebral closure with no cyst, marked by a tuft of hair. D, Incomplete vertebral closure with a cyst of meninges and cerebrospinal fluid (CSF)—meningocele. E, Incomplete vertebral closure with a cyst containing a malformed spinal cord—myelomeningocele. Table 7-1 Basic Definitions of Myelodysplastic Defects 302 Defect Definition Spina bifida Vertebral defect in which posterior elements of the vertebral arch fail to close; no sac; vertebral defect usually not associated with an abnormality of the spinal cord occulta Spina bifida Vertebral defect with a protruding cyst of meninges or spinal cord and meninges cystica Meningocele Cyst containing cerebrospinal fluid and meninges and usually covered with epithelium; clinical symptoms variable Myelomeningocele| Cyst containing cerebrospinal fluid, meninges, spinal cord, and possibly nerve roots; cord incompletely formed or malformed; most common in the lumbar area; the higher the lesion, the more deficits present Adapted from Ryan KD, Ploski C, Emans JB: Myelodysplasia: The musculoskeletal problem: Habilitation from infancy to adulthood. Phys Ther 71:935-946, 1991. With permission of the American Physical Therapy Association. 303 Incidence The incidence of MMC has declined over the last decade due to better nutrition and increased screening. MMC is the most common neural tube defect (NTD). About 1500 babies are born annually in the United States with MMC. Incidence appears to be stable at 3.4 per 10,000 live births (Boulet et al., 2008). If a sibling has already been born with MMC, the risk of recurrence in the family is 2% to 3%. Worldwide incidence of all NTDs occurs at a rate of 0.17 to 6.39 per 1000 live births (Bowman et al., 2009a). These figures include defects of closure of the neural tube at the cephalic end, as well as in the thoracic, lumbar, and sacral regions. One province in China has reported a very high prevalence of NTDs (Li et al., 2006). Prevalence is the number of people with a disorder in a population. The lack of closure cephalically results in anencephaly, or failure of the brain to develop beyond the brain stem. These infants rarely survive for any length of time after birth. An encephalocele results when the brain tissue protrudes from the skull. It usually occurs in the occipital and results in visual impairment. Prevalence of NTDs is highest in Hispanic people (4.17 per 10,000), followed by non-Hispanic whites (3.22 per 10,000) and finally non-Hispanic blacks (2.64 per 10,000) (Centers for Disease Control and Prevention [CDC], 2010). 304 Etiology Many factors have been implicated in spina bifida and MMC, but no definitive cause has been identified (Fenichel, 2009). More than likely, the cause is a combination of environmental and genetic factors. Following mandatory fortification of food with folic acid, there has been a 31% decrease in prevalence of MMC in the U.S. (Boulet et al., 2008). It is recommended that a woman with a history of having had a child with an NTD takes 4 mg of folic acid a day at least a month before conception and throughout the first trimester (Fenichel, 2009). Additional factors that may play a role in MMC are exposure to alcohol (Main and Mennuti, 1986), certain seizure or acne medications (Ornoy, 2006), and being obese (Shaw et al., 2003). Some genetic disorders, such as trisomy 13 and trisomy 18, have been associated with MMC (Luthy et al., 1991), and a few genes have been identified that may play a role in MMC (Copp and Greene, 2010). 305 Prenatal diagnosis A neural tube defect can be diagnosed prenatally by testing for levels of alpha-fetoprotein. If levels of the protein are too high, it may mean that the fetus has an open NTD. This suspicion can be confirmed by high-resolution ultrasonography to visualize the vertebral defect. When an open NTD is detected, the infant should be delivered by cesarean section before labor begins in order to decrease the risk of central nervous system infection and to minimize trauma to the spinal cord during the delivery process. This practice has decreased the trauma (Hinderer et al., 2012). Testing for levels of acetylcholinesterase from amniotic fluid is more accurate than testing alpha-fetoprotein because it can detect a closed NTD. Chromosome analysis of cells in the amniotic fluid can confirm if there is an associated chromosome error and provide more information to parents who are considering terminating the pregnancy. Because of improved medical care, the prevalence of MMC in the population has increased even though the likelihood of having an infant with MMC has declined. Fetal surgery to repair the defect in MMC has been performed in selected centers since 2003 (Walsh and Adzick, 2003; Tulipan, 2003). The goal of the intrauterine surgery is to decrease the need for placing a shunt for hydrocephalus, which typically develops after closure of the MMC, and to improve lower extremity function. In the recent randomized control trial of prenatal versus postnatal repair, fetal surgery was performed before 26 weeks of gestation (Adzick et al., 2011). The Management of Myelomeningocele Study (MOMS) compared the efficacy and safety between the standard postnatal repair and prenatal repair. The study was halted because the efficacy of the prenatal repair was proven. The need for shunt surgery was reduced, and improved motor outcomes were demonstrated at 30 months in the group who had prenatal surgical repair. Despite the associated maternal and fetal risks, the outcomes support prenatal repair. 306 Clinical features Neurologic Defects and Impairments The infant with MMC presents with motor and sensory impairments as a result of the spinal cord malformation. The extent of the impairment is directly related to the level of the cyst and the level of the spinal cord defect. Unlike in complete spinal cord injuries, which have a relatively straightforward relationship between the level of bony vertebra involvement and the underlying cord involvement, no clear relationship is present in infants with MMC. Some bony defects may involve more than one vertebral level. The spinal cord may be partially formed or malformed, or part of the spinal cord may be intact at one of the involved levels and may have innervated muscles below the MMC. If the nerve roots are damaged or the cord is dysplastic, the infant will have a flaccid type of motor paralysis with lack of sensation, the classic lower motor neuron presentation. However, if part of the spinal cord below the MMC is intact and has innervated muscles, the potential exists for a spastic type of motor paralysis. In some cases, the child may actually demonstrate an area of flaccidity at the level of the MMC, with spasticity present below the flaccid muscles. Either type of motor paralysis presents inherent difficulty in managing range of motion and in using orthoses for ambulation. Functional Movement Related to Level In general, the higher the level of the lesion, the greater the degree of muscular impairment and the less likely the child will ambulate functionally. A child with thoracic involvement at T12 has some control of the pelvis because of the innervation of the quadratus and complete innervation of the abdominal muscles. The gluteus maximus would not be active because it is innervated by L5 to S1. A high lumbar level lesion (L1 to L2) affects the lower extremities, but hip flexors and hip adductors are innervated. A midlumbar level lesion at L3 means that the child can flex at the hips and can extend the knees but has no ankle or toe movement. In a low lumbar level of paralysis at L4 or L5, the child adds the ability to flex the knees and dorsiflex the ankles, but only weakly extend the hips. Children with sacral level paralysis at S1 have weak plantar flexion for push-off and good hip abduction. To be classified as having an S2 or S3 level lesion, the child’s plantar flexors must have a muscle grade of at least 3/5 and the gluteal muscles a grade of 4/5 on a manual muscle test scale (Hinderer et al., 2012). The lesion is considered “no loss” when the child has normal function of bowel and bladder and normal strength in the lower extremity muscles. Musculoskeletal Impairments Muscle paralysis results in an impairment of voluntary movement of the trunk and lower extremities. Children with the classic lower motor neuron presentation of flaccid paralysis have no lower extremity motion, and the legs are drawn into a frog-leg position by gravity. Because of the lack of voluntary movement, the lower extremities assume a position of comfort—hip abduction, external rotation, knee flexion, and ankle plantar flexion. Table 7-2 provides a list of typical deformities caused by muscle imbalances seen with a given level of lesion. Rather than memorizing the table, one would be better served to review the appropriate anatomy and kinesiology and determine in what direction the limbs would be pulled if only certain muscles were innervated. For example, if there was innervation of only the anterior tibialis (L4 motor level) with no opposing pull from the gastrocnemius or posterior tibialis, in what position would the foot be held? It would be pulled into dorsiflexion and inversion, resulting in a calcaneovarus foot posture. In this situation, what muscle is most likely to become shortened? This may be one of the few instances in which the anterior tibialis needs to be stretched to maintain its resting length. Table 7-2 Function Related to Level of Lesion Level of Lesion Muscle Function Potential Deformi' Thoracic Trunk weakness Positional deformities of hips, knees, and ankles secondary to frog-leg T7-T9 upper abdominals posture T9-T12 lower abdominals T12 has weak quadratus lumborum 307 High lumbar (L1-_| Unopposed hip flexors and some adductors Hip flexion, adduction L2) Hip dislocation Lumbar lordosis Knee flexion and plantar flexion Midlumbar (L3) Strong hip flexors, adductors Hip dislocation, subluxation Weak hip rotators Genu recurvatum Antigravity knee extension Low lumbar (L4) Strong quadriceps, medial knee flexors against gravity, ankle dorsiflexion and Equinovarus, calcaneovatus, or calcaneocavus foot inversion Low lumbar (L5) Weak hip extension, abduction Equinovarus, calcaneovalgus, or calcaneocavus foot Good knee flexion against gravity Weak plantar flexion with eversion Sacral (S1) Good hip abductors, weak plantar flexors = Sacral (S2-S3 Good hip extensors and ankle plantar flexors = The child with MMC may also have congenital lower limb deformities, in addition to being at risk of acquiring additional deformities because of muscle imbalances. These deformities may include hip dislocation, hip dysplasia and subluxation, genu varus, and genu valgus. Congenital foot deformities associated with MMC are talipes equinovarus or congenital clubfoot, pes equinus or flatfoot, and convex pes valgus or rocker-bottom foot, with a vertical talus. These are depicted in Figure 7-2. Clubfoot is the most common foot deformity seen in children with MMC who have an L4 or L5 motor level (Tappit-Emas, 2008). The physical therapist may perform taping and gentle manipulation during the early management of this foot problem. The physical therapist assistant may or may not be involved with providing gentle corrective range of motion. Because of pressure problems over the bony prominences, splinting is recommended instead of serial casting. Surgical correction of the foot deformity is probably indicated in all but the mildest cases (Tappit-Emas, 2008). CLUBFOOT: EQUINOVARUS CALCANEOVALGUS VERTICAL TALUS i C FIGURE 7-2 Common lower extremity deformities. Most children with MMC begin to ambulate between 1 and 2 years of age. A plantigrade foot, one that can be flat and in contact with the ground, is essential to ensure ambulation. In addition, the foot needs to be able to exhibit 10 degrees of dorsiflexion for toe clearance. This does not, however, 308 have to be active range. If the child has a spastic type of motor paralysis, limb movements may result from muscle spasms, but such movements are not under the child’s voluntary control. Various limb positions may result, depending on which muscles are spastic. The deforming forces will be stronger if spasticity is present. For example, in a child with an L1 or L2 motor level, the hip flexors and adductors may pull so strongly because of increased tone that the hip is dislocated. Muscle imbalances due to the level of innervation may be intensified by increased tone. Osteoporosis As in adults with spinal cord injury, the loss of the ability to produce a muscle contraction is devastating for voluntary movement, but it also has ramifications for the ongoing development and function of the skeletal system. The skeletal system, including the long bones and axial skeleton, depends on muscle pull and weight bearing to maintain structural integrity and to help balance normal bone loss with new bone production. Children, like adults with spinal cord injury, are at risk of developing osteoporosis (Hinderer et al., 2012). Osteoporosis predisposes a bone to fracture; therefore, children with MMC are at greater risk of developing fractures secondary to loss of muscle strength and inactivity (Dosa et al., 2007). Researchers have found that children who are household or community ambulators have higher bone mineral density than children who walk only therapeutically (Rosenstein et al., 1987). The reader is referred to Chapter 12 for the definition of the various levels of ambulation. Walking ability is a significant determinant of bone density in children with MMC (Ausili et al., 2008). A recent review found that the risk of low bone mineral density and fractures was related to higher neurologic levels, inactivity, previous spontaneous fracture, not walking, and contractures (Marrieos et al., 2012). With aging, there is a risk for developing Charcot joints (Nagarkatti et al., 2000). A Charcot joint is a joint deformity caused by a condition involving the spinal cord. The joint is painful and unstable. Neuropathic Fractures Twenty percent of children with MMC are likely to experience a neuropathic fracture (Lock and Aronson, 1989). Neuropathic fractures relate to the underlying neurologic disorder. Paralyzed muscles cannot generate forces through long bones, so that essentially no weight bearing takes place, with resulting osteoporosis. Osteoporosis makes it easier for the bone to fracture. Low bone density for age is strongly related to risk for fractures (Szalay and Cheema, 2011). Possible causes of neuropathic fractures in this population include overly aggressive therapeutic exercise and lack of stabilization during transfers (Garber, 1991). Prolonged immobilization following surgery can also predispose the child to pathologic fractures. Proper nutrition is always important but even more so if the child is taking seizure medications that disrupt the metabolism of vitamin D and calcium. The following clinical example illustrates another possible situation involving a neuropathic fracture. Once, when placing the lower extremities of a child with MMC into his braces, a clinician felt warmth along the child’s tibial crest. The child was biracial, so no redness was apparent, but a definite separation was noted along the tibia. The child was in no pain or distress. His mother later recounted that it had been particularly difficult to put his braces on the day before. A radiograph confirmed the therapist’s clinical suspicion that the child had a fracture. The limb was put in a cast until the fracture healed. While the child was in his cast, therapy continued, with an emphasis on upper extremity strengthening and trunk balance. Presence of a cast protecting a fracture is usually not an indication to curtail activity in children with MMC. In fact, it may spark creativity on the part of the rehabilitation team to come up with ways to combat postural insecurity and loss of antigravity muscle strength while the child’s limb is immobilized. Spinal Deformities Children with MMC can have congenital or acquired scoliosis. Congenital scoliosis is usually related to vertebral anomalies, such as a hemivertebra, that are present in addition to the bifid spine. This type of scoliosis is inflexible. Acquired scoliosis results from muscle imbalances in the trunk, producing a flexible scoliosis. A rapid onset of scoliosis can also occur secondary to a tethered spinal cord or to a condition called hydromyelia. These conditions are explained later in the text. The physical therapist assistant must be observant of any postural changes in treating a child with 309 MMC. Acquired scoliosis should be managed by some type of orthosis until spinal fixation with instrumentation is appropriate. Children with MMC go through puberty at a younger age than typically developing children, and this allows for earlier spinal surgery with little loss of the child’s mature trunk height. Other spinal deformities, such as kyphosis and lordosis, may also be seen in these children. The kyphosis may be in the thoracic area or may encompass the entire spine, as seen in a baby. The lordosis in the lumbar area may be exaggerated or reversed. Spinal deformities of all kinds are more likely to be present in children with higher-level lesions. Spinal alignment and potential for deformity must always be considered when one uses developmentally appropriate positions, such as sitting and standing. If the child cannot maintain trunk alignment muscularly, then some type of orthosis may be indicated. The child’s sitting posture should be documented during therapy, and sitting positions to be used at home should be identified. Spinal deformities may not always be preventable, but attention must be paid to the effect of gravity on a malleable spine when it is in vulnerable developmental postures. Arnold-Chiari Malformation In addition to the spinal cord defect in MMC, most children with this neuromuscular problem have an Arnold-Chiari type II malformation. The Arnold-Chiari malformation involves the cerebellum, the medulla, and the cervical part of the spinal cord (Figure 7-3). Because the cerebellum is not fully developed, the hindbrain is downwardly displaced through the foramen magnum. The flow of CSF is obstructed, thus causing fluid to build up within the ventricles of the brain. The abnormal accumulation of CSF results in hydrocephalus, as shown in Figure 7-3. A child with spina bifida, MMC, and an Arnold-Chiari type II malformation has a greater than 90% chance of developing hydrocephalus. The Arnold-Chiari type II malformation may also affect cranial nerve and brain stem function because of the pressure exerted on these areas by the accumulation of CSF within the ventricular system. Clinically, this involvement may be manifested by swallowing difficulties. | | | —S Aqueduct \t ne eT Aqueduct * ia ~ —7/— Cerebellum — Fourth ventricle ; Wot Fourth ventricle YA) ‘ Spinal cord Cerebral tonsils e Cerebral tonsils ae Spinal cord BRAIN STEM Mesencephaion (midbrain) Pons Medulla A B FIGURE 7-3 A, Normal brain with patent cerebrospinal fluid (CSF) circulation. B, Arnold-Chiari type II malformation with enlarged ventricles, a condition that predisposes a child with myelomeningocele to hydrocephalus. The brain stem, the fourth ventricle, part of the cerebellum, and the cerebral tonsils are displaced downward through the foramen magnum, and this leads to blockage of CSF flow. Additionally, pressure on the brain stem housing the cranial nerves may result in nerve palsies. (From Goodman CC, Boissonnault WG, Fuller KS: Pathology: implications for the physical therapist, St. Louis, 2015, WB Saunders.) Hydrocephalus Hydrocephalus can occur in children with MMC with or without the Arnold-Chiari malformation. Hydrocephalus is treated neurosurgically with the placement of a ventriculoperitoneal shunt, which drains excess CSF into the peritoneal cavity (Figure 7-4). You will be able to palpate the shunt tubing along the child’s neck as it goes under the clavicle and down the chest wall. All shunt systems have a one-way valve that allows fluid to flow out of the ventricles but prevents backflow. The child’s movements are generally not restricted unless such restriction is specified by the physician. However, the child should avoid spending prolonged periods of time in a head-down position, such as hanging upside down, because this may disrupt the valve function or may interfere with the flow of the fluid (Williamson, 1987). Knowledge of signs of shunt malfunction is 310 important when working with children with MMC. “Approximately 40% of new shunts fail within a year, and 80% fail within 10 years” (Sandler, 2010, p. 890). Za * FIGURE 7-4 A ventriculoperitoneal shunt provides primary drainage of cerebrospinal fluid from the ventricles to an extracranial compartment, usually either the heart or the abdominal or peritoneal cavity, as shown here. Extra tubing is left in the extracranial site to uncoil as the child grows. A unidirectional valve designed to open at a predetermined intraventricular pressure and to close when the pressure falls below that level prevents backflow of fluid. (From Goodman CC, Boissonnault WG, Fuller KS: Pathology: implications for the physical therapist, St. Louis, 2015, WB Saunders.) Shunts can become blocked or infected, so the clinician must be aware of signs that could indicate shunt malfunction. These signs are listed in Table 7-3. Ninety-five percent of children with shunts will have at least one shunt revision (Bowman et al., 2001). Many of the signs and symptoms, such as irritability, seizures, vomiting, and lethargy, are seen regardless of the age of the child. Other signs are unique to the age of the child. Infants may display bulging of the fontanels secondary to increased intracranial pressure. The sunset sign of the eyes refers to the finding that the iris is only partially visible because of the infant’s downward gaze. Older children may exhibit personality or memory changes. Shunt malfunction can occur years after implantation even without symptoms (Tomlinson and Sugarman, 1995). Table 7-3 Signs and Symptoms of Shunt Malfunction 311 Sign or Symptom Infants Toddlers School-Age Children Excessive rate of growth of head circumference a Memory changes Central Nervous System Deterioration In addition to being vigilant about watching for signs of shunt malfunction as the child grows, the clinician must investigate any change in motor and sensory status or functional abilities because it may indicate neurologic deterioration. Common causes of such deterioration are hydromyelia and a tethered spinal cord. All areas of the child’s function, such as mobility, activities of daily living (ADLs), and school performance, can be affected by either of these two conditions. Hydromyelia Hydromyelia is characterized by an accumulation of CSF in the central canal of the spinal cord. The condition can cause rapidly progressing scoliosis, upper extremity weakness, and increased tone (Long and Toscano, 2001). Other investigators have reported sensory changes (Ryan et al., 1991) and ascending motor loss in the lower extremities (Krosschell and Pesavento, 2013). The incidence of hydromyelia in children with MMC ranges from 20% to 80% (Byrd et al., 1991). Any time a child presents with rapidly progressing scoliosis, alert your supervising therapist, who will inform the child’s physician so that the cause of the symptoms can be investigated and treated quickly. Scoliosis in this disorder is often an indication of a progressing neurologic problem. Tethered Spinal Cord The relationship of the spinal cord to the vertebral column normally changes with age. At birth, the end of the spinal cord is at the level of L3, rising to L1 in adulthood as a result of skeletal growth. Because of scarring from the surgical repair of the back lesion, adhesions can form and can anchor the spinal cord at the lesion site. The spinal cord is then tethered and is not free to move upward within the vertebral canal as the child grows. Progressive neurologic dysfunction, such as a decline in motor and sensory function, pain, or loss of previous bowel and bladder control, may occur. Other signs may include rapidly progressive scoliosis, increased tone in the lower extremities, and changes in gait pattern. Clinical signs are most commonly seen between the ages of 6 and 12 (Sandler, 2010). Prompt surgical correction can usually prevent any permanent neurologic damage and relieve pain (Schoenmakers et al., 2003; Bowman et al., 2009b). Any deterioration in 312 neuromuscular or urologic performance from the child’s baseline or the rapid onset of scoliosis should immediately be reported to the supervising physical therapist. Sensory Impairment Sensory impairment from MMC is not as straightforward in children as it is in adults with a spinal cord injury. The sensory losses exhibited by children are less likely to correspond to the motor level of paralysis. Do not presume that because one part of a dermatome is intact, the entire dermatome is intact to sensation. “Skip” areas that have no sensation may be present within an innervated dermatome (Hinderer et al., 2012). Often, the therapist has tested for only light touch or pinprick, because the child with MMC is usually unable to differentiate between the two sensations. If the therapist has tested for vibration, intact areas of sensation may be present below those perceived as insensate for either light touch or pinprick (Hinderer and Hinderer, 1990). The functional implications of loss of sensation are enormous. An increased potential exists for damaging the skin and underlying tissue secondary to extremes of temperature and normal pressure. A child with MMC loses the ability to feel that he has too much pressure on the buttocks from sitting too long. This loss of sensation can lead to the development of pressure ulcers. The consequences of loss of time from school and play and of independent function because of a pressure ulcer can be immeasurable. The plan of care must include teaching skin safety and inspection as well as pressure-relief techniques. These techniques are essential to good primary prevention of complications. The use of seat cushions and other joint protective devices is advised. Insensitive skin needs to be protected as the child learns to move around and explore the environment. The family needs to be made aware of the importance of making regular skin inspection part of the daily routine. As the child grows and shoes and braces are introduced, skin integrity must be a high priority when one initiates a wearing schedule for any orthotic devices. Bowel and Bladder Dysfunction Most children with MMC have some degree of bowel and bladder dysfunction. The sacral levels of the spinal cord, 52 to 54, innervate the bladder and are responsible for voiding and defecation reflexes. With loss of motor and sensory functions, the child has no sensation of bladder fullness or of wetness. The reflex emptying and the inhibition of voiding can be problematic. If tone in the bladder wall is increased, the bladder cannot store the typical amount of urine and empties reflexively. Special attention must be paid to the treatment of urinary dysfunction because mismanagement can result in kidney damage. By the age of 3 or 4 years, most children begin to work on gaining urinary continence by using clean intermittent catheterization (CIC). By 6 years, the child should be independent in self-intermittent catheterization (SIC). Functional prerequisites for this skill include sitting balance with no hand support and the ability to do a toilet transfer. These functional activities should be incorporated into early and middle stages of physical therapy management. Latex Allergy It has been estimated that up to 50% of children with MMC are allergic to latex (Cremer et al., 2002; Sandler, 2010). This may be because the infant with MMC is exposed repeatedly to latex products. Exposure to latex can produce an anaphylactic reaction that can be life-threatening (Dormans et al., 1995), with the risk increasing as the child gets older (Mazon et al., 2000). All contact with latex products should be avoided from the beginning, including catheters, surgical gloves, and Theraband. Any surgery should be performed in a latex-free environment. Toys that contain latex, such as rubber balls and balloons, should be avoided. With the concentrated effort to avoid all latex, children born more recently have lower rates of latex sensitivity (Blumchen et al., 2010). 313 Physical therapy intervention Three stages of care are used to describe the continuum of physical therapy management of the child with myelodysplasia. Although similarities exist between adults with spinal cord injuries and children with congenital neurologic spinal deficits, inherent differences are also present. The biggest difference is that the anomaly occurs during development of the body and its systems. Therefore, one of the major foci of a physical therapy plan of care should be to minimize the impact and ongoing development of bony deformation, postural changes, and abnormal tone. Optimizing development encompasses not only motor development but cognitive and social-emotional development as well. Other therapeutic considerations are the same as for an adult who has sustained a spinal cord injury, such as strengthening the upper extremities, developing sitting and standing balance, fostering locomotion, promoting self-care, encouraging safety and personal hygiene, and teaching a range of self-performed motion and pressure relief. First Stage of Physical Therapy Intervention This stage includes the acute care the infant receives after birth and up to the time of ambulation. Initially, after the birth of a child with MMC, parents deal with multiple medical practitioners, each with his or her own contribution to the health of the infant. The neurosurgeon performs the surgery to remove and close the MMC within 24 hours of the infant’s birth to minimize the risk of infection. The placement of a shunt to relieve the hydrocephalus may be performed at the same time or may occur within the first week of life. The orthopedist assesses the status of the infant's joints and muscles. The urologist assesses the child’s renal status and monitors bowel and bladder function. Depending on the amount of skin coverage available to close the defect, a plastic surgeon may also be involved. Once the back lesion is repaired and a shunt is placed, the infant is medically stabilized in preparation for discharge home. Communication among all members of the team working with the parents and infant is crucial. Information about the infant’s present level of function must be shared among all personnel who evaluate and treat the infant. The physical therapist establishes motor and sensory levels of function; evaluates muscle tone, degree of head and trunk control, and range-of-motion limitations; and checks for the presence of any musculoskeletal deformities. General physical therapy goals during this first stage of care include the following: 1. Prevent secondary complications (contractures, deformities, skin breakdown). 2. Promote age-appropriate sensorimotor development. 3. Prepare the child for ambulation. 4, Educate the family about appropriate strategies to manage the child’s condition. If the physical therapist assistant is involved at this stage of the infant’s care, a caring and positive attitude is of utmost importance to foster healthy, appropriate interactions between the parents and the infant. The most important thing to teach the parents is how to interact with their infant. Parents have many things to learn before the infant is discharged from the acute care facility: positioning, sensory precautions, range of motion, and therapeutic handling. Parents need to be comfortable in using handling techniques to promote normal sensorimotor development, especially head and trunk control. Giving parents a sense of competence in their ability to care for their infant is everyone’s job and ensures carryover of instructions to the home setting. Prevention of Deformities: Postoperative Positioning Positioning after the surgical repair of the back lesion should avoid pressure on the repaired area until it is healed. Therefore, the infant initially is limited to prone and side-lying positions. You can show the child’s parents how to place the infant prone on their laps and gently rock to soothe and stimulate head lifting. Holding the infant high on the shoulder, with support under the arms, fosters head control and may be the easiest position for the infant with MMC to maintain a stable head. Handling and carrying strategies may be recommended by the physical therapist and practiced by the assistant before being demonstrated to the parents. Parents are naturally anxious when handling an infant with a disability. Use gentle encouragement, and do not hesitate to correct any errors in hand placement. The infant’s head should be supported when the infant is picked up and put down. As the child’s head control improves, support can gradually be withdrawn. As the 314 back heals, the infant can experience brief periods of supine and supported upright sitting without any interference with wound healing. When the shunt has been inserted, you should always follow any positioning precautions according to the physician’s orders. Prone Positioning Prone positioning is important to prevent development of potentially deforming hip and knee flexion contractures. Prone is also a position from which the infant can begin to develop head control. Depending on the child’s level of motor paralysis and the presence of hypotonia in the neck and trunk, the infant may have more difficulty in learning to lift the head off the support surface in prone than in a supported upright position. Movement in the prone position, as when the infant is placed over the caregiver’s lap or when the infant is carried while prone, will also stimulate head control by encouraging lifting the head into extension. Intervention 7-1 demonstrates a way to position an infant in lying prone with lateral supports to maintain proper alignment. Encouraging the infant to use the upper extremities for propping on elbows and for pushing up to extended arms provides a good beginning for upper extremity strengthening. Intervention 7-1 Prone Lying with Support 315 Infant in prone lying position with lateral supports to maintain proper trunk and lower extremity alignment. (From Williamson GG: Children with spina bifida: early intervention and preschool programming, Baltimore, 1987, Paul H. Brookes.) Effects of Gravity When the infant is in the supine position, the paralyzed lower extremities will tend to assume positions of comfort, such as hip abduction and external rotation, because of the effect of gravity. In children with partial innervation of the lower extremities, hip flexion and adduction can produce hip flexion contractures and can lead to hip dislocation because of the lack of muscle pull from hip extensors or abductors. Certain postures should be avoided, as listed in Box 7-1. Genu recurvatum is seen when the quadriceps muscles are not opposed by equally strong hamstring pull to balance the knee-extension posture. When only anterior tibialis function is present, a calcaneovarus foot results. Some of these foot deformities are depicted in Figure 7-2. Box 7-1 Positions to be Avoided in Children with 316 Myelomeningocele Frog-leg position in prone or supine W sitting Ring sitting Heel sitting Cross-legged sitting (From Hinderer KA, Hinderer SR, Shurtleff DB: Myelodysplasia. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders, pp. 703-755.) Orthoses for Lower Extremity Positioning Orthoses may be needed early to prevent deformities, or the caregiver may simply need to position the child with towel rolls or small pillows to help maintain a neutral hip, knee, and ankle position. An example of a simple lower extremity splint is seen in Figure 7-5. Early on, it is detrimental to adduct the hips completely because the hip joints are incompletely formed and may sublux or dislocate if they are adducted beyond neutral. Maintaining a neutral alignment of the foot is critical for later plantigrade weight bearing. Children with higher-level lesions may benefit initially from a total body splint, to be worn while they are sleeping (Figure 7-6). Many clinicians recommend night splints for this reason. Any orthosis should be introduced gradually because of lack of skin sensation, and the skin should be monitored closely for breakdown. FIGURE 7-5 Simple abduction splint. A, A pad is placed between the child’s legs with a strap underneath. B, The straps are wrapped around the legs and attached with Velcro, C, bringing the legs into neutral hip rotation. 317 FIGURE 7-6 Total body splint. (From Schneider JW, Pesavento MJ: Spina bifida: A congenital spinal cord injury. In Umphred DA, Lazaro RT, Roller ML, Burton GU, editors: Umphred's neurological rehabilitation, ed 6. St Louis, 2013, CV Mosby.) Prevention of Skin Breakdown Lack of awareness of pressure may cause the infant to remain in one position too long, especially once sitting is attained. However, the supine position may pose more danger of skin breakdown over the ischial tuberosities, the sacrum, and the calcaneus. Side lying can be a dangerous position because of the excess pressure on the trochanters. Because of the lack of sensation and decreased awareness of excessive pressure from being in one position for too long, the skin of children with MMC must be closely monitored for redness. Infants need to have their position changed often. Check for red areas, especially over bony prominences and after the infant wears any orthosis. If redness persists longer than 20 minutes, the orthosis should be adjusted (Tappit-Emas, 2008). Sensory Precautions Parents often find it difficult to realize that the infant lacks the ability to feel below the level of the injury. Encouraging parents to play with the infant and to tickle different areas of the child’s body will help them understand where the baby has feeling. It is not appropriate to demonstrate the infant’s lack of sensitivity by stroking the skin with a pin, even though the therapist may use this technique during formal sensory testing. Socks or booties are a good idea for protecting the feet from being nibbled as the infant finds his toes at around 6 months. Teach the parents to keep the infant’s lower extremities covered to protect the skin when the infant is crawling or creeping. Close inspection of the floor or carpet for small objects that could cause an accidental injury is a necessity. Protecting the skin with clothing also helps with temperature regulation, which is impaired. Skin that is anesthetic does not sweat and cannot conserve heat or give off heat and therefore must be protected. Parents must always be instructed to test bath water before placing the infant into the tub because a burn could easily result. Proper shoe fit is imperative to prevent pressure areas and 318 abrasions. Children with MMC may continue to have a chubby baby foot, so extra room may be needed in shoes. Prevention of Contractures: Range of Motion Passive range of motion should be done two to three times a day in an infant with MMC. To decrease the number of exercises in the home program, exercises for certain joints, such as the hip and knee, can be combined. For example, hip and knee flexion on one side can be combined with hip and knee extension on the other side while the infant is supine. Hip abduction can be done bilaterally, as can internal and external rotation. Performing these movements when the infant is prone provides a nice stretch to the hip flexors. Range of motion of the foot and ankle should be done individually. Always be sure that the subtalar joint is in a neutral position when doing ankle dorsiflexion range, so that the movement occurs at the correct joint. If the foot is allowed to go into varus or valgus positioning when stretching a tight heel cord, the motion caused by your stretching will take place in the midfoot, rather than the hindfoot. You may be causing a rocker-bottom foot by allowing the motion to occur at the wrong place. Be sure that your supervising physical therapist demonstrates the correct technique to stretch a heel cord while maintaining subtalar neutral. Range-of-motion exercises should be done gently, with your hands placed close to the child’s joints, to provide a short lever arm. Hold the motion briefly at the end of the available range. Even in the presence of contractures, aggressive stretching is not indicated. Serial casting may be needed as an adjunct to therapy if persistent passive range-of-motion exercise does not improve the range of motion. Always keep your supervising therapist apprised of any problems in this area. Range-of- motion exercises are easy to forget when the infant becomes more active, but these simple exercises are an important part of the infant’s program. Once able, the child should be responsible for doing her own daily range of motion. Promotion of Age-Appropriate Sensorimotor Development Therapeutic Handling: Development of Head Control Any of the techniques outlined in Chapter 5 to encourage head control can be used in a child with MMC. Some early cautions include being sure that the skin over the back defect is well healed and that care is taken to prevent shearing forces on the lower extremities or the trunk when the infant is positioned for head lifting. Additionally, the caregiver should provide extra support if the child’s head is larger than normal, secondary to hydrocephalus. The infant can be carried at the caregiver's shoulder to encourage head lifting as the body sways, just as you would with any newborn. The caregiver can also support the infant in the prone position during carrying or gentle rocking on the lap to promote head control using vestibular input. Extra support can be given to the infant’s head at the jaw or forehead when the child is in the prone position (Intervention 7-2). Intervention 7-2 Prone Carrying 319 Prone carrying with extra support for jaw or forehead. (From Burns YR, MacDonald J: Physiotherapy and the growing child, London, 1996, WB Saunders.) Although head control in infants usually develops first in the prone position, it may be more difficult for an infant with myelodysplasia to lift the head from this position because of hydrocephalus and hypotonic neck and trunk muscles. Extra support from a bolster or a small half- roll under the chest provides assistance in distributing some of the weight farther down the trunk as well as help in bringing the upper extremities under the body to assume a prone-on-elbows position (Figure 7-7). Additional support can be provided under the child’s forehead, if needed, to give the infant a chance to experience this position. Rolling from supine to side lying with the head supported on a half-roll also gives the child practice in keeping the head in line with the body during rotation around the long axis of the body. Head control in the supine position is needed to balance the development of axial extension with axial flexion. Positioning the child in a supported supine position on a wedge can encourage a chin tuck or forward head lift into flexion. Every time the infant is picked up, the caregiver should encourage active head and trunk movements on the part of the child. Carrying should also be seen as a therapeutic activity to promote postural control, rather than as a passive action performed by the caregiver. The clinician or caregiver should watch for signs that could indicate medical complications while interacting with and handling a child with MMC and a shunt. Signs of shunt obstruction may include the setting-sun sign and increased muscle tone in the upper or lower extremities. FIGURE 7-7 Prone position over a half-roll. 320 Therapeutic Handling: Developing Righting and Equilibrium Reactions If the infant uses too much shoulder elevation as a substitute for head control, developing righting reactions of the head and trunk becomes more difficult. Try to modify the position to make it easier for the infant to use neck muscles for stability, rather than the elevated shoulder position. In addition, give more support proximally at the child’s trunk to provide a stable base on which the head can work. The infant may use an elevated position of the shoulders when in propped sitting, with the arms internally rotated and the scapula protracted. Although this posture may be positionally stable, it does not allow the infant to move within or from the posture with any degree of control, thus making it difficult to reach or to shift weight in sitting. As the infant with MMC develops head control in prone, supine, and side-lying positions, righting reactions should be seen in the trunk. Head and trunk righting can be encouraged in prone by slightly shifting the infant’s weight onto one side of the body and seeing whether the other side shortens. Righting of the trunk occurs only as far down the body as the muscles are innervated. The clinician should note any asymmetry in the trunk, because this will need to be taken into account for planning upright activities that could predispose the child to scoliosis. As the infant is able to lift the head off the supporting surface, trunk extension develops down the back. The extension of the infant’s back and the arms should be encouraged by enticing the child to reach forward from a prone position with one or both arms. As the infant becomes stronger, and depending on how much of the trunk is innervated, less and less anterior trunk support can be given while still encouraging lifting and reaching with the arms and upper trunk. (The goal is to have the child “fly,” as in the Landau reflex.) By placing the infant on a small ball or over a small bolster and shifting weight forward, you may elicit head and trunk lifting (Intervention 7-3, A), reaching with arms (Intervention 7-3, B), or propping on one extended arm and reaching with the other (Intervention 7-3, C). If the infant is moved quickly, protective extension of the upper extremities may be elicited. For the infant with a lower level lesion and hip innervation, hip extension should be encouraged when the child is in the prone position. Intervention 7-3 Ball Exercises 321 A. Prone positioning on a ball with the child’s weight shifted forward for head lifting. B. Reaching with both arms over a ball. C. Reaching with one arm while propping on the other over a ball. Trunk rotation must be encouraged to support the child’s transition from one posture to another, such as in rolling from supine to prone and back and in coming to sit from side lying. Trunk rotation in sitting encourages the development of equilibrium reactions that bring the center of gravity back within the base of support. Equilibrium reactions are trunk reactions that occur in developmental postures. In prone and supine, trunk incurvation and limb abduction result from a lateral weight shift. Again, the trunk responds only to the degree to which it is innervated, so one should encourage rotation in all directions. Trunk rotation is also used in protective reactions of the upper extremities when balance is lost. Handling: Developing Trunk Control in Sitting Acclimation to upright sitting is begun as close as possible to the developmentally appropriate time (6 to 8 months). Ideally, the infant should have sufficient head control and sufficient ability to bear weight on extended arms. Propped sitting is a typical way to begin developing independence in sitting. Good postural alignment of the back should be maintained when the child is placed in a sitting position. A floor sitter, a type of adaptive equipment, can be used to support the child’s back if kyphosis is present. Some floor sitters have extensions that provide head support if head control is inconsistent. Floor sitters with head support allow even the child with poor head control to be placed in a sitting position on the floor to play. In children with good head control, sitting balance can be trained by varying the child’s base of support and the amount of hand support. Often, a bench or tray placed in front of the child can provide extra support and security as confidence is gained while the child plays in a new position. Certain sitting positions should be avoided because of their potentially deforming forces. These positions are listed in Box 7-1. Once propped sitting is achieved, hand support is gradually but methodically decreased. Reaching for objects while supporting with one hand can begin in the midline, and then the range can be widened as balance improves. Weight shifting at the pelvis in sitting can be used to elicit head and trunk righting reactions and upper-extremity protective reactions. Trunk rotation with extension is needed to foster the ability to protect in a backward direction. Later, the child can work on transferring objects at the midline with no hand support, an ultimate test of balance. Always remember to protect the child’s back and skin during weight bearing in sitting. Skin inspection should be done after sitting for short periods of time. If the child cannot maintain an upright trunk muscularly, an orthosis may be indicated for alignment in sitting and for prevention of scoliosis. Preparation for Ambulation: Acclimation to Upright and Weight Bearing Acclimation to upright and weight bearing begins with fostering development of head and trunk control and includes sensory input to the lower extremities despite the lack of sensation. Brief periods of weight bearing on properly aligned lower extremities should be encouraged throughout the day. These periods occur in supported standing and should be done often. Providing a symmetric position for the infant is important for increasing awareness of body position and sensory input. Handling should promote symmetry, equal weight bearing, and equal sensory input. Weight bearing in the upright position provides a perfect opportunity to engage the child in cognitively appropriate play. The physical therapist assistant can serve as a vocal model for speech by making sounds, talking, and describing objects and actions in the child’s environment. By interacting with the child, you are also modeling appropriate behavior for the caregiver. Upper Extremity Strengthening During early development, pulling and pushing with the upper extremities are excellent ways to foster increasing upper extremity strength. The progression of pushing from prone on elbows to prone on extended arms and onto hands and knees can provide many opportunities for the child to use the arms in a weight-bearing form of work. Providing the infant with your hands and requesting her to pull to sit can be done before she turns and pushes up to sit. Pulling on various resistances of latex-free Theraband can be a fun way to incorporate upper extremity strengthening into the child’s treatment plan. Other objects can be used for pulling, such as a dowel rod or cane. Pushing on the floor on a scooter board can provide excellent resistance training. 322 Mat Mobility Moving around in supine and prone positions is important for exploring the environment and self- care activities, but mat mobility includes movement in upright sitting. Mat mobility needs to be encouraged once trunk balance begins in supported sitting. The child can be encouraged to pull herself up to sitting by using another person, a rope tied to the end of the bed, or an overhead trapeze. Children can and should use pushup blocks or other devices to increase the strength in their upper extremities (Intervention 7-4). They need to have strong triceps, latissimus dorsi, and shoulder depressors to transfer independently. Moving around on the mat or floor is good preparation for moving around in upright standing or doing push-ups in a wheelchair. Connecting arm motion with mobility early gives the child a foundation for coordinating other, more advanced transfer and self-care movements. Intervention 7-4 Strengthening Upper Extremities with Push-up Blocks Push-ups on wooden blocks to strengthen scapular muscles. Push-ups prepare for transfers and pressure relief. (From Williamson GG: Children with spina bifida: early intervention and preschool programming, Baltimore, 1987, Paul H. Brookes.) Standing Frames Use of a standing frame for weight bearing can begin when the child has sufficient head control and exhibits interest in attaining an upright standing position. Normally, infants begin to pull to stand at around 9 months of age. By 1 year, all children with a motor level of L3 or above should be fitted with a standing frame or parapodium to encourage early weight bearing. The Toronto A-frame is the preambulation orthosis of choice for most children with MMC (Figure 7-8). A standing frame is usually less expensive than a parapodium and is easier to apply (Ryan et al., 1991). The tubular 323 frame supports the trunk, hips, and knees and leaves the hands-free. Some children with L4 or lower lesions may be fitted with some type of hip-knee-ankle-foot orthosis (HKAFO) to begin standing in preparation for walking. The orthotic device pictured in Figure 7-9 has a thoracic support. Having the child stand four or five times a day for 20 to 30 minutes seems to be manageable for most parents (Tappit-Emas, 2008). A more detailed explanation of standing frames is presented later in this chapter. | ih Se baal ' a * , P =: a FIGURE 7-8 Standing frame. A, Anterior view. B, The frame is adapted to accommodate the child’s leg-length discrepancy and tendency to lean to the right. (From Ryan KD, Ploski C, Emans JB: Myelodysplasia: The musculoskeletal problem: Habilitation from infancy to adulthood. Phys Ther 71:935-946, 1991. With permission of the American Physical Therapy Association.) 324 FIGURE 7-9 Hip-knee-ankle-foot orthosis with a thoracic strap. A, Front view. B, Side view. C, Posterior view. (From Nawoczenski DA, Epler ME: Orthotics in functional rehabilitation of the lower limb, Philadelphia, 1997, WB Saunders.) Family Education The family must be taught sensory precautions, signs of shunt malfunction, range of motion, handling, and positioning. Most of these activities are not particularly difficult. However, the difficulty comes in trying not to overwhelm the parents with all the things that need to be done. Parents of children with a physical disability need to be empowered to be parents and advocates for their child. Parents are not surrogate therapists and should not be made to think they should be. Literature that may be helpful is available from the Spina Bifida Association of America. As much as possible, many of the precautions, range-of-motion exercises, and developmental activities should become part of the family’s everyday routine. Range-of-motion exercises and developmental activities can be shared between the spouses, and a schedule of standing time can be outlined. Siblings are often the best partners in encouraging developmentally appropriate play. Second Stage of Physical Therapy Intervention The ambulatory phase begins when the infant becomes a toddler and continues into the school years. The general physical therapy goals for this second stage include the following: 1. Ambulation and independent mobility. 2. Continued improvements in flexibility, strength, and endurance. 3. Independence in pressure relief, self-care, and ADLs. 4. Promotion of ongoing cognitive and social-emotional development. 5. Identification of perceptual problems that may interfere with learning. 6. Collaboration with family, school, and health-care providers for total management. Box 7-2 lists vital components of a physical therapy program. 325 Box 7-2 Vital Components of a Physical Therapy Program Proper positioning in sitting and sleeping Stretching Strengthening Pressure relief and joint protection Mobility for short and long distances Transfers and activities of daily living Skin inspection Self-care Play Recreation and physical fitness (Modified from Hinderer KA, Hinderer SR, Shurtleff DB: Myelodysplasia. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders, pp. 703-755.) Orthotic Management The health-care provider's philosophy of orthosis use may determine who receives what type of orthosis and when. Some clinicians do not think that children with high levels of paralysis, such as those with thoracic or high lumbar (L1 or L2) lesions, should be prescribed orthoses because studies show that by adolescence these individuals are mobile in a wheelchair and have discarded walking as a primary means of mobility. Others think that all children, regardless of the level of lesion, have the right to experience upright ambulation even though they may discard this type of mobility later. Orthotic Selection The physical therapist, in conjunction with the orthopedist and the orthotist, is involved with the family in making orthotic decisions for the child with MMC. Many factors have to be considered when choosing an orthosis for a child who is beginning to stand and ambulate, including level of lesion, age, central nervous system status, body proportions, contractures, upper limb function, and cognition. Financial considerations also play a role in determining the initial type of orthosis. Any time prior approval is needed, the process must begin in sufficient time so as not to interfere with the child’s developmental progress. Even though it is not your responsibility to make orthotic decisions as a physical therapist assistant, you do need to be aware of what goes into this decision making. Level of Lesion The level of motor function demonstrated by the toddler does not always correspond to the level of the lesion because of individual differences in nerve root innervation. A thorough examination needs to be completed by the physical therapist prior to making orthotic recommendations. A chart of possible orthoses to be considered according to the child’s motor level is found in Table 7-4. Age recommendations for each device vary considerably among different sources and are often linked to the philosophy of orthotic management espoused by a particular facility or clinic. Contractures can prevent a child from being fitted with orthoses. The child cannot have any significant amount of hip or knee flexion contractures and must have a plantigrade foot—that is, the ankle must be able to achieve a neutral position or 90 degrees—to be able to wear an orthotic device for standing and ambulation. Standers may be used to counteract hip flexor tightness seen in children with MMC. Addition of a 15-degree wedge to increase passive stretch of the gastrocnemius muscles can be used in conjunction with a stander (Paleg et al., 2014). Table 7-4 Predicted Ambulation of Children with Spina Bifida Motor Level Orthosis/Assistive Device Thoracic May use THKAFO or HKAFO for supported standing when young May use KAFO, RGO with walker or crutches for short distances in house when young May use KAFO with walker or crutches for short distances in house and community L4 Uses AFO and crutches in communit Community, W/C for long distances May or may not use AFO, FO in community, crutches for long distances Community, W/C for sports Sacral May or may not use FO in communit Communit 326 Sources: Data from Ratliffe, 1998; Drnach, 2008; Krosschell and Pesavento, 2013. AFO, Ankle-foot orthosis; FO, foot orthosis; HKAFO, hip-knee-ankle-foot orthosis; KAFO, knee-ankle-foot orthosis; RGO, reciprocating gait orthosis; THKAFO, trunk-hip-knee-ankle-foot orthosis; W/C, wheelchair. Age The type of orthosis used by a child with MMC may vary according to age. A child younger than 1 year of age can be fitted with a night splint to maintain the lower extremities in proper alignment. By 1 year, all children should be fitted with a standing frame or parapodium to encourage early weight bearing. Most children exhibit a desire to pull to stand at around 9 months of age, and the therapist and the assistant should anticipate this desire and should be ready with an orthosis to take advantage of the child’s readiness to stand. When a child with MMC exhibits a developmental delay, the child should be placed in a standing device when her developmental age reaches 9 months. If, however, the child does not attain a developmental age of 9 months by 20 to 24 months of chronologic age, standing should be begun for physiologic benefits. A parapodium is the orthosis of choice in this situation (Figure 7-10). FIGURE 7-10 Front view of the Toronto parapodium. (From Knutson LM, Clark DE: Orthotic devices for ambulation in children with cerebral palsy and myelomeningocele. Phys Ther 71:947-960, 1991. With permission of the American Physical Therapy Association.) The level of MMC is correlated with the child’s age to determine the appropriate type of orthotic device. A child with a thoracic or high lumbar (L1, 2) motor level requires an HKAFO with thoracic support (see Figure 7-9). Often, the child begins gait training in a parapodium and progresses to a reciprocating gait orthosis (RGO) (Figure 7-11). Household ambulation may be possible but at a very high energy cost. Children with a high motor level should be engaged in activities to prepare them for wheelchair propulsion, such as transfers and increasing upper body strength. A child with a midlumbar (L3 or L4) motor level may begin with a parapodium and may make the transition to 327 standard knee-ankle-foot orthoses (KAFOs) or ankle-foot-orthoses (AFOs) (Figures 7-12 and 7-13, A), depending on quadriceps strength. A child with a low motor level, such as L4 to L5 or $2, may begin standing without any device. When learning to ambulate, children with low lumbar motor levels benefit from AFOs or supramalleolar molded orthoses (SMOs) to support the foot and ankle (Figure 7-13, A and B). A child with an L5 motor level has hip extension and ankle eversion and may need only lightweight AFOs to ambulate. Although the child with an S2 motor level may begin to walk without any orthosis, she may later be fitted with a foot orthosis (Figure 7-13, C). FIGURE 7-11 Reciprocating gait orthosis with a thoracic strap, posterior view. (From Nawoczenski DA, Epler ME: Orthotics in functional rehabilitation of the lower limb, Philadelphia, 1997, WB Saunders.) 328 FIGURE 7-12 Oblique view of knee-ankle-foot orthoses with anterior thigh cuffs. (From Knutson LM, Clark DE: Orthotic devices for ambulation in children with cerebral palsy and myelomeningocele. Phys Ther 71:947—960, 1991. With permission of the American Physical Therapy Association.) 329 n < 8 FIGURE 7-13 A, Fixed molded ankle-foot orthosis with an ankle strap to restrain the heel. Extrinsic toe elevation to unload the metatarsal heads is optional. B, Supramalleolar orthosis extending proximally to the malleoli. Well- molded medial and lateral walls that wrap over the dorsum of the foot (a) help to control the midtarsal joint and to keep the heel seated. Dorsal flaps also disperse pressure and may reduce sensitivity of the foot. Intrinsic toe elevation (b) can prevent stimulating the plantar grasp reflex. C, Foot orthosis designed to oppose pronation by molding the heel cup to grasp the calcaneus firmly (a) and wedging, or posting, the heel medially (6). (From Knutson LM, Clark DE: Orthotic devices for ambulation in children with cerebral palsy and myelomeningocele. Phys Ther 71:947—960, 1991. With permission of the American Physical Therapy Association.) Types of Orthoses Parapodiums, RGOs, and swivel walkers are all specially designed HKAFOs. They encompass and control the child’s hips, knees, ankles, and feet. A traditional HKAFO consists of a pelvic band, external hip joints, and bilateral long-leg braces (KAFOs). Additional trunk components may be attached to an HKAFO if the child has minimal trunk control or needs to control a spinal deformity. The more extensive the orthosis, the less likely the child will be to continue to ambulate as she grows older. The amount of energy expended to ambulate with a cumbersome orthosis is high. Although the child is young, she may be highly motivated to move around in the upright position. As time progresses, it may become more important to keep up with a peer group, and she may prefer an alternative, faster, and less cumbersome means of mobility. Parapodium The parapodium (see Figure 7-10) is a commonly used first orthotic device for standing and ambulating. Its wide base provides support for standing and allows the child to acclimate to upright while leaving the arms free for play. The child’s knees and hips can be unlocked for sitting at a table or on a bench, a feature that allows the child to participate in typical preschool activities such as snack and circle time. The Toronto parapodium has one lock for the hip and knee, whereas the Rochester parapodium has separate locks for each joint. Reciprocating Gait Orthosis An RGO is the orthosis of choice for progressing a child who begins ambulating with a parapodium. The RGO is more energy efficient than a traditional HKAFO, because it employs a 330 cable system to cause hip extension reciprocally on the stance side when hip flexion is initiated on the swing side. At least weak hip flexors are needed to operate the cable system in the standard RGO, according to Hinderer et al. (2012). If an isocentric RGO is used, a lateral and backward weight shift causes the unweighted leg to swing forward (Tappit-Emas, 2008). RGOs are used with individuals with L1 to L3 levels and in some facilities for individuals with thoracic lesions. This type of gait pattern requires no active movement of the lower extremities. The RGO requires use of an assistive device, reverse walker, rolling walker, Lofstrand crutches, or canes. The energy cost must be considered individually and recognition that community ambulation for children with thoracic to L3 levels is accomplished using a wheelchair. Swivel Walker This device is similar to a parapodium, except that the base and footplate assembly allow a swivel motion. An Orthotic Research and Locomotor Assessment Unit (ORLAU) swivel walker is pictured in Figure 7-14. It is prescribed for children with a high level of MMC who require trunk support. By shifting weight from side to side, the child can ambulate without crutches. If arm swing is added, the child can increase the speed of forward progression, and with crutches, the child may be able to learn a swing-to or swing-through gait pattern. Sitting is not possible because this type of orthosis has no locks at the hips and knees. Some adults with MMC continue to use this device into adulthood. sve FIGURE 7-14 Front view of the Orthotic Research and Locomotor Assessment Unit (ORLAU) swivel walker. (From Knutson LM, Clark DE: Orthotic devices for ambulation in children with cerebral palsy and myelomeningocele. Phys Ther 71:947-960, 1991. With permission of the American Physical Therapy Association.) Donning and Doffing of Orthoses 331 Ambulating with orthoses and assistive devices requires assistance to don the braces. Teaching donning and doffing of orthoses can be accomplished when the child is supine or sitting. The child may be able to roll into the orthosis by going from prone to supine. Sitting is preferable for independent donning of the orthosis if the child can boost into the brace. Next, the child places each foot into the shoe with the knees of the orthosis unlocked, laces or closes the foot piece, locks the knees, and fastens the thigh cuffs or waist belt, if the device has one. Cotton knee-high socks or tights should be worn under the orthosis to absorb perspiration and to decrease any skin irritation. It takes a great deal of practice on the part of the child to become independent in donning the orthosis. Wearing Time of Orthoses Caregivers should monitor the wearing time of orthoses, including the gradual increase in time, with periodic checks for any areas of potential skin breakdown. The child can begin wearing the orthosis for 1 or 2 hours for the first few days and can increase wearing time from there. A chart is helpful so that everyone (teacher, aide, family) knows the length of time the child is wearing the orthosis and who is responsible for checking skin integrity. Check for red marks after the child wears the orthosis and note how long it takes for these marks to disappear. If they do not resolve after 20 to 30 minutes, contact the orthotist about making an adjustment. The orthosis should not be worn again until it is checked by the orthotist. Upper Limb Function Two thirds of children with MMC exhibit impaired upper limb function that can be linked to cerebellar dysmorphology (Dennis et al., 2009). The difficulties in coordination appear to be related to the timing and smooth control of the movements of the upper extremities. These children do not perform well on tests that are timed and exhibit delayed or mixed hand dominance (Dennis et al., 2009). Children with MMC have hand weakness (Effgen and Brown, 1992), poor hand function (Grimm, 1976), and impaired kinesthetic awareness (Hwang et al., 2002). Difficulties with fine- motor tasks and those related to eye-hand coordination are documented in the literature. Some authors relate the perceptual difficulties to the upper limb dyscoordination rather than to a true perceptual deficit (Hinderer et al., 2012). Motor planning and timing deficits are documented (Peny- Dahlstrand et al., 2009; Jewell et al., 2010). The low muscle tone often exhibited in the neck and trunk of these children could also add to their coordination problems. The child with MMC must have sufficient upper extremity control to be able to use an assistive device, such as a walker, and the ability to learn the sequence of using a walker for independent gait. Practicing fine-motor activities has been found to help with the problem and carries over to functional tasks (Fay et al., 1986). Occupational therapists are also involved in the treatment of these children. Cognition The child must also be able to understand the task to be performed to master upright ambulation with an orthosis and assistive device. Cognitive function in a child with MMC can vary with the degree of nervous system involvement and hydrocephalus. Results from intelligence testing place them in the low normal range but below the population mean (Tappit-Emas, 2008), which is an IO of greater than 70 (Barf et al., 2004). The remaining 25% are in the mild intellectual disability category, with an IQ of between 55 and 70. Children with MMC are at risk for a myriad of developmental disabilities including what is often called nonverbal learning disability. They can demonstrate better reading than math and often demonstrate impairments in executive function, which includes problem solving, staying on task, and sequencing actions. Some of the poor performance by children with MMC may be related to their attention difficulties, slow speed of motor response, and memory deficits secondary to cerebellar dysgenesis. Vision and Visual Perception Twenty percent of children with MMC have strabismus, which may require surgical correction (Verhoef et al., 2004). Infants with MMC delay in orienting to faces (Landry et al., 2003) and, when they are older, have difficulty orienting to external stimuli and once engaged cannot easily break their focus (Dennis et al., 2005). In visual perceptual tasks, the child with MMC finds it more difficult if the task is action-based rather than object-based. They may have a more developed 332 “what” neural pathway than a “where” neural pathway. Spatial perception usually depends on moving through an environment, something that may be delayed in the child with MMC. Jansen- Osmann et al. (2008) found that children with MMC had difficulty constructing a situation model of space, which may relate to deficits in figure-ground perception. Cocktail Party Speech You may encounter a child who seems verbally much more intelligent than she really is when formally tested. “Cocktail party speech” can be indicative of “cocktail party personality,” a behavioral manifestation associated with cognitive dysfunction. The therapist assistant must be cautious not to mistake verbose speech for more advanced cognitive ability in a child with MMC. These children are often more severely impaired than one would first think based on their verbal conversation. When they are closely questioned about a topic such as performing daily tasks within their environment, they are unable to furnish details, solve problems, or generalize the task to new situations. Principles of Gait Training Regardless of the timing and type of orthosis that is used, general principles of treatment can be discussed for this second or middle stage of care. Gait training begins with learning to perform and control weight shifts in standing. If the toddler has had only limited experience in upright standing, a standing program may be initiated simultaneously with practicing weight shifting. If the toddler is already acclimated to standing and has a standing frame, one can challenge the child’s balance while the child is in the frame. The therapist assistant moves the child in the frame and causes the child to respond with head and trunk reactions (Intervention 7-5). This maneuver can be a good beginning for any standing session. Parents should be taught how to challenge the child’s balance similarly at home. The child should not be left unattended in the frame because she may topple over from too much self-initiated body movement. By being placed at a surface of appropriate height, the child can engage in fine-motor activities such as building block towers, sorting objects, lacing cards, or practicing puzzles. Intervention 7-5 Weight Shifting in Standing 333 Weight shifting the child while in a standing frame can promote head and trunk righting reactions. These movements prepare the child for later weight shifting during ambulation. (From Burns YR, MacDonald J: Physiotherapy and the growing child, London, 1996, WB Saunders.) Children with moderate to severe central nervous system deficits and delayed head and upper extremity development may continue to use the standing frames until age 3 or 4 or until they no longer fit into them (Tappit-Emas, 2008). In this case, an ORLAU swivel walker is used as the ambulation orthosis, with progression to an RGO with thoracic support and a rollator walker. The physical therapist assistant can play an important role during this second stage of physical therapy management by teaching the child with MMC to ambulate with the new orthosis, usually a parapodium. The child is first taught to shift weight laterally onto one side of the base of the parapodium and to allow the unweighted portion of the base to pivot forward. This maneuver is called a swivel gait pattern. Children can be taught this maneuver in appropriately high parallel bars or with a walker. However, use of the parallel bars may encourage the child to pull rather than push and may make it more difficult to progress to using a walker. The therapist assistant may also be seated on a rolling stool in front of the child and may hold the child’s hands to encourage the weight-shifting sequence. Once the child has mastered ambulation with the new orthosis, consideration can be given to changing the type of assistive device. The child’s gait pattern in a parapodium is progressed from a swivel pattern to a swing-to pattern, which requires a walker. Tappit-Emas (2008) recommends using a rollator walker as the initial assistive device for gait training a child with MMC. This type of walker provides a wide base of stability and two wheels; therefore, the child can advance the walker without picking it up. “The child with an L4 or L5 motor level is often able to begin ambulation after one or two sessions of gait training with a rollator walker” (Tappit-Emas, 2008). A child should be independent with one type of orthosis and assistive device before moving on to a different orthosis or different device. After success with a swing-to gait pattern using a walker, the 334 child can be progressed to using the same pattern with Lofstrand crutches. Once the child has mastered the gait progression with a parapodium and a walker, plans can be made for progression to a more energy-efficient orthosis or a less restrictive assistive device, but not at the same time. A swing-through gait pattern is the most efficient, but it requires using forearm or Lofstrand crutches. The earliest a child may be able to understand and succeed in using Lofstrand crutches is 3 years of age. Tappit-Emas (2008) recommends waiting until the child is 4 or 5 years of age because the use of Lofstrand crutches is complicated. She thinks that the additional time allows the child to be confident in and have perfected additional skills in the upright position. Lofstrand crutches provide much greater maneuverability than a walker, so whenever possible, the child should be progressed from a walker to forearm crutches. Orthotic choices following the use of a parapodium include an HKAFO/RGO or a KAFO. The main advantage of the RGO is energy efficiency. A child with only hip flexors can walk faster and has less fatigue using an RGO than using either conventional KAFOs or a parapodium. A walker may still be the assistive device of choice to provide the child with sufficient support during forward locomotion. Transition to an RGO is not recommended before the child is 30 to 36 months of developmental age, according to Knutson and Clark (1991). If the child has some innervated knee musculature, such as a child with an L3 motor level, ambulation with KAFOs protects the knees. A long-term goal may be walking with the knees unlocked, and if quadriceps strength increases sufficiently, the KAFOs could be cut down to AFOs. If the child is able to move each lower extremity separately, a four-point or two-point gait pattern can be taught. Gait instruction progresses from level ground to uneven ground to elevated surfaces, such as curbs, ramps, and stairs. Level of Ambulation Three levels of ambulation have been identified (Hoffer et al., 1973). These are therapeutic, household, and community. The names of the levels are descriptive of the type and location in which the ambulation takes place and are defined in Chapter 12. The functional ambulatory level for a child with MMC is linked to the motor level. Table 7-4 relates the level of lesion to the child’s long-term ambulation potential. Early on a child with thoracic-level involvement can be a therapeutic ambulator. However, children with high thoracic involvement (above T10) rarely ambulate by the time they are teenagers; they prefer to be independently mobile in a wheelchair to be able to keep up with their peers. Children with upper lumbar innervation (L1 or L2) can usually ambulate within the household or classroom but long- term prognosis is community ambulation in a wheelchair. At L3 level, the strength of the quadriceps determines the level of functional ambulation in this group. Early on ambulation is household and short distances in the community but again, wheelchair independence is the long- term prognosis. Children with L4 or below levels of innervation are community ambulators and should be able to maintain this level of independence throughout adulthood. Those at L4, L5, and sacral levels may also use a wheelchair for long distances or for sports participation. Ambulation is a major goal during early childhood, and most children with MMC are successful. Nevertheless, many children need a wheelchair to explore and have total access to their environments. Studies have shown that early introduction of wheeled mobility does not interfere with the acquisition of upright ambulation. In fact, wheelchair use may boost the child’s self- confidence. It enables the child to exert control over her environment by independently moving to acquire an object or to seek out attention rather than passively waiting for an object to be brought by another person. Movement through the spatial environment is crucial for the development of perceptual cognitive development. Mobility is crucial to the child with MMC who may have difficulty with visual spatial cues, and several options should be made available, depending on the child’s developmental status. Box 7-3 shows a list of mobility options. Box 7-3 Mobility Options for Children with Myelomeningocele Caster cart Prone scooter Walker Mobile vertical stander 335 Manual wheelchair Electric wheelchair Adapted tricycle Cyclone Wheelchair training for the toddler or preschooler should consist of preparatory and actual training activities, as listed in Boxes 7-4 and 7-5. The child should have sufficient sitting balance to use her arms to propel the chair or to operate an electric switch. Arm strength is necessary to propel a manual chair and to execute lateral transfers with or without a sliding board. Training begins on level surfaces within the home and classroom. Safety is always a number one priority; therefore, the child should wear a seat belt while in the wheelchair. Box 7-4 Preparatory Activities for Wheelchair Mobility Sitting balance Arm strength Ability to transfer Wheelchair propulsion or operating an electric switch or joystick Box 7-5 Wheelchair Training for Toddlers and Preschoolers Ability to transfer Mobility on level surfaces Exploration of home and classroom Safety (From Hinderer KA, Hinderer SR, Shurtleff DB: Myelodysplasia. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders, pp. 702-755.) Strength, Flexibility, and Endurance All functional activities in which a child participates require strong upper extremities. Traditional strengthening activities can be modified for the shorter stature of the child, and the amount of weight used can be adjusted to decrease the strain on growing bones. Weights, pulleys, latex-free tubing, and push-up blocks can be incorporated into games of “tug of war” and mat races. Trunk control and strength can be improved by use of righting and equilibrium reactions in developmentally appropriate positions. Refer to the descriptions earlier in this chapter. Monitoring joint range of motion for possible contractures is exceedingly important at all stages of care. Be careful with repetitive movements because this population is prone to injury from excessive joint stress and overuse. Begin early on to think of joint conservation when the child is performing routine motions for transfers and ADLs. Learning to move the lower extremities by attaching strips of latex-free bands to them can be an early functional activity that fosters learning of self-performed range of motion. Independence in Pressure Relief Pressure relief and mobility must also be monitored whether the child is wearing an orthosis or not. When the child has the orthosis on, can she still do a push-up for pressure relief? Does the seating device or wheelchair currently used allow enough room for the child to sit without undue pressure from the additional width of the orthosis, or does it take up too much room in the wheelchair? How many different ways does the child know to relieve pressure? The more ways that are available to the child, the more likely the task is to be accomplished. The obvious way is to do push-ups, but if the child is in a regular chair at school, the chair may not have arms. If the child sits in a wheelchair at the desk, the chair must be locked before the child attempts a push-up. Forward leans can also be performed from a seated position. Alternative positioning in kneeling, standing, or lying prone can be used during rest and play periods. Be creative! 336 Independence in Self-Care and Activities of Daily Living Skin care must be a high priority for the child with MMC, especially as the amount of sitting increases during the school day. Skin inspection should be done twice a day with a handheld mirror. Clothing should be nonrestrictive and sufficiently thick to protect the skin from sharp objects and wheelchair parts and orthoses. An appropriate seat cushion must be used to distribute pressure while the child sits in the wheelchair. Pressure-reducing seat cushions do not, however, decrease the need for performing pressure-relief activities. Children with MMC do not accomplish self-care activities at the same age as typically developing children (Okamoto et al., 1984; Sousa et al., 1983; Tsai et al., 2002) and are not independent in their daily performance (Peny-Dahlstrand et al., 2009). Children with MMC were found to be “unable to perform self-chosen and well-known everyday activities in an effortless, efficient, safe, and independent manner” (Peny-Dahlstrand et al., 2009, p. 1677). Daily self-care includes dressing and undressing, feeding, bathing, and bowel and bladder care. Interpretation of the data further suggests that the delays may be the result of lower performance expectations. Parents often do not perceive their children as competent compared to typically developing children and may therefore expect less from them. Parents must be encouraged to expect independence from the child with MMC. Peny-Dahlstrand et al. (2009) suggested that children with MMC need help to learn how to do tasks and encouragement to persevere in order to complete the task. By the time the child goes to preschool, she will be aware that her toileting abilities are different from those of her peers of the same age (Williamson, 1987). Bowel and bladder care is usually overseen by the school nurse where available, but everyone working with a child with MMC needs to be aware of the importance of these skills. Consistency of routine, privacy, and safety must always be part of any bowel and bladder program for a young child. Helping the child to maintain a positive self-image while teaching responsible toileting behavior can be especially tricky. The child should be given responsibility for as much of her own care as possible. Even if the child is still in diapers, she should also wash her hands at the sink after a diaper change. Williamson (1987) suggests these ways to assist the child to begin to participate: 1. Indicate the need for a diaper change. 2. Assist in pulling the pants down and in removing any orthotic devices, if necessary. 3. Unfasten the soiled diaper. 4. Refasten the clean diaper. 5. Assist in donning the orthosis if necessary and in pulling up the pants. 6. Wash hands. Williamson (1987) provides many excellent suggestions for fostering self-care skills in the preschooler with MMC. The reader is refer to the text by this author for more information. ADL skills include the ability to transfer. We tend to think of transferring from mat to wheelchair and back as the ultimate transfer goal, but for the child to be as independent as possible, he should also be able to perform all transfers related to ADLs, such as to and from a bed, a dressing bench or a regular chair, a chair and a toilet, a chair and the floor, and the tub or shower. Promotion of Cognitive and Social-Emotional Growth Preschoolers are inquisitive individuals who need mobility to explore their environment. They should be encouraged to explore the space around them by physically moving through it, not just visually observing what goes on around them. Scooter boards can be used to help the child move her body weight with the arms while receiving vestibular input. The use of adapted tricycles that are propelled by arm cranking allows movement through space and they could be used on the playground rather than a wheelchair. Difficulty with mobility may interfere with self-initiated exploration and may foster dependence instead of independence. Other barriers to peer interaction or factors that may limit peer interaction are listed in Box 7-6. Box 7-6 Limitations to Peer Interaction Mobility Activities of daily living, especially transfers Additional equipment Independence in bowel and bladder care 337 Hygiene Accessibility Having a child with MMC can be stressful for the family (Holmbeck and Devine, 2010; Vermaes et al., 2008). Caregivers describe children with MMC as being less adaptable, more negative when initially responding to new or novel stimuli, more distractible, and less able to persist when completing a task compared to same-age peers without MMC (Vachha and Adams, 2005). Parents report that their children with MMC are less competent physically and cognitively than typically developing children (Landry et al., 1993). Clinicians can provide guidance to parents to interpret the child’s signals and provide appropriate responses. Many children with MMC experience healthy emotional development (Williamson, 1987) and exhibit high levels of resilience (Holmbeck and Devine, 2010). The task of infancy, according to Erikson, is to develop trust that basic needs will be met. Parents, primary caregivers, and health- care providers need to ensure that these emotional needs are met. If the infant perceives the world as hostile, she may develop coping mechanisms such as withdrawal or perseveration. If the child is encouraged to explore the environment and is guided to overcome the physical barriers encountered, she will perceive the world realistically as full of a series of challenges to be mastered, rather than as full of unsurmountable obstacles. In the case of children with MMC, the motor skills that they have the most difficulty with are those that involve motor planning and adaptation. Parents need to foster autonomy in daily life in their children with MMC. Identification of Perceptual Problems School-age children with MMC are motivated to learn and to perform academically to the same extent as any other children. During this time, perceptual problems may become apparent. Children with MMC have impaired visual analysis and synthesis (Vinck et al., 2006; Vinck et al., 2010). Visual perception in a child with MMC should be evaluated separately from her visuomotor abilities, to determine whether she truly has a perceptual deficit (Hinderer et al., 2012). For example, a child’s difficulty with copying shapes, a motor skill, may be more closely related to her lack of motor control of the upper extremity than to an inaccurate visual perception of the shape to be copied. Perception and cognition are connected to movement. Development of visual spatial perception and spatial cognition can occur because children with MMC have impaired movement. For example, children with MMC have been found to have problems with figure-ground (find the hidden shapes) and route finding as in a maze (Dennis et al., 2002; Jansen-Osmann et al., 2008). Collaboration for Total Management The management of the child with MMC in preschool and subsequently in the primary grades involves everyone who comes in contact with that child. From the bus driver to the teacher to the classroom aide, everyone has to know what the child is capable of doing, in which areas she needs assistance, and what must be done for her. Medical and educational goals should overlap to support the development of the most functionally independent child possible, a child whose psychosocial development is on the same level as that of her able-bodied peers and who is ready to handle the tasks and issues of adolescence and adulthood. Third Stage of Physical Therapy Intervention The third stage of management involves the transition from school age to adolescence and into adulthood. General physical therapy goals during this last stage are as follows: 1. Reevaluation of ambulation potential 2. Mobility for home, school, and community distances 3. Continued improvements in flexibility, strength, and endurance 4. Independence in ADLs 5. Physical fitness and participation in recreational activities Reevaluation of Ambulation Potential The potential for continued ambulation needs to be reevaluated by the physical therapist during the student’s school years and, in particular, as she approaches adolescence. Children with MMC go through puberty earlier than their peers who are able-bodied. Surgical procedures that depend on 338 skeletal maturity may be scheduled at this time. The long-term functional level of mobility of these students can be determined as their physical maturity is peaking. The assistant working with the student can provide valuable data regarding the length of time that upright ambulation is used as the primary means of mobility. Any student in whom ambulation becomes unsafe or whose ambulation skills become limited functionally should discontinue ambulation except with supervision. Physical therapy goals during this time are to maintain the adolescent’s present level of function if possible, to prevent secondary complications, to promote independence, to remediate any perceptual-motor problems, to provide any needed adaptive devices, and to promote self- esteem and social-sexual adjustment (Krosschell and Pesavento, 2013). Developmental changes that may contribute to the loss of mobility in adolescents with MMC are as follows: 1. Changes in length of long bones, such that skeletal growth outstrips muscular growth 2. Changes in body composition that alter the biomechanics of movement 3. Progression of neurologic deficit 4. Immobilization resulting from treatment of secondary problems, such as skin breakdown or orthopedic surgery 5. Progression of spinal deformity 6. Joint pain or ligamentous laxity Physical therapy during this stage focuses on making a smooth transition to primary wheeled mobility if that transition is needed to save energy for more academic, athletic, or social activities. Individuals with thoracic, high lumbar (L1 or L2), and midlumbar (L3 or L4) lesions require a wheelchair for long-term functional mobility. They may have already been using a wheelchair during transport to and from school or for school field trips. School-age children can lose function because of spinal-cord tethering, so they should be monitored closely during rapid periods of growth for any signs of change in neurologic status. An adolescent with a midlumbar lesion can ambulate independently within a house or a classroom but needs aids to be functional within the community. Long-distance mobility is much more energy-efficient if the individual uses a wheelchair. Individuals with lower-level lesions (L5 and below) should be able to remain ambulatory for life, unless too great an increase in body weight occurs, thereby making wheelchair use a necessity. Hinderer et al. (1988) found a potential decline in mobility resulting from progressive neurologic loss in adolescents even with lower-level lesions, so any adolescent with MMC should be monitored for potential progression of neurologic deficit (Rowe and Jadhav, 2008). Weight gain can severely impair the teen’s ability to ambulate. Youths with MMC engage in unhealthy behaviors that persist into their late 20s (Soe et al., 2012). Unhealthy behaviors included less healthy diets, sedentary activities, and less exercise compared to national estimates. Symptoms of depression were related to drinking alcohol. Wheelchair Mobility When an adolescent with MMC makes the transition to continuous use of a wheelchair, you should not dwell on the loss of upright ambulation as something devastating but focus on the positive gains provided by wheeled mobility. Most of the time, if the transition is presented as a natural and normal occurrence, it is more easily accepted by the individual. The wheelchair should be presented as just another type of “assistive” device, thereby decreasing any negative connotation for the adolescent. The mitigating factor is always the energy cost. The student with MMC may be able to ambulate within the classroom but may need a wheelchair to move efficiently between classes and keep up with her friends. “Mobility limitations are magnified once a child begins school because of the increased community mobility distances and skills required” (Hinderer et al., 2000). This requirement becomes a significant problem once a child is in school because the travel distances increase and the skills needed to maneuver within new environments become more complicated. A wheelchair may be a necessity by middle school or whenever the student begins to change classes, has to retrieve books from a locker, and needs to go to the next class in a short time. For the student with all but the lowest motor levels, wheeled mobility is a must to maintain efficient function. Johnson et al. (2007) found that 57% to 65% of young adults with MMC use lightweight wheelchairs, both manual and power-assisted. Environmental Accessibility All environments in which a person with MMC functions should be accessible—home, school, and 339 community. The Americans with Disabilities Act was an effort to make all public buildings, programs, and services accessible to the general public. Under this Act, reasonable accommodations have to be made to allow an individual with a disability to access public education and facilities. Public transportation, libraries, and grocery stores, for example, should be accessible to everyone. Assistive technology can play a significant role in improving access and independence for the youth with MMC. Timers, cell phones, and computer access can be used to support personal-care routines as well as organization skills (Johnson et al., 2007). Driver Education Driver education is as important to a person with MMC as it is to any 16-year-old teenager, and may be even more so. Some states have programs that evaluate the ability of an individual with a disability to drive, after which recommendations to use appropriate devices, such as hand controls and type of vehicle, will be given. A review of car transfers should be part of therapy for adolescents along with other activities that prepare them for independent living and a job. The ability to move the wheelchair in and out of the car is also vital to independent function. Flexibility, Strength, and Endurance Prevention of contractures must be aggressively pursued during the rapid growth of adolescence because skeletal growth can cause significant shortening of muscles. Stretching should be done at home on a regular basis and at school if the student has problem areas. Areas that should be targeted are the low back extensors, the hip flexors, the hamstrings, and the shoulder girdle. Proper positioning for sitting and sleeping should be reviewed, with the routine use of the prone position crucial to keep hip and knee flexors loose and to relieve pressure on the buttocks. More decubitus ulcers are seen in adolescents with MMC because of increased body weight, less strict adherence to pressure-relief procedures, and development of adult patterns of sweating around the buttocks. Strengthening exercises and activities can be incorporated into physical education free time. A workout can be planned for the student that can be carried out both at home and at a local gym. Endurance activities such as wind sprints in the wheelchair, swimming, wheelchair track, basketball, and tennis are all appropriate ways to work on muscular and cardiovascular endurance while the student is socializing. If wheelchair sports are available, this is an excellent way to combine strengthening and endurance activities for fun and fitness. Check with your local parks and recreation department for information on wheelchair sports available in your area. Hygiene Adult patterns of sweating, incontinence of bowel and bladder, and the onset of menses can all contribute to a potential hygiene problem for an adolescent with MMC. A good bowel and bladder program is essential to avoid incontinence, odor, and skin irritation, which can contribute to low self-esteem. Adolescents are extremely body conscious, and the additional stress of dealing with bowel and bladder dysfunction, along with menstruation for girls, may be particularly burdensome. Scheduled toileting and bathing and meticulous self-care, including being able to wipe properly and to handle pads and tampons, can provide adequate maintenance of personal hygiene. Socialization Adolescents are particularly conscious about their body image, so they may be motivated to maintain a normal weight and to provide extra attention to their bowel and bladder programs. Sexuality is also a big concern for adolescents. Functional limitations based on levels of innervation are discussed in Chapter 12. Abstinence, safe sex, use of birth control to prevent pregnancy, and knowledge of the dangers of sexually transmitted diseases must all be topics of discussion with the teenager with MMC. This is no different from discussing with the teenager without MMC. The clinician must always provide information that is as accurate as possible to a young adult. Social isolation can have a negative effect on emotional and social development in this population (Holmbeck et al., 2003). Socialization requires access to all social situations at school and in the community. Peer interaction during adolescence can be limited by the same things identified as potential limitations on interaction early in life, as listed in Box 7-6. Additional challenges to the adolescent with MMC can occur if issues of adolescence such as personal identity, sexuality, and 340 peer relations, and concern for loss of biped ambulation are not resolved. Adult development is hindered by having to work through these issues during early adulthood (Friedrich and Shaffer, 1986; Shaffer and Friedrich, 1986). Independent Living Basic ADLs (BADLs) are those activities required for personal care such as ambulating, feeding, bathing, dressing, grooming, maintaining continence, and toileting (Cech and Martin, 2012). Instrumental ADLs (I[ADLs) are those skills that require the use of equipment such as the stove, washing machine, or vacuum cleaner, and they relate to managing within the home and community. Being able to shop for food or clothes and being able to prepare a meal are examples of IADLs. Mastery of both BADL and IADL skills is needed to be able to live on one’s own. Functional limitations that may affect both BADLs and IADLs may become apparent when the person with MMC has difficulty in lifting and carrying objects. Vocational counseling and planning should begin during high school or even possibly in middle school. The student should be encouraged to live on her own if possible after high school as part of a college experience or during vocational training. “Launching” of a young adult with MMC has been reported in the literature. Launching is the last transition in the family life cycle in which “the late adolescent is launched into the outside world to begin to develop an autonomous life” (Friedrich and Shaffer, 1986). Challenges during this time include discussion regarding guardianship if ongoing care is needed, placement plans, and a redefinition of the roles of the parents and the young adult with MMC. Employment of only 25% of adults with MMC was reported by Hunt (1990), and few persons described in this report were married or had children. Buran et al. (2004) describe adolescents with MMC as having hopeful and positive attitudes toward their disability. However, they found the adolescents were not engaging in sufficient decision making and self-management to prepare themselves for adult roles. This lack of preparation might be the reason many individuals with MMC are underemployed and not living independently as young adults (Buran et al., 2004). Each period of the life span brings different challenges for the family with a child with MMC. Box 7-7 is a review of the responsibilities and challenges in the care of a child with MMC across the life span. In light of the recent research, more emphasis may need to be placed on decision making during adolescence. Box 7-7 Responsibilities and Challenges in the Care of a Child with Myelomeningocele over the Life Span Infancy (birth to 2 years) Initial crisis: grieving; intensive medical services including surgery; hospitalizations that may interfere with bonding process Subsequent crisis: procurement of therapy services; delay in locomotion and bowel or bladder training Preschool (3-5 years) Ongoing medical monitoring; prolonged dependency of the child requiring additional physical care Recurrent hospitalizations for CSF shunt revisions and orthopedic procedures School age (6-12 years) School programming; ongoing appraisal of the child’s development Establishment of family roles: dealing with discrepancies in sibling’s abilities; parental tasks Potential for limited peer involvement Recurrent hospitalizations for CSF shunt revisions and orthopedic procedures Adolescence (13-20 years) Accepting “permanence” of disability Personal identity Child’s increased size affecting care More need for adaptive equipment Issues of sexuality and peer relations Issues concerning potential loss of biped ambulatory skills Recurrent hospitalizations for CSF shunt revision and orthopedic procedures Launching (21 years and beyond) 341 Discussion of guardianship issues relating to ongoing care of the young adult Placement plans for the young adult Parents redefine roles regarding young adult and themselves From Friedrich W, Shaffer J: Family adjustments and contributions. In Shurtleff DB, editor: Myelodysplasias and exstrophies: significance, prevention, and treatment, Orlando, FL, 1986, Grune & Stratton, pp. 399-410. Quality of Life Locomotion and, hence, ambulation potential impact the quality of life of an individual with MMC. Rendeli et al. (2002) found that children with MMC had significantly different cognitive outcomes based on their ambulatory status. Those that walked with or without assistive devices had higher performance IQ than those who did not ambulate. There was no difference between the two groups on total IQ. It has been suggested that self-produced locomotion facilitates development of spatial cognition. Others have found that independent ambulatory status was the most important factor in determining health-related quality of life (HRQOL) (Schoenmakers et al., 2005; Danielsson et al., 2008). HRQOL is a broad multidimensional concept that usually includes self-reported measures of physical and mental health (NBDPN, 2012). Children with MMC were found to have a lower HROQOL than other children with a chronic illness (Oddson et al., 2006). Seventy-two percent of youths and young adults with MMC had decreased participation in structured activities and required assistive technology to assist their mobility (Johnson et al., 2007). The presence of spasticity in the muscles around the hip and knee, quadriceps muscle weakness, level of lesion, and severity of neurologic symptoms affected ambulatory ability and functional ability, which in turn decreased HRQOL (Danielsson et al., 2008). Flanagan et al. (2011) found that the parentally perceived HRQOL of children with MMC differed based on the motor level of the child. Children with motor levels at L2 and above had decreased HRQOL scores compared to children with motor levels at L3 to L5. They used the Pediatric Quality of Life Inventory (Peds QL) and the Pediatric Outcomes Data Collection Instrument Version 2.0 (PODCI) as measures of HRQOL. Categories in which there were score differences included sports and physical function, transfers and basic mobility, health, and global function. In contrast, Kelley et al. (2011) found that participation in children with MMC did not differ based on motor level, ambulation status, or bowel and bladder problems. They divided their subjects into age groups, 2 to 5 years, 6 to 12 years, and 13 to 18 years. There were differences between groups in participation scores for skill-based activities (physical and recreational activities), with younger children participating more in skill-based and physical activities and the middle age group participating more in recreational activities than the older group. Bowel and bladder problems were found to limit the participation of the children of 6 to 12 years old in social and physical activities. Kelley et al. (2011) used different measures for participation than Flannagan et al. It also appears that a higher percentage of children in the study of Kelley et al were at a L3 motor level, which according to the study of Flannagan et al have a higher HRQOL. Regardless, physical function does affect the quality of life of individuals with MMC. Clinicians need to be more focused on breaking down community barriers to participation and promoting optimal mobility and health so that children with MMC transition into independent adults. Chapter summary The management of the person with MMC is complex and requires multiple levels of intervention and constant monitoring. Early on, intensive periods of intervention are needed to establish the best outcome and to provide the infant and child with MMC the best developmental start possible. Physical therapy intervention focuses primarily on the attainment of motor milestones of head and trunk control within the boundaries of the neurologic insult. While the achievement of independent ambulation may be expected of most people with MMC during their childhood years, this expectation needs to be tempered based on the child’s motor level and long-term potential for functional ambulation. Fostering cognitive and social-emotional maturity should occur simultaneously. Children with MMC can develop social abilities despite a reduced level of self-care or impaired motor function. The physical therapist monitors the student’s motor progress throughout the school years and intervenes during transitions to a new setting. Each new setting may demand increased or different functional skills. Monitoring the student in school also includes looking for any evidence of deterioration of neurologic or musculoskeletal status that may prevent 342 optimum function in school or access to the community. Examples of appropriate intervention times are occasions when the student needs assistance in making the transition to another level of function, such as using a wheelchair for primary mobility and evaluating a work site for wheelchair access. The physical therapist assistant may provide therapy to the individual with MMC that is aimed at fostering functional motor abilities or teaching functional skills related to use of orthoses or assistive devices, transfers, and ADLs. The physical therapist assistant can provide valuable data to the therapist during annual examinations as well as ongoing information regarding function to manage the needs of the person with MMC from birth through adulthood most efficiently. Review questions 1. What type of paralysis can be expected in a child with MMC? 2. What complications are seen in a child with MMC that may be related to skeletal growth? 3. What are the signs of shunt malfunction in a child with MMC? 4. What position is important to use in preventing the development of hip and knee flexion contractures in a child with MMC? 5. What precautions should be taken by parents to protect skin integrity in a child with MMC? 6. What determines the type of orthosis used by a child with MMC? 7. What is the relationship of motor level to level of ambulation in a child with MMC? 8. When is the functional level of mobility determined for an individual with MMC? 9. What developmental changes may contribute to a loss of mobility in the adolescent with MMC? 10. When is the most important time to intervene therapeutically with an individual with MMC? Case Studies Rehabilitation Unit Initial Examination and Evaluation: PL History Chart Review PL is a talkative, good-natured, 3-year-old boy. He is in the care of his grandmother during the day because both of his parents work. He is the younger of two children. PL presents with a low lumbar (L2) MMC with flaccid paralysis. Medical history includes premature birth at 32 weeks of gestation, bilateral hip dislocation, bilateral clubfeet (surgically repaired at 1 year of age), scoliosis, multiple hemivertebrae, and shunted (ventriculoperitoneal [VP]) hydrocephalus (at birth). Subjective Mother reports that PL’s previous physical therapy consisted of passive and active range of motion for the lower extremities and learning to walk with a walker and braces. She expresses concern about his continued mobility now that he is going to preschool. Objective Systems Review Communication/Cognition: PL communicates in 5- to 6-word sentences. Paul has an IQ of 90. Cardiovascular/Pulmonary: Normal values for age. Integumentary: Healed 7-cm scar on the lower back, no areas of redness below L2. Musculoskeletal: AROM and strength within functional limits in the upper extremities. AROM limitations present in the lower extremities, secondary to neuromuscular weakness. Neuromuscular: Upper extremities grossly coordinated, lower extremity paralysis. Tests and Measures Anthropometric: Height 36 inches, weight 35 Ibs, BMI 19 (20 to 24 is normal). Circulation: Skin warm to touch below L2, pedal pulses present bilaterally, strong radial pulse. 343 Integumentary: No ulcers or edema present. Shunt palpable behind right ear. Motor Function: PL’s motor upper extremity skills are coordinated. He can build an 8-cube tower. He sits independently and moves in and out of sitting and standing independently. He is unable to transfer into and out of the tub independently. Neurodevelopmental Status: Peabody Developmental Motor Scales (PDMS) Developmental Motor Quotient (DMQ) = 69. Age equivalent = 12 months. Fine motor development is average for his age (PDMS DMQ = 90). Reflex integrity: Patellar 1 +, Achilles 0 bilaterally. No abnormal tone is noted in the upper extremities; tone is decreased in the trunk, flaccid in the lower extremities. Range of Motion: Active motion is within functional limits (WFL) for the upper extremities and for hip flexion and adduction. Active knee extension is complete in side lying. Passive motion is WEL for remaining joints of the lower extremities. Muscle Performance: As tested using functional muscle testing. If the child could move the limb against gravity and take any resistance the muscle was graded 3 +. If the limb could only move through full range in the gravity-eliminated position, the muscle was graded a 2. Right Left Partial symmetrical curl up Hips llio psoas Gluteus maximus Adductors Abductors Sensory Integrity: Pinprick intact to L2, absent below. Posture: PL exhibits a mild right thoracic—left lumbar scoliosis. Gait, Locomotion, and Balance: PL sits independently and stands with a forward facing walker and bilateral HKAFOs. PL can demonstrate a reciprocal gait pattern for approximately 10 feet when he ambulates with a walker and HKAFOs but prefers a swing-to pattern. Using a swing-to pattern, he can ambulate 25 feet before wanting to rest. He creeps reciprocally but prefers to drag-crawl. PL can creep up stairs with assistance and comes down head first on his stomach. Head and trunk righting is present in sitting, with upper extremity protective extension present in all directions to either side. PL exhibits minimal trunk rotation when balance is disturbed laterally in sitting. Self-care: PL assists with dressing and undressing and is independent in his sitting balance while performing bathing and dressing activities. He feeds himself but is dependent in bowel and bladder care (wears a diaper). Play/Preschool: PL exhibits cooperative play and functional play but is delayed in pretend play. He presently attends morning preschool 3 days a week and will be attending every day within 1 month. 344 Assessment/evaluation PL is a 3-year-old boy with a repaired L2 MMC with a VP shunt, and he is currently ambulating with a forward-facing walker and HKAFOs. He is making the transition to a preschool program. He is seen one time a week for 30 minutes of physical therapy. Problem List 1. Unable to ambulate with Lofstrand crutches 2. Decreased strength and endurance 3. Dependent in self-care and transfers 4. Lacking knowledge of pressure relief Diagnosis: PL exhibits impaired motor function and sensory integrity associated with nonprogressive disorders of the central nervous system—congenital in origin, which is guide pattern 5C. Prognosis: PL will improve his level of functional independence and functional skills in the preschool setting. He has excellent potential to achieve the following goals within the school year. Short-Term Objectives (actions to be accomplished by midyear review) 1. PL will propel a prone scooter up and down the hall of the preschool for 15 consecutive minutes. 2. PL will perform 20 consecutive chin-ups during free play on the playground daily. 3. PL will kick a soccer ball 5 to 10 feet, 4 or 5 attempts during free play daily. 4. PL will wash and dry hands after toileting. 5. PL will be independent in pressure relief. Long-Term Functional Goals (end of the first year in preschool) 1. PL will ambulate to and from the gym and the lunch room using a reciprocal gait pattern and Lofstrand crutches daily. 2. PL will exhibit pretend play by verbally engaging in story time 3 times a week. 3. PL will assist in managing clothing during toileting and clean intermittent catheterization. Plan Coordination, Communication, and Documentation The therapist and physical therapist assistant will communicate with PL’s mother and teacher on a regular basis. Outcomes of interventions will be documented on a weekly basis. Patient/Client Instruction PL and his family will be instructed in a home exercise program including upper extremity and trunk strengthening exercises, practicing trunk righting and equilibrium reactions in sitting and standing, dressing, transfers, improving standing time, and ambulation using the preferred pattern. Procedural Interventions 1. Mat activities that incorporate prone push-ups, wheelbarrow walking, movement transitions from prone to long sitting, and back to prone, sitting push-ups with push-up blocks, and pressure relief techniques. 2. Using a movable surface such as a ball, promote lateral equilibrium reactions to encourage active trunk rotation. 3. Resistive exercises for upper and lower extremities using latex-free Theraband or cuff weights. 4. Resisted creeping to improve lower extremity reciprocation and trunk control. 5. Increased distances walked using a reciprocal gait pattern by 5 feet every 2 weeks, first with a walker, progressing to Lofstrand crutches. 6. 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Discuss the importance of functional activity training through the life span of a child with a genetic disorder. po2 Introduction More than 6000 genetic disorders have been identified to date. Some are evident at birth, whereas others present later in life. Most genetic disorders have their onset in childhood. The physical therapist assistant working in a children’s hospital, outpatient rehabilitation center, or school system may be involved in providing physical therapy for these children. Some of the genetic disorders discussed in this chapter include Down syndrome (DS), fragile X syndrome (FXS), Rett syndrome, cystic fibrosis (CF), Duchenne muscular dystrophy (DMD), osteogenesis imperfecta (Ol), and autism spectrum disorder (ASD). After a general discussion of the types of genetic transmission, the pathophysiology and clinical features of these conditions are outlined, followed by a brief discussion of the physical therapy management. A case study of a child with DS is presented at the end of the chapter to illustrate the physical therapy management of children with low muscle tone. A second case study of a child with DMD is presented to illustrate the physical therapy management of a child with a progressive genetic disorder. Genetic disorders in children are often thought to involve primarily only one body system — muscular, skeletal, respiratory, or nervous—and to affect other systems secondarily. However, genetic disorders typically affect more than one body system, especially when those systems are embryonically linked, such as the nervous and integumentary systems, both of which are derived from the same primitive tissue. For example, individuals with neurofibromatosis have skin defects in the form of café-au-lait spots in addition to nervous system tumors. Genetic disorders that primarily affect one system, such as the muscular dystrophies, eventually have an impact on or stress other body systems, such as the cardiac and pulmonary systems. Because the nervous system is most frequently involved in genetic disorders, similar clinical features are displayed by a large number of affected children. In addition to the cluster of clinical symptoms that constitute many genetic syndromes, children with genetic disorders often present with what is termed a behavioral phenotype. The term has been around quite a while in medical genetics but may not be familiar to the physical therapist assistant; “...a behavioral phenotype is a profile of behavior, cognition, or personality that represents a component of the overall pattern seen in many or most individuals with a particular condition or syndrome” (Baty et al., 2011). Just as facial features may be different in children with DS or FXS, there may be behavioral and cognitive differences related to the different genetic syndromes. These are just beginning to be detailed in the literature. 353 Genetic transmission Genes carry the blueprint for how body systems are put together, how the body changes during growth and development, and how the body operates on a daily basis. The color of your eyes and hair is genetically determined. One hair color, such as brown, is more dominant than another color, such as blond. A trait that is passed on as dominant is expressed, whereas a recessive trait may be expressed only under certain circumstances. All cells of the body carry genetic material in chromosomes. The chromosomes in the body cells are called autosomes. Because each of us has 22 pairs of autosomes, every cell in the body has 44 chromosomes, and two sex chromosomes. Reproductive cells contain 23 chromosomes—22 autosomes and either an X or a Y chromosome. After fertilization of the egg by the sperm, the genetic material is combined during meiosis, thus determining the sex of the child by the pairing of the sex chromosomes. Two X chromosomes make a female, whereas one X and one Y make a male. Each gene inherited by a child has a paternal and a maternal contribution. Alleles are alternative forms of a gene, such as H or h. If someone carries identical alleles of a gene, HH or hh, the person is homozygous. If the person carries different alleles of a gene, Hh or hH, the person is heterozygous. 354 Categories The two major categories of genetic disorders are chromosomal abnormalities and specific gene defects. Chromosomal abnormalities occur by one of three mechanisms: nondisjunction, deletion, and translocation. When cells divide unequally, the result is called a nondisjunction. Nondisjunction can cause DS. When part or all of a chromosome is lost, it is called a deletion. When part of one chromosome becomes detached and reattaches to a completely different chromosome, it is called a translocation. Chromosome abnormalities include the following: trisomies, in which three of a particular chromosome are present instead of the usual two; sex chromosome abnormalities, in which there is an absence or addition of one sex chromosome; and partial deletions. The most widely recognized trisomy is DS, or trisomy 21. Turner syndrome and Klinefelter syndrome are examples of sex chromosome errors, but they are not discussed in this chapter. Partial deletion syndromes that are discussed include cri-du-chat syndrome and Prader-Willi syndrome (PWS). A specific gene defect is inherited in three different ways: (1) as an autosomal dominant trait; (2) as an autosomal recessive trait; or (3) as a sex-linked trait. Autosomal dominant inheritance requires that one parent be affected by the gene or that a spontaneous mutation of the gene occurs. In the latter case, neither parent has the disorder, but the gene spontaneously mutates or changes in the child. When one parent has an autosomal dominant disorder, each child born has a 1 in 2 chance of having the same disorder. Examples of autosomal dominant disorders include OI, which affects the skeletal system and produces brittle bones, and neurofibromatosis, which affects the skin and nervous system. Autosomal recessive inheritance occurs when either parent is a carrier for the disorder. A carrier is a person who has the gene but in whom it is not expressed. The condition is not apparent in the person. The carrier may pass the gene on without having the disorder or knowing that he or she is a carrier. In this situation, the carrier parent is said to be heterozygous for the abnormal gene, and each child has a 1 in 4 chance of being a carrier. The heterozygous parent is carrying a gene with alleles that are dissimilar for a particular trait. If both parents are carriers, each is heterozygous for the abnormal gene, and each child will have a 1 in 4 chance of having the disorder and an increased chance that the child will be homozygous for the disorder. Homozygous means that the person is carrying a gene with identical alleles for a given trait. Examples of autosomal recessive disorders that are discussed in this chapter are CF, phenylketonuria, and three types of spinal muscular atrophy (SMA). Sex-linked inheritance means that the abnormal gene is carried on the X chromosome. Just as autosomes can have dominant and recessive expressions, so can sex chromosomes. In X-linked recessive inheritance, females with only one abnormal allele are carriers for the disorder, but they usually do not exhibit any symptoms because they have one normal X chromosome. Each child born to a carrier mother has a 1 in 2 chance of becoming a carrier, and each son has a 1 in 2 chance of having the disorder. The most common examples of X-linked recessive disorders are DMD and hemophilia, a disorder of blood coagulation. FXS is the most common X-linked disorder that causes intellectual disability in males. Rett syndrome is also X-linked and seen predominately in females. A discussion of genetically transmitted disorders follows—first chromosome abnormalities and then specific gene defects. 355 Down syndrome DS is the leading chromosomal cause of intellectual disability and the most frequently reported birth defect (CDC, 2006; Gardiner et al., 2010). Increasing maternal and paternal age is a risk factor. DS occurs in 1 in every 700 live births and is caused by a genetic imbalance resulting in the presence of an extra 21st chromosome or trisomy 21 in all or most of the body’s cells. Ninety-five percent of DS cases result from a failure of chromosome 21 to split completely during formation of the egg or sperm (nondisjunction). A gamete is a mature male or female germ cell (sperm or egg). When the abnormal gamete joins a normal one, the result is three copies of chromosome 21. Fewer than 5% of children have a third chromosome 21 attached to another chromosome. This type of DS is caused by a translocation. The least common type of DS is a mosaic type in which some of the body’s cells have three copies of chromosome 21 and others have a normal complement of chromosomes. The severity of the syndrome is related to the proportion of normal to abnormal cells. Clinical Features Characteristic features of the child with DS include hypotonicity, joint hypermobility, upwardly slanting epicanthal folds, and a flat nasal bridge and facial profile (Figure 8-1). The child has a small oral cavity that sometimes causes the tongue to seem to protrude. Developmental findings include delayed development and impaired motor control. Feeding problems may be evident at birth and may require intervention. Fifty percent of children with DS also have congenital heart defects of the wall between the atrias or the ventricles (Vis et al., 2009), which can be corrected by cardiac surgery. Musculoskeletal manifestations may include pes planus (flatfoot), thoracolumbar scoliosis, and patellar dislocation as well as possible atlantoaxial instability (AAI). The incidence of AAI ranges from 10% to 15% (Mik et al., 2008). Beginning at the age of 2 years, a child’s cervical spine can and should be radiographed to determine whether AAI is present. If instability is present, the family should be educated for possible symptoms, which are listed in Box 8-1. The child’s activity should be modified to avoid stress or strain on the neck such as that which may occur when diving, doing gymnastics, and playing any contact sport. Most cases are asymptomatic (Glanzman, 2014). 356 FIGURE 8-1 Profile of a child with Down syndrome. Box 8-1 Symptoms of Atlantoaxial Instability Hyperreflexia Clonus Babinski sign Torticollis Increased loss of strength Sensory changes Loss of bowel or bladder control Decrease in motor skills (Source: Glanzman A: Genetic and developmental disorders. In Goodman CC, Fuller KS, editors: Pathology: implications for the physical therapist, ed 2. Philadelphia, 2003, WB Saunders, pp. 1161-1210.) After over a decade of support for screening for AAT in children with DS, the American Academy of Pediatrics’ Committee on Sports Medicine and Fitness withdrew support of this practice in 1995. Others still recommend the practice and support family and community awareness of the potential problems with AAI in children with DS (Cassidy and Allanson, 2001; Glanzman, 2014; Pueschel, 1998). As physical therapists and physical therapist assistants working with families of children with DS, we have a responsibility to provide such education and advocate for screening. Major sensory systems, such as hearing and vision, may be impaired in children with DS. Visual impairments may include nearsightedness (myopia), cataracts, crossing of the eyes (esotropia), nystagmus, and astigmatism. Mild to moderate hearing loss is not uncommon. Either a sensorineural loss, in which the eighth cranial nerve is damaged, or a conductive loss, resulting 357 from too much fluid in the middle ear, may cause delayed language development. These problems must be identified early in life and treated aggressively so as to not hinder the child’s ability to interact with caregivers and the environment and to develop appropriate language skills. Intelligence As stated earlier, DS is the major cause of intellectual disability in children. Intelligence quotients (IQs) within this population range from 25 to 50, with the majority falling in the mild to moderate range of intellectual disability (Ratliffe, 1998). To be diagnosed with an intellectual disability, a child’s IQ has to be 70 to 75 or below. The American Association on Intellectual Developmental Disabilities has been trying to move away from defining intellectual disability based only on IQ scores. Their definition of intellectual disability means the person is limited in intelligence and in adaptive skills. Adaptive skills can include but not be limited to communication, self-care, and ability to engage in social roles. If effective early intervention programs can be designed and used in the preschool years, the subsequent educational progress of a child with DS may be altered significantly. An “educable” person is defined as one who is capable of learning such basic skills as reading and arithmetic and is quite capable of self-care and independent living (those with mild intellectual disability are generally considered educable). Although trainable (moderate intellectual disability) persons are very limited in educational attainments, they can benefit from simple training for self-care and vocational tasks (Bellenir, 2004). Development Motor development is slow, and without intervention the rate of acquisition of skills declines. Difficulty in learning motor skills has always been linked to the lack of postural tone and, to some extent, to hypermobile joints. Ligamentous laxity with resulting joint hypermobility is thought to be due to a collagen defect. The hypotonia is related not only to structural changes in the cerebellum but also to changes in other central nervous system structures and processes. These changes are indicative of missing or delayed neuromaturation in DS. As a result of the low tone and joint laxity, it is difficult for the child with DS to attain head and trunk control. Weight bearing on the limbs is typically accomplished by locking extremity joints such as the elbows and knees. These children often substitute positional stability for muscular stability, as in W sitting, to provide trunk stability in sitting, rather than dynamically firing trunk muscles in response to weight shifting in a position. Children with DS often avoid activating trunk muscles for rotation and prefer to advance from prone to sitting over widely abducted legs (Figure 8-2). Table 8-1 compares the age at which motor tasks may be accomplished by children with DS and typically developing children. Infant intervention has been shown to have a positive impact on developing motor skills and overall function in these children (Connolly et al., 1993; Hines and Bennett, 1996; Ulrich et al., 2001; Ulrich et al., 2008). 358 FIGURE 8-2 A-D, Common abnormal prone-to-sitting maneuver pattern noted in children with Down syndrome. (Reprinted from Lydic JS, Steele C: Assessment of the quality of sitting and gait patterns in children with Down syndrome. Phys Ther 59:1489-1494, 1979. With permission of the APTA.) Table 8-1 Predicted Probability (%) of Children with DS Achieving Milestones Based on Logistic Regression Age (months) Skil 6 12 18 24 aD OIC ca CE sic_[ [7 [0 [io] sof so 09000 ‘caval 10 [19 [50 [5 [rm [oe [oe | [0 nets [oa [oo [> [or [ow [0 pose [oie [oe fv From Palisano RJ, Walter SD, Russell DJ, et al: Gross motor function of children with Down syndrome: Creation of motor growth curves. Arch Phys Med Rehabil 82:494—500, 2001. Individuals with DS can live in group communities that foster independence and self-reliance. Some individuals with DS have been employed in small and medium-sized offices as clerical 359 workers or in hotels and restaurants. Batshaw et al. (2013) credit the introduction of supported employment in the 1980s with providing the potential for adults with DS to obtain and to hold a job. In supported employment, the individual has a job coach. Crucial to the individual's job success is the early development and maintenance of a positive self-image and a healthy self-esteem, along with the ability to work apart from the family and to participate in personal recreational activities. Fitness is decreased in individuals with DS. Dichter et al. (1993) found that a group of children with DS had reduced pulmonary function and fitness compared with age-matched controls without disabilities. Other researchers have found children with DS to be less active, and 25% of them become overweight (Pueschel, 1990; Sharav and Bowman, 1992). Lack of cardiorespiratory endurance and weak abdominal muscles have been linked to the reductions in fitness (Shields et al., 2009). Because of increased longevity, fitness in every person with a disability needs to be explored as another potential area of physical therapy intervention. Barriers to exercise for people with DS have been identified as lack of a support person and appropriate levels of interaction (Heller et al., 2002; Menear, 2007). When physical therapy students mentored adolescents with DS to exercise, the student’s attitudes toward working with a person with disabilities improved considerably. Life expectancy for individuals with DS has increased to 60 years (Bittles et al., 2006). The increase has occurred despite the higher incidence of other serious diseases in this population. Children with DS have a 15% to 20% higher chance of acquiring leukemia during their first 3 years of life. Again, the cure rate is high. The last major health risk faced by these individuals is Alzheimer disease. Every person with DS who lives past 40 years develops pathologic signs of Alzheimer disease, such as amyloid plaques and neurofibrillary tangles. Individuals with DS produce more of the B-amyloid that makes up the plaques because the gene that produces the protein is located on the 21st chromosome (Head and Lott, 2004). Adults with DS over 50 years old are more likely to regress in adaptive behavior than are adults with intellectual disability without DS (Zigman et al., 1996). This could be explained by the inability of the adult with DS to counteract oxidative stress from abundance of free radicals in the brain (Pagano and Castello, 2012). Three- fourths of adults who live past 65 years of age have signs of dementia (Lott and Dierssen, 2010). Child’s Impairments and Interventions The physical therapist’s examination and evaluation of a child with DS typically identifies the following impairments to be addressed by physical therapy intervention: 1. Delayed psychomotor development 2. Hypotonia 3. Hyperextensible joints and ligamentous laxity 4. Impaired respiratory function 5. Impaired exercise tolerance Early physical therapy is important for the child with DS. A case study of a child with DS is presented at the end of the chapter to illustrate general intervention strategies with a child with low muscle tone, because the impairments demonstrated by these children are similar. These interventions could be used with any child who displays low muscle tone or muscle weakness secondary to genetic disorders such as cri-du-chat syndrome, PWS, and SMA. Body-Weight Support Treadmill Training Children with DS walk independently between 18 months and 3 years (Palisano et al., 2001). Research has shown that infants with DS who participant in body-weight support treadmill training walk early than typically developing children with DS. Early ambulation in this population is beneficial as it supports development in other areas such as language and cognition. Ulrich et al. (2001) were the first to show that using treadmill training accelerated the developmental outcome of independent ambulation in children with DS. As little as 8 minutes five times a week produced change. When a higher intensity was compared with a lower intensity, the children in the higher intensity group walked 3 months earlier than the children in the lower intensity group (Ulrich et al., 2008). Orthoses Children with DS have low tone and joint hypermobility. Instability in the lower extremity may not 360 allow the child to experience a stable base while in standing or when attempting to walk. Martin (2004) studied use of supramalleolar orthoses (SMOs) in children with DS to determine the effect of their use on independent ambulation. Children showed significant improvement in standing and walking, running, and jumping on the Gross Motor Function Measure, both at the initial fitting and after wearing the orthoses for 7 weeks. Balance improved at the end of the 7-week period. Looper and Ulrich (2010) found that too early use of SMOs while the child engaged in treadmill training actually deterred onset of walking. However, in order to use an orthosis with the children, the treadmill training did not begin until the child pulled to standing, a milestone that is delayed in children with DS. More recently, Looper et al. (2012) compared the effect of two types of orthoses on the gait of children with DS. They compared a foot orthosis (FO) and an SMO. The results were not clearly in favor of one orthosis over another. There were strong correlations found between the use of each orthosis and specific gait parameters. Body-weight support treadmill training appears to have a positive effect on achievement of early ambulation; however, use of an orthosis during treadmill training may not be indicated. After achievement of independent ambulation, an orthosis may be needed to address gait deviations, such as foot angle, walking speed, amount of pronation during stance phase (Selby-Silverstein et al., 2001). As pointed out by Nervik and Roberts (2012), the best practice continues to be individualized recommendations for use of orthoses and trials of different orthoses in order to make the best decision. 361 CRI-DU-Chat syndrome When part of the short arm of chromosome 5 is deleted, the result is the cat-cry syndrome, or cri- du-chat syndrome. The chromosome abnormality primarily affects the nervous system and results in intellectual disability. The incidence is 1 in 20,000 to 1 in 50,000 live births (Online Mendelian Inheritance in Man [OMIM], 2014). One percent of institutionalized individuals with intellectual disability may have this disorder (Carlin, 1995). Characteristic clinical features include a catlike cry, microcephaly, widely spaced eyes, and profound intellectual disability. The cry is usually present only in infancy and is the result of laryngeal malformation, which lessens as the child grows. Although usually born at term, these children exhibit the result of intrauterine growth retardation by being small for their gestational age. Microcephaly is diagnosed when the head circumference is less than the third percentile. Together, these features constitute the cri-du-chat syndrome, but any or all of the signs can be noted in many other congenital genetic disorders. Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with cri-du-chat syndrome typically identifies the following impairments or potential problems to be addressed by physical therapy intervention: 1. Delayed psychomotor development 2. Hypotonia 3. Delayed development of postural reactions 4. Hyperextensible joints 5. Contractures and skeletal deformities 6. Impaired respiratory function Musculoskeletal problems that may be associated with cri-du-chat syndrome include clubfeet, hip dislocation, joint hypermobility, and scoliosis. Muscle tone is low—a feature that may predispose the child to problems related to musculoskeletal alignment. In addition, motor delays also result from a lack of the cognitive ability needed to learn motor skills. Postural control is difficult to develop because of the low tone and nervous system immaturity. Physically, the child’s movements are laborious and inconsistent. Gravity is a true enemy to the child with low tone. Congenital heart disease is also common, and severe respiratory problems can be present (Bellamy and Shen, 2013). Life expectancy has improved to almost normal with better medical care (Chen, 2013). 362 Prader-willi syndrome and angelman syndrome PWS is the other example of a syndrome caused by a partial deletion of a chromosome; in this case, a microdeletion of a part of the long arm of chromosome 15. The incidence of this syndrome originally described by Prader et al. in 1956 is thought to be about 1 in 10,000 to 1 in 30,000 (Batshaw et al., 2013). The disorder is more common than cri-du-chat syndrome. In fact, it is one of the most common microdeletions seen in genetic clinics (Dykens et al., 2011). Diagnosis is usually made based on the child’s behavior and physical features and confirmed by genetic testing. Features include obesity, underdeveloped gonads, short stature, hypotonia, and mild to moderate intellectual disability. These children become obsessed with food at around the age of 2 years and exhibit hyperphagia (excessive eating). Before this age they have difficulty in feeding secondary to low muscle tone, gain weight slowly, and may be diagnosed as failure to thrive. Children with PWS are very delayed in attainment of motor milestones during the first 2 years of life and often do not sit until 12 months and do not walk until 24 months (Dykens et al., 2011). Obesity can lead to respiratory compromise with impaired breathing and cyanosis. PWS is the most common genetic form of obesity. Maladaptive behavior is part of the behavioral phenotype of this genetic condition and includes temper tantrums, obsessive compulsive disorder, self-harm, and lability. If a child inherits the deletion from the father, the child will have PWS, but if the child inherits the deletion from the mother, the child will have Angelman syndrome. This variability of expression depending on the sex of the parent is called genomic imprinting. This phenomenon is a result of differential activation of genes on the same chromosome. Angelman syndrome (AS) is characterized by significantly delayed development, intellectual disability, ataxia, severe speech problems, and progressive microcephaly. Delays are not apparent until around 6 to 12 months of age. There may be problems with sucking and swallowing, drooling, or tongue thrusting in 20% to 80% of children (Bellamy and Shen, 2013). They have a happy affect and display hand-flapping movements. Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with PWS typically identifies the following impairments or potential problems to be addressed by physical therapy intervention: 1. Impaired feeding (before age 2) 2. Hypotonia 3. Delayed psychomotor development 4. Obesity (after age 2) 5. Impaired respiratory function Intervention must match the needs of the child based on age. The infant may need oral motor therapy to improve the ability to feed. Positioning for support and alignment is necessary for feeding and carrying. Techniques for fostering head and trunk control should be taught to the caregivers. As the child’s appetite increases, weight control becomes crucial. The aim of a preschool program is to provide interventions to establish and improve gross-motor abilities. Food control must be understood by everyone working with the child with PWS. Attention in the school years is focused on training good eating habits while improving tolerance for aerobic activity. This is continued throughout adolescence, when behavioral control appears to be the most successful means for controlling weight gain. “Interventions should be directed toward increasing muscle strength, aerobic endurance, postural control, movement efficiency, function, and respiration to manage obesity and minimize cardiovascular risk factors and osteoporosis” (Lewis, 2000). Suggested activities for strength training at various ages can be found in Table 8-2. These activities would be appropriate for most children with weakness. Aquatic exercise is also an ideal beginning aerobic activity for the child with severe obesity (Lewis, 2000). Additional aerobic activities for different age groups are found in Table 8-3. They, too, have general applicability to most children with developmental deficits. Box 8- 2 details outcome measures that could be used to document changes in strength and aerobic conditioning in the PWS population. Some of these measures may be applicable with children with other developmental diagnoses, while others may be difficult due to lack of motor control. Table 8-2 363 Activities for Strength Training Activities to Strengthen Muscles of Respiration Blood pressure | Younger children Wheelbarrow walks Squats Sit-ups Blowing Breath Push/pull a wagon Vertical jumping Bridges bubbles holding Vertical drawing Stair climbing Trunk rotations Straw Stabilization Lifting objects Walking on toes Stand up from supine sucking Scooter board Ball kicking Swing a weighted bat Blowing Walking sideways balloons Cotton ball hockey Singing Chair pushups Blood pressure | Older Elastic bands, hand weights, Elastic bands, ankle weights, games, music, Swiss ball Breath children/younger games, music, dance dance Incline sit-ups holding adolescents Broad jumping Foam rollers Blood pressure | Older Strength training: bicep curls, Strength training: hamstring curls, quadriceps, | Strength training: abdominal adolescents/young, triceps, latissimus pulls extensions, squats, toe raises crunches, obliques adults Modified from Lewis CL: Prader-Willi syndrome: A review for pediatric physical therapists. Pediatr Phys Ther 12:87—95, 2000; Young HJ: The effects of home fitness programs in preschoolers with disabilities. Chapel Hill, NC, Program in Human Movement Science with Division of Physical Therapy. University of North Carolina, Chapel Hill, 1996:50. Thesis. Table 8-3 Activities for Aerobic Conditioning Ages Activities Younger children Bunny hopping Running long jump Running up and down steps or incline Running up and down hills Riding a tricycle Sitting on a scooter board and propelling with the feet] Older children/younger adolescents} Bike riding Stationary bike riding Brisk walking Water aerobics Roller skating Roller-blading Ice skating Cross-country skiing Downhill skiing Older adolescents/younger adults | Same as above, plus: Dancing Low-impact step aerobics Jazzercise Aerobic circuit training From Lewis CL: Prader-Willi syndrome: A review for pediatric physical therapists. Pediatr Phys Ther 12:87—95, 2000, p. 92. Box 8-2 Clinical Outcome Measures Measures of Strength Training = Grip dynamometer: before and after training (average of five trials) = Myometer of target muscles: before and after training (average of five trials) = One or six repetition maximum (1 RM, 6 RM)*: before and after training (average over three different days)' = Standing long jump distance: before and after training (average of five trials)’ Measures of Aerobic Conditioning = Heart rate: measure the radial pulse or use a heart rate monitor; establish baseline over a 5-day period = Improved cardiovascular function documented by decreased resting heart rate; decreased heart rate during steady state (2 minutes into the activity); time it takes for heart rate to return to preactivity level 364 = Timed performance of activities such as 50-foot sprint, seven sit-ups, stair climbing = Two- or 6-minute walk/run/lap swim time: maximum distance covered divided by time m Determine energy expenditure index (EEI) of gait: working HR minus resting HR divided by speed (From Lewis CL: Prader-Willi syndrome: A review for pediatric physical therapists. Pediatr Phys Ther 12:87-95, 2000, p. 92.) —— LE ee | “1 RM is the maximum amount of weight that can be lifted one time; 6 RM is the maximum amount of weight that can be lifted six times. * From 1985 School Population Fitness Survey. Washington, DC, 1985, President’s Council on Physical Fitness and Sports. + Rose J, Gamble J, Lee J, et al: The energy expenditure index: A method to quantitate and compare walking energy expenditure for children and adolescents. J Pediatr Orthop 11:571-578, 1991. 365 Arthrogryposis multiplex congenita One-third of arthrogryposis multiplex congenita (AMC) cases have a genetic cause. The gene that causes the neuropathic form is found on chromosome 5 (Tanamy et al., 2001). Another form, distal AMC, is inherited as an autosomal dominant trait with the defective gene being traced to chromosome 9 (Bamshad et al., 1994). AMC is a nonprogressive neuromuscular syndrome that the physical therapist assistant may encounter in practice. AMC results in multiple joint contractures and usually requires surgical intervention to correct misaligned joints. AMC is also known as multiple congenital contractures. The incidence of the disorder is 1 in 3000 to 6000 live births according to Hall (2007). A 1 in 4300 prevalence has been reported in Canada (Lowry et al., 2010). Pathogenesis has been related to the muscular, nervous, or joint abnormalities associated with intrauterine movement restriction, but despite identification of multiple causes, the exact cause is still unknown. Pathophysiology and Natural History As early as 1990, Tachdjian postulated that the basic mechanism for the multiple joint contractures seen in AMC was a lack of fetal movement. That hypothesis has been accepted in that AMC can result from any condition that limits fetal movement (Glanzman, 2014). Myopathic and neuropathic causes have been linked to multiple nonprogressive joint contractures. If muscles around a fetal joint do not provide enough stimulation (muscle pull), the result is joint stiffness. If the anterior horn cell does not function properly, muscle movement is lessened, and contractures and soft tissue fibrosis occur. Muscle imbalances in utero can lead to abnormal joint positions. The first trimester of pregnancy has been identified as the most likely time for the primary insult to occur to produce AMC. Although the contractures themselves are not progressive, the extent of functional disability they produce is significant, as seen in Figure 8-3. Limitation in mobility and in activities of daily living (ADLs) can make the child dependent on family members. FIGURE 8-3 A, An infant with arthrogryposis multiplex congenita (AMC) with flexed and dislocated hips, extended knees, clubfeet (equinovarus), internally rotated shoulders, flexed elbows, and flexed and ulnarly deviated wrists. B, An infant with AMC with abducted and externally rotated hips, flexed knees, clubfeet, internally rotated shoulders, extended elbows, and flexed and ulnarly deviated wrists. (From Donohoe M: Arthrogryposis multiplex congenita. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders.) Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with AMC typically identifies the 366 following impairments to be addressed by physical therapy intervention: 1. Impaired range of motion 2. Impaired functional mobility 3. Limitations in ADLs, including donning and doffing orthoses Early physical therapy intervention focuses on assisting the infant to attain head and trunk control. Depending on the extent of limb involvement, the child may have difficulty in using the arms for support when initially learning to sit or catch himself or herself when losing balance. Most of these children become ambulatory, but they may need some assistance in finding ways to go up and down the stairs. An adapted tricycle can provide an alternative means of mobility before walking is mastered (Figure 8-4). Functional movement and maintenance of range of motion are the two major physical therapy goals for a child with this physical disability. No cognitive deficit is present; therefore, the child with AMC should be able to attend regular preschool and school. Table 8-4 gives an overview of the management of the child with AMC across the life span. FIGURE 8-4 Adapted tricycle. (Reprinted by permission of the publisher from Connor FP, Williamson GG, Siepp JM, editors: Program guide for infants and toddlers with neuromotor and other developmental disabilities, p. 361. [New York, Teachers College Press, © 1978 Teachers College, Columbia University. All rights reserved.]) Table 8-4 Management of Arthrogryposis Multiplex Congenita, or Multiple Congenital Contractures 367 Time Period Goals Strategies Medical/Surgical Home Program Infancy Maximize strength Teach rolling Clubfoot surgery by age 2__| Stretching 3-5 times a day Increase ROM Floor scooting years Standing 2 hours a day Enhance sensory and motor Streng thening Splints adjusted every 4-6 Positioning development Stretching weeks Positioning Preschool Decrease disability Solve ADL challenges Stroller for community Stretching twice a day Enhance ambulation Gait training Articulating AFOs Positioning Maximize ADLs Stretching, positioning Splints Play groups, sleepovers, sports Establish peer relationships Promote selfesteem School-age and Strengthen peer relationships Adaptive physical education Manual wheelchair for Sports, social activities adolescent Independent mobility Environmental adaptations, community Selfdirected stretching and prone Preserve ROM stretching Power mobility positioning Compensatory for ADLs Surgery Personal hygiene Adulthood Independent in ADLs with/without Joint protection and Flexibility assistive devices conservation Positioning Ambulation/mobility Assess accessibility Endurance Driving Assistive technology Data from Donohoe M: Arthrogryposis multiplex congenita. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders, pp. 313-332. ADLs, Activities of daily living; AFOs, ankle-foot orthoses; ROM, range of motion. Range of Motion Range-of-motion exercises and stretching exercises are the cornerstone of physical therapy intervention in children with AMC. Initially, stretching needs to be performed three to five times a day. Each affected joint should be moved three to five times and held for 20 to 30 seconds at the end of the available range. Because these children have multiple-joint involvement, range of motion requires a serious commitment on the part of the family. Incorporating stretching into the daily routine of feeding, bathing, dressing, and diaper changing is warranted. As the child grows older, the frequency of stretching can be decreased. The school-age child should begin to take over responsibility for his or her own stretching program. Although stretching is less important once skeletal growth has ceased, flexibility remains a goal to prevent further deformities from developing. Joint preservation and energy conservation techniques are legitimate strategies for the adult with AMC. Positioning Positioning options depend on the type of contractures present. If the joints are more extended in the upper extremity, this will hamper the child’s acceptance of the prone position and will require that the chest be supported by a roll or a wedge. Too much flexion and abduction in the lower extremities may need to be controlled by lateral towel rolls or a Velcro strap (Figure 8-5). Quadruped is not a good posture to use because it reinforces flexion in the upper and lower extremities. Prone positioning is an excellent way to stretch hip flexion contractures while encouraging the development of the motor abilities of the prone progression. A prone positioning program should be continued throughout the life span. 368 FIGURE 8-5 This child with arthrogryposis multiplex congenita is wearing a wide Velcro band strapped around the thighs to keep the legs in more neutral alignment. (From Donohoe M, Bleakney DA: Arthrogryposis multiplex congenita. In Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 2. Philadelphia, 2000, WB Saunders.) Functional Activities and Gait Rolling and scooting on the bottom are used as primary means of floor mobility. Development of independent sitting is often delayed because of the child’s inability to attain the position, but most of these children do so by 15 months of age. Placement in sitting and encouragement of static sitting balance with or without hand support should begin early, at around 6 months of age. Focus on dynamic balance and transitions into and out of sitting while using trunk flexion and rotation should follow. Nine months is an appropriate age for the child to begin experiencing weight bearing in standing. For children with plantar flexion contractures, shoes can be wedged to allow total contact of the foot with the support surface. In some cases, a standing frame or parapodium, as is used with children with myelomeningocele, can be beneficial (Figure 8-6). Other children benefit from use of supine or prone standers. The standing goal for a 1-year-old child is 2 hours a day (Donohoe and Bleakney, 2000). Strengthening of muscles needed for key functional motor skills, such as rolling, sitting, hitching (bottom scooting), standing, and walking, is done in play. Reaching to roll, rotation in sitting and standing, and movement transitions into and out of postures can facilitate carryover into functional tasks. Toys should be adapted with switches to facilitate the child’s ability to play, and adaptive equipment should be used to lessen dependence during ADLs. 369 FIGURE 8-6 A child with arthrogryposis multiplex congenita who is using a standing frame. (From Donohoe M: Arthrogryposis multiplex congenita. In Campbell SK, Palisano RJ, Orline MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders.) Ambulation is achieved by most children with AMC by 18 months of age (Donohoe and Bleakney, 2000). Because clubfoot is often a part of the presentation in AMC, its presence must be dealt with in the development of standing and walking. Early surgical correction of the deformity often requires later surgical revisions, so investigators have suggested that surgery occur after the child is stronger and wants to walk, at around the end of the first year of life. The operation should be performed by the time the child is 2 years old to avoid the possibility of having to do more bony surgery, as opposed to soft-tissue corrections. Use of orthoses for ambulation depends on the strength of the lower extremity extensors and the types of contractures found at the hip, knee, and ankle. Less than fair muscle strength at a joint usually indicates the need for an orthosis at that joint. For example, if the quadriceps muscles are scored less than 3 out of 5 on manual muscle testing, then a knee-ankle-foot orthosis (KAFO) is indicated. Children with knee extension contractures tend to require less orthotic control than those with knee flexion contractures (Donohoe, 2012). Children with weak quadriceps or knee flexion contractures may need to walk with the knees of the KAFO locked. Functional ambulation also depends on the child’s ability to use an assistive device. Because of upper extremity contractures, this may not be possible, and adaptations to walkers and crutches may be needed. Polyvinyl chloride pipe can often be used to fabricate lightweight walkers or crutches to give the child maximal independence (Figure 8-7). Power mobility may provide easy and efficient environmental access for a child with weak lower extremities and poor upper extremity function. Some school-age children or adolescents routinely use a manual wheelchair to keep up with peers in a community setting. 370 FIGURE 8-7 Thermoplastic forearm supports can be customized to the walker for the child with arthrogryposis multiplex congenita. (From Donohoe M: Arthrogryposis multiplex congenita. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders.) 371 Osteogenesis imperfecta Ol is an autosomal dominant disorder of collagen synthesis that affects bone metabolism. The original classification scheme of four types was devised by Sillence et al. (1979) based on clinical examination, x-ray findings, and type of inheritance. Recent research in molecular genetics has resulted in the identification of three more types, expanding the number of types from four to seven. The first four types are listed in Table 8-5. Type V and VI represent only a small percentage of cases and type VII is only found in a certain population. Types I and IV account for 95% of all cases (Martin and Shapiro, 2007). All four types are inherited as an autosomal dominant trait, which occurs in 1 per 10,000 live births. Each type has a different degree of severity. Depending on the type of OI, the infant may be born with multiple fractures or may not experience any broken bones until reaching preschool age. The more fragile the skeletal system, the less likely it is that a physical therapist assistant will be involved in the child’s therapy. It would be more likely for an assistant to treat children with types I and IV because these are the most common. Individuals with OI have “brittle bones.” Many also exhibit short stature, bowing of long bones, ligamentous joint laxity, and kyphoscoliosis. Average or above-average intelligence is typical. Table 8-5 Classification of Osteogenesis Imperfecta Type Characteristics Severity Most severe (perinatal lethal) Po Data from Donohoe M: Osteogenesis Imperfecta. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders, pp. 332-352; Engelbert et al., 2000; Glanzman, 2014. AD, Autosomal dominant. Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with OI typically identifies the following impairments to be addressed by physical therapy intervention: . Impaired range of motion . Impaired strength . Pathologic fractures . Delayed motor development . Impaired functional mobility . Limitations in ADLs . Impaired respiratory function . Scoliosis Children with milder forms of OI are seen for strengthening and endurance training in a preschool or school setting. Every situation must be viewed as being potentially hazardous because of the potential for bony fracture. Safety always comes first when dealing with a potential hazard; therefore, orthoses can be used to protect joints, and playground equipment can be padded. No extra force should be used in donning and doffing orthoses. Signs of redness, swelling, or warmth may indicate more than excessive pressure and could indicate a fracture. ANDO WNH Caution Fracture risk is greatest during bathing, dressing, and carrying. Baby walkers and jumper seats should be avoided. All trunk or extremity rotations should be active, not passive. 372 Social interaction may need to be structured if the child with OI is unable to participate in many, if any, sports-related activities. Being the manager of the softball or soccer team may be as close as the child with OI can be to participating in sports. Table 8-6 provides an overview of the management of a child with OI across the life span. Table 8-6 Therapeutic Management of Osteogenesis Imperfecta Time Period Goals Therapeutic Interventions Infancy Safe handling and positioning Even distribution of body weight Development of age-appropriate skills) Padded carrier Prone, side-lying, supine, sitting positions Pull-to-sit transfer contraindicated Preschool Protected weight bearing Use of contour-molded orthoses for compression and support in standing, Safe independent self-mobility Adaptive devices Light weights, aquatic thera) School age and adolescence] Maximizing independence Mobility cart, HKAFOs, clamshell braces, air splints Maximizing endurance Ambulation without orthoses as fracture rate declines Maximizing strength Wheelchair for community ambulation Peer relationships Adaptive physical education Boy Scouts, Girl Scouts, 4-H Adulthood Appropriate career placement Career counseling Job site evaluation Data from Donohoe M: Osteogenesis imperfecta. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders, pp. 333-352. HKAFOs, Hip-knee-ankle-foot orthoses. Handling and Positioning Parents of an infant with OI must be taught to protect the child while carrying him or her on a pillow or in a custom-molded carrier. Handling and positioning are illustrated in Intervention 8-1. All hard surfaces must be padded. Protective positioning must be balanced with permitting the infant’s active movement. Sandbags, towel rolls, and other objects may be used. Greatest care is needed when dressing, diapering, and feeding the child. When handling the child, caregivers should avoid grasping the child around the ankles, around the ribs, or under the arms because this may increase the risk of fractures. Clothing should be roomy enough so that it fits easily over the child’s head. Temperature regulation is often impaired, so light, absorbent clothing is a good idea. A plastic or spongy basin is best for bathing. Despite all precautions, infants may still experience fractures. The physical therapist assistant will most likely not be involved in the initial stages of physical therapy care for the infant with OI because of the patient’s fragility. However, if the physical therapist assistant is involved later, he or she does need to be knowledgeable about what has been taught to the family. Intervention 8-1 Handling a Child with Osteogenesis Imperfecta 373 A. In handling a young child with osteogenesis imperfecta, support the neck and shoulders and the pelvis with your hands; do not lift the child from under the arms. B. Placing the child on a pillow may make lifting and holding easier. (From Myers RS: Saunders manual of physical therapy practice, Philadelphia, 1995, WB Saunders.) Positioning should be used to minimize joint deformities. Using symmetry with the infant in supine and side lying positions is good. A wedge can be placed under the chest when the infant is in prone to encourage head and trunk movement while providing support (Figure 8-8). The child’s feet should not be allowed to dangle while sitting but should always be supported. Water beds are not recommended for this population because the pressure may cause joint deformities. FIGURE 8-8 Prone positioning of a child on a wedge encourages head and trunk movement and upper extremity weight bearing. 374 Range of Motion and Strengthening By the time the child is of preschool age, not only are the bones still fragile, the joints lax, and the muscles weak, but the child also has probably developed disuse atrophy and osteoporosis from immobilization secondary to fractures in infancy or childhood. OI has a variable time of onset depending on the type. Range of motion and strengthening are essential. Active movement promotes bone mineralization, and early protected weight bearing seems to have a positive effect on the condition. Range of motion in a straight plane is preferable to diagonal exercises, with emphasis placed on the shoulder and pelvic girdles initially. Light weights can be used to increase strength, but they need to be placed close to the joint to limit excessive torque. Pool exercise is good because the water can support the child’s limbs, and flotation devices can be used to increase buoyancy. Water is an excellent medium for active movement progressing to some resistance as tolerated. The child’s respiratory function can be strengthened in the water by having the child blow bubbles and hold his or her breath. Deep breathing is good for chest expansion, which may be limited secondary to chest wall deformities. The water temperature needs to be kept low because of these children’s increased metabolism (Donohoe, 2012). Increased endurance, protected weight bearing, chest expansion, muscle strengthening, and improved coordination are all potential benefits of aquatic intervention. Initial sessions in the pool are short, lasting for only 20 to 30 minutes (Cintas, 2005). Functional Activities and Gait Developmental activities should be encouraged within safe limits (Intervention 8-2). Use proximal points from which to handle the child and incorporate safe, lightweight toys for motivation. Reaching in supine, side lying, and supported sitting can be used for upper extremity strengthening, as well as for encouraging weight shifting. Rolling is important as a primary means of floor mobility. Prepositioning one upper extremity beside the child’s head as the child is encouraged to roll can be beneficial. All rotations should be active, not passive (Brenneman et al., 1995). Performing a traditional pull-to-sit maneuver is contraindicated. The assistant or caregiver should provide manual assistance at the child’s shoulders to encourage head lifting and trunk activation when the assistant is helping the child into an upright position. Intervention 8-2 Developmental Activities for a Child with Osteogenesis Imperfecta A. The emphasis is on sitting with an erect trunk. B. All rotations should be active. C. Weight bearing on the arms and legs is indicated as tolerated. 375 (From Myers RS: Saunders manual of physical therapy practice, Philadelphia, 1995, WB Saunders.) Sitting needs to be in erect alignment, as compared with the typical progression of children from prop sitting to no hands, because propping may lead to a more kyphotic trunk posture. External support may be necessary to promote tolerance to the upright position, such as with a corner seat or a seat insert. Sling seats in strollers and other seating devices should be avoided because they do not promote proper alignment. Once head control is present, short sitting or sitting straddling the caregiver's leg or a bolster can be used to encourage active trunk righting, equilibrium, and protective reactions. These sitting positions can also be used to begin protected weight bearing for the lower extremities, such as that seen in Figure 8-9. Scooting on a bolster or a bench can be the start of learning sitting transfers. Sitting and hitching are primary means of floor mobility for the child with OI after rolling and are used until the child masters creeping. A scooter propelled by a child’s arms or legs can be used for mobility (Figure 8-10). FIGURE 8-9 Straddle roll activity of supported sit-to-stand for lower extremity strengthening and weight bearing. (From Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders, p. 343.) 376 @, FIGURE 8-10 Scooter used for mobility that can be propelled by a child’s legs or arms. (From Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders, p. 344.) Transition to Standing The child with OI should have sufficient upright control to begin standing during the preschool period. Prior to that time, standing and walking with insufficient support will put too much weight on the lower extremities and will produce further bending and bowing of the long bones. Susceptibility to fractures of these long bones is greatest between 2 years and 10 to 15 years (Jones, 2006). A child with OI should be fitted with a standing or ambulatory device by the age of 2 or 3 years (Pauls and Reed, 2004). Hip-knee-ankle-foot orthoses (HKAFOs) are used in conjunction with some type of standing frame such as a prone stander. Ambulation is often begun in the pool because of the protection afforded by the water. The child is then progressed to shallow water. Water can also be used to teach ambulation for the first time or to retrain walking after a fracture, but lightweight plastic splints should also be used. Duffield (1983) suggested the following progression in water: (1) in parallel bars or a standing frame, with a weight shift from side to side, forward, and backward, and (2) forward walking. Motor skill development is delayed because of fractures and also because muscles are poorly developed and joints are hypermobile. The disease type and ability to sit by 9 or 10 months of age are the best predictors of ambulatory status (Daley et al., 1996; Engelbert and Uitervaal, 2000). Most children with type I OI will be ambulatory within their household and about half will become community ambulators without the need for any assistive device (Glanzman, 2014). This is in contrast to children with type III, in which almost 50% will depend on power mobility. Medical Management Typically developing children without disabilities form 7% more bone than is resorbed when their bones grow and remodel. Children with mild forms of OI only form 3% more bone than they resorb 377 (Batshaw et al., 2013). Prior to the last decade, there had really not been any substantive medical management of children with OI other than surgical. Many types of therapy have been tried to enhance bone formation, such as prescribing calcitonin, fluoride, and vitamin D, but none of these have been found to be successful. Pamidronate therapy has become the standard of care for those children with moderate to severe OI (Glorieux, 2007). Pamidronate is a bisphosphonate that is a powerful anitresorptive agent. It has been found to increase bone density, decrease bone pain, and increase the ability of the patients to ambulate (Land et al., 2006; DiMeglio and Peacock, 2006). Pamidronate is administered intravenously in 3-day cycles (Glorieux, 2007). Positive effects have not been documented in mild cases. Orthotic and Surgical Management Orthoses are made of lightweight polypropylene and are created to conform to the contours of the child’s lower extremity. Initially, the orthosis may have a pelvic band and no knee joints for maximum stability. As strength and control increase, the pelvic band may be removed, and knee joints may be used. Some orthoses have a clamshell design that includes an ischial weight-bearing component, a feature borrowed from lower extremity prostheses. The ambulation potential of a child with OI is highly variable, so orthotic choices are, too. From using a standing frame and orthosis, the child progresses to some type of KAFO with the knees locked in full extension (Figure 8-11). The child first ambulates in the safety of the parallel bars, then moves to a walker, and finally progresses to crutches as limb strength and coordination improve. “Most children ambulate without braces when the fracture rate decreases” (Donohoe, 2012, p. 345). FIGURE 8-11 Acchild with osteogenesis imperfecta who is using long-leg braces and a rollator posture walker. (From Bleakney DA, Donohoe M: Osteogenesis imperfecta. In Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 3. Philadelphia, 2006, WB Saunders.) 378 Healing time for fractures in children with OI is normally 4 to 6 weeks, the same as in children without the condition. What is not normal is the number of fractures these children can experience. Intramedullary rod fixation is the best way to stabilize fractures that occur in the long, weight- bearing bones. Special telescoping rods developed by Bailey and Dubow (1965) allow the child’s bones to grow with the rod in place. This type of surgical procedure is usually performed after the child is 4 or 5 years of age to allow for sufficient growth of the femur. However, one study suggests that the operation be performed when the child is between the ages of 2 and 3.5 years, potentially to improve the child’s neuromotor development (Engelbert et al., 1995). Fortunately, the frequency of fractures tends to decrease after puberty (Glorieux, 2007). Scoliosis or kyphosis occurs in 50% of children with OI (Tachdjian, 2002). Often, the child cannot use an orthosis to manage a spinal curve, because the forces from the orthosis produce rib deformities rather than controlling the spine. Curvatures can progress rapidly after the age of 5 years, with maximum deformity present by age 12 (Gitelis et al., 1983). Surgical fixation with Harrington rods is often necessary (Marini and Chernoff, 2001). In addition to compounding the short stature in the child with OI, spinal deformities can significantly impair chest wall movement and respiratory function. School Age and Adolescence The goals during this period are to maximize all abilities from ambulation to ADLs. One circumstance that may make this more difficult is overprotection of the school-age child by anyone involved with managing the student’s care. Strengthening and endurance exercises are continued during this time to improve ambulation. At puberty, the rate of fractures decreases, thus making ambulation without orthoses a possibility for the first time. Despite this change, a wheelchair becomes the primary means of mobility for most individuals for community mobility. This allows the child with OI to have the energy needed to keep up and socialize with her peer group. Proper wheelchair positioning must be assured to protect exposed extremities from deformities or trauma. The school-age child with OI has to avoid contact sports, for obvious reasons, but still needs to have some means of exercising to maintain cardiovascular fitness. Swimming and wheelchair court sports, such as tennis, are excellent choices. Strengthening and fitness programs have been undertaken in children with type I and IV OI which have resulted in functional gains. Van Brussel et al. (2008) conducted a study of a 12-week graded exercise program in children with the mildest forms of OI. In this random control trial, children who participated in 30 sessions of 45 minutes of graded exercise showed significant improvements in aerobic capacity and muscle force and a decrease in subjective fatigue. The improvements were not sustained after the intervention ended, which supports the need for ongoing exercise in this group. Caudill et al. (2010) found that weak plantar flexion in children with type I OI was correlated with function as measured by the Pediatric Outcome Data Collection Instrument, the Gillette Functional Assessment Questionnaire, and the revised Faces Pain Scale. Ambulatory children with OI need to participate in progressive strengthening and functional fitness programs. Children with OI who are not ambulatory need to increase core strength and their ability to sit and hitch or sit-scoot as these are essential for transfers and self-care into adulthood. Whole body vibration has been recommended as an intervention for immobilized children and adolescents with OI (Semler et al., 2007). Adulthood The major challenge to individuals with OI as they move into adulthood is dealing with the secondary problems of the disorder. Spinal deformity may be severe and may continue to progress. Scoliosis is present in close to 80% to 90% of teens and adults with OI (Albright, 1981). Career planning must take into account the physical limitations imposed by the musculoskeletal problems. Assisting youth with developmental disabilities to transition into the adult care system, work, and community is a relatively new role for the physical therapist (Cicirello et al., 2012). 379 380 Cystic fibrosis CF is an autosomal recessive disorder of the exocrine glands that is caused by a defect on chromosome 7. The pancreas does not secrete enzymes to break down fat and protein in 85% of these individuals. CF produces respiratory compromise, because abnormally thick mucus builds up in the lungs. This buildup creates a chronic obstructive lung disorder. A parent can be a carrier of this gene and may not express any symptoms. When one parent is a carrier or has the gene, the child has a 1 in 4 chance of having the disorder. The incidence is 1 in 3000 live births in whites. Five percent of the population carries a single copy of the CF gene which equates to 12 million people in the United States. Newborn screening is mandated in every state. Diagnosis CF is the most lethal genetic disease in whites. Diagnosis can be made on the basis of a positive sweat chloride test. Children with CF excrete too much salt in their sweat, and this salt can be measured and compared with normal values. Values greater than 60 mEq/L indicate CF. Some mothers have even stated that the child tastes salty when kissed. Because of the difficulty with digesting fat, the child may have foul-smelling stools and may not be able to gain weight. Before being diagnosed with CF, the child may have been labeled as failing to thrive because of a lack of weight gain. Prenatal diagnosis is available, and couples can be screened to detect whether either is a carrier of the gene. Pathophysiology and Natural History Even though the genetic defect has been localized, the exact mechanism that causes the disease is still unidentified. The ability of salt and water to cross the cell membrane is altered, and this change explains the high salt content present when these children perspire. Thick secretions obstruct the mucus-secreting exocrine glands. The disease involves multiple systems: gastrointestinal, reproductive, sweat glands, and respiratory. The two most severely impaired organs are the lungs and the pancreas. Diet and pancreatic enzymes are used to manage the pancreatic involvement. With life expectancy increasing, there has been an increased incidence of CF-related diabetes (CFRD) due to damage of the beta cells in the pancreas (Moran et al., 2009). The percentage of individuals with CFRD rises with increasing age such that 40% to 50% of adults with CF have this condition. The structure and function of the lungs are normal at birth. Only after thick secretions begin to obstruct or block airways, which are smaller in infants than in adults, is pulmonary function adversely affected. The secretions also provide a place for bacteria to grow. Inflammation of the airways brings in infiltrates that eventually destroy the airway walls. The combination of increased thick secretions and chronic bacterial infections produces chronic airway obstruction. Initially, this condition may be reversed with aggressive bronchial hygiene and medications. Eventually, repeated infections and bronchitis progress to bronchiectasis, which is irreversible. Bronchiectasis stretches the breathing tubes and leads to abnormal breathing patterns. Pulmonary function becomes more and more severely compromised over the life span, and the person dies of respiratory failure. Life expectancy for an individual with CF has increased over the last several decades. The median survival is into the late 30s with current newborns diagnosed with CF projected to live into their 40s (Volsko, 2009). Increase in longevity can be related to improved medical care, pharmacologic intervention, and heart and lung transplantation. The pulmonary manifestations of the disease are those that result in the greatest mortality. Sixty-seven percent of adolescents and sixteen percent of adults who receive lung transplants have CF (Boucek et al., 2003). The two biggest factors for prognosticating survival are nutrition and pulmonary function (Mahadeva et al., 1998), a higher exercise capacity has been linked to improved survival (Nixon et al., 1992). Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with CF typically identifies the following impairments to be addressed by physical therapy intervention: 381 1. Retained secretions 2. Impaired ability to clear airways 3. Impaired exercise tolerance 4. Chest wall deformities 5. Nutritional deficits Chest Physical Therapy Central to the care of the child with CF is chest physical therapy (CPT). It consists of bronchial drainage in specific positions with percussion, rib shaking, vibration, and breathing exercises and retraining. Treatment is focused on reducing symptoms. Respiratory infections are to be avoided or treated aggressively. Signs of pulmonary infection include increased cough and sputum production, fever, and increased respiration rate. Additional findings could include increased white blood cell count, new findings on auscultation or radiographs, and decreased pulmonary function test values. Unfortunately, bacteria can become resistant to certain medications over time. Parents are taught to perform postural drainage three to five times a day. Adequate fluid intake is important to keep the mucus hydrated and therefore make it easier to move and be expectorated. The child with CF receives medications to provide hydration, to break up the mucus, to keep the bronchial tubes open, and to prevent bronchial spasms. These drugs are usually administered before postural drainage is performed. Antibiotics are a key to the increased survival rate in patients with CF and must be matched to the organism causing the infection. Postural Drainage Postural drainage is the physical act of using gravity or body position to aid in draining mucus from the lungs. The breathing tubes that branch off from the two main stem bronchi are like branches of an upside-down tree, each branch becoming smaller and smaller the farther away it is from the main trunk. The position of the body for postural drainage depends on the direction the branch points. Each segment of the lobes of the lungs has an optimal position for gravity to drain the secretions and allow them to travel back up the bronchial tree to be expelled by coughing. Postural drainage or positioning for drainage is almost always accompanied by percussion and vibration. Manual vibration is shown in Intervention 8-3. Percussion is manually applied with a cupped hand while the person is in the drainage positions for 3 to 5 minutes. Proper configuration of the hand for percussion is shown in Figure 8-12. Percussion dislodges secretions within that segment of the lung, and gravity usually does the rest. The classic 12 positions are shown in Figure 8-13. Percussion and vibration should be applied only to those areas that have retained secretions. Treatment usually lasts no more than 30 minutes total, with the time divided among the lung segments that need to be drained. Intervention 8-3 Manual Vibration 382 Vibration is used in conjunction with positioning to drain secretions out of the lungs. The chest wall should be vibrated as the child exhales to encourage coughing. FIGURE 8-12 Proper configuration of the hand for percussion. (From Hillegass EA, Sadowsky HS: Essentials of cardiopulmonary physical therapy, Philadelphia, 1994, WB Saunders.) 383 _—_—— ] Position 1: Upper lobes, apical segments J Position 2: Upper lobes, posterior segments Position 3: Upper lobes, anterior segments Position 12: Lower kibes, superior segrnents lateral Dasal segments FIGURE 8-13 Postural drainage positions. Positions 10 and 11: Lower lobes. al Dasal Coughing as a form of forced expiration is necessary to clear secretions. Laughing or crying can stimulate coughing. Although most children with CF cough on their own, some may need to be encouraged to do so through laughter. If this technique is unsuccessful, the tracheal “tickle” can be used by placing a finger on the trachea above the sternal notch and gently applying pressure. If you attempt this maneuver on yourself, you will feel the urge to clear your throat. To make coughing more functional and productive, the physical therapist assistant can teach the child a forced expiration technique. When in a gravity-aided position, the child is asked to “huff” several times after taking a medium-sized breath. This is followed by several relaxed breaths using the diaphragm. The sequence of huffing and diaphragmatic breathing is repeated as long as secretions are being expectorated. The force of the expirations (huffs) can be magnified by manual resistance over the epigastric area or by having the child actively adduct the arms and compress the chest wall laterally. This technique can be taught to children who are 4 to 5 years of age. Alternative forms of airway clearance are undergoing research in an effort to increase effectiveness and patient usage and reduce time demands on caregivers. These alternatives include positive expiratory pressure (PEP) delivered via a mask (Figure 8-14), autogenic drainage, and use of a Flutter device (Figure 8-15). PEP is easy to use, takes less time than typical chest physical therapy, and is accepted by patients (McIlwaine et al., 1997). Most importantly, it is effective in removing secretions (Gaskin et al., 1998). “The PEP device maintains pressure in the lungs, keeping the airways open and allowing air to get behind the mucous” (Packel and von Berg, 2014). PEP is combined with the forced expiratory technique of huffing to expectorate mucus. This technique was described earlier in the postural drainage section. Autogenic drainage is a sequence of breathing exercises performed at different lung volumes. The reader is referred to Frownfelter and Dean (2012) for a more detailed description of this breathing exercise. Oscillating PEP either using the Flutter or Acapella is a popular airway clearance technique (Morrison and Agnew, 2009). The Flutter device does the same thing as the PEP mask and is also used with autogenic drainage (Packel and von Berg, 2014). The last way that high frequency vibration can be used for airway 384 clearance is through use of an inflatable vest that fits snugly around the chest wall. A pump generates high-frequency oscillations. This technique is called high-frequency chest wall oscillation, or HFCWO, and has been successful in short-term studies (Grece, 2000; Tecklin et al., 2000). > Se, : a FIGURE 8-14 Preparation for PEP therapy. (From Frownfelter D, Dean E: Principles and practice of cardiopulmonary physical therapy, ed 3. Philadelphia, 1996, WB Saunders, p. 356.) 385 FIGURE 8-15 A, Use of Flutter valve. B, Close-up construction of valve. (A, From Frownfelter D, Dean E: Principles and practice of cardiopulmonary physical therapy, ed 3. Philadelphia, 1996, WB Saunders, p. 356.) Strengthening specific muscles can assist respiration. Target the upper body, with emphasis on the shoulder girdle and chest wall muscles such as the pectoralis major and minor, intercostals, serratus, erector spinae, rhomboids, latissimus dorsi, and abdominals. Stretches to maintain optimal length-tension relationships of chest wall musculature are helpful. Respiratory efficiency can be lost when too much of the work of breathing is done by the accessory neck muscles. Part of pulmonary rehabilitation is to teach breathlessness positions, use of the diaphragm, and lateral basal expansion. Breathlessness positions allow the upper body to rest to allow the major 386 muscle of inspiration, the diaphragm, to work most easily. Typical postures are seen in Intervention 8-4. Diaphragmatic breathing can initially be taught by having the child in a supported back-lying position and by using manual cues on the epigastric area (Intervention 8-5, A). The child should be progressed from this position to upright sitting, to standing, and then to walking (Intervention 8-5, B, C). The diaphragm works maximally when the child breathes deeply. Manual contacts on the lateral borders of the ribs can be used to encourage full expansion of the bases of the lungs (Intervention 8-6). Intervention 8-4 Breathlessness Postures A, B. Breathlessness postures for conserving energy, promoting relaxation, and ease of breathing. (From Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, Saunders.) Intervention 8-5 Diaphragmatic Breathing 387 A. Initially, the child can be taught diaphragmatic breathing in a supported back-lying position, with manual cues on the epigastric area. B, C. Then the child should be progressed to upright sitting, standing, and eventually walking while continuing to use the diaphragm for breathing. Intervention 8-6 Lateral Basal Chest Expansion 388 Manual contacts on the lateral borders of the ribs can be used to encourage full expansion of the bases of the lungs. Exercise Most individuals with CF can participate in an exercise program. Exercise tolerance does vary with the severity of the disease. Exercise for cardiovascular and muscular endurance plays a major role in keeping these individuals fit and in slowing the deterioration of lung function. Using exercise early on provides the child with a positive attitude toward exercise. Bike riding, swimming, tumbling, and walking are all excellent means of providing low-impact endurance training. With decreases in endurance resulting from disease progression, other activities, such as table tennis, can be suggested. Exercise programs for those with CF should be based on the results of an exercise test performed by a physical therapist. Children with CF may cough while exercising, causing brief oxygen desaturation. Coughing during exercise is not an indication to stop the exercise (Philpott et al., 2010). Some children with CF also have asthma. The results of the exercise test may indicate the need to monitor oxygen saturation using an ear or finger pulse oximeter while the child exercises. 389 Oxygen saturation should remain at 90% during exercise. Exercise improves not only lung function but also the habitual activity of children with CF (Paranjape et al., 2012). When monitoring exercise tolerance with an individual with CF, use the perceived exertion rating scale and level of dyspnea scale to assess how hard the child is working. These ratings are found in Tables 8-7 and 8-8. If the child is known to desaturate with exercise, monitoring with an oximeter is indicated. If the oxygen saturation level drops below 90%, exercise should be terminated, and the supervising therapist should be notified before additional forms of exercise are attempted. Use of bronchodilating medication 20 minutes prior to exercise may also be beneficial, but again, guidelines for use of any medication should be sought from the supervising therapist in consultation with the child’s physician. Table 8-7 Rating of Perceived Exertion Scale Pe No exertion at all Extremely light ref hel (From Borg RPE scale, © Gunnar Borg, 1970, 1985, 1998, 2006.) 390 Table 8-8 Dyspnea Scale Mild, noticeable to patient but not observer Mild, some difficulty, noticeable to observer Moderate difficulty, but can continue Severe difficulty, patient cannot continue From American College of Sports Medicine: Guidelines for exercise testing and prescription, ed 4. Philadelphia, 1991, Lea & Febiger. Reprinted with permission. As life expectancy has increased, sports and exercise have become an even bigger part of the management of children, adolescents, and adults with CF (Hebestreit et al., 2006; Philpott et al., 2010; Orenstein et al., 2004). Webb and Dodd (1999) report that most students with CF can participate in school sports. These patients are able to continue to pursue cycling, swimming, and even running marathons as adults. Good nutrition and pulmonary function must always be considered. Caloric intake may need to be increased to avoid weight loss since individuals with CF expend more energy to perform exercises than individuals without CF. Fluid replacement during exercise is crucial and needs to include electrolytes not just water. Exercise improves airway clearance, delays decline in pulmonary function, delays onset of dyspnea and prevents decreases in bone density. However, the best reason to exercise is to improve aerobic fitness since it correlates with increased survival (Nixon et al., 1992, 2001). Some sports to be avoided are those such as skiing, bungee jumping, parachute jumping, and scuba diving. These have inherent risks due to altitude, increasing vascular pressure, or air trapping. Sports activities should be curtailed during an infective exacerbation (Packel and von Berg, 2014). Exercising in hot weather is not contraindicated but, again, fluid and electrolytes must be sufficiently replaced. Heavy breathing is a typical response to intense exercise. Deconditioned individuals with CF may demonstrate heavy breathing at lower workloads; this is not pathologic (Orenstein, 2002). In general, individuals with CF should be encouraged to exercise and set their own limits. Quality of life is associated with fitness and physical activity in this population (Hebestreit et al., 2014). 391 Spinal muscular atrophy SMA is a progressive disease of the nervous system inherited as an autosomal recessive trait. Although most of the genetic disorders discussed so far have involved the central nervous system, in SMA, the anterior horn cell undergoes progressive degeneration. Children with SMA exhibit hypotonia of peripheral, rather than of central, origin. Damage to lower motor neurons produces low muscle tone or flaccidity, depending on whether some or all of the anterior horn cells degenerate. Muscle fibers have little or no innervation from the spinal nerve if the anterior horn cell is damaged, and the result is weakness. Children with SMA have normal intelligence. Although many types of SMA are recognized, the following discussion is limited to three types of SMA. All three types of SMA are really variations of the same disorder involving a gene mutation on chromosome 5. The earliest-occurring type of SMA is infantile-onset or acute SMA, also known as Werdnig-Hoffman syndrome. Type II SMA is a chronic or intermediate form. Type III SMA is known as Kugelberg-Welander syndrome and is the mildest form. All types of SMA differ in age at onset and severity of symptoms. As a group of disorders, SMA occurs in 1 of 10,000 live births, is the second most common fatal recessive genetic disorder seen in children, after cystic fibrosis, and the leading cause of death in infants and toddlers (Practice committee, Section on Pediatrics, APTA, 2012). The prevalence of SMA in the population is 1 in 6000 with 1 in 40 people carrying the gene (Beroud et al., 2003). A routine test for prenatal diagnosis has recently been developed. SMA is a result of the loss of the Survival of Motor Neuron (SMN) 1 protein. SMA Type | The earliest-occurring and therefore the most physically devastating form is type 1, acute infantile SMA. The incidence is 1 in 6000 to 10,000 births (Pearn, 1973, 1978) with an onset between birth and 2 months. The child’s limp, “frog-legged” lower extremity posture is evident at birth, along with a weak cry. Most children have a history of decreased fetal movements. Deep tendon reflexes are absent, and the tongue may fasciculate (quiver) because of weakness. Most infants are sociable and interact appropriately because they have normal intelligence. Motor weakness progresses rapidly, and death results from respiratory compromise. Infants with type ISMA usually die within the first 2 years of life (D’ Amico et al., 2011). Life may be extended if the family chooses mechanical ventilation and gastrostomy feedings (Oskoui et al., 2007). In the infant with SMA type I, positioning and family support are the most important interventions. Physical therapy focuses on fostering normal developmental activities and providing the infant with access to the environment. Positioning for feeding, playing with toys, and interacting with caregivers are paramount. Poor head control may make positioning in prone too difficult. The prone position may also be difficult for the child to tolerate because it may inhibit diaphragm movement. These infants rely on the diaphragm to breathe because their intercostal and neck accessory muscles are weak. Creative solutions to adaptive equipment needs can often be the result of brainstorming sessions with the entire healthcare team and the family. Positioning in side lying to play may be very appropriate as seen in Figure 8-16. Equipment should be borrowed rather than purchased because the length of time it will be used is limited. Because of the poor prognosis of children with this type of SMA, listening to the family’s concerns is an integral part of the role of physical therapy clinicians. 392 FIGURE 8-16 An overhead sling supports the forearm of a youngster with type | spinal muscle atrophy and allows her to fish with a magnet puzzle. (Adapted from Bach JR: Management of patients with neuromuscular disease, Philadelphia, 2004, Hanley & Belfus.) SMA Type Il Chronic type II SMA has a later onset, which is reported to occur between 6 and 18 months. This type is characterized by the onset of proximal weakness, similar to the infantile type and has the same incidence in the population. There is a range of severity with some just able to sit unsupported. Most children with this type develop the ability to sit and, in some cases, stand but cannot walk independently. Because of trunk muscle weakness, scoliosis is a pervasive problem and may require surgical intervention. Furthermore, with a reported 12% to 15% fracture rate, weight bearing is also recommended as part of any therapeutic intervention to prevent fractures (Ballestrazzi et al., 1989). Standers and lower-extremity braces can be used to start standing at age 2 in children with type II SMA (Granata et al., 1987). Stuberg (2012) recommended a supine stander for children who lack adequate head control. Life expectancy is variable with some reaching adulthood and others succumbing in childhood. Survival is dependent on the support provided and presence of respiratory compromise. The course of the disease is rapid at first and then stabilizes; therefore, the range of disability can be varied. Intellectually and socially, these children need to be stimulated just as much as their typically-developing peer group. The child’s ability to participate in preschool and school is often hampered by inadequate positioning and lack of ability to access play and academic materials. Assistive technology can be very helpful in providing easier access. Power mobility can be used as early as 18 months (Jones et al., 2003; Jones et al., 2012). Goals can be related to improved access using switches, overhead slings, and adaptive equipment. Because the child will continue to weaken, any changes or decreases in strength should be reported by the physical therapist assistant to the supervising therapist (Ratliffe, 1998). Physical therapy goals can also be directed toward attaining some type of functional mobility. Power mobility may be indicated even at a young age (Jones et al., 2003, 2012) for a child who is not strong enough to propel a manual chair. The physical therapist assistant can play a vital role in promoting the child’s independence by teaching the child to control a power wheelchair both in and out of the classroom. Appropriate trunk support when seated must be ensured to decrease the progression of spinal deformities. Because of the tendency of the child to lean in the wheelchair even with lateral supports, one should consider alternating placement of the joystick from one side to the other (Stuberg, 2000). Although scoliosis cannot always be prevented, every effort should be made to minimize any progression of deformities and therefore to maintain adequate respiratory function. 393 Prognosis in this type of SMA depends on the degree and frequency of pulmonary complications. Postural drainage positioning can be incorporated into the preschool, school, and home routines. Deep breathing should be an integral part of the exercise program. Scoliosis can compound pulmonary problems, with surgical correction indicated only if the child has a good prognosis for survival. Respiratory compromise remains the major cause of death, although cardiac muscle involvement may contribute to mortality. SMA Type III The third type of SMA is Kugelberg-Welander syndrome, which has an onset after 18 months (D’Amico et al., 2011). This is the least involved form with an incidence of 6 in 100,000 live births. Type III can have its onset anywhere from 2 to 15 years. Characteristics include proximal weakness, which is greatest in the hips, knees, and trunk. Developmental progress is slow, with independent sitting achieved by 1 year and independent walking by 3 years. The gait is slow and waddling, often with bilateral Trendelenburg signs. These children have good upper extremity strength, a finding that can differentiate this type of SMA from DMD. The progression of the disease is slow in type III. Physical therapy goals in the toddler and preschool period are directed toward mobility, including walking. Appropriate orthoses for ambulation could include KAFOs, parapodiums, and reciprocating gait orthoses. The reader is referred to Chapter 7 for a discussion of these devices. The physical therapist assistant may be involved in training the child to use and to apply orthotic devices. Orthotic devices assist ambulation, as does the use of a walker. Safety can be a significant issue as the child becomes weaker, so appropriate precautions such as close monitoring must be taken. Goals for the school-aged and adolescent with SMA include support of mobility, access to and completion of academic tasks such as using a computer, positioning to prevent scoliosis and promote pulmonary hygiene, and vocational planning. The physical therapist assistant may not be treating a child with SMA that is in a regular classroom on a weekly basis since therapy may be provided in a consultative service delivery model. However, the assistant may be asked to adjust orthoses, adapt equipment or teach transfers when guided by the supervising physical therapist. Driver training may be indicated as part of the adolescent's prevocational plan. Even though children with type III SMA usually ambulate, half will lose the ability by age 10 and, by midadulthood, become wheelchair-dependent (Glanzman, 2014). Life expectancy is normal for individuals with type III so vocational planning is realistic. The physical therapy needs are determined by the specific type of SMA, the functional limitations present, and the age of the child. While the needs of the child with infantile SMA type I are limited, the child with type II or III may very well survive into adolescence and require ongoing physical therapy intervention. Management includes positioning, functional strengthening and mobility training, standing and walking if possible, pulmonary hygiene, and ventilatory support. 394 Phenylketonuria One genetic cause of intellectual disability that is preventable is the inborn error of metabolism called phenylketonuria (PKU). PKU is caused by an autosomal recessive trait that can be detected at birth by a simple blood test. The infant’s metabolism is missing an enzyme that converts phenylalanine to tyrosine. Too much phenylalanine causes mental and growth retardation along with seizures and behavioral problems. Once the error is identified, infants are placed on a phenylalanine-restricted diet. If dietary management is begun, the child will not develop intellectual disability or any of the other neurologic signs of the disorder. If the error is undetected, the infant’s mental and physical development will be delayed, and physical therapy intervention is warranted. 395 Duchenne muscular dystrophy DMD is transmitted as an X-linked recessive trait, which means that it is manifested only in boys. Females can be carriers of the gene, but they do not express it, although some sources state that a small percentage of female carriers do exhibit muscle weakness. DMD affects 20 to 30 in 100,000 male births (Glanzman, 2014). Two-thirds of cases of DMD are inherited, whereas one-third of cases result from a spontaneous mutation. Boys with DMD develop motor skills normally. However, between the ages of 3 and 5 years, they may begin to fall more often or experience difficulty in going up and down stairs, or they may use a characteristic Gower maneuver to move into a standing position from the floor (Figure 8-17). The Gower maneuver is characterized by the child using his arms to push on the thighs to achieve a standing position. This maneuver indicates presenting muscle weakness. The diagnosis is usually made during this time. Elevated levels of creatine kinase are often found in the blood as a result of the breakdown of muscle. This enzyme is a measure of the amount of muscle fiber loss. The definitive diagnosis is usually made by muscle biopsy. E = FIGURE 8-17 A-—E, The Gower maneuver. The child needs to push on his legs to achieve an upright position because of pelvic girdle and lower extremity weakness. Pathophysiology and Natural History Children with DMD lack the gene that produces the muscle protein dystrophin. Absence of this protein weakens the cell membrane and eventually leads to the destruction of muscle fibers. The lack of another protein, nebulin, prevents proper alignment of the contractile filaments during muscle contraction. As muscle fibers break down, they are replaced by fat and connective tissue. Fiber necrosis, degeneration, and regeneration are characteristically seen on muscle biopsy. The replacement of muscle fiber with fat and connective tissue results in a pseudohypertrophy, or false hypertrophy of muscles that is most readily apparent in the calves (Figure 8-18). With progressive loss of muscle, weakness ensues, followed by loss of active and passive range of motion. Limitations in range and ADLs begin at around 5 years of age (Hallum and Allen, 2013); an inability to climb stairs is seen between 7 and 10 years of age. The ability to ambulate is usually lost between the ages of 9 and 13 years (Stuberg, 2012; Glanzman, 2014). Intellectual function is less than normal in about one-third of these children. 396 FIGURE 8-18 Pseudohypertrophy of the calves. (From Stuberg W: Muscular dystrophy and spinal muscular atrophy. In Campbell SK, Palisano RJ, Orlin MN, editors: Physical therapy for children, ed 4. Philadelphia, 2012, WB Saunders.) Smooth muscle is also affected by the lack of dystrophin; 84% of boys with DMD exhibit cardiomyopathy, or weakness of the heart muscle. Cardiac failure results either from this weakness or from respiratory insufficiency. As the muscles of respiration become involved, pulmonary function is compromised, with death from respiratory or cardiac failure usually occurring before age 25. Life can be prolonged by use of mechanical ventilation, but this decision is based on the individual’s and the family’s wishes. Bach et al. (1991) reported that satisfaction with life was positive in a majority of individuals with DMD who used long-term ventilatory support. Survival is being prolonged by use of noninvasive ventilator support (Bach and Martinez, 2011). Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with DMD typically identifies the following impairments, activity limitations, or participation restrictions to be addressed by physical therapy intervention: 1. Impaired strength 2. Impaired active and passive range of motion 3. Impaired gait 4. Limitations in functional abilities 5. Impaired respiratory function 6. Spinal deformities—apparent or potential 7. Potential need for adaptive equipment, orthoses, and wheelchair 8. Emotional trauma of the individual and family The family’s understanding of the disease and its progressive nature must be taken into consideration when the physical therapist plans an intervention program. The ultimate goal of the program is to provide education and support for the family while managing the child’s impairments. Each problem or impairment is discussed, along with possible interventions. The physical therapy goals are to prevent deformity, to prolong function by maintaining capacity for ADLs and play, to facilitate movement, to assist in supporting the family and to control discomfort. Management is a total approach requiring blending of medical, educational, and family goals. Treatment has both preventive and supportive aspects. Weakness Proximal muscle weakness is one of the major clinical features of DMD and is most clearly apparent in the shoulder and pelvic girdles (see Figure 8-18). The loss of strength eventually progresses distally to encompass all the musculature. Whether exercise can be used to counteract the 397 pathologic weakness seen in muscular dystrophies is unclear. Strengthening exercises have been found to be beneficial by some researchers and not by others. More important, however, although exercise has not been found to hasten the progression of the disease, the role of exercise remains controversial (Ansved, 2003). Some therapists do not encourage active resistive exercises (Florence, 1999) and choose instead to focus on preserving functional levels of strength by having the child do all ADLs. Other therapists recommend that submaximal forms of exercise are beneficial but advocate these activities only if they are not burdensome to the family. Movement in some form must be an integral part of a physical therapy plan of care for the child with DMD. Theoretically, exercise should be able to assist intact muscle fibers to increase in strength to make up for lost fibers. Key muscles to target, if exercise is going to be used to treat weakness, include the abdominals, hip extensors and abductors, and knee extensors. In addition, the triceps and scapular stabilizers should be targeted in the upper extremities. Recreational activities, such as bike riding and swimming, are excellent choices and provide aerobic conditioning. Even though the exact role of exercise in these children is unclear, clinicians generally agree that overexertion, exercising at maximal levels, and immobility are detrimental to the child with DMD. High resistance and eccentric training should also be avoided (Ansved, 2003). Exercise capacity is probably best determined by the stage and rate of disease progression (Ansved, 2003; McDonald, 2002). Exercise may be more beneficial early as opposed to later in the disease process. Mobility status is related to knee extension strength and gait velocity in children with DMD. Boys with less than antigravity (3/5) quadriceps strength lost the ability to ambulate (McDonald et al., 1995, McDonald, 2002). Walking should be done for a minimum of 2 to 3 hours a day, according to many sources (Siegel, 1978; Ziter and Allsop, 1976). The speed of walking has been used to predict the length of time that will pass before a child with DMD will require the use of a wheelchair. A high percentage of boys who walked 10 meters in less than 6 seconds were more than 2 years away from using a wheelchair whereas all of the boys who took 12 seconds or more to walk 10 meters required a wheelchair within a year (McDonald et al., 1995). The longer a child can remain ambulatory, the better. Range of Motion The potential for muscle contractures is high, and every effort should be made to maintain range of motion at all joints. Specifically, attention should be paid to the gastrocnemius-soleus complex and the tensor fasciae latae. Tightness in these muscle groups results in gait deviations and a widened base of support. Stretching of the illiopsoas, iliotibial band, and tensor fasciae latae is demonstrated in Intervention 8-7. Although contractures cannot be prevented, their progression can be slowed (Stuberg, 2012). A prone positioning program is crucial for managing the detrimental effect of gravity. Time in prone counteracts the potential formation of hip and knee flexion contractures, which develop from too much sitting. The physical therapist assistant may teach a home program to the child’s parents and may monitor position changes within the classroom. Prolonged sitting can all too quickly lead to lower extremity flexion deformities that can hinder ambulation. Intervention 8-7 Stretching of the Iliopsoas, Iliotibial Band, and Tensor Fasciae Latae 398 —— Prone stretching of the hip flexors, iliotibial band, and tensor fasciae latae. The hip first is positioned in abduction and then is moved into maximal hip extension and then hip adduction. The knee can be extended to provide greater stretch for the iliotibial and tensor muscles. (From Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 3. Philadelphia, 2006, WB Saunders.) Alternatives to a sitting position should be scheduled several times a day. When the child is in preschool, the prone position can be easily incorporated into nap or rest time. A prone stander can be used during class time when the child is standing and working on the blackboard can be incorporated into the child’s daily classroom routine. Prone positioning over a wedge can also be used. At home, sleeping in the prone position should be encouraged as long as it does not compromise the child’s respiratory function. Skin Care Skin integrity must always be monitored. Pressure relief and use of a cushion must be part of the daily routine once the child is using a wheelchair for any length of time. If the child is using a splint or orthosis, wearing times must be controlled and the skin must be inspected on a routine basis. Gait Children with DMD ambulate with a characteristic waddle because the pelvic girdle muscles weaken. Hip extensor weakness can lead to compensatory lordosis, which keeps the center of mass posterior to the hip joint, as seen in Figure 8-18. Excessive lateral trunk lean during gait may be seen in response to bilateral Trendelenburg signs indicative of hip abductor weakness. Knee hyperextension may be substituted for quadriceps muscle strength, and it can further increase the lumbar lordosis. Failure to keep the body weight in front of the knee joint or behind the hip joint results in a loss of the ability to stand. Plantar flexion contractures can compromise toe clearance, can lead to toe walking and may make balance even more precarious. Functional rating scales can be helpful in documenting the progression of disability. Several are available. Box 8-3 depicts simple scales for the upper and lower extremities. The Pediatric Evaluation of Disability Inventory (Haley et al., 1992) or the School Function Assessment (Coster et al., 1998) can be used to obtain more specific information about mobility and self-care. The supervising physical therapist may use this information for treatment planning, and the physical therapist assistant may be responsible for collecting data as part of the ongoing assessment. The physical therapist assistant also provides feedback to the primary therapist for appropriate 399 modifications to the child’s plan of care. Box 8-3 Vignos Classification Scales for Children with Duchenne Muscular Dystrophy Upper extremity functional grades 1. Can abduct arms in a full circle until they touch above the head. 2. Raises arms above the head only by shortening the lever arm or using accessory muscles. 3. Cannot raise hands above the head but can raise a 180-mL cup of water to mouth using both hands, if necessary. 4. Can raise hands to mouth but cannot raise a 180-mL cup of water to mouth. 5. Cannot raise hands to mouth, but can use hands to hold a pen or pick up a coin. 6. Cannot raise hands to mouth and has no functional use of hands. Lower extremity functional grades 1. Walks and climbs stairs without assistance. 2. Walks and climbs stairs with aid of railing. 3. Walks and climbs stairs slowly with aid of railing (more than 12 seconds for four steps). 4, Walks unassisted and rises from a chair but cannot climb stairs. 5. Walks unassisted but cannot rise from a chair or climb stairs. 6. Walks only with assistance or walks independently in long-leg braces. 7. Walks in long-leg braces but requires assistance for balance. 8. Stands in long-leg braces but is unable to walk even with assistance. 9. Must use a wheelchair. 10. Bedridden. (Data from Vignos PJ, Spencer GE, Archibald KC: Management of progressive muscular dystrophy in childhood. JAMA 184:89-96, 1963.) © 1963 American Medical Association. Medical Management No known treatment can stop the progression of DMD. Steroid therapy has been used to slow the progression of both the Duchenne and Becker forms of muscular dystrophy. Becker is a milder form of muscular dystrophy with a later onset, slower progression, and longer life expectancy. Prednisolone has been shown to improve the strength of muscles and to decrease the deterioration of muscle function (Dubowitz et al., 2002; Backman and Hendriksson, 1995; Hardiman et al., 1993). Two additional promising approaches for the treatment of DMD are myoblast transplantation and gene therapy. Both approaches have met with many difficulties, mostly involving immune reactions (Moisset et al., 1998). No reports have been published to date of improved strength in individuals with DMD using the myoblast transfer (Smythe et al., 2000). A report of a pilot study of myoblast transfer in the treatment of subjects with Becker muscular dystrophy stated that myoblast implantation has had limited success (Neumeyer et al., 1998). Surgical and Orthotic Management As the quality of the child’s functional gait declines, medical management of the child with DMD is broadened. Surgical and orthotic solutions to the loss of range or ambulation abilities are by no means universal. Many variables must be factored into a final decision whether to perform surgery or to use an orthosis. Some clinicians think that it is worse to try to postpone the inevitable, whereas others support the child’s and family’s right to choose to fight for independence as long as resources are available. Surgical procedures that have been used to combat the progressive effects of DMD are Achilles tendon lengthening procedures, tensor fasciae latae fasciotomy, tendon transfers, tenotomies, and, most recently, myoblast transfers. These procedures must be followed by vigorous physical therapy to achieve the best gains. Ankle-foot orthoses (AFOs) are often prescribed following heel cord lengthening. Use of KAFOs has also been tried; one source reported that early surgery followed by rehabilitation negated the need for KAFOs (Bach and McKeon, 1991). Orthoses can be prescribed to maintain heel cord length while the patient is ambulating. A night 400 splint may be fabricated to incorporate the knees, because knee flexion contractures can also be a problem. In the majority of cases, however, as the quadriceps muscles lose strength, the child develops severe lordosis as compensation. This change keeps the body weight in front of the knee joints and allows gravity to control knee extension. The child’s gait becomes lurching, and if the ankles do not have sufficient range to keep the feet plantigrade, dynamic balance becomes impaired. Surgical release of the Achilles tendon followed by use of polypropylene AFOs may prolong the length of time a child can remain ambulatory. However, once ambulation skills are lost, the child will require a wheelchair. Adaptive Equipment The physical therapist assistant may participate in the team’s decision regarding the type of wheelchair to be prescribed for the child with DMD. The child may not be able to propel a manual wheelchair because of upper extremity weakness, so consideration of a lighter sports wheelchair or a power wheelchair may be appropriate. Energy cost and insurance or reimbursement constraints must be considered. The child may be able to propel a lighter wheelchair during certain times of the day or use it to work on endurance, but in the long term, he may be more mobile in a power wheelchair, as seen in Figure 8-19. If reimbursement limitations are severe and only one wheelchair is possible, power mobility may be a more functional choice. Other adaptive equipment such as mobile arm supports for feeding or voice-activated computer and environmental controls may also be considered to augment the child’s level of function. x tye fe . ” FIGURE 8-19 A boy with Duchenne muscular dystrophy using a power chair. (From Stuberg W: Muscular dystrophy and spinal muscular atrophy. In Campbell SK, editor: Physical therapy for children, Philadelphia, 1994, WB Saunders.) Respiratory Function 401 Respiratory function must be targeted for aggressive management. Breathing exercises and range of motion should be part of a home exercise program and incorporated into any therapy session. Flexion of the arms or legs can be paired with inspiration, while extension can be linked to expiration. Diaphragmatic breathing is more efficient than use of accessory muscles and therefore should be emphasized along with lateral basal chest expansion. Chest wall tightness can be discouraged by active trunk rotation, passive counterrotation, and manual stretching (Intervention 8-8). On occasion, postural drainage with percussion may be needed to clear the lungs of retained secretions. Children often miss school because of respiratory involvement. Parents should be taught appropriate airway clearance techniques, as described in the section on CF. Intervention 8-8 Chest Wall Stretching Chest wall mobility can be promoted by active trunk rotation, passive counterrotation, and manual stretching. Stretching counteracts the tendency to tightness that occurs as the child becomes more sedentary. Activities that promote cardiovascular endurance are as important as stretching and functional activities. Always incorporate deep breathing and chest mobility into the child’s upper- or lower- extremity exercises. Wind sprints can be done when the child is in a wheelchair. These are fast, 402 energetic pushes of the wheelchair for set distances. The child can be timed and work to improve or maintain his best time. An exercise program for a child with DMD needs to include an aerobic component, because the respiratory system ultimately causes the child to die from the effects of the disease. Swimming is an excellent aerobic exercise for children with DMD. At least biannual reexaminations are used to document the inevitable progression of the disease. Documenting progression of the disease is critical for timing of interventions as the child declines from one functional level to another. Whether to have surgical treatment or to use orthotic devices remains controversial. Accurate data must be kept to allow one to intervene aggressively to provide adequate mobility and respiratory support for the individual and his family. Table 8-9 outlines some of the goals, strategies, and interventions that could be implemented over the life span of a patient with DMD. Table 8-9 Management of Duchenne Muscular Dystrophy Time Period Goals Strategies Medical/Surgical Home Program Schoolage | Prevent deformity Stretching Splints/AFOs ROM program Preserve independent Strengthening Monitor spinal alignment Night splints mobility Breathing exercises Manual wheelchair as walking becomes Cycling or swimming Preserve vital capacity difficult Prone positioning Motorized scooter Blow bottles Adolescence | Manage contractures Stretching AFOs/KAFOs before ambulation ceases ROM program Maintain ambulation Guard during stair climbing or general Surgery to prolong ambulatory ability Night splints Assist with transfers and walking Proper wheelchair fit and support Prone positioning ADLs Positioning ADLs, ADL modifications Surgery for scoliosis management Blow bottles Strengthening shoulder depressors and Assistance with transfers and triceps ADLs Adulthood | Monitor respiratory function] Breathing exercises, postural drainage, assisted | Mechanical ventilation Hospital bed Manage mobility and coughing Monitoring oxygen saturation Ball-bearing feeder transfers Assistive technology Power mobility Hoyer lift From Stuberg WA: Muscular dystrophy and spinal muscular atrophy. In Campbell SK, Vander Linden DW, Palisano RJ, editors: Physical therapy for children, ed 2. Philadelphia, 2000, WB Saunders, pp. 339-369. ADLs, Activities of daily living; AFOs, ankle-foot orthoses; KAFOs, knee-ankle-foot orthoses; ROM, range of motion. 403 Becker muscular dystrophy Children with Becker muscular dystrophy (BMD) have an onset of symptoms between 5 and 10 years of age. This X-linked dystrophy occurs in 5 per 100,000 males, so it is rarer than DMD. Dystrophin continues to be present but in lesser amounts than normal. Laboratory findings are not as striking as in DMD; one sees less elevation of creatine kinase levels and less destruction of muscle fibers on biopsy. Another significant difference from DMD is the lower incidence of intellectual disability with the Becker type of muscular dystrophy. Physical therapy management follows the same general outline as for the child with DMD; however, the progression of the disorder is much slower. Greater potential and expectation exist for the individual to continue to ambulate until his late teens. Prevention of excessive weight gain must be vigorously pursued to avoid use of a wheelchair too early, because life expectancy reaches into the 40s. Providing sufficient exercise for weight control may be an even greater challenge in this population because the use of power mobility is more prevalent. The transition from adolescence to adulthood is more of an issue in BMD because of the longer life expectancy. Individuals with BMD live into their 40s with death secondary to pulmonary or cardiac failure (Glanzman, 2014). Vocational rehabilitation can be invaluable in assisting with vocational training or college attendance, depending on the patient’s degree of disability and disease progression. Regardless of vocational or avocational plans, the adult with BMD needs assistance with living arrangements. Evaluation of needs should begin before the completion of high school. 404 Fragile X syndrome Fragile X syndrome (FXS) is the leading inherited cause of intellectual disability. It occurs in 1 per 4000 males and 1 per 8000 females (Jorde et al., 2010). Detection of a fragile site on the X chromosome at a cellular level makes it possible to confirm this entity as the cause of a child’s intellectual disability. The fragile X gene (FMR) codes for a fragile X mental retardation protein (FMRP). FXS is characterized by intellectual disability, unusual facies, poor coordination, a generalized decrease in muscle tone, and enlarged testes in male patients after puberty. These children may have a long, narrow face with a prominent forehead, jaw, and ears (Figure 8-20). The clinical manifestations of the disorder vary depending on the completeness of the mutation. The FMR gene determines the number of repeats of a series of three amino acids. When the FMR gene is inherited the number of repeats can go from normal (6 to 40 repeats) to a permutation (50 to 200 repeats) to a full blown mutation of greater than 200 repeats. In the full blown mutation almost no FMRP is produced. The less FMRP produced, the more severe the intellectual disability. Over successive generations there is an increased risk of the number of repeats expanding so that the disease appears to worsen in successive generations. Genetic counseling for the family of a child with fragile X is extremely important for them to understand the reproductive risks. FIGURE 8-20 A 6-year-old boy with fragile X syndrome. (From Hagerman R: Fragile X syndrome. In Allen PJ, Vessey JA, and Schapiro NA, editors: Primary care of the child with a chronic condition, ed 5, St. Louis, 2010, Mosby, pp 514-526.) Connective tissue involvement can include joint hypermobility, flatfeet, inguinal hernia, pectus excavatum, and mitral valve prolapse (Goldstein and Reynolds, 2011). Symptoms in girls are not as severe as in boys. Girls do not usually present with dysmorphic features (structural differences often seen in the face) or connective tissue abnormalities. Females with fragile X are more likely to 405 have normal intelligence but may have a learning disability. Children of female carriers, however, have a greater risk of the disorder than those of male carriers which again reinforces the importance of genetic counseling for this condition. Behavioral characteristics of both males and females with FXS include a short attention span, impulsivity, tactile defensiveness, hyperactivity and perseveration in speech and motor actions (Goldstein and Reynolds, 2011). FXS is the most common single gene defect associated with autism spectrum disorder. Thirty percent of children with FXS will be diagnosed with autism (Harris et al., 2008). Most children with FXS demonstrate autistic-like behavior. There appears to be a shared molecular overlap between autism, FXS, and fragile X permutation (Gurkan and Hagerman, 2012). There is greater impairment of cognition, language, and adaptive behavior in those with FXS and autism compared with those with FXS without autism (Hagerman et al., 2008). Intelligence Intellectual disability in children with FXS can range from severe to borderline normal. The average IQ falls between 20 and 60, with a mean of 30 to 45. Additional cognitive deficits may include attention deficit-hyperactivity disorder, learning disability, and autistic-like mannerisms. In fact, girls may be incorrectly diagnosed as having infantile autism or may exhibit only a mild cognitive deficit, such as a learning disability (Batshaw et al., 2013). Motor Development Gross and fine motor development is delayed in the child with FXS. The average age of walking is 2 years (Levitas et al., 1983), with 75% of boys exhibiting a flatfooted and waddling gait (Davids et al., 1990). The child’s motor skills are at the same developmental age level as the child’s mental ability. Even before the diagnosis of FXS is made, the physical therapist may be the first to recognize that the child has more problems than just delayed development. Maintaining balance in any developmental posture is a challenge for these children because of their low tone, joint hypermobility, and gravitational insecurity. Individuals who are mildly affected may present with language delays and behavioral problems, especially hyperactivity (Schopmeyer and Lowe, 1992). Tactile Defensiveness Regardless of the severity of the disorder, 90% of these children avoid eye contact and 80% display tactile defensiveness. The characteristics of tactile defensiveness are listed in Table 8-10. Touch can be perceived as aversive, and light touch may elicit a withdrawal response rather than an orienting response. Treatment involves the use of different-textured surfaces on equipment that the child can touch during play. Vestibular stimulation, firm pressure, and increasing proprioceptive input through weight bearing and movement are helpful (Schopmeyer and Lowe, 1992). Table 8-10 Tactile Defensiveness Major Symptom Child’s Behavior Avoidance of touch Avoids scratchy or rough clothing, prefers soft material, long sleeves or pants Prefers to stand alone to avoid contact with other children Avoids play activities that involve body contact Aversive responses to non-noxious touch Turns away or struggles when picked up, hugged, or cuddled Resists certain ADLs, such as baths, cutting fingernails, haircuts, and face washing} Has an aversion to dental care Has an aversion to art materials such as finger-paints, paste, or sand Atypical affective responses to nonnoxious tactile stimuli] Responds aggressively to light touch to arms, face, or legs Increased stress in response to being physically close to people Objects to or withdraws from touch contact. From Royeen CB: Domain specifications of the construct of tactile defensiveness. Am J Occup Ther 39:596—599, 1985. © 1985 American Occupational Therapy Association. Reprinted with permission. ADLs Activities of daily living. Sensory Integration In addition to tactile defensiveness, other sensory integration problems are evident in the decreased ability of these children to tolerate being exposed to multiple sensory inputs at one time. These 406 children become easily overwhelmed because they cannot filter out environmental stimuli. When gaze aversion occurs, it is thought to be related to the child’s high degree of anxiety, rather than to autism or social dysfunction. Because low tolerance for frustration often leads to tantrums in these children; always be alert to the child’s losing control and institute appropriate behavior modification responses that have been decided on by the team. Learning Visual learning is a strength of children with FXS, so using a visual cue with a verbal request is a good intervention strategy. Teaching any motor skill or task should be done within the context in which it is expected to be performed, such as teaching hand washing at a sink in the bathroom. Examples of inappropriate contexts are teaching tooth brushing in the cafeteria or teaching ball kicking in the classroom. The physical, social, and emotional surroundings in which learning takes place are significant for the activity to make sense to the child. Teaching a task in its entirety, rather than breaking it down into its component parts, may help to lessen the child’s difficulty with sequential learning and tendency to perseverate, defined as repeating an action over and over. 407 Rett syndrome Rett syndrome is a neurodevelopmental disorder that almost exclusively affects females. It occurs in approximately 1 in 12,000 females. The presentation in females suggests an X-linked dominant means of inheritance but this has been disproven (Goldstein and Reynolds, 2011). Males with Rett syndrome have been described in the literature (Clayton-Smith et al., 2000; Moog et al., 2003). Rett syndrome is characterized by intellectual disability, ataxia, and growth retardation. It is a major cause of intellectual disability in females (Shahbazian and Zoghbi, 2001). Despite the intellectual disability, Rett syndrome is not a neurodegenerative disorder (Zoghbi, 2003). It represents a failure of postnatal development due to a mutation in the MECP2 gene, which is responsible for development of synaptic connections in the brain. Intellectual disability is in the severe, profound range. There is a prestage in which the child’s development appears normal. This prestage lasts 6 months and is followed by four stages of decline. Stage 1 has been characterized as early onset stagnation where there is loss of language and motor skills between 6 and 18 months. Stage 2 is rapid destruction of previously acquired hand function. It is during this stage that children develop stereotypical hand movements, such as flapping, wringing, and slapping, as well as mouthing. Decline in function during childhood includes a decreased ability to communicate, seizure activity, and later, scoliosis. There is a plateau during stage 3, which lasts until around the age of 10 years, followed by late motor deterioration in stage 4. Expression of the syndrome varies in severity. Girls with Rett syndrome live into adulthood (Goldstein and Reynolds, 2011). 408 Autism Spectrum Disorder Infants and children diagnosed with autism have deficits in social, communication, and motor and behavioral development. Autism spectrum disorders (ASDs) include autistic disorder, pervasive developmental delay not otherwise specified (PDD-NOS), and Asperger syndrome (CDC, 2014). Autism must be differentiated from developmental delay in order to provide an accurate diagnosis and implementation of the appropriate interventions (Mitchell et al., 2011). The diagnosis of autism at the age of 2 years has been found to be stable, reliable, and valid (Kleinman et al., 2008), yet the diagnoses of Asperger and PDD-NOS are usually not made until later, around age 6 years and 4 years, respectively (Batshaw et al., 2013). Early detection allows for early intervention and the potential for positive developmental change and a substantially better prognosis (Kleinman et al., 2008). ASD is more common in boys than girls and occurs in all ethnic, racial, and socioeconomic groups. It is estimated that 1 in 68 children have ASD. According to the Diagnostic and Statistical Manual of Mental Disorders (DSM-5), in order to be diagnosed with ASD, a child has to demonstrate impaired social interaction, communication, and restricted, repetitive behaviors. Motor impairment is not part of the diagnostic criteria despite the fact that difficulty with motor control has been recognized in early descriptions of autism (Kanner, 1943). Many recent studies have highlighted the impaired motor function demonstrated by young children with ASD (Bhat et al., 2012; Lloyd et al., 2011; Provost et al., 2007). However, some researchers have not reported delays in motor development in children with ASD compared with typically developing children (Ozonoff et al., 2008) and others only found delays in the motor age equivalents not on scaled scores (Lane et al., 2012). Motor imitation is delayed in children with ASD (Carey et al., 2014). Early motor delays in siblings of children with autism were found to predict risk for later communication delays (Bhat et al., 2012). Slow reach-to-grasp movements were found in lower functioning children with autism (Mari et al., 2003). Older children with ASD have been found to demonstrate difficulty with motor planning (praxis) (MacNeil and Mostofsky, 2012). There is evidence that some degree of motor delay is present in most children with autism. There is currently not enough evidence to support whether the presence of an early delay in motor development can be predictive of autism. Physical therapists need to be involved in the evaluation of motor skills in this group. Genetic disorders such as DS and fragile X have been found to be associated with ASD. The cause of ASD is as yet unknown. A diagnosis of autism along with a genetic disorder can compound developmental problems, although services may be more readily available with a diagnosis of autism because of the increased prevalence. Children with autism do not exhibit the ability to pretend play but can be taught to engage in pretend play by peer and adult modeling (Barton and Pavilanis, 2012). Best practice includes use of social scripts to model social skills for children with autism (Reichow and Volkmar, 2010). The most commonly targeted skills are communication and social interaction. However, based on the findings regarding motor development in children with autism, physical therapy intervention should include posture and balance training as well as motor imitation and planning in conjunction with sensory integration provided by occupational therapy. Parents should be taught to foster social play in addition to social interaction and communication. Play is age-appropriate and can take advantage of movement and language skills as well as engaging the imagination. 409 Genetic disorders and intellectual disability One to three percent of the total population of the United States has psychomotor or intellectual disability. Intellectual disability is “a substantial limitation in present function characterized by subaverage intelligence and related limitations in two or more of the following areas: communication, self-care, home living, social skills, community use, health and safety, academics, leisure, and work,” as defined by the American Association on Intellectual and Developmental Disabilities (AAIDD, 2010). A person must have an IQ of 70 to 75 or less to be diagnosed as having intellectual disability. The foregoing definition emphasizes the effect that a decreased ability to learn has on all aspects of a person’s life. Educational definitions of intellectual disability may vary from state to state because of differences in eligibility criteria for developmental services. An IQ score tells little about the strengths of the individual and may artificially lower the expectations of the child’s capabilities. Despite the inclusion of the deficits in adaptive abilities seen in individuals with intellectual disability, four classic levels of retardation are reported in the literature. These levels, along with the relative proportion of each type within the population with intellectual disability, are listed in Table 8-11. Table 8-11 Classification of Intellectual Disability Level of Intellectual Disability IQ __ Percentage of Disabled Population| i 55-70] 70% Moderate 40-55] 20% Severe 25-40] 5% Profound <25 Based on data from Grossman HJ: Classification in mental retardation. Washington, DC, 1983, American Association on Mental Retardation; Jones ED, Payne JS: Definition and prevalence. In Patton JR, Payne JS, Beirne-Smith M, editors: Mental retardation, ed 2. Columbus, OH, 1986, Charles E. Merrill, pp. 33-75. The two most common genetic disorders that produce intellectual disability are DS and FXS. DS results from a trisomy of one of the chromosomes, chromosome 21, whereas FXS is caused by a defect on the X chromosome. This major X-linked disorder explains why the rate of intellectual disability is higher in males than females. The defect on the X chromosome is expressed in males when no normal X chromosome is present. Most genetic disorders involving the nervous system produce intellectual disability, and children present with low muscle tone as a primary clinical feature. Child’s Impairments and Interventions The physical therapist’s examination and evaluation of the child with low muscle tone secondary to a genetic problem, regardless of whether the child has associated intellectual disability, typically identifies similar impairments or potential problems to be addressed by physical therapy intervention: 1. Delayed psychomotor development (only motor delay in SMA) 2. Hypotonia or weakness 3. Delayed development of postural reactions 4. Hyperextensible joints 5. Contractures and skeletal deformities 6. Impaired respiratory function Intervention to address these impairments is discussed here both generally and within the context of a case study. Intellectual disability is the preferred term rather than mental retardation. Psychomotor Development Promotion of psychomotor development in children with genetic disorders resulting in delayed motor and cognitive development is a primary focus of physical therapy intervention. Children with intellectual disability are capable of learning motor skills and life skills. However, children with intellectual disability learn fewer things, and those things take longer to learn. Principles of motor learning can and should be used with this population. Practice and repetition are even more critical in the child with intellectual disability than in a child with a motor delay without intellectual 410 disability. The clinician must always ensure that the skill or task being taught is part of the child’s everyday function. Breaking the task into its component parts improves the potential for learning the original task and for that task to carry over into other skills. The ability to generalize a skill to another task is decreased in children with intellectual disability. Each task is new; no matter how similar we may think it is, the process of teaching must start again. Skills that are not practiced on a regular basis will not be maintained, which is another reason for tasks to be made relevant and applicable to everyday life. Hypotonia and Delayed Postural Reactions Early in therapy, functional goals are focused on the development of postural control. The child must learn to move through the environment safely and to perform tasks such as manipulating objects within the environment. The intellectual disability, hypotonia, joint hypermobility, and delayed development characteristically seen in children with genetic disorders such as DS interact to produce poor postural control. The child with low postural tone cannot easily support a posture against gravity, move or shift weight within a posture, or maintain a posture to use limbs efficiently. Making the transition from one posture to another is accomplished only with a great deal of effort and unusual movement patterns. By improving postural tone in therapy, the therapist provides the child with a foundation for movement. Children with DS benefit from being taught or trained to achieve motor milestones and to improve postural responses. Table 8-2 lists the ages at attainment of developmental milestones in children with DS compared with the typical age at attainment of the same skills. Ann, as shown in Figure 8-21, is a 17-month-old child with DS. She provides a model for treatment of children with genetic disorders in which hypotonia and delayed motor development are the overriding impairments. Ann is seen weekly for physical therapy. She creeps and pulls to stand but is not yet walking independently. While Ann undresses, the therapist encourages Ann’s ability to balance while her weight is shifted to one side (see Figure 8-21). In addition, typical help with sock removal is greatly appreciated (Figure 8-22). 411 FIGURE 8-21 Trunk weight shift while undressing. 412 FIGURE 8-22 Child with Down syndrome removing her sock. Stability Preparation for movement in children consists of weight bearing in appropriate joint alignment. Splints of various materials may be used to maintain the required alignment without any mechanical joint locking if the child is unable to do so on her own. Gentle intermittent approximation by manual means helps prepare a body part to accept weight. Approximation is shown in Intervention 8-9. Approximation through the extremities during weight bearing can reinforce the maintenance of a posture and can provide a stable base on which to superimpose movement, in the form of a weight shift or a movement transition. Intervention 8-10 shows the therapist guiding Ann’s movement from sitting to upper extremity weight bearing and Ann reaching with a return to sitting. Intervention 8-9 Approximation 413 A. Approximation in a modified plantigrade position. B. Approximation of the foot to the floor in a squatting position. C. Approximation from the knees to the feet while the child sits on a bolster. D. Approximation at the hips in standing. E. Approximation through the shoulder. Intervention 8-10 Movement Transition 414 Cc A-D. The child practices active trunk rotation within a play task. Guided movement from sitting to upper extremity weight bearing and reaching with a return to sitting. Mobility The child with intellectual disability needs to be mobile to explore the environment. Manual manipulation of objects and the ability to explore the surrounding environment are assumed to contribute positively to the development of cognition, communication, and emotion. Even if motor and cognition develop separately, they facilitate one another, so by fostering movement, understanding of an action is made possible. Ann is encouraged to come to stand at a bench to play both by pulling up and by coming to stand from sitting on the therapist’s knee (Intervention 8-11). The use of postural supports such as a toy shopping cart can entice the child into walking (Intervention 8-12). Mobility options facilitate the child’s mastery of the environment. Intervention 8-11 Coming to Stand 415 The child is encouraged to stand as follows: A, B. By pulling up from the floor. C. By coming to standing from sitting on the therapist’s knee. Intervention 8-12 Walking 416 A, B. The use of postural supports, such as a toy shopping cart, can encourage walking. Alternative means of mobility, such as a power wheelchair, a cart, an adapted tricycle, or a prone scooter, can be used to give the child with moderate to severe intellectual disability and impaired motor abilities a way to move independently. McEwen (2000) stated that children with intellectual disability who have vision and cognition at the level of an 18-month-old are able to learn how to use a powered means of mobility. Orientation in an upright position is important for social interaction with peers and adults. McEwen (1992) also found that teachers interacted more with children who were positioned nearer the normal interaction level of adults, that is, in a wheelchair, than with children who were positioned on the floor. Postural Control The child with low tone should be handled firmly, with vestibular input used when appropriate to encourage development of head and trunk control. Joint stability must always be taken into consideration when the clinician uses vestibular sensation or movement to improve a child’s balance. The therapist and family should use carrying positions that incorporate trunk support and allow the child’s head either to lift against gravity or to be maintained in a midline position. An infant can be carried over the adult’s arm, at the adult’s shoulder, or with the child’s back to the adult’s chest (Intervention 8-13). Gathered-together positions in which the limbs are held close to the body and most joints are flexed promote security and reinforce midline orientation and symmetry. Prone on elbows, prone on extended arms, propping on arms in sitting, and four-point are all good weight-bearing positions. When the child cannot fully support the body’s weight, the use of an appropriate device, such as a wedge, a bolster, or a half-roll, can still allow the physical therapist assistant to position the child for weight bearing. Upright positioning can enhance the child’s arousal and therefore can provide a more optimal condition for learning than being recumbent (Guess et al., 1988). Intervention 8-13 Carrying Positions 417 A. Carrying the child with her back to the adult’s chest promotes stability. B. Carrying the child over the arm promotes head lifting and improves tolerance for the prone position. To develop postural control of the trunk, the clinician must balance trunk extensor strength with trunk flexor strength. Trunk extension can be facilitated when the child is in the prone position over a ball by asking the child to reach for an object (Intervention 8-14, A). Protective extension of the upper extremities can also be encouraged at the same time, as seen in Intervention 8-14, B. The ball can also be used to support body weight partially for standing after the hips have been prepared with some gentle approximation (Intervention 8-15). A balanced trunk allows for the possibility of eliciting balance reactions. These reactions can be attempted on a movable surface (Intervention 8- 16). The reader is referred to Chapter 5 for descriptions of additional ways to encourage development of motor milestones and ways to facilitate protective, righting, and equilibrium reactions within developmental postures. Intervention 8-14 Trunk Extension and Protective Extension A B 418 A. Trunk extension can be facilitated with the child in the prone position over a ball by asking the child to reach for an object. The difficulty of the task can be increased by having more of the child’s trunk unsupported. B. Protective extension of the upper extremities can also be encouraged from the same position over a ball if the child is moved quickly forward. Intervention 8-15 Standing with Support from the Ball A. Preparing the hips for standing, with some gentle approximation. B. Use of the ball as a support for standing. Intervention 8-16 Eliciting Balance Reactions A B Cc A. Ensure a neutral pelvis, neither anteriorly nor posteriorly tilted. B. Shift weight to one side, keeping the weight on the downside hip. This allows the child to respond with lateral head and trunk righting. 419 C. When the child exhibits lateral righting, trunk rotation can be encouraged as part of an equilibrium reaction. When trunk extension is not balanced by abdominal strength, trunk stability may have to be derived from hip adduction and hip extension by using the hamstrings (Moerchen, 1994). If a child has such low tone that the legs are widely abducted in the supine position, the hip flexors will quickly tighten. This tightness impairs the ability of the abdominal oblique muscles to elongate the rib cage. The result is inadequate trunk control, a high-riding rib cage, and trunk rotation. Inadequate trunk control in children with low tone not only impairs respiratory function but also impedes the development of dynamic postural control of the trunk, usually manifested in righting and equilibrium reactions. Contractures and Deformities Avoiding contractures and deformities may seem to be a relatively easy task because these children exhibit increased mobility. However, muscles can shorten in overly lengthened positions. Because of low tone and excessive joint motion, the child’s limbs are at the mercy of gravity. When the child is supine, gravity fosters external rotation of the limbs and the tendency for the head to fall to one side, thus making it difficult for the child with low tone to maintain the head in midline. Simple positioning devices such as a U-shaped towel roll can be used to promote a midline head position. Intervention should be aimed at normal alignment and maintenance of appropriate range of motion for typical flexibility and comfort. Positions that provide stability at the cost of continuing excessive range, such as wide abducted sitting, propping on hyperextended arms in sitting, or standing with knee hyperextension, should be avoided. Modify the positions to allow for more typical weight bearing and use of muscles for postural stability rather than maintaining position. Narrow the base of sitting when the child sits with legs too widely abducted. Use air splints or soft splits to prevent elbow or knee hyperextension. Another possibility is to use a vertical stander to support the child so that the knees are in a more neutral position. Good positioning can positively affect muscle use for maintaining posture, for easier feeding, and for breathing. Respiratory Function Chest wall tightness may develop in a child who is not able to sit supported at the appropriate time developmentally (6 months). Gravity normally assists in changing the configuration of the chest wall in infants from a triangle to more of a rectangle. If this change does not occur, the diaphragm will remain flat and will not work as efficiently. The child may develop rib flaring as a consequence of the underuse of all the abdominal muscles or the overuse of the centrally located rectus abdominis muscle. If the structural modifications are not made, the diaphragm cannot become an efficient muscle of respiration. The child may continue to belly breathe and may never learn to expand the chest wall fully. Fatigue during physical activity in children with low tone may be related to the inefficient function of the respiratory system (Dichter et al., 1993). Because these children work harder to breathe than other children, they have less oxygen available for the muscular work of performing functional tasks. Any child with low muscle tone may have difficulty in generating sufficient expiratory force to clear secretions. Children who are immobile because of the severity of their neuromuscular deficits, such as those with SMA or late-stage muscular dystrophy, can benefit greatly from chest physical therapy including postural drainage with percussion and vibration. The positions for postural drainage are found in Figure 8-11. Additional expiratory techniques are described in the section of this chapter dealing with CF. Chapter summary Working with children with genetic disorders can be challenging and rewarding because of the many variations exhibited within the different disorders. The commonality of clinical features exhibited by children with these disorders, such as low muscle tone, delayed development, and some degree of intellectual disability, except for the children with SMA, allows for discussion of some almost universally applicable interventions. Because motor development in children with genetic disorders is generally characterized by immature patterns of movement rather than by abnormal patterns, as seen in children with cerebral palsy, physical therapy management is geared 420 to fostering the normal sequence of sensorimotor development including postural reactions while safeguarding joint alignment. Because of the progressive nature of some of the genetic disorders, physical therapy management must also be focused on preserving motor function or on optimizing function in any body system that is compromised. The physical therapist assistant can play a valuable role in implementing physical therapy interventions for children with any of the genetic disorders discussed in this chapter. Review questions 1. What is the leading cause of inherited intellectual disability? 2. When one parent is a carrier for CF, what chance does each child have of being affected? 3. What genetic disorder produces muscle weakness without cognitive impairment? 4, What are the three mechanisms by which chromosome abnormalities occur? 5. What are the two most common clinical features in children with most genetic disorders involving the central nervous system? 6. What principles of motor learning are important to use when working with children with cognitive impairment? 7. What types of interventions are appropriate for a child with low tone? 8. What interventions can be used to prevent secondary complications in children with low tone? 9. What interventions are most often used with a child with OI? 10. What physical therapy goal is most important when working with a child with a progressive genetic disorder? 11. What constitutes an autism spectrum disorder? Case studies Rehabilitation Unit Initial Examination and Evaluation: AG History Chart Review AG is a 17-month-old girl with DS. AG and her parents have been participants in an infant program since she was 3 months old. AG was born at term with a pneumothorax. During her stay in the neonatal intensive care unit, the DS diagnosis was confirmed by genetic testing. She has had no rehospitalizations. Her health continues to be good. Immunizations are up to date. Subjective The child’s mother reports that AG laughs and sings. She smiles easily and is a good eater. She previously had difficulty with choking on food. Her mother’s biggest concern is knowing when to expect AG to walk. Objective Systems Review Communication/Cognition: AG has 10 words in her vocabulary. She understands “no.” AG’s mental development index on the Bayley scale is < 50, based on a raw score of 75, which is mildly delayed performance. Cardiovascular/pulmonary: Values normal for age. Integumentary: Skin intact, no scars or areas of redness. Musculoskeletal: AROM greater than normal, strength decreased throughout. Neuromuscular: Coordination and balance impaired. Test and Measures Anthropometric: Height 32", weight 30 lbs, BMI 21 (20-24 is normal). 421 Motor Function: AG rolls from supine to prone and pushes herself into sitting over her abducted legs. She pulls to stand by furniture but is unable to come to stand from sitting without pulling with her arms. AG sits independently with a wide base of support. She is unable to stand from a squat. Neurodevelopmental Status: Peabody Developmental Motor Scales (PDMS) Gross Motor Developmental Motor Quotient (DMQ) is below average (DMQ = 65), age equivalent is 9 months. Fine Motor DMQ = 69, with an age equivalent of 9 months. Range of Motion: PROM is WFL in all joints, with joint hypermobility present in the hips, knees, and ankles of the lower extremities and in the shoulders and elbows of the upper extremities. No asymmetry is noted. Reflex Integrity: Biceps, patellar, and Achilles 1 + bilaterally. Low muscle tone is present throughout her extremities and trunk. No asymmetry is noted. Cranial Nerve Integrity: AG turns her head toward sound. Visually, she tracks in all directions, although she tends to move her head with her eyes. Quick changes in position such as when she is being picked up or in an inverted position are tolerated without crying. She has no difficulty swallowing liquids or solids by parent report. Sensory Integrity: Sensation appears to be intact to light touch. Posture: When she is ring sitting on the floor, her trunk is kyphotic. Her posture is slightly lordotic in quadruped position. Gait, Locomotion, and Balance: AG creeps on her hands and knees for up to 30 feet. She pivots in sitting. AG occasionally exhibits trunk rotation when making the transition from hands-and- knees to side sitting. AG exhibits head righting reactions in all directions. Trunk righting reactions are present, but equilibrium reactions are delayed and are incomplete in sitting position and quadruped position. Upper extremity protective reactions are present in all directions in sitting but are delayed. Balance in standing requires support of a person or object. She leans forward, flexing her hips and keeping her knees hyperextended. Self-care: AG finger-feeds. She assists with dressing by removing some clothes. Play: AG plays with toys appropriate for a 9- to 12-month-old. She looks at pictures in a book and squeezes a doll to make it squeak. Assessment/evaluation AG is a 17-month-old girl with DS who is functioning below her age level in gross and fine motor development and cognitive development. She is creeping reciprocally and pulling to stand but not walking independently. She is classified at a GMFCS level 1. She has a supportive family and is involved in an infant intervention program. Frequency of treatment is one time a week for an hour. Problem List 1. Delayed gross and fine motor development, secondary to hypotonia 2. Hypermobile joints 3. Dependent in ambulation 4. Delayed postural reactions Diagnosis AG demonstrates impaired neuromotor development which is guide pattern 5B. Down syndrome is a genetic syndrome which is included in this pattern, as is delayed development and cognitive delay. Prognosis AG will improve her level of functional independence and functional skills in her home. Her potential is good for the following goals. Short-term goals (1 month) 1. AG will walk while pushing an object 20 feet 80% of the time. 2. AG will demonstrate trunk rotation when moving in and out of side sitting 80% of the time. 3. AG will rise to standing from sitting on a stool without pulling with her arms 80% of the time. Long-term goals (6 months) 1. AG will ambulate independently without an assistive device for unlimited distances. 2. AG will go up stairs alternating feet while holding on to a rail independently. 3. AG will assist in dressing and undressing as requested. 4. AG will exhibit beginning pretend play by substituting one object for another while playing with a doll. 422 Plan Coordination, communication, and documentation The physical therapist and physical therapist assistant will be in frequent and constant communication with the family and the early childhood educator regarding AG’s program. Outcomes of interventions will be documented on a weekly basis. Patient/client instruction Discuss family instruction regarding positions to avoid and a home exercise program. The program is to include movement/games that encourage exploration and play in postural positions that challenge AG’s balance. Procedural interventions 1. Using a small treadmill, the parents will support AG as she is encouraged to take steps 15 minutes twice a day. 2. Using appropriate verbal and manual cues, AG will assist with removing her clothes before therapy and putting them back on after therapy. 3. Work on movement transitions from four-point to kneeling, kneeling to half-kneeling, half- kneeling to standing, standing from sitting on a stool, standing to a squat, and returning to standing. 4. Use weight bearing through the upper and lower extremities in developmentally appropriate postures such as four-point, kneeling, and standing to increase support responses. Maintain joint alignment to prevent mechanical locking of joints and encourage muscular holding of positions. 5. Use alternating isometrics and rhythmic stability in sitting, quadruped, and standing positions to increase stability. 6. AG will be encouraged to push a weighted toy shopping cart during play. 7. AG will be engaged in play with a doll and functional objects, such as a cup and spoon. Questions to think about a What activities could be part of AG’s home exercise program? = How can fitness be incorporated into AG’s physical therapy program? AROM, Active range of motion; BMI, body mass index; GMFCS, gross motor functional classification system; PROM, passive range of motion; WFL, within functional limitations. Case studies Rehabilitation Unit Initial Examination and Evaluation: DJ History Chart review DJ is an 8-year-old boy diagnosed with DMD at the age of 3. He attends a regular school and is in the second grade. He has had one recent hospitalization for pneumonia which lasted 3 days. He continues on an antibiotic for the recent lung infection and has just begun taking Prednisone.* Subjective DJ’s mother reports that he lives with his parents and one younger sister. He ambulates independently and wants to play basketball with his classmates during recess. He is being seen in school for physical therapy one time a week. His mother and father are active participants in his home exercise program, which consists of active and passive range of motion and aerobic exercise. DJ’s orthopedist is considering surgery to release his tight heel cords. Objective Systems review Communication/Cognition: DJ is talkative and friendly. His IQ is 80. Cardiovascular/Pulmonary: RR is 20 beats/min with adventitious breath sounds. HR and BP are normal for age. Integumentary: Intact. Musculoskeletal: AROM and PROM impaired. Strength impaired proximally. Neuromuscular: Coordination diminished. 423 Tests and measures Appearance and Anthropometric: Height 50”, weight 49 lbs, BMI 14 (20-24 is normal). Pseudohypertrophy noted in calf muscles bilaterally. Cardiovascular/Pulmonary: Rales and crackles evident at bases bilaterally. Diaphragm strength is fair with a functional cough. Vital capacity is 75% of predicted for age. Motor Function: DJ ambulates independently but fatigues easily. Starting with arms at the sides, he can abduct his arms in a full circle until they touch above his head. He can lift a 10-Ib weight to a shelf above eye level. He stands up from lying supine in 60 seconds demonstrating a Gower sign. He climbs stairs with the aid of a railing foot over foot. Muscle Performance: Muscle testing is performed in sitting unless otherwise specified as per standard manual muscle testing procedures (Berryman, 2005). Shoulders = Flexors « Abductors Elbow « Flexors « Extensors Wrist = Flexors = Extensors rip - Flexors 3— (tested in prone) « Extensors - a ; ies 4— (tested in side lying) = Extensors « Flexors Ankle « Plantar flexors * Dorsi flexors Range of Motion: Active and passive range of motion is WFL except for 15-degree hip flexion contracture bilaterally. He exhibits iliotibial band tightness and 5-degree plantar flexion contractures with 15 degrees of active dorsiflexion bilaterally. Reflex Integrity: Patellar 2 +, Achilles 1 +, Babinski is absent bilaterally. Sensory Integrity: Intact. 424 Posture: In standing, DJ exhibits a forward head and lordosis; weight is shifted forward onto the toes and his heels are off the ground. Gait, Locomotion, and Balance: He walks with no arm swing, does not run easily or well. He walks a total of 60 feet in 3 minutes with one rest of 1-minute duration. He can walk 30 feet as fast as he can without falling in 2 minutes. On average, he walks 2.5 hours a day. He takes a protective step in any direction when standing balance is disturbed. Self-care: DJ dresses, feeds, and toilets himself independently. Play: He plays with videogames, likes action figures, and is involved in cub scouts. He reads at grade level. He enjoys swimming, going to the zoo, and riding his bicycle around the neighborhood. He participates in physical education at school. Assessment/evaluation DJ is an 8-year-old boy with DMD who attends school regularly and receives physical therapy in the school setting as needed to prevent pulmonary complications and maintain present level of function. He recently had an upper respiratory infection that required hospitalization. He is ambulatory but has lower extremity contractures that are beginning to interfere with upright function. His physician is considering surgical intervention to release his heel cords. He is being seen once a week for 30 minutes and is participating in a home exercise program. Problem list 1. Lower extremity contractures 2. Decreased strength and endurance 3. Decreased pulmonary function 4. At risk for decreased locomotion Diagnosis DJ exhibits impaired muscle performance, which is guide pattern 4C because it includes myopathies. He also could be classified under 5B, because muscular dystrophy is a genetic disorder, or 6A, which is a prevention/risk reduction pattern for cardiovascular/pulmonary disorders. Prognosis DJ will improve or maintain his present level of function and prevent a recurrence of respiratory infection, which might lead to permanent respiratory compromise. His potential is fair for the following goals. Short-term goals (Actions to be Accomplished by Midyear Review) 1. DJ will increase active and passive dorsiflexion to 20 degrees bilaterally so that he can stand to write math problems on the board. 2. DJ will play on the playground equipment safely. 3. DJ will be independent in breathing exercises. 4. DJ’s family will demonstrate correct postural drainage and assisted coughing techniques. 5. DJ will ambulate 50 feet times 3 with custom molded AFOs during the school day with only one rest. Long-term goals (End of 2nd Grade) 1. DJ will maintain lower extremity muscle strength. 2. DJ will swim across the pool, breathing every other stroke. 3. DJ will exhibit no decline in vital capacity. 4. DJ will ambulate 50 feet times 4 with AFOs during the school day. 5. DJ will increase total standing time by 30 minutes a day. Plan Coordination, communication and documentation The physical therapist and physical therapist assistant will be in frequent and constant communication with DJ’s family and his teacher. The therapist will communicate with the physician and orthotist prior to and after surgery to lengthen his heel cords. If another therapist/assistant is involved during the acute care phase, the school therapist would need to establish and maintain communication. Outcomes of interventions will be documented on a weekly basis. Patient/client instruction Teach how to don and doff AFOs independently following surgery; implement wearing schedule; 425 and check for skin integrity. Teach safety on the playground. Teach and review techniques of chest wall stretching, diaphragmatic breathing, inspiratory and expiratory muscle training, postural drainage, and assistive cough. Have DJ stand a total of 3 hours a day, part of which should occur at home. Procedural interventions 1. Positioning a. Standing on a small wedge for increasing amounts of time to stretch heel cords. b. Use a prone stander for one or two class periods to provide stretch to hip and knee flexors and dorsiflexors. c. Wear lower extremity night splints before and after surgery. d. Monitor for development of scoliosis. Strengthening a. Do concentric movements of quadriceps, hamstrings, and dorsiflexors against gravity; add manual resistance or Theraband if suitable. b. Use marching, kicking, and heel walking. c. Pull on Theraband with upper extremities. d. Monitor for change in strength. Aerobic and functional activities a. Move through an obstacle course while being timed. 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Science. 2003;302(5646):826-830. 432 433 SECTION 3 Adults 434 CHAPTER 9 435 Proprioceptive Neuromuscular Facilitation” Objectives After reading this chapter, the student will be able to: ¢ State the philosophy of proprioceptive neuromuscular facilitation. ¢ List the proprioceptive neuromuscular facilitation patterns for the extremities and trunk. ¢ Describe applications of extremity and trunk patterns in neurorehabilitation. ¢ Explain the use of proprioceptive neuromuscular facilitation patterns and techniques within postures of the developmental sequence. ¢ Identify which proprioceptive neuromuscular facilitation techniques are most appropriate to promote the different stages of motor control. ¢ Understand the rationale for using the proprioceptive neuromuscular facilitation approach in neurorehabilitation to address movement impairment. ¢ Discuss the motor learning strategies used in proprioceptive neuromuscular facilitation. 436 Introduction The purpose of this chapter is to present one of the most frequently used treatment interventions in neurologic rehabilitation, proprioceptive neuromuscular facilitation (PNF). PNF can be used to improve performance of functional tasks by increasing strength, flexibility, and range of motion. Integration of these gains assists the patient to: (1) establish head and trunk control, (2) initiate and sustain movement, (3) control shifts in the center of gravity, and (4) control the pelvis and trunk in the midline while the extremities move. Using the developmental sequence as a guide, the goal of these techniques is to promote achievement of progressively higher levels of proficiency and functional independence in bed mobility, transitional movements, sitting, standing, and walking. 437 History of proprioceptive neuromuscular facilitation Dr. Herman Kabat, a medical physician, applied his background in neurophysiology to conceptualize this therapeutic approach in the early 1940s. He was joined by two physical therapists, Margaret Knott in 1947 and Dorothy Voss in 1953. The team collaborated in expanding and refining treatment techniques and procedures to improve motor function. Knott and Voss authored the first book introducing PNF in 1956. The initial focus of these founders was on development and application of integral concepts including resistance, stretch reflexes, approximation, traction, and manual contacts to facilitate movement. Their goal and the goal of their treatment approach was to promote improvement in patient efficiency in motor function and independence in activities of daily living (Kabat, 1961). PNF was based on the understanding of the central nervous system at the time and grew to become a viable treatment method. Kabat, Knott, and Voss continued to treat patients, review the literature, and refine their approach during the ensuing years. Today, clinicians and researchers continue to provide input that allows PNF to grow and evolve. This chapter presents a combination of the traditional interventions used by clinical practitioners and the tenets embraced by the International PNF Association. 438 Basic principles of PNF Motor learning is enhanced through skilled application of ten essential components (Knott and Voss, 1968). These concepts are often referred to as the key elements of PNF (Table 9-1). anual contacts ody position and body mechanics tretch anual resistance rradiation oint facilitation iming of movement atterns of movement isual cues erbal input Manual Contacts Placing the hands on the skin stimulates pressure receptors and provides information to the patient about the desired direction of movement. Optimally, manual contacts are placed on the skin overlying the target muscle groups and in the direction of the desired movement (Adler et al., 2008). For example, to facilitate shoulder flexion, one or both of the clinician’s hands are placed on the anterior and superior surface of the upper extremity; to facilitate trunk flexion, the hands contact the anterior surface of the trunk. A lumbrical grip is preferred to control movement and provide optimal resistance, especially regarding rotation, while avoiding excessive pressure or producing discomfort (Figure 9-1). FIGURE 9-1 Lumbrical grip. A lumbrical grip is one in which the metacarpophalangeal joints are flexed and adducted while the fingers are in relaxed extension. This position allows flexion forces to be generated through the clinician’s hand without squeezing (which provides ambiguous sensory stimulation regarding muscle group and direction) or exerting excessive pressure. This grip provides optimal control of the three-dimensional movements that occur in PNF patterns. 439 Body Position and Body Mechanics Dynamic clinician movement that mirrors the patient’s direction of movement is essential to effective facilitation. The pelvis, shoulders, arms, and hands of the clinician should be placed in line with the movement. When this is not possible, the arms and hands of the clinician should be in alignment with the movement. Resistance is created through use of the clinician’s body weight while the hands and arms remain relatively relaxed (Adler et al., 2008). Stretch Kabat proposed that the stretch reflex could be used to facilitate muscle activity. He hypothesized that if the muscle is placed in an elongated position, a stretch reflex could be elicited by producing slight movement farther into the elongated range. A stretch facilitates the muscle that is elongated, synergistic muscles at the same joint and facilitates other associated muscles (Loofbourrow and Gellhorn, 1948). Although quick stretch tends to increase motor response, prolonged stretch can potentially decrease muscle activity; therefore, patient response should be closely monitored. The presence of joint hypermobility, fracture, or pain contraindicates the use of facilitatory stretch. Stretch, especially quick stretch, should be applied with caution in the presence of spasticity because individual responses vary, and may result in undesired motor activity. Manual Resistance Resistance is defined by Sullivan and Markos (1995) as “an internal or external force that alters the difficulty of moving.” The status of the involved tissue regarding stiffness, length, and neurologic influences dictates the internal resistance that the patient encounters during movement. Manual, mechanical, or gravitational forces can be used to apply resistance external to the body surface. Some PNF procedures focus on reducing internal resistance by altering neural firing patterns; other activities or techniques provide external resistance to increase motor unit recruitment. Therefore, in the context of PNF, resistance may be considered either a means of neuromuscular facilitation or a tool through which muscle strengthening can be promoted. Through complex interactions among neural and contractile components, resistance may influence movement initiation, postural stability, timing of functional movement patterns, motor learning, endurance, and muscle mass (Sullivan and Markos, 1995). Appropriate resistance facilitates the maximum motor response that allows proper completion of the defined task (Knott and Voss, 1968). If the goal of intervention is mobility, appropriate resistance is the greatest amount of resistance that allows the patient to move smoothly and without pain through the available range of motion (Kisner and Colby, 2007). The amount and direction of the applied force must adapt to the changes in muscle function and patient ability that may occur throughout the range. If the goal of intervention is stability, appropriate resistance is the greatest amount that allows the patient to isometrically maintain the designated position. Irradiation Irradiation is a neurophysiologic phenomenon defined as an increase in activity in related muscles in response to external resistance. This term is often used synonymously with overflow and reinforcement (Adler et al., 2008; Sullivan et al., 1982). The magnitude of the response increases as the stimulus increases in intensity and duration (Sherrington, 1947). PNF uses the process of irradiation to increase muscular activity in the agonist muscle(s) or to inhibit opposing antagonist muscle groups. Each person’s response to resistance varies; therefore, different patterns of overflow occur among individuals. By watching patient response, the clinician can identify the manual contacts and amount of resistance that maximize a patient's ability to generate the desired movement. Examples of activities and typical patterns of response include the following: 1. Resistance to trunk flexion produces overflow into the hip flexors and ankle dorsiflexors. 2. Resistance to trunk extension produces overflow into the hip and knee extensors. 3. Resistance to upper extremity extension and adduction produces overflow into the trunk flexors. 4. Resistance to hip flexion, adduction, and external rotation produces overflow into the dorsiflexors. 440 Joint Facilitation Traction and approximation stimulate receptors within the joint and periarticular structures. Traction creates elongation of a body segment, which can be used to facilitate motion and decrease pain (Sullivan et al., 1982). Approximation produces compression of body structures, which can be used to promote weight bearing and muscle cocontraction (Adler et al., 2008). Individual responses to traction and approximation vary. These forces may be applied during performance of extremity patterns or superimposed upon body positions. Timing of Movement Normal movement requires smooth sequencing and gradation of muscle activation. Timing of most functional movements occurs in a distal to proximal direction, as in picking up a pencil. The pencil is grasped in the hand and then positioned for use by actions of the elbow and shoulder. A related consideration is that development of postural control proceeds from cephalad to caudal and from proximal to distal (Shumway-Cook and Woollacott, 2012). These issues must be considered when assessing, facilitating, and teaching movement strategies in the neurologically impaired individual (Carr and Shepherd, 1998). Adequate muscle strength and joint range of motion may be present to allow execution of a specified functional task; however, sequencing of the components within a movement pattern may be faulty. Also, sufficient control of the trunk and proximal extremity joints must be attained before mastery of tasks that require precise movements of the distal joints. Patterns of Movement PNF is characterized by its unique diagonal patterns of movement. Kabat and Knott recognized that groups of muscles work synergistically in functional contexts. They combined these related movements to create PNF patterns. Furthermore, because muscles are spiral and diagonal in both structure and function, most functional movements do not occur in cardinal planes. For example, reaching with an upper extremity and walking are two common activities that occur as triplanar versus uniplanar movements. PNF patterns, therefore, more closely simulate the demands incurred during functional movements. Visual Cues Visual cues can help an individual control and correct body position and movement. Eye movement influences head and body position. Feedback from the visual system may be used to promote a stronger muscle contraction (Adler et al., 2008) and to facilitate proper alignment of body parts, such as the head and trunk, through postural reactions. Verbal Input A verbal command is used to provide information to the patient. The command should be concise and should provide a directional cue. The verbal command consists of three phases: preparation, action, and correction. The preparatory phase readies the patient for action. The action phase provides information about the desired action and signals the patient to initiate the movement. The correction phase tells the patient how to modify the action if necessary. PNF uses the knowledge of the effects of voice volume and intonation to promote the desired response, such as relaxation or greater effort (Adler et al., 2008). Application of Proprioceptive Neuromuscular Facilitation Principles When considered as a group, the preceding principles provide a template for the clinical application of PNF techniques. The clinician’s hands are placed on the surface of the patient’s body in the direction of the desired diagonal movement using a lumbrical grip (see Figure 9-1). The clinician positions the patient to allow for dynamic movement by aligning the patient’s body with the diagonal movement pattern. The body segment is elongated before requesting the patient to move, and a quick stretch is applied if appropriate. A concise verbal command is given and timed to 44] coincide with the initiation of the desired movement. The amount of resistance is graded (increased or decreased to match the patient's ability to generate force) to allow for the desired response. Normal timing is considered and reinforced during the movement pattern. The clinician monitors the patient’s response and may add a visual cue to enhance the response. Table 9-2 lists key points to use as a tool for clinical application. This checklist may help the clinician select specific PNF techniques to address individual patient needs. Table 9-2 PNF Checklist for Clinical Use Component Correct Incorrect] Patient position Clinician position Clinician’s body mechanics| Manual contacts Stretch || Resistance /-— 442 Biomechanical considerations Other considerations that affect relative ease or difficulty of movement include biomechanical factors such as the base of support (BOS), center of gravity (COG), number of weight-bearing joints, and length of lever arm. The BOS involves both the body surface in contact with the supporting surface and the area enclosed by the contacting body segments. COG refers to the distance of the center of mass of the patient’s body to the supporting surface. The number of weight-bearing joints involved indicates the complexity and degree of control inherent in the activity. In general, the greater the number of joints through which the line of force passes, the greater the degree of muscle control required to efficiently perform a related task. The lever arm is affected by gravity, body weight, and the site of application of the resistive force. The resultant force on the moving segment increases as the distance between the applied force and the target muscles increases. All of these factors must be considered when selecting and progressing activities and techniques within a therapeutic exercise program. A relative increase in difficulty is experienced by the patient when the height of the COG, number of weight-bearing joints, and length of lever arm are increased or the BOS is decreased. Within the developmental sequence, the natural progression of postures is that of increasing challenge to the stabilizing muscles. Quadruped, therefore, is a more demanding position than prone-on-elbows because of COG location relative to the support surface and differences in surface area within the BOS. 443 Patterns Early development of PNF techniques included analysis of typical movement strategies (Knott and Voss, 1968). The results of these observations were integrated into specific combinations of joint movements called patterns. Although often combined in clinical practice, patterns focus on either the extremities or the trunk. All PNF patterns consist of a combination of motions occurring in three planes. The rotation component is especially important and should be recruited during the beginning range of the pattern. Early rotation reinforces normal distal to proximal timing of extremity movements while recruiting greater participation of the trunk musculature. Extremity Patterns The two extremity diagonal patterns are diagrammed in Figure 9-2. These are named diagonal 1 (D,,) and diagonal 2 (D,). Extremity patterns are named for the direction of movement occurring in the proximal joint and represent the movement that results from performing the pattern. Each diagonal is further subdivided into flexion and extension directions. For example, in D, flexion in the upper extremity (UE), the shoulder moves into flexion, and in D, extension, the shoulder moves into extension. The middle or intermediate joint may be flexed or extended. Straight arm and leg patterns are used to emphasize the proximal component of the pattern and recruit greater trunk activity. When the intermediate joints are flexed, more emphasis can be placed on the intermediate or distal components. The UE patterns will be described in a supine position. Figure 9-2 illustrates and identifies the components of the UE patterns. y \ d =a\ Sf Radial deviation 0, Flexion (wrist) UNS) Ulnar deviation UNS (wrist) 0, Extension Ulnar doviation D, Extension A B (wrist) (A) (> Ye 7 4 =p A=A4 ASA Radial deviaton D, Flexion Racal Geviation D, Flexion (wrist) nN (wrist) “ 204 (a , — 7 External rotation ABDUCTION ys Y WY C 0, Extension Ulnar deviation (wrist) D 0, Extension Ulnar deviation (wrist) FIGURE 9-2 Upper extremity diagonal patterns. The two major diagonal patterns (D, and D2) of the upper extremity are depicted in the four pictured diagrams. The reader should orient herself to the illustration as if the reader is the person (patient role) moving the left arm with the head at the top of the diagram. The posture of the hands is used to help the reader guide the movements in the correct combinations. The shaded areas represent 444 the shoulder components of the pattern in bold type: (A) D, Flexion, (B) D; Extension, (C) Dz Flexion, and (D) Dz Extension. For example, to perform D, Flexion, the reader begins with the hand in the D, extension hand position in which the left hand is thrust slightly out from the left side of the body as if in preparation to stop a fall and performs the shaded movements depicted in diagram A, i.e., shoulder external rotation and adduction, so that the hand ends up in the D, hand position (the left hand has performed a movement similar to grasping a scarf and bringing it across the body and over the right shoulder). To perform D, Extension, the reader looks at Figure 9-2, B, and starts in the D, Flexion hand position, performing the shaded movements in a reverse sequence. To perform D» Flexion, the reader starts with the left hand curled in a fist next to the right hip with the arm across the body and then moves the arm up and to the left as if in preparation to throw something over the left shoulder. Dz Extension is performed in a reverse sequence. Upper Extremity Patterns The UE D, flexion pattern consists of shoulder flexion/adduction/external rotation. The arm begins in an extended position slightly out to the side, about one fist width from the hip. The shoulder is extended/abducted/internally rotated with the forearm pronated, and the wrist ulnarly deviated. The clinician requests that the patient “squeeze my hand and pull up.” It may be helpful for the clinician to suggest that the patient think about reaching up to bring a scarf over the opposite shoulder. The UE D, extension pattern is the reverse of the flexion pattern and consists of extension/abduction/internal rotation. The patient starts with the arm flexed with the elbow across the midline of the body at about nose level. The forearm is supinated with the wrist and fingers flexed and the wrist radially deviated. The clinician requests that the patient “open your hand and push down and out.” The UE D, flexion diagonal pattern is often thought of as functional for feeding and the UE D, extension pattern as functional for performing a protective reaction when in a sitting position. Detailed descriptions of the UE D, flexion pattern and the UE D, extension pattern are found in Tables 9-3 and 9-4, respectively. Performance of the UE D, flexion pattern and UE D, extension pattern are depicted in Interventions 9-1 and 9-2, respectively. Table 9-3 Upper Extremity D, Flexion—Flexion/Abduction External Rotation—Elbow Extended Starting Position Ending Position p Posterior depression Anterior elevation Extension/abduction/internal rotation] Flexion/adduction/external rotation| Elbow Extension Extension Pronation Supination Wrist Extension/ulnar deviation Flexion/radial deviation Fingers | Extension Flexion Table 9-4 Upper Extremity D, Extension—Extension/Adduction/Internal Rotation—Elbow Extended Joint Starting Position Ending Position Scapula_| Anterior elevation Posterior depression Shoulder] Flexion/adduction/external rotation] Extension/abduction/internal rotation} Elbow Extension Extension Forearm | Supination Pronation Wrist Flexion/radial deviation Extension/ulnar deviation Fingers | Flexion Extension Intervention 9-1 Upper Extremity D, Flexion 445 446 447 The pattern begins in the lengthened position of the primary muscles involved (extension) and ends in the shortened position of the same muscle groups (flexion). The patient’s left upper extremity is being treated. The clinician’s right hand is placed distally; her left hand proximally. A. Beginning. The clinician stands in the diagonal position and faces the patient’s feet. The clinician’s right palm contacts the patient’s left palm, similar to holding hands as if going for a walk. The palmar surface of the clinician’s left hand is placed on the anterior aspect of the patient’s arm just proximal to the elbow. The verbal command is given to “turn your hand up and pull up and across your body.” B. Midrange. As the patient pulls the left upper extremity across the body, the clinician remains in the diagonal position while pivoting to face the patient. Manual contacts may shift slightly to accommodate patient effort. C. End range. The patient completes the range with hand placements consistent with the previous description of midrange. Intervention 9-2 Upper Extremity D, Extension 448 449 450 The pattern begins in the lengthened range of the involved muscle groups (flexion) and ends in the shortened range (extension). The patient's left upper extremity is treated. The clinician’s left hand contacts the dorsal aspect of the patient’s hand, including the fingers. The clinician’s right palm contacts the patient’s dorsal arm, just proximal to the elbow. A. Beginning. The clinician stands in the diagonal position and faces the patient. The given verbal command is “turn your hand down and push down and out to the side.” The patient extends the wrist and fingers and pronates the forearm, as if pushing the clinician away. Note that some clinicians prefer to face the patient's feet in the starting position of this pattern. B. Midrange. The clinician shifts body weight and position to accommodate movement through the range. Manual contacts continue on the dorsal hand or fingers and the dorsal and distal aspect of the patient’s humerus. C. End range. The clinician pivots toward the patient’s feet while remaining in the diagonal position. Manual contacts remain as previously. It is important that during the latter part of this pattern that as the clinician facilitates or resists wrist extension that the force is parallel to the patient’s forearm. CAUTION: Care must be taken to avoid application of force perpendicular to the forearm, which can result in resistance to the shoulder flexors. This input disrupts the flow of the pattern and often confuses the patient as to the intent of the movement. The D, flexion pattern consists of shoulder flexion/abduction/external rotation. The arm begins 451 extended across the body with the elbow crossing the midline, forearm pronated, wrist and fingers flexed, and wrist ulnarly deviated. The clinician asks the patient to “lift your wrist and arm up.” The UE D, extension pattern is the reverse of the flexion pattern and consists of shoulder extension/adduction/internal rotation. The arm begins in flexion about one fist width lateral to the ipsilateral ear. The shoulder is externally rotated with the forearm supinated, wrist and fingers extended, and the wrist radially deviated. The clinician requests that the patient “squeeze my hand and pull down and across.” Students can remember these diagonals functionally by thinking of D, flexion as throwing a wedding bouquet over the same shoulder and D, extension as placing a sword in its sheath. Detailed descriptions of the UE D, flexion pattern and UE D, extension pattern are found in Tables 9-5 and 9-6, respectively. Performance of the UE D, flexion pattern and UE D, extension pattern are depicted in Interventions 9-3 and 9-4, respectively. Table 9-5 Upper Extremity D, Flexion—Flexion/Abduction/External Rotation—Elbow Extended Starting Position Ending Position Anterior depression Posterior elevation Elbow Extension Extension Wrist Flexion/ulnar deviation Extension/radial deviation Fingers | Flexion Extension Table 9-6 Upper Extremity D, Extension—Extension/Adduction/Internal Rotation—Elbow Extended Joint Starting Position Ending Position Scapula_| Posterior elevation Anterior depression Elbow Extension Extension Wrist Extension/radial deviation Flexion/ulnar deviation Fingers | Extension Flexion Intervention 9-3 Upper Extremity D, Flexion 452 453 454 The pattern is pictured as applied to the patient’s left upper extremity. The clinician’s right hand contacts the dorsal aspect of patient’s hand, with the left hand on the dorsal humeral region. A. Beginning. The clinician stands in the diagonal position and faces the patient’s left hip. The clinician’s right palm contacts the patient’s dorsal hand, and then places the dorsal aspect of her left hand against the patient’s dorsal humerus, just proximal to the elbow. The given command is “open your hand and lift your thumb up and out.” B. Midrange. As the patient moves into midrange, the clinician shifts backward. The clinician’s left hand naturally supinates with the movement, allowing the palm to now contact the patient’s dorsal arm. The clinician’s right thumb may be used to facilitate or resist thumb abduction. C. End range. Movement continues through range with manual contacts remaining similar to those at midrange. The clinician shifts farther posteriorly as needed to accommodate patient movement. Intervention 9-4 Upper Extremity D, Extension 455 456 457 The patient’s left upper extremity participates, starting with the shoulder in a flexed position overhead. A. Beginning. The clinician stands in the diagonal position and faces the patient. She then places the left hand in the patient’s palm and the dorsal aspect of the right hand on the anterior surface of the patient’s arm, just proximal to the elbow. The pattern commences upon the command to “squeeze my hand, turn your thumb down and toward your opposite hip.” The patient then flexes her fingers to grasp the clinician’s hand, flexes the wrist, and pronates the forearm. B. Midrange. As the patient extends and adducts her shoulder, the clinician pivots to face the patient’s feet and supinates the forearm such that the patient’s dorsal arm now lies within the clinician’s open hand. C. End range. The patient completes the motion as the clinician shifts her weight backward to resist the patient’s efforts as appropriate. The clinician maintains similar manual contacts as described for midrange. The following associations may help students remember the movement combinations in the upper extremity. Flexion patterns are always paired with shoulder external rotation, forearm supination, and radial deviation of the wrist. Conversely, UE extension patterns are always paired with shoulder internal rotation, forearm pronation, and ulnar deviation of the wrist. Scapular Patterns 458 The scapula moves in diagonal patterns in keeping with scapulohumeral biomechanics. The scapular pattern associated with D, flexion is anterior elevation. The scapula elevates and protracts as the arm comes across the body. The scapular pattern associated with D, extension is the opposite of anterior elevation or posterior depression. The scapula is depressed and retracted. To help visualize these movements, consider shrugging your shoulder forward toward your ear as being associated with the UE D, flexion pattern and putting the inferior angle of your right scapula in the left hip pocket as related to D, extension. These patterns are pictured in Interventions 9-5 and 9-6, respectively. Intervention 9-5 Scapular Anterior Elevation 459 The patient is pictured in left side-lying with the cervical spine in neutral position. The right scapular region is addressed. The clinician stands behind the patient, approximately at level with the patient’s pelvis. The clinician stands in the diagonal position and faces the patient’s head. A. Beginning. The clinician’s right hand contacts the patient’s right acromial region. The clinician’s left hand is placed on top of and reinforces her right. The patient is asked to “shrug your shoulder forward toward your ear.” B. End. The patient completes the motion while the clinician shifts her body weight onto the forward foot, mirroring patient movement. Intervention 9-6 Scapular Posterior Depression 460 The patient is lying on the left side and the right shoulder region is treated. The clinician stands in the diagonal position, behind the patient and facing her head. A. Beginning. The clinician’s right hand is placed on the patient’s right acromion with her left hand contacting the inferior and medial border of the scapula. The pattern begins upon the command “pull your shoulder blade down and back.” B. End. As the patient continues through the range, the clinician shifts her body weight onto the back leg to counter patient effort. The scapular pattern associated with D, flexion is posterior elevation. As the arm is lifted up and externally rotated, the scapula is posteriorly elevated. Shrugging with the shoulder held back is approximately the same motion as the scapula is elevated and retracted. Scapular anterior depression 461 is part of the D, extension pattern and is the opposite of posterior elevation. The scapula is depressed and protracted as when pushing up to sitting from side-lying. These patterns are shown in Interventions 9-7 and 9-8, respectively. Intervention 9-7 Scapular Posterior Elevation The pattern is performed with the right scapula with the patient lying on the left side. The clinician stands in the diagonal position at the end of the table adjacent and slightly posterior to the 462 patient’s head. A. Beginning. The clinician’s left hand is placed slightly posterior to the patient’s right acromion; the right hand covers the left hand. The patient is asked to “shrug your shoulder up and back.” B. End. As the patient elevates and adducts the scapula, the clinician shifts her body weight backward. Intervention 9-8 Scapular Anterior Depression 463 The pattern is applied to the patient’s right scapula while the patient is left side-lying. The clinician stands at the head of the table adjacent and slightly posterior to the patient’s head. A. Beginning. Manual contacts are positioned slightly anterior to the patient’s right acromion with the left hand under the right. The verbal command “push your shoulder blade down and forward” is given. B. End. The clinician shifts her weight forward as the patient depresses and adducts the scapula. A clock is a useful way to visualize the scapula moving on the thorax. The patient is positioned in left side-lying. Twelve o’clock is toward the patient’s head, and six o’clock is toward the feet. Figure 9-3 depicts the placement of the scapular diagonals on a clock face. Posterior elevation is at eleven o'clock, and diagonally opposite at five o’clock is anterior depression. Anterior elevation is at one o’clock, and diagonally opposite at seven o’clock is posterior depression. 12 Posternor _—_—_—___' Anterior elevation 1.1 = 1 cievation tk > y, \ ff Y . \\ ~ moderate assist of 1 — minimal assist of 1 — standby assist of 1 as he progresses q. Patient will be asked to walk toward an object or place of interest; orientation will be incorporated in this exercise by having patient walk to get a newspaper or objects he may need in the home r. As patient progresses, simulated shopping may be included with gait activities s. Patient will be asked to make a list of items or remember a list given to him verbally to make the task more cognitively challenging 5. Dynamic balance activities: a. In a standing position, patient will shoot baskets and count baskets made b. Patient will carry objects while ambulating 6. Discharge planning: a. Patient will be discharged to home with supervision by caregiver b. A home assessment will be performed if needed c. Equipment will be secured as necessary d. If a proper caregiver cannot be obtained for discharge to home, patient will be discharged to assisted-living facility e. Vocational rehabilitation will be contacted Questions to think about = How can the therapists facilitate the performance of functional activities? a What other therapeutic interventions can be used to help the patient with motor learning? = How can aerobic conditioning be included in the patient's treatment program? m What types of activities or exercises would be included as part of the patient’s home exercise program? 719 References American Physical Therapy Association: Position paper: protecting student athletes from concussions act of 2013 (HR 3530). Available from www.apta.org/PolicyResources/PositionPapers/ConcussionsStudentA thletes. Accessed November 3, 2014. Bland DC, Zampieri-Gallagher C, Damiani DL. Effectiveness of physical therapy for improving gait and balance in ambulatory individuals with traumatic brain injury: a systematic review of the literature. Brain Inj. 2011;25:664-679. Bleiberg J: The road to rehabilitation. Part 3. 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Clin Manag. 1988;8:6-9. 721 CHAPTER 12 722 Spinal Cord Injuries Objectives After reading this chapter, the student will be able to: ¢ Discuss the causes, clinical manifestations, and possible complications of spinal cord injury. ¢ Differentiate between complete and incomplete types of spinal cord injuries. ¢ Discuss the various levels of spinal cord injury. * Relate segmental level of muscle innervation to level of function in the patient with a spinal cord injury. ¢ Instruct patients with a spinal cord injury in pulmonary exercises, strengthening exercises, and mat activities. ¢ Teach gait training and wheelchair mobility interventions to the patient, as appropriate. 723 Introduction An estimated 12,000 new cases of spinal cord injury (SCI) occur annually. Within the United States, currently more than 273,000 people are living with SCIs (National Spinal Cord Injury Statistical Center, 2013). SCIs are most likely to occur in young adults between the ages of 16 and 30 years. However, as the population in the United States continues to age, the average age at time of injury has also increased to 42.6 years. Approximately 81% of the individuals with SCIs are male (National Spinal Cord Injury Statistical Center, 2013). The etiology of SCIs continues to change. Previously, injuries that were due to motor vehicle accidents and sporting activities were identified as the most likely causes. More recent statistics suggest that motor vehicle accidents (36.5%), falls (28.5%), acts of violence (14.3%), and sports-related injuries (9.2%) are the most common causes of SCIs in the United States (National Spinal Cord Injury Statistical Center, 2013). Life expectancies for individuals with SCIs are still below those without SCI, and there has not been an improvement in this statistic since the 1980s. Individuals with SCIs can experience a lifetime of disability and life-threatening medical complications. Potential causes of death that significantly affect life expectancy include pneumonia and septicemia. The cost of medical care for these individuals is in the billions of dollars. Lifetime medical expenses for individuals with high cervical injuries are approximately $4.6 million, and $2.2 million for individuals with paraplegia. These figures can exceed the maximum insurance benefit allowed by many insurance policies. In addition to the direct costs of medical care, there are indirect costs associated with lost wages, employee benefits, and productivity —costs that can average $70,575 a year (National Spinal Cord Injury Statistical Center, 2013). 724 Etiology To understand the etiology of SCIs, it is necessary to review the anatomy of the region. There are 31 pairs of spinal nerves within the peripheral nervous system. The first seven pairs of spinal nerves, which originate in the cervical area, exit above the first seven cervical vertebrae. Spinal nerve C8 exits between C7 and T1, because there is no eighth cervical vertebra. The remaining spinal nerve roots exit below the corresponding bony vertebrae. This holds true through L1. At this point, the spinal cord becomes a mass of nerve roots known as the cauda equina. Figure 12-1 illustrates segmental and vertebral levels. Occipital bone 1st cervical nerve —— 1st cervical cord segment 1st thoracic cord Vertebra T1 segment 1st thoracic nerve 1st lumbar cord segment Vertebra L1 1st sacral cord 1st lumbar ———— segment nerve QO D do pe Vertebra S1 © 1st sacral nerve ————— \ FIGURE 12-1 Segmental and vertebral levels compared. Spinal nerves 1 to 7 emerge above the corresponding vertebrae, and the remaining spinal nerves emerge below them. (From Fitzgerald MJT: Neuroanatomy: basic and clinical, Clinical neuroanatomy and related neuroscience, ed 4, London, 2002, WB Saunders.) Certain areas of the spinal column are more susceptible to injury than others. In the cervical spine, the spinal segments of C1, C2, and C5 through C7 are often injured, and in the thoracolumbar area, T12 through L2 are most often affected. The biomechanics of the vertebral column accentuates this situation. Movement (rotation) is greatest at these segments and leads to instability within the regions. In addition, the spinal cord is larger in these areas because of the large number of nerve cell bodies which are located here. Figure 12-2 illustrates this configuration. 725 Ventral root of spinal nerve C1 Dorsal root of spinal nerve c2 Cervical enlargement | if Dorsal root be 1 | Dorsal root 6 Lumbar enlargement Dorsal root u Conus medullaris Dorsal root $1 Filum terminale Coccygeal nerve FIGURE 12-2 Posterior view of the spinal cord showing the attached dorsal roots and spinal ganglia. (From Carpenter MB, Sutin J: Human neuroanatomy, ed 8, Baltimore, 1983, Williams & Wilkins.) 726 Naming the level of injury To name the level of an individual’s injury, the health-care professional first identifies the vertebral or bony spine segment involved. For example, cervical injuries are designated with C, thoracic injuries with T, and lumbar injuries with L. This designation is followed by the last spinal nerve root segment in which innervation is present. Therefore, if a patient has an injury in the cervical region and has innervation of the biceps, the lesion would be classified as a C5 injury. Medical personnel have used the following terms to describe the extent of involvement a patient may be experiencing. Individuals with injuries to the cervical region of the spine are classified as having tetraplegia, which is the preferred term. Tetraplegia encompasses impairments to the upper extremities, lower extremities, trunk, and pelvic organs. Injuries involving the thoracic spine can produce paraplegia. With paraplegia upper extremity function is spared, but there are varying degrees of lower extremity, trunk, and pelvic organ involvement. Injuries at L1 or below are called cauda equina injuries (Burns et al., 2012). The American Spinal Injury Association (ASIA) has developed standards to assist health-care providers in naming the level of the injury. The ASIA International Standards for Neurological Classification of Spinal Cord Injury assessment tool is the instrument that clinicians use to classify SClIs (Figure 12-3). The neurologic level is defined as the “most caudal segment of the cord with intact sensation and antigravity (3 or more) muscle function strength, provided that there is normal intact sensory and motor function rostrally respectively” (ASIA, 2013). Determination of the neurologic level is determined by testing key dermatomes (sensory areas) and myotomes (muscles) in a supine position. A patient’s sensory level is determined by assessing both light touch and pinprick sensation bilaterally (ASIA, 2013). Astin’ clsnrasned or wana cease ISC@S PsMCsca) SENSORY RIGHT 222%, omen Lape Touch LTR) PP POR *@ J cy TH UU ES EI i JF * aa \ AN c™“s Mp fences L2 LER foes eters LS a j | | | ey eer — {\ (“,) +g om iS) | | wi Aette pnts tears 81 ry If alc Vy Ii Seu t (ORO) Vokartary anal contraction d} or] Aa I os monrromus |_| manne i ™ - ~ motor —— ulin _—, __ SENSORY sunscones va ~}+0e + ves vora [__} veel _Joans[ sumsrom (|) ued en [ orom[_] a —_J+ emf | waar re war aa Fr r a = - NEUROLOGICAL rn 2 NEUROLOGICAL ‘ [San RS 20ME sone a Mewes UAVIL OF aR ] rac co 5 cat sao — Pty Sasa mmunwenT scace acy [_) ,_PRESERWATION woron UJI freely bet shout! et be ated eaheut permason fem the Americar Spe! mary Ansocaton FIGURE 12-3 ASIA Standard Neurological. Classification of Spinal Cord Injury. (From American Spinal | Injury Association: International standards for neurological classification of spinal cord injury, revised. Atlanta, GA, 2013, American Spinal Injury Association.) Normal muscle function is further defined as the lowest key muscle with a manual muscle testing grade of fair (3/5), provided that the key muscles above this level have intact (normal, 5/5) strength. ASIA has chosen these muscles because they are consistently innervated by the designated segments of the spinal cord and are easily tested in a clinical setting (ASIA, 2013). Table 12-1 lists the ASIA key muscles for the upper and lower extremities. For example, the elbow extensors (C7) are a key muscle group. Patients with C7 innervation have the potential to transfer independently without a sliding board because of their ability to extend the elbow and perform a lateral push-up. The ASIA standards also recognize that muscles are innervated by more than one spinal cord segment. Thus, assigning one muscle or group to represent a single spinal nerve is not appropriate 727 and leads to over simplification. Muscle innervation by one spinal nerve in the absence of additional innervation will result in muscle weakness (Burns et al., 2012). It is possible that an individual may have partial innervation of motor or sensory function in up to three segments below the injury site. In areas where there are not specific myotomes to test, the motor level is presumed to correspond to the sensory level if the muscles above that level are judged to have normal strength (ASIA, 2013). Table 12-1 ASIA Identification of Key Muscles That Can Provide Greatest Functional Improvements Level Key Muscles Elbow flexors Wrist extensors Elbow extensors Finger flexors Finger abductors Hip flexors Knee extensors Ankle dorsiflexors Big toe extensors Ankle plantar flexors Data from American Spinal Cord Injury Association: International standards for neurological classification of spinal cord injury, revised. Atlanta, GA, 2013, American Spinal Injury Association. 728 Mechanisms of injury Traumatic impact is a common cause of SCI. Trauma can be precipitated by compression, penetrating injury, and hyperextension or hyperflexion forces. The resultant injury to the spinal cord can be temporary or permanent. Associated injuries to the vertebral bodies may also lead to spinal cord damage. Vertebral subluxation (separation of the vertebral bodies), compression fractures, and fracture-dislocations can further damage the spinal cord by encroachment or additional compression of the spinal cord. Severe injuries to the vertebral column can also result in partial or complete transection of the spinal cord. Cervical Flexion and Rotation Injuries In the cervical region, the most common type of injury is one that involves flexion and rotation. With this type of force, the posterior spinal ligaments rupture, and the uppermost vertebra is displaced over the one below it. Rupture of the intervertebral disc and, in severe cases, the anterior longitudinal ligament can also occur. Transection of the spinal cord is often associated with this type of injury. Rear-end motor vehicle accidents frequently produce flexion and rotation injuries. Figure 12-4, A, provides an example of a flexion and rotation mechanism of injury. A Cervical 8 Hyperfiexion C Hyperextension D Compression flexion-rotahon injury injury injury injury FIGURE 12-4 A-D, Types of spinal cord injuries. Cervical Hyperflexion Injuries A pure hyperflexion force causes an anterior compression fracture of the vertebral body with stretching of the posterior longitudinal ligaments. The ligaments remain intact, however. The force sustained by the bony structures leads to a wedge-type fracture of the vertebral bodies. This type of injury frequently severs the anterior spinal artery and results in an incomplete anterior cord syndrome. A head-on collision or a blow to the back of the head is a cause of this type of injury. Figure 12-4, B, depicts an example. Cervical Hyperextension Injuries Hyperextension injuries are common in the older adult as a result of a fall. The individual’s chin often strikes a stationary object, and this leads to neck hyperextension. The force ruptures the anterior longitudinal ligament and compresses and ruptures the intervertebral disc. The spinal cord can become compressed between the ligamentum flavum and the vertebral body, with a resulting central cord type of injury. Figure 12-4, C, shows an example. Compression Injuries Vertical compressive forces can also injure the cervical or lumbar spine. Diving accidents cause injuries that are a combination of compression and flexion forces. Falls from elevated surfaces can also produce this type of injury. With vertical compression, one sees fracture of the vertebral end plates and movement of the nucleus pulposus into the vertebral body. Bone fragments can be produced and displaced outward. The longitudinal ligaments are stretched but remain intact 729 (Figure 12-4, D). Compression injuries caused by the effects of osteoporosis, osteoarthritis, or rheumatoid arthritis can also produce SCIs in the older adult. A discussion of the pathologic processes that lead to these conditions is beyond the scope of this text. 730 Medical intervention Following an acute SCI, the patient should be immobilized and transferred to a trauma center. Advances in the acute medical management include the administration of pharmacologic interventions which can limit the extent of initial injury by decreasing the effects of posttraumatic hemorrhage and ischemia, and thereby enhance blood flow. Methylprednisolone, a corticosteroid, and drugs that block opiate receptors can decrease the impact of hemorrhagic shock (Fuller, 2009). Once the patient is medically stable, a primary concern of the physician is stabilization of the spine to prevent further spinal cord or nerve root damage. Surgery is indicated in the following situations: (1) to restore the alignment of bony vertebral structures; (2) to decompress neural tissue; (3) to stabilize the spine by fusion or instrumentation; (4) to minimize deformities; and (5) to allow the individual earlier opportunities for mobilization (Somers, 2010). Several different stabilization procedures are available to the surgeon. Skeletal traction may be used on an interim basis while the patient’s medical condition is fragile. Traction can reduce the overlapping of fracture fragments and can assist with spinal alignment. Once the patient is medically stable, the physician may schedule the patient for surgery. During surgery, fusion of the fracture fragments is performed. Bone grafting from the iliac crests, combined with placement of internal fixation devices, is often employed during this procedure. In some situations, surgery is not indicated, and external fixation with a halo jacket, a hard cervical collar, or a rigid body jacket may be all that is needed to stabilize the involved spinal segments. Bony fusion is usually complete in 6 to 8 weeks. Figure 12-5 shows various types of spinal orthoses. Cc FIGURE 12-5 A, Halo vest. B, Aspen collar. C, Philadelphia collar. D, Custom-made body jacket. (B-D, From Umphred DA, editor: Neurological rehabilitation, Umphred's neurological rehabilitation, ed 6. St Louis, 2013, Elsevier, pp. 464, 466.) 731 732 Pathologic changes that occur following injury Initially after the injury, hemorrhage into the gray matter of the spinal cord occurs. There is necrosis of the axons that were damaged by the actual injury. Edema develops within the white matter and exerts pressure on the nerve fiber tracts that carry various cutaneous sensations to the cerebral cortex and motor impulses from the cortex to the body. Secondary tissue destruction and trauma ensues and can expand the injured area. Ischemia, hypoxia, and biochemical changes further deprive the white and gray matter of needed oxygen (Somers, 2010). The myelin sheathes begin to disintegrate, and the axons begin to shrink. The immune system is also thought to contribute to additional cell death as monocytes and macrophages emit chemical substances that “trigger apoptosis or programmed cell death” (Fuller, 2009). Eventually, a scar forms around the injury site (Fuller, 2009). It is extremely important to monitor the patient’s level of injury for the first 24 to 48 hours. The injury may ascend one or two levels because of vascular changes. If loss of function is apparent more than two spinal cord segments above the initial level of the injury, it may mean that the spinal cord was damaged in more than one place. Immediate notification of the patient’s primary nurse and physician is necessary. Immediately after an SCI, the patient exhibits spinal shock. The condition results from interruption of the pathways between higher cortical centers and the spinal cord (Fulk et al., 2014). Spinal shock is characterized by a period of flaccidity, areflexia, loss of bowel and bladder function, and autonomic deficits including decreased arterial blood pressure and poor temperature regulation below the level of the injury. Spinal shock normally lasts for approximately 24 to 48 hours; however, certain sources state that it may last up to several weeks. Because of suppressed reflex activity, one cannot accurately assess the patient's level of injury during spinal shock. As spinal shock resolves, reflex activity below the level of the lesion will return, reaching a peak at 1 to 6 months after injury, and if motor and sensory tracts have been salvaged, function in these areas will also be evident (Fulk et al., 2014). 729 Types of lesions SCIs are classified into two primary types: complete and incomplete. Because of the vast differences in clinical presentations, the ASIA Impairment Scale (AIS) was developed to allow for improved communication between health care professionals with respect to patient impairments (Fulk et al., 2014). The AIS is summarized in Table 12-2. Table 12-2 ASIA Impairment Scale Grade Impairment A= Complete No motor or sensory function is preserved in the sacral segments S4-S5. B = Sensory Sensory but not motor function is preserved below the neurologic level and includes the sacral segments S4-S5, And no motor is preserved more than three levels Incomplete below the motor level on either side of the body. C= Motor Motor function is preserved below the neurologic level, and more than half of key muscle functions below the neurologic level have a muscle grade less than 3. Incomplete D= Motor Motor function is preserved below the neurologic level, and at least half of key muscle functions below the neurologic level have a muscle grade of 3 or more. Incomplete E=Normal Motor and sensory functions are normal in all segments, and the patient had prior deficits. From American Spinal Cord Injury Association: International standards for neurological classification of spinal cord injury, revised. Atlanta, GA, 2013, American Spinal Injury Association. Complete Injuries If an injury is complete, sensory and motor function will be absent below the level of the injury and in the lowest sacral segments of S4 and S5. Complete injuries are most often the result of complete spinal cord transection, spinal cord compression, or vascular impairment. The most caudal segment with some sensory or motor function (or both) is defined as the zone of partial preservation. This condition applies only to complete injuries (Burns et al., 2012). Incomplete Injuries Incomplete injuries are described as those injuries in which there is partial preservation of some motor or sensory function (sacral sparing) below the neurologic level and in the lowest sacral segments of $4 and S5. Perianal sensation or voluntary contraction of the external anal sphincter indicates an incomplete injury (Burns et al., 2012). Investigators have estimated that more than 40.6% of patients have incomplete tetraplegia and 18.7% have incomplete paraplegia (National Spinal Cord Statistical Center, 2013). The clinical picture of incomplete injuries is highly variable and unpredictable. The area of the spinal cord damaged and the number of spinal cord tracts that remain intact dictate the amount of motor and sensory functions preserved. Several clinical findings help to confirm a diagnosis of an incomplete injury. Sacral sparing is one such finding. Because the sacral tracts run most medially within the spinal cord, they are often salvaged. Patients with sacral sparing may have perianal sensation and/or the ability to have voluntary control over the rectal sphincter muscle (Finkbeiner and Russo, 1990). These spared motor and sensory functions can be of great functional benefit to the patient because they may provide for normal bowel, bladder, and sexual activities. Another clinical finding observed in patients with incomplete injuries is abnormal tone or muscle spasticity. Resistance to passive stretching, clonus, increased deep tendon reflexes, and muscle spasms may be present. Decreased inhibition from descending supraspinal pathways, loss of sensory information associated with weight bearing, “loss of descending facilitation of afferents from Golgi tendon organs,” sprouting of synaptic terminals, and increased responsiveness to neurons distal to the injury may be possible explanations for these findings (Somers, 2010). Brown-Séquard Syndrome Brown-Séquard syndrome results from an injury involving half of the spinal cord (Figure 12-6, A). Penetrating injuries, such as injuries sustained from gunshot or stab wounds, are common causes. The patient loses motor function, proprioception, and vibration on the same side as the injury because the fibers within the corticospinal tract and dorsal columns do not cross at the spinal cord level. Pain and temperature sensations are absent on the opposite side of the injury a few segments 734 lower. The reason for the loss of pain and temperature sensations in this distribution is that the lateral spinothalamic tract ascends several spinal segments on the same side of the spinal cord before it crosses to the contralateral side (Fuller, 2009). Light touch sensation may or may not be preserved in these patients. Prognosis for recovery with this type of injury is good. Many individuals become independent in activities of daily living (ADLs) and are continent of bowel and bladder. A Brown-Séquard B Anterior cord syndrome syndrome C Central cord D Dorsal column syndrome syndrome FIGURE 12-6 A-—D, Types of incomplete spinal cord injuries. Anterior Cord Syndrome Anterior cord syndrome results from a flexion injury to the cervical spine in which a fracture- dislocation of the cervical vertebrae occurs. The anterior spinal cord or anterior spinal artery may be damaged (Figure 12-6, B). The patient loses motor, pain, and temperature sensations bilaterally below the level of the injury as a result of injury to the corticospinal and spinothalamic tracts. The posterior (dorsal) columns remain intact, and therefore the patient retains the ability to perceive position sense and vibration below the injury. The prognosis for functional return is limited because all voluntary motor function is lost. Central Cord Syndrome Central cord syndrome is another type of incomplete injury and is the most common. This type of SCI can result from progressive stenosis or compression that is a consequence of hyperextension injuries. Bleeding into the central gray matter causes damage to the spinal cord (Figure 12-6, C). Characteristically, the upper extremities are more severely involved than the lower extremities. This is because the cervical tracts are located more centrally in the gray matter. Injury to the central spinal cord damages three different motor and sensory tracts: the spinothalamic tract, the corticospinal tract, and the dorsal column. Sensory deficits tend to be variable. Bowel, bladder, and sexual functions are preserved if the sacral portions of the tracts are spared. Ambulation is possible for many patients. Functional independence in ADLs depends on the amount of upper extremity innervation the patient regains. Dorsal Column Syndrome 739 Dorsal column syndrome or posterior cord syndrome is a rare incomplete injury that results from damage to the posterior spinal artery by a tumor or vascular infarct (Figure 12-6, D). A patient with this type of injury loses the ability to perceive proprioception and vibration. The ability to move and to perceive pain remains intact. Conus Medullaris Syndrome Patients with injuries to the conus medullaris present with flaccid paralysis and areflexic bowel and bladder function. In some situations, the sacral reflexes are present. Cauda Equina Injuries A cauda equina injury usually occurs after the patient sustains a direct trauma from a fracture- dislocation below the L1 vertebrae. This type of injury often results in an incomplete lower motor neuron lesion. Flaccidity, areflexia, and loss of bowel and bladder function are the common clinical manifestations. Regeneration of the involved peripheral nerve root is possible, but it depends on the extent of initial damage. Table 12-3 summarizes the causes and clinical findings seen in patients with incomplete injuries. Table 12-3 Types of Incomplete Spinal Cord Injuries Cause Findings ype Brown-Séquard syndrome Penetrating injury: gunshot or stab wounds Loss of motor function, proprioception, and vibration on the same side as the injury Pain and temperature lost on the opposite side Anterior cord syndrome Flexion injury with fracture-dislocation of the cervical Loss of motor, pain, and temperature sensation bilaterally below the level of the injury vertebrae Position and vibration sense intact Central cord syndrome Progressive stenosis or hyperextension injuries Damage to all three tracts Upper extremities more involved than lower Sensory deficits variable Dorsal column or posterior cord | Compression of the posterior spinal artery by tumor or Loss of proprioception and vibration bilaterally syndrome vascular infarction Cauda equina injuries Direct trauma from a fracture-dislocation below L1 Upper and lower motor neuron signs possible including flaccidity, areflexia, loss of bowel and bladder function Conus medullaris syndrome Damage to the sacral aspect of the spinal cord and the Flaccidity of the lower extremities, areflexive bowel and bladder function lumbar nerve roots Sacral reflexes remain intact in some individuals Root Escape Damage to the nerve root within the vertebral foramen can lead to a peripheral nerve injury. Root escape is the term used to describe the preservation or return of motor or sensory function in various nerve roots at or near the site of injury. Therefore, a patient may experience some improved function or a return of function in the muscles innervated by the peripheral nerve several months after the initial injury. This increased motor or sensory return should not, however, be mistaken for return of spinal cord function. 736 Clinical manifestations of spinal cord injuries The clinical picture of a patient who has experienced an SCI is variable. Much depends on the level of the injury and the muscle and sensory functions that remain. In addition, one must consider whether the injury is complete or incomplete. In general, the following signs or symptoms may be present in an individual who has sustained an SCI: (1) motor paralysis or paresis below the level of the injury or lesion; (2) sensory loss (sensory function may remain intact two spinal cord segments below the level of the injury); (3) cardiopulmonary dysfunction; (4) impaired temperature control; (5) spasticity; (6) bladder and bowel dysfunction; and (7) sexual dysfunction. 737 Resolution of spinal shock Reflex activity below the injury resumes after spinal shock subsides. The earliest reflexes that return are the sacral level reflexes. As a result, reflexive bowel and bladder function may return. Flexor withdrawal responses may also become apparent. Initially, these reflexes are evoked by a noxious stimulus, and as recovery progresses, they may be evoked by other, less noxious means. As time goes on, upper or lower extremity spasticity can develop in muscle groups that lack innervation. Flexor spasticity in the lower extremities often develops first, secondary to interruption of the vestibulospinal tract. In time, extensor tone usually dominates (Decker and Hall, 1986). Additional muscle tightness and shortening become evident as a result of static positioning and muscle imbalances. For example, tightness in the hip flexors can develop as the patient spends increased amounts of time sitting upright in a wheelchair. 738 Complications Multiple complications can result following an SCI. Careful prevention of possible secondary complications can improve a patient's rehabilitation potential and quality of life. Pressure Ulcers One of the most common complications seen after SCI is the development of pressure ulcers. Pressure areas develop over bony prominences in response to the patient’s inability to perceive the need to shift weight or relieve pressure. Additionally, changes in collagen degradation and decreased peripheral blood flow makes the skin more vulnerable to injury (Somers, 2010). The treatment of open wounds that develop as a consequence of excessive pressure is a leading reason for increased lengths of hospital stays and medical costs (Fulk et al., 2014). For health-care professionals, prevention of pressure ulcers is of the utmost importance. Patients must be instructed in pressure relief techniques, or family members and caregivers must be taught how to assist the patient with weight-shifting activities. Patients should be instructed to perform 1 minute of pressure relief for every 15 to 20 minutes of sitting (Somers and Bruce, 2014). Patients who are able should perform skin inspection independently with the use of a handheld mirror. Patients who require physical assistance with skin inspection should be advised to instruct others in the performance of this activity. Protective padding can also be applied during the performance of functional activities to decrease sheer forces and the possibility of trauma. Equipment including specialized beds, mattresses, custom wheelchairs, cushions, and lower extremity splints and padding may be necessary to provide patients with some pressure-reducing capacities. Autonomic Dysreflexia Autonomic dysreflexia occurs in patients with injuries above T6. This pathologic autonomic reflex is caused by sympathetic nervous system instability. All sympathetic outflow occurs below the T6 level. Consequently, in cervical and upper thoracic injuries, descending excitatory and inhibitory input from the medulla to sympathetic neurons are lost. Autonomic responses are discharged as a result of a noxious sensory stimulus applied below the level of the lesion. This noxious sensory input causes autonomic stimulation, vasoconstriction, and a rapid and massive rise in the patient’s blood pressure. Normally, an increase in an individual’s blood pressure would stimulate the baroreceptors in the carotid sinus and aorta and would cause an adjustment in peripheral vascular resistance, thereby lowering the patient’s blood pressure. Because of the patient’s condition, impulses are unable to travel below the level of the injury to decrease the patient’s blood pressure. Thus, hypertension persists unless the noxious stimulus is removed or the patient receives medical intervention. This condition can cause life-threatening complications including renal failure, seizures, subarachnoid hemorrhage, and even death if left untreated. Common causes of autonomic dysreflexia include bladder or bowel distention, bowel impaction, disruption of the patient’s catheter, urinary tract infections, noxious cutaneous stimulation, pressure sores, kidney malfunction, environmental temperature changes, and a passive stretch applied to the patient’s hip (Somers, 2010). Symptoms of autonomic dysreflexia include significant hypertension, severe and pounding headache, bradycardia, vasoconstriction below the level of the lesion, vasodilation (flushing) and profuse sweating above the level of the injury, constricted pupils, goose bumps (piloerection), blurred vision, and a runny nose. Immediate recognition and treatment of these signs or symptoms is essential. The first thing one should do is to look for the likely source of noxious stimulation. Often, the patient’s catheter is kinked or the catheter bag may need emptying. If the source of the problem cannot be identified immediately, one should try to lower the patient’s blood pressure by sitting or standing the patient. Monitoring of the patient’s vital signs is necessary. Application of a nitroglycerin patch, a potent vasodilator, or administration of antihypertensive drugs including nifedipine, nitrates and captropril can assist in lowering the patient’s blood pressure (Fulk et al., 2014). The patient’s primary nurse and physician must be notified as soon as possible. Prevention of recurrent episodes and patient and family education are critical. Medications or surgical intervention may be needed to assist the patient in the regulation of this condition. 739 Postural Hypotension Another possible complication is postural hypotension. Patients who have experienced an SCI often develop low blood pressure. Lack of an efficient skeletal muscle pump, combined with an absent vasoresponse in the lower extremities, leads to venous pooling. Consequently, the amount of blood circulating in the body is decreased, thereby precipitating decreases in stroke volume and cardiac output. Postural hypotension can develop when patients are transferred to sitting, when they are placed in upright standing, or during exercise. Thus, careful monitoring of blood pressure responses must occur during treatment activities. The application of an abdominal binder before beginning upright activities promotes venous return by minimizing the drops in intraabdominal pressure that can occur when the patient’s position is changed. In addition, elastic stockings can be worn by the patient to prevent venous pooling in the lower extremities. Medications (vasopressors or mineralocorticoids) increase the patient’s blood pressure and increasing fluid intake in the presence of hypovolemia may be prescribed to manage this condition (Somers and Bruce, 2014). Pain Pain is acommon problem seen in patients after spinal cord injury. It has been reported that 26% to 96% of all individuals with SCI experience chronic pain (Fulk et al., 2014). Pain can limit the patient's ability to participate in rehabilitation and may have negative consequences on one’s ability to perform ADLs, sleep, and one’s overall quality of life. Two types of pain have been identified: nociceptive and neuropathic. Nociceptive pain is associated with musculoskeletal structures (i.e., muscles, bones, tendons) and can develop as a result of the initial injury, inflammation, poor handling and positioning, or muscle spasm. Over time, the patient with SCI can develop musculoskeletal pain and overuse pain syndromes, especially in the upper extremity. Common conditions seen include rotator cuff tears, shoulder impingement, lateral epicondylitis, carpal tunnel syndrome, and tendonitis of the wrist. These overuse injuries develop as a result of repetitive upper extremity movements and weight-bearing conditions needed to complete functional tasks including wheelchair propulsion, transfers, and pressure relief (Somers, 2010; Fulk et al., 2014). Neuropathic pain develops as a consequence of injury to the central and or peripheral nervous system and can occur at, above, or below the level of the initial injury. Neuropathic pain above the injury site is often due to damage to a peripheral nerve from compression or entrapment. The nature of the pain can be variable and may be constant or intermittent, and can be sharp, shooting, or burning in nature. Treatment of neuropathic pain is challenging for health-care practitioners. Medical interventions include patient education about the nature of the pain and pharmacologic management. The physician may prescribe acetaminophen or other nonsteroidal antiinflammatory drugs, including ibuprofen (Motrin), naproxen (Naprosyn), and indomethacin (Indocin); anticonvulsants such as gabapentin (Neurontin), pregabalin (Lyrica), and valproic acid (Depakote); the antidepressant amitriptyline (Elavil); and analgesics (tramadol). Psychological pain management techniques, transcutaneous electrical nerve stimulation, acupuncture, and mental imagery may also be helpful in the management of chronic pain (Fulk et al., 2014; Somers, 2010). Contractures Patients tend to develop flexion contractures as a result of the flexor reflex activity that develops after the injury and also as a consequence of prolonged sitting. Muscle imbalances around a joint may also predispose an individual to contracture formation. Prevention of contractures is important to maintain maximal function. Patients should be instructed in a good stretching program that they can perform independently or with the assistance of a family member or caregiver. In addition, all patients should be encouraged to perform a regular prone positioning program. Patients should spend at least 20 minutes each day on their stomachs to stretch the hip flexors. The prone position also relieves pressure on the ischial tuberosities and can provide aeration to the buttocks. Heterotopic Ossification Heterotopic ossification is another potential secondary complication. Bone can form in the soft tissues below the level of the injury. Usually, heterotopic bone develops adjacent to a large lower extremity joint, such as the hip or knee. The etiology of heterotopic ossificans is unknown, although spasticity, 740 trauma, complete injury, and urinary tract infection are thought to contribute to its development. Clinical signs of heterotopic ossification include range-of-motion limitations, swelling, warmth, and pain; fever may or may not be present. The management of this condition entails pharmacologic intervention with bisphosphonates; physical therapy and range-of-motion exercises to maintain available range; and surgical resection if the patient has a significant limitation (Fulk et al., 2014; Somers, 2010). Deep Vein Thrombosis The development of deep vein thrombosis is a common and life-threatening complication. The risk appears to be greatest during the first 2 to 3 months after injury. Because patients are often immobile and are medically fragile during this period, prophylactic anticoagulants, such as oral warfarin (Coumadin) or intravenous heparin, may be used for the first few months after the injury to prevent blood clotting. Surgical implantation of a vena cava filter may also be necessary to decrease the risk of pulmonary embolus. Regularly scheduled turning programs and early mobilization including sitting up in bed and transferring to a wheelchair are important to prevent venous pooling. Elastic supports and sequential compression devices for the lower extremities may also be prescribed to assist the patient with venous return. Osteoporosis Osteoporosis can be seen after SCIs because of changes in calcium metabolism. Although the exact etiology is not clear, decreased opportunities for weight bearing and limited muscle activity are thought to contribute to decreased bone density. The reduction in bone mass also places patients at an increased risk for fractures, with an incidence as high as 46% of all patients experiencing a pathologic fracture (Somers, 2010). Early mobilization, therapeutic standing, use of functional electric stimulation, administration of calcium supplements, and good dietary management can minimize the development of these potential complications (Fulk et al., 2014). Respiratory Compromise Serious and sometimes life-threatening complications can develop as a result of a patient’s decreased respiratory capabilities. These complications develop in response to decreased innervation of the muscles of respiration and immobility. The diaphragm, innervated by cervical nerve roots C3 through C5, is the primary muscle of inspiration. Therefore, patients with high cervical injuries may lose the ability to breathe on their own, secondary to paralysis or weakness of the diaphragm muscle. The external intercostal muscles assist with inspiration and are innervated segmentally starting at T1. They act to lift the ribs and increase the dimension of the thoracic cavity. Patients with paraplegia below T12 have innervation of the external intercostals and should be able to exhibit a normal breathing pattern using the chest and diaphragm equally. This is often described as a two-chest two-diaphragm breathing pattern (Wetzel, 1985). The abdominals are the other important muscle group needed for respiration. The upper abdominal muscles are innervated by T7 through T9, and the lower abdominals are innervated by spinal segments T9 through T12. The abdominals are activated when the patient attempts forceful expiration, such as coughing. Patients who are unable to generate an adequate amount of muscle force to cough will be susceptible to accumulation of bronchial secretions. This can lead to pneumonia, atelectasis, and respiratory compromise in many individuals. Weakness in the muscles of respiration can also lead to a decreased inspiratory effort and impairment of the patient’s ability to tolerate exercise—a factor that ultimately affects endurance for functional activities. Multiple interventions are used to minimize the effects of impaired respiratory function. These include early acclimation to the upright position, abdominal corsets and binders to assist with positioning of the abdominal contents, assisted cough techniques taught to the patient and caregivers, diaphragmatic strengthening, and incentive spirometry techniques. A more in-depth discussion of these techniques occurs in the treatment section of this chapter. Bladder and Bowel Dysfunction Bladder and bowel dysfunction may be considered a clinical finding or a complication of SCI. 741 Patients with SCIs often experience difficulties with this area of function, and urinary tract infections are a major cause of mortality in individuals with SCI (Fulk et al., 2014). The bladder is innervated by the lower sacral segments, specifically S2 through $4. During the period of spinal shock, the bladder is flaccid or areflexic. Once spinal shock is over, two possible situations can prevail, depending on the location of the injury. If the patient’s injury is above S2, the sacral reflex arc remains intact, and the patient is said to have a hyperreflexic or spastic bladder. In this condition, the bladder empties reflexively when the pressure inside it reaches a certain level. Patients can apply specific cutaneous stimulation techniques to the suprapubic region to assist with bladder emptying. If the patient’s injury is to the cauda equina or the conus medullaris, the patient is said to have a nonreflexive or flaccid bladder. The sacral reflex arc is not intact, and thus the bladder remains flaccid, requiring manual emptying at predetermined time periods (Fulk et al., 2014). Bladder-training programs are important components of the patient’s rehabilitation program. Intermittent catheterization, timed voiding programs, and manual stimulation can be used to empty the bladder and allow the patient to be catheter-free. Residual volumes of urine must be monitored to aid in the prevention of urinary tract infections (Fulk et al., 2014). Bowel dysfunction is a major concern for many patients and can impact one’s involvement in social activities and how one views his overall quality of life. In patients with injuries above S2, the patient will have a spastic or reflex bowel. Reflexive emptying of stool will occur once the rectum is full. In injuries at S2 to S4, patients have a flaccid or areflexive bowel, and as such the bowels do not empty reflexively, leading to possible impaction or incontinence (Fulk et al., 2014). The establishment of a regular bowel program is also part of the patient’s comprehensive plan of care. Patients are often placed on a regular schedule of bowel evacuation. High-fiber diets, adequate intake of fluids, use of stool softeners, and manual stimulation or evacuation may be suggested to assist the patient in the establishment of a bowel program (Fulk et al., 2014). The rehabilitation team needs to be aware of the patient’s schedule for bladder and bowel training. Therapies should not be scheduled during times designated for these activities. Sexual Dysfunction A common concern expressed by patients following SCI is the impact the injury will have on sexual relationships. As stated previously, physical function depends on the patient’s motor level. Males with upper motor neuron injuries have the potential for reflex erections (ones that occur in response to external stimulation) if the sacral reflex arc remains intact. Psychogenic erections are possible through cognitive activity at the level of the cortex. The ability to ejaculate is limited for patients with both upper and lower motor neuron injuries. Therefore, men experience significant challenges with fertility. Advances in medications, topical agents, and mechanical devices are available to improve erectile function. Women with SCIs continue to experience menstruation and thus are able to become pregnant. Women who do become pregnant and are ready to deliver are often hospitalized as a precautionary measure, because they may not be able to feel uterine contractions (depending on their neurologic level) that would indicate the onset of labor (Fulk et al., 2014). Physical therapists (PTs) and physical therapist assistants (PTAs) must be comfortable discussing this information with their patients. Because of the time we spend working with our patients, questions related to sexual activity may be directed to us. We must answer questions honestly and accurately. If you do not feel comfortable fielding these types of questions, you need to refer the patient to someone who can. Spasticity Spasticity is a common sequela of SCI. The prevalence of spasticity is higher in patients with cervical and incomplete injuries, specifically those classified as ASI B and C (Somers, 2010). Research suggests that increased tone is the result of residual influence of supraspinal centers (cortex, red nucleus, reticular system, and vestibular nuclei) on the spinal cord and ineffective modulation of spinal pathways (Craik, 1991). Spasticity may also be greater in patients who have experienced significant and multiple complications. Investigators have also shown that noxious stimuli tend to exacerbate abnormal muscle tone. In most instances, PTs and PTAs focus treatment on ways to decrease or minimize the effects of abnormal muscle tone. However, in some instances, an increase in muscle tone can be advantageous to the patient. Spasticity can help maintain muscle bulk, prevent atrophy, and assist in the maintenance of circulation. Spasticity can also assist the 742 patient in performing functional activities including transfers, basic bed mobility, and standing when the patient has adequate innervation and sufficient trunk control. In addition, spasticity can provide increased tone to the anal sphincter, tone that may aid the patient in performing a bowel program. The management of spasticity can be challenging. At this time, no treatment is available that completely ameliorates the effects of abnormal tone. Physicians may recommend a multitude of interventions to help the patient. Elimination of the stimuli or factors that contribute to increased sensory input is beneficial. Physical therapy interventions may include positioning, static stretching, weight bearing, cryotherapy, aquatics, and functional electrical stimulation. These different treatment interventions are discussed in more depth in the treatment section of this chapter. Pharmacologic intervention may be necessary for some patients with significant abnormal tone. The most common oral medications prescribed include dantrolene sodium, which targets muscle contractility; baclofen (Lioresal) and diazepam (Valium), which target y-aminobutyric acid receptors in the central nervous system; and clonidine (Catapres), which decreases spasticity through its effects on alpha receptors in the spinal cord (Somers, 2010). All these medications have documented side effects, including hepatotoxicity, bradycardia, sedation, decreased attention and memory, hypotension, and reduced muscle strength and coordination (Somers, 2010 p. 50; Katz, 1988, 1994; Scelza and Shatzer, 2003; Yarkony and Chen, 1996). Patients frequently experiment with these medications and then discontinue their use because of adverse side effects. Intrathecal baclofen pumps and botulism injections are other forms of treatment for spasticity. With the intrathecal pump, a pump and small catheter are implanted subcutaneously into the patient’s abdominal wall. Baclofen is then delivered directly into the subarachnoid space of the spinal cord, thereby reducing the dosage needed and some of the side effects. Baclofen has been found to be more effective in reducing tone in the lower extremities compared with the upper extremities because of catheter placement (Katz, 1988; Scelza and Shatzer, 2003). Botulinum toxin A is injected directly into the spastic muscle. This neurotoxin inhibits the release of acetylcholine at the neuromuscular junction, thereby causing temporary muscle paralysis (Cromwell and Paquette, 1996). Surgical intervention is a final type of management of abnormal tone. Neurectomies, rhizotomies, myelotomies, tenotomies, and nerve and motor point blocks may be administered to assist the patient with management of abnormal tone. Neurectomy is the surgical excision of a segment of nerve. Rhizotomy is a surgical procedure in which the dorsal or sensory root of a spinal nerve is resected. In myelotomy, the tracts within the spinal cord are severed. Tenotomy is the surgical release of a tendon. Nerve blocks are performed with injectable phenol and reduce spasticity on a temporary basis (3 to 6 months). A more detailed description of these procedures is beyond the scope of this text (Katz, 1988, 1994; Yarkony and Chen, 1996). 743 Functional outcomes A patient’s functional outcome following an SCI depends on many factors. Age, the type and level of the injury, the motor and sensory function preserved, the patient’s general health and preinjury activity level, status before the injury, body build, support systems, financial security, motivation, access to medical and rehabilitation services, and preexisting personality traits—all play a role in the patient’s eventual outcome (Somers and Bruce, 2014; Lewthwaite et al., 1994). In patients with motor complete injuries (AIS A), the neurologic level is the most important factor in determining the patient’s eventual functional outcome (Somers and Bruce, 2014). Key Muscles by Segmental Innervation Before we can begin to talk about functional capabilities in an individual with SCI, we must review key muscles and their actions. The innervation of key muscle groups allows patients to achieve a certain level of functional skill and independence. Table 12-4 highlights key muscles at each spinal level. Table 12-4 Key Muscles by Segmental Innervation Spinal Level Muscles Cl1-C2 Facial muscles, partial sternocleidomastoid, capital muscles C3 Sternocleidomastoid, partial diaphragm, upper trapezius C4 Diaphragm, partial deltoid, sternocleidomastoid, upper trapezius C5 Deltoid, biceps, rhomboids, brachioradialis, teres minor, infraspinatus C6 Extensor carpi radialis, pectoralis major (clavicular portion), teres major, supinator, serratus anterior, weak pronator| CF Triceps, flexor carpi radialis, latissimus, pronator teres C8 Flexor carpi ulnaris, extensor carpi ulnaris, patient may have some hand intrinsics T1-T8 Hand intrinsics, top half of the intercostals, pectoralis major (sternal portion T7-T9 Upper abdominals T9-T12 Lower abdominals T12 Lower abdominals, weak quadratus lumborum L2 Iliopsoas, weak sartorius, weak adductors, weak rectus femoris L3 Sartorius, rectus femoris, adductors L4 Gluteus medius, tensor fascia latae, hamstrings, tibialis anterior L5 Weak gluteus maximus, long toe extensors, tibialis posterior SI Gluteus maximus, ankle plantar flexors (gastrocnemius, soleus $2 Anal sphincter Functional Potentials Each successive motor level provides the patient with the potential for greater function. Strength of a muscle must be at least fair-plus to perform a functional activity (Alvarez, 1985). Table 12-5 provides a review of functional potentials based on the patient’s motor innervation and limitations encountered because of decreased muscle strength or range of motion. A description of each level and the patient’s potential for achievement of functional activities is provided. It is important to keep in mind that these functional expectations should serve only as a guide and that individual patient differences must be considered when developing patient goals or plan of care. Table 12-5 Functional Potential for Patients with Spinal Cord Injuries 744 Present Potential Level Muscles C1-C2: Facial muscles Vital capacity 20%-30% of normal ca C3: Stemocleidomestoid, Power recline wheelchair with breath or chin control and portable upper trapezius ventilator Ability to perform pressure relief in wheelchair with power recline feature Fulltime attendant required Ability to direct care verbally Use of environmental control units with setup Vital capacity 30%-50% of normal Power wheelchair with mouth stick or chin control 30° of cervical motion needed to drive a wheelchair with a chin control Maximal assistance with bed mobility Independent pressure relief with power reclining wheelchair Fulltime attendant required Ability to direct care verbally Use of environmental contro! units with setup Vital capacity 40°%-60% of normal Power wheelchair with hand controls Manual wheelchair with rim projections Moderate assistance for bed mobility Maximal assistance needed for transfers (sliding board or sit pivot) Independent forward raise for pressure relief with loops attached to the back of the wheelchair Possible independence with some grooming tasks with adaptive equipment (wrist splints) and setup Attendant needed Use of environmental contro! units Vital capacity: 60°%-80% of normal Independent rolling Independent pressure relief via weight shift Independent sliding board transfers possible or patient may require minimal assist Modified independent manual wheelchair propulsion with rim projections Modified independent feeding with adaptive equipment Independent upper extremity dressing Requires assistance for lower extremity dressing Ability to drive automobile with hand controls Vocation outside the home possible Prehension with flexor hinge splint Attendant needed for an and pm care Assistance needed for commode transfers Vital capacity 80% of normal Independent living possible Independent pressure relief via lateral pushup Independent self-range of motion of lower extremities Modified independent transters, wheelchair propulsion, pressure relief, and upper and lower extremity dressing Same potential as individual at C7 Independent living Negotiation of 2- to 4-inch curbs in wheelchair Wheelies in wheelchair Hand intrinsics Independent in manual wheelchair propulsion on all levels and surfaces Top half of intercostals (6-inch curbs) Pectoralis major (sternal Therapeutic ambulation with orthoses in paralle! bars (T6-T8) portion) Abdominals Independent wheelchair mobility Therapeutic ambulation with orthoses and assistive devices possible T10 vital capacity 100% Quadratus lumborum Household ambulation Independent in coming to stand and ambulation with orthoses L3: thopsoas and rectas Commanity ambulation with orthoses Quadriceps, medial hamstrings 4-15 Community ambulation: may only need ankle-oot orthoses and canes for ambulation Ambulation with articulated ankle-foot orthoses ADLs, Activities of daily living. C1 Through C3 Limitations Dependent on ventilator No upper extremity innervation Dependent in all ADLs Dependent in bed mobility and transfers Has only elbow flexors, prone to elbow flexion contractures Must consider energy and time requirements for activity completion Dependent in bathing and dressing No elbow extension or hand function (patient prone to contractures) No finger muscles Transfers to floor require moderate or maximum assistance Assist needed to right wheelchair Some assistance needed for wheelchair propulsion on ramps and uneven terrain Some intrinsic hand function Writing, fine-motor coordination activities can be difficult Assistance with floor transfers No lower abdominal muscle function Minimal assistance to independent with floor transfers and righting wheelchair No hip flexor function No quadriceps function Wheelchair used for community ambulation Loss of bowel and bladder function A patient with an injury above C4 has limited muscle innervation. Because the diaphragm is only 745 minimally innervated by C3, most patients with injuries at these levels will likely require mechanical ventilation. Some patients with high cervical lesions may, however, be able to tolerate electric stimulation to the phrenic nerve (phrenic nerve pacing). Stimulation to the phrenic nerve causes the diaphragm to contract, thereby reducing the patient’s reliance on mechanical ventilation (Atrice et al., 2013). Patients with injuries at C1 through C3 require full-time attendants and will be totally dependent in all ADLs, bed mobility, and transfers. A power wheelchair with a reclining feature will be needed to allow for pressure relief and rest. The patient should have adequate breath support or neck range of motion to operate a power wheelchair by a sip-and-puff mechanism or with a chin cup. With a sip-and-puff unit, the patient either sips or blows into a straw mounted in front of his or her face to provide the stimulus for the wheelchair to move. A few patients may be able to use a chin cup. The device requires that the patient have at least 30 degrees of active cervical motion. Patients with injuries at C1 through C3 may or may not have sufficient active range of motion in the cervical spine. Advances in technology have improved the capabilities of all patients with SCls, especially those with injuries at higher levels. Environmental control units that can be operated from the wheelchair allow some patients an increase in control over their home and work environments. These control units can be networked with one’s personal computer and can operate appliances, lights, speaker phones, and so forth. Individuals with injuries at this level must be empowered to direct their care through instructions provided to attendants and caregivers. This provides the patient with a certain level of independence and autonomy regarding his or her situation and care. C4 A patient with a C4-level injury likely has some innervation of the diaphragm. This has significant functional implications because it means that a patient may not have to depend on a ventilator. The vital capacity of patients with diaphragmatic innervation is still markedly decreased. Individuals at this level should be able to operate a power wheelchair using a chin cup, chin control, or mouth stick. Patients still must have sufficient range of motion to drive a wheelchair with a chin control. Environmental control units may also be prescribed for these patients. Individuals with C4 innervation continue to require full-time attendants because they are completely dependent in all transfers and ADLs. C5 Patients with C5 innervation have some functional abilities. A patient with C5 innervation has deltoid, biceps, and rhomboid function. However, even though these muscles are innervated at this level, they may not have normal strength. Each patient has different motor capabilities, and the PT must thoroughly examine muscle function. Because of innervation of these key muscles, a patient with innervation at C5 should be able to flex and abduct the shoulders to 90 degrees, flex the elbows, and adduct the scapulae. The ability to flex and abduct the shoulders means that the patient will be able to raise his or her arms to assist with rolling and can also bring his or her hand to the mouth. He or she cannot, however, extend the elbow because the triceps are not innervated. The patient will be able to operate a power wheelchair with a hand control. A few patients are able to propel a manual wheelchair with rim projections. Although manual wheelchair propulsion may be possible, one must consider the high energy costs associated with this activity. For this reason, power wheelchairs are preferred for patients with innervation at this level. The individual with C5 innervation may be able to be independent with some self-care activities, but the patient will require setup of the activity by an attendant or a family member. Patients also need to use adaptive equipment, including splints and built-up ADL devices, to perform self-care activities. Our experience has shown that even though patients may be able to perform a self-care activity independently after setup, the time and energy required to complete the task are often too great to continue performance on a regular basis. Individuals with innervation at the C5 level can provide minimal assistance with sliding board transfers from their wheelchairs and will require assistance for bed mobility. They can perform independent pressure relief by leaning forward in the wheelchair or by looping one of their upper extremities over the push handles on the back of the wheelchair and performing a weight shift. The rhomboids provide limited scapular stabilization for upper extremity self-care activities and for assuming functional positions, such as prone on elbows and long sitting with extended arm support. Driving is possible with a van and adaptive hand controls. 746 C6 Patients with C6 innervation have some greater functional abilities. Because of innervation of the wrist extensors, the pectoralis major, and the teres major, patients at this level are able to be independent with rolling, feeding, and upper extremity dressing. The patient should be able to propel a manual wheelchair independently with rim projections, and the potential exists for the person to be independent with sliding board transfers. Patients may need assistance in the morning and at night with self-care activities, and some patients need assistance for transfers, especially to the commode. Assistance is also required for lower extremity dressing. The ability to drive a motor vehicle with adaptive controls and gainful employment outside the home are possible for individuals with innervation at this level. C7 An individual with a C7 injury has the potential for living independently because patients at this level have innervation of the triceps. With triceps strength, the patient can use his or her upper extremities to lift the body during transfers. In addition, the person will be able to perform a wheelchair push-up for pressure relief. Independence in self-care activities is possible, including upper and lower extremity dressing. A person should become independent in transferring from the wheelchair to the bed or mat, at first with a sliding board and eventually without the use of a board. Additional functional capabilities include independence with pressure relief, self—-range of motion to the lower extremities, and operation of a standard motor vehicle with adapted hand controls. C8 With innervation at C8, a patient can live independently. An individual is able to perform everything that a patient with innervation at a C7 level is able to complete. With the addition of some increased finger control, the patient may also be able to perform wheelies and negotiate 2- to 4-inch curbs in the wheelchair. T1 Through T9 We look at capabilities of individuals with T1 through T9 innervation as a group. With increased motor return in the thoracic region, the patient demonstrates improved trunk control and breathing capabilities including the ability to clear secretions because of increasing innervation of the intercostals. Individuals are able to operate a manual wheelchair on all levels and surfaces and should be able to transfer into and out of the wheelchair to the floor. Patients with innervation at the T1 through T9 level may also be candidates for physiologic standing and limited therapeutic ambulation in the parallel bars with physical assistance and orthoses. Therapeutic ambulation is defined as walking for the physiologic benefits that standing and weight bearing provide. The section of this chapter on ambulation discusses this concept in greater detail. T10 Through L2 Patients with innervation at the T10 through L2 level have abilities similar to those mentioned for individuals with T1 through T9 function. Therapeutic ambulation and ambulation in the home with orthoses and assistive devices may be possible, although manual wheelchair propulsion is the typical mode of functional mobility. L3 Through L5 The quadriceps are partially innervated by L3. The presence of lower extremity innervation improves the patient’s capacity for ambulation activities. Patients with innervation at this level should be independent in household ambulation and may become independent in community ambulation at the L3 level. Knee-ankle-foot orthoses or ankle-foot orthoses are necessary. Patients with injuries at the L4 and L5 levels should be independent with all functional activities, including gait. These individuals can ambulate in the community with some type of orthoses and assistive device. 747 748 Physical therapy intervention: acute care The acute-care management of the patient with an SCI centers around the following goals: 1. Prevention of joint contractures and deformities 2. Improvement of muscle and respiratory function 3. Acclimation of the patient to an upright position 4, Prevention of secondary complications 5. Pain management 6. Patient and family education The patient’s initial physical therapy examination includes information on the patient’s respiratory function, muscle strength, muscle tone, reflex activity, skin status, cardiac function, and functional mobility skills. The PT develops a plan of care to address the patient’s primary impairments, functional limitations, and activity restrictions. In this early stage, interventions should focus on breathing exercises, selective strengthening and range-of-motion exercises, functional mobility training, activities to improve the patient's tolerance to upright, and patient and family education. A patient with a cervical or thoracic injury may not immediately undergo surgical stabilization; therefore, the PT may be involved in the care of the patient in the intensive care unit. Any patient with an unstable spine must be carefully assessed by the physician for the appropriateness of physical therapy innervation. Because of the acuity of the patient’s condition and the potential for unpredictable patient responses, it is best for the patient to be treated by the PT at this stage. Cotreatments with the PTA or other members of the team may be appropriate. Breathing Exercises Exercises performed in the acute stage should emphasize maximizing respiratory function. Much depends on the patient’s current level of muscle innervation. For those patients with innervation between C4 and T1, emphasis is on increasing the diaphragm’s strength and efficiency. These patients possess diaphragm function and often demonstrate a diaphragmatic breathing pattern. If the diaphragm is weak, use of accessory muscles, such as the sternocleidomastoid and scalenes, may be evident. A good way to assess respiratory function is to observe the epigastric area and to watch for epigastric rise. An exaggerated movement of the abdominal area indicates that the diaphragm is working. The PTA can place a hand over this area to determine how much movement is actually occurring, as depicted in Figure 12-7. If the patient is having difficulty, a quick stretch applied before the diaphragm contracts can help facilitate a response. If the patient is able to move the epigastric area at least 2 inches, the strength of the diaphragm is said to be fair (Wetzel, 1985). To strengthen this muscle even more, the PTA can apply manual resistance during the inspiratory phase of respiration. If the patient is able to take resistance to the diaphragm during inspiration, the strength of the muscle is considered good. Care must be taken to gauge the amount of manual resistance applied. Early on, patients may experience difficulties in breathing as a consequence of diaphragm weakness. In addition, respiratory muscle fatigue may become evident. Observation of the neck area can provide the clinician with valuable information regarding accessory muscle use. Patients often use accessory muscles extensively when the diaphragm is weak. Visible contraction of the sternocleidomastoids, scalenes, or platysma indicates accessory muscle use. 749 FIGURE 12-7 Placement of the hand for diaphragmatic breathing. (From Myers RS: Saunders Manual of Physical Therapy Practice. Philadelphia, 1995, WB Saunders.) Glossopharyngeal Breathing Patients with injuries at the C1 through C3 level and some patients with injuries at C4 require mechanical ventilation. These patients need to be taught a technique to assist their ability to tolerate short periods of breathing while they are off the ventilator. Glossopharyngeal breathing is a technique that can be taught to patients with high-level tetraplegia. The patient takes a breath of air and closes the mouth. The patient raises the palate to trap the air. Saying the words “ah” or “oops” accomplishes this. The larynx is then opened as the tongue forces the air through the open larynx and into the lungs. This technique is extremely beneficial if, for some reason, the patient needs to be disconnected from the ventilator for a short time because of equipment failure, power outage, showering, or another unforeseeable circumstance. This technique allows the patient to receive adequate breath support until mechanical ventilation can be resumed. Lateral Expansion For patients who have some intercostal innervation (T1 through T12), lateral expansion or basilar breathing should be emphasized. Patients are encouraged to take deep breaths as they try to expand the chest wall laterally. PTAs can place their hands on the patient's lateral chest wall and can palpate the amount of movement present. Manual resistance can eventually be applied as the patient gains strength in the intercostal muscles. Progression to a two-diaphragm, two-chest breathing pattern is desirable if the patient has innervation through T12 (external intercostals). Incentive Spirometry Another activity that can be used to improve the function of the pulmonary system is incentive spirometry. Blow bottles at the patient’s bedside can encourage deep breathing. A measurement of vital capacity can be taken with a handheld spirometer. Vital capacity is the maximum amount of air expelled after maximum inhalation. Measurements of the patient’s vital capacity can be taken throughout rehabilitation to document changes in ventilation (Wetzel, 1985). Patients can also be instructed to vary their breathing rate and to hold their breath as a means to promote improved respiratory function. Chest Wall Stretching Spasticity and muscle tightness within the chest wall can develop. Manual chest stretching may be indicated to increase chest expansion. The PTA can place one hand under the patient’s ribs and the 750 other on top of the chest. The clinician then brings the hands together in a wringing type of motion, moving segmentally up the chest. This procedure, however, is contraindicated in the presence of rib fractures (Wetzel, 1985). Intervention 12-1 illustrates a clinician performing this technique. Intervention 12-1 Chest Wall Stretching A. Starting position for manual chest stretching with one hand under the patient’s ribs and the other on top of the patient's ribs. B. Ending position of the clinician’s hands after applying a wringing motion to the patient’s chest for manual stretching. C. The last hand position after the clinician progresses up the patient’s chest for manual chest stretching with the clinician’s top hand just inferior to the patient’s clavicle. (From Adkins HV, editor: Spinal cord injury, New York, 1985, Churchill Livingstone.) Postural Drainage Postural drainage with percussion and vibration may be necessary to aid in clearing secretions. Many facilities employ respiratory therapists who are responsible for these activities. However, the PT or PTA may be the health-care provider responsible for the patient’s bronchial hygiene (removal of secretions). Postural drainage positions are outlined in Chapter 8. Physical therapy plays an important role in teaching the patient assisted cough techniques. For patients who lack abdominal innervation, it is imperative to identify ways in which the patient can expel secretions. If the patient is unable to perform these assistive cough techniques independently, a caregiver or a family member should be instructed in the technique. These techniques are discussed in the next section. Maintaining good bronchial hygiene assists in the prevention of secondary complications, such as pneumonia. Coughs Coughs are classified into three different categories, based on the amount of force the individual is able to generate. Functional coughs are those that are strong enough to clear secretions. Weak functional coughs produce an adequate amount of force to clear the upper airways. Nonfunctional coughs are ineffective in clearing the airways of bronchial secretions (Wetzel, 1985). 751 Assisted Cough Techniques Several methods are available to assist patients with the ability to cough. Depending on the patient’s medical status, these techniques can be initiated in the acute-care setting or during the early phases of rehabilitation. Technique 1 The patient inhales two or three times and, on the second or third inhalation, attempts to cough. Intrathoracic pressure increases which allows the patient to generate a greater force to expel secretions. Technique 2 The patient places his or her forearms over the abdomen. As the patient tries to cough, the patient pulls downward with the upper extremities to assist with force production. This can be completed in either a supine or a sitting position. This technique can also be modified by having the patient fall toward his or her knees as he or she attempts to cough. This is illustrated in Intervention 12-2, A. Intervention 12-2 Assistive Cough Techniques foe Cc A. Self-manual coughing by the patient.* B. Assisted cough technique in long sitting.* C. Assistive cough technique administered by the therapist.* __—_—_—_—————————————————| t (From Sisto SA, Druin E, Sliwinski MM: Spinal cord injury: management and rehabilitation, St Louis, 2009, Mosby.) + (From Adkins HV, editor: Spinal cord injury, New York, 1985, Churchill Livingstone.) Ina prone-on-elbows position, the patient raises his or her shoulders, extends his or her neck, and inhales. As the patient coughs, the patient flexes the neck downward and leans onto the elbows. 750 Technique 4 If the patient is unable to master any of the previously mentioned assistive cough techniques, a caregiver can assist the patient with secretion expulsion. A modified Heimlich maneuver can be performed by placing the caregiver’s hands on the patient’s abdomen just below the rib cage and providing resistance in a downward-and-upward direction as the patient coughs (Intervention 12-2, B). Range of Motion Range-of-motion exercises are an important component of the early stage of rehabilitation. For patients with tetraplegia, stretching of the shoulders, elbows, wrists, and fingers is essential. Patients immobilized in a halo will be limited in their ability to perform active or passive range of motion of the shoulder. The halo vest sits over the patient’s shoulders, thus limiting shoulder flexion and abduction to approximately 90 degrees. The following shoulder ranges of motion are necessary to maximize function in the patient with tetraplegia. Approximately 60 degrees of shoulder extension and 90 degrees of shoulder external rotation are desirable. The patient needs shoulder extension to perform transfers from supine to the long-sitting position. External rotation of the shoulder is needed so the patient can perform the elbow-locking maneuver to assume a sitting position. Full elbow extension is essential to ensure that the patient is able to use elbow locking for the long-sitting position and for transfers. Patients who lack innervation of the triceps (patients with C5 and C6 tetraplegia) use the elbow-locking mechanism to improve their functional potentials. Adequate forearm pronation is necessary for feeding. Patients who lack finger function need 90 degrees of wrist extension. When an individual extends the wrist, passive insufficiency causes a subsequent flexing of the finger flexors referred to as tenodesis (Figure 12-8). Tenodesis can be used functionally to allow a patient to grip objects with built-up handles using passive or active wrist extension. As a result of this functional movement, stretching of the extrinsic finger flexors in combination with wrist extension should be avoided. If the finger flexors become overstretched, the patient will lose the ability to achieve a tenodesis grasp. Sitting on the mat with an open hand will overstretch the finger flexors. The patient should be encouraged to maintain the proximal interphalangeal joints and the distal interphalangeal joints in flexion. Overstretching of the thumb web space should also be avoided, because tightness in the thumb adductors and flexors allows the thumb to oppose the first and second fingers during tenodesis. Patients are then able to use the thumb as a hook for functional activities. FIGURE 12-8 Fundamental principle of tetraplegia hand function. A, With gravity-assisted wrist flexion the fingers and thumb passively open for grasp. B, With volitional wrist extension, the thumb and fingers passively close for grasp. The tenodesis hand function provides sufficient force for light objects. Once the halo is removed, clinicians should also avoid overstretching the cervical extensors. Stretching of the cervical extensors predisposes one to forward head posturing. This head position interferes with the patient’s sitting balance and can limit the patient’s respiratory capabilities by inhibiting the use of accessory muscles. Passive Range of Motion Passive range of motion must be performed to the lower extremities when they are paralyzed. Special attention must be given to the hamstrings. The desired amount of passive hamstring 754 flexibility needed to maintain a long-sitting position and to dress the lower extremities is 110 degrees, although the amount of hamstring range required depends on the length of the patient’s upper and lower extremities. When stretching the lower extremities, the PTA should make sure that the patient’s pelvis is stabilized so movement is from the hamstrings and not from the low back. Some tightness in the low-back musculature is desirable because this assists the patient with rolling, transfers, and maintenance of sitting positions. Tightness in the low back provides the patient with a certain degree of passive trunk stability. In addition, maintenance of a “tight” back and the presence of adequate hamstring flexibility prevents the patient from developing a posterior pelvic tilt that can lead to sacral sitting and pressure problems when sitting in the wheelchair. Stretching of the hip extensors, flexors, and rotators is necessary because gravity and increased tone may predispose patients to contractures. Hip flexion range of 100 degrees is needed to perform transfers into and out of the wheelchair. The patient needs 45 degrees of hip external rotation for dressing the lower extremities. Early in rehabilitation, it may not be possible to position the patient in prone to stretch the hip flexors because of respiratory compromise. The prone position can inhibit the diaphragm’s ability to work. However, as soon as the patient can safely maintain this position, it should be initiated. Stretching of the ankle plantar flexors is necessary to provide passive stability of the feet during transfers, to allow proper positioning of the feet on the wheelchair footrests, and to allow the use of orthoses if the patient will be ambulatory. Table 12-6 provides a review of passive range-of-motion requirements. Table 12-6 Range-of-Motion Requirements Movement Range Needed Shoulder external rotation | 90° Forearm pronation Full forearm pronation Wrist extension 90° Hip extension 10° 45° Hip external rotation Passive straight leg raising] 110° Knee extension Full knee extension Ankle dorsiflexion To neutral Caution If the patient’s cervical spine is unstable, passive range-of-motion exercises to the shoulders should be limited to 90 degrees of flexion and abduction to avoid possible movement of the cervical vertebrae. Instability in the lumbar spine requires that passive hip flexion be limited to 90 degrees with knee flexion and 60 degrees with the knees straight (Somers, 2010). Passive straight leg raising should be limited to ranges which do not produce movement (lifting of the pelvis). Once the spine is stabilized, more aggressive range-of-motion exercises can begin. Strengthening Exercises Strengthening exercises are another essential component of the patient’s rehabilitation. During the acute phase, certain muscles must be strengthened cautiously to avoid stress at the fracture site and possible fatigue. Initially, muscles may need to be exercised in a gravity-neutralized (antigravity) position secondary to weakness. Intervention 12-3, A and B, illustrates triceps strengthening in a gravity-neutralized position. Application of resistance may be contraindicated in the muscles of the scapulae and shoulders in patients with tetraplegia and in the muscles of the hips and trunk in patients with paraplegia, depending on the stability of the fracture site. When the PT is designing the patient’s plan of care, exercises that incorporate bilateral upper extremity movements are beneficial. For example, bilateral upper extremity exercises performed in a straight plane or in proprioceptive neuromuscular facilitation patterns offer the patient many advantages. These types of exercises are often more efficiently performed and reduce the asymmetric forces applied to the spine during upper extremity exercises. Key muscles to be strengthened for patients with tetraplegia include the anterior deltoids, shoulder extensors, and biceps. Key muscles to be emphasized for patients with paraplegia include shoulder depressors, triceps, and latissimus dorsi. 759 Intervention 12-3 Triceps and Upper Extremity Strengthening A and B. Triceps strengthening performed in the gravity-neutralized position. The patient’s forearm must be carefully guarded. Weakness in the upper extremity may cause the patient’s hand to flex toward her face. C. Using a Velcro weight for additional resistance during triceps strengthening. D. Using an elastic band for biceps strengthening. During this early stage of rehabilitation, the PTA may use manual resistance as the primary means of strengthening weakened muscles. In addition, Velcro weights or elastic bands may be used (Intervention 12-3, C and D). As the patient progresses, these items may be left at the patient’s bedside to allow the patient the opportunity to exercise at other times during the day. If you do decide to leave one of these items for the patient, make sure that the patient can apply the device independently. Often, when a patient has decreased hand function, applying one of these devices can be difficult. Fairly rigorous upper extremity exercises can be performed by patients with paraplegia. Barbells, exercise equipment, free weights, and elastic bands can be used for resistive exercise. Acclimation to Upright In addition to passive stretching and strengthening exercises, the patient should also begin sitting activities. Because of the initial trauma and secondary medical conditions, the patient may have been immobilized in a supine position for several days or weeks. As a consequence, the patient may experience orthostatic hypotension. Initially, nursing and physical therapy can work on raising the head of the patient’s bed. One should monitor the patient’s vital signs during the performance of upright activities. Baseline pulse, blood pressure, and respiration rates should be recorded. As stated previously, as long as the patient’s blood pressure does not drop below 80/50 mm Hg, kidney perfusion is adequate (Finkbeiner and Russo, 1990). If the patient can tolerate sitting with the head 756 of the bed elevated, the patient can be progressed to sitting in a reclining wheelchair with elevating leg rests. Often, the patient is transferred to the wheelchair with a draw sheet or mechanical lift initially. Transfers into and out of hospital beds are often difficult, based on the height of the bed and the presence of a halo. As the patient is better able to tolerate sitting, the time and degree of elevation can be increased. The tilt table can also be used to acclimate the patient to the upright position (Figure 12-9). FIGURE 12-9 The tilt table is used to help a patient gradually build up tolerance to the upright position. (From Fairchild SL: Pierson and Fairchild’s principles and techniques of patient care, ed 5. St. Louis, 2013, Elsevier.) Weight bearing on the lower extremities has many therapeutic benefits, including reducing the effects of osteoporosis, assisting with bowel and bladder function, and decreasing abnormal muscle tone that may be present. To assist the patient with blood pressure regulation during any of these upright activities, it may be necessary to have the patient wear an abdominal binder, elastic stockings, or elastic wraps. The abdominal binder helps support the abdominal contents during upright activities by minimizing the effects of gravity. The top of the binder should cover the two lowest ribs, and the bottom portion should be placed over the patient’s anterior superior iliac spines. The binder should be tighter more distally. Elastic wraps or elastic stockings assist the lower extremities with venous return in the absence of skeletal muscle action in the lower extremities. The patient should also be carefully monitored for possible autonomic dysreflexia during these early attempts at upright positioning. 7o/ 758 Physical therapy interventions during inpatient rehabilitation Once the patient is medically stable, the patient will likely be transferred to a comprehensive rehabilitation center. Most patients spend approximately 11 days in an acute care center. During the inpatient rehabilitation phase of the patient’s recovery, the emphasis is on maximizing functional potential. The average length of stay for inpatient rehabilitation is approximately 36 days (National Spinal Cord Injury Statistical Center, 2013). Activities that were initiated during the acute phase of recovery continue. Interventions should focus on maximizing respiratory function, range of motion, positioning, and strength of innervated muscles. Additional interventions are incorporated to assist the patient in the development of motor control, acquisition of self-care and functional activities including gait (if appropriate), therapeutic exercises to improve flexibility and overall fitness, patient and family education and training, and recommendations for equipment. Physical Therapy Goals The goals of intervention at this stage are many and variable. Much depends on the patient's level of innervation and resultant muscle capabilities. Examples of goals for this stage of the patient's recovery include the following: Increased strength of key muscle groups Independence in skin inspection and pressure relief Increased passive range of motion of the hamstrings and shoulder extensors Increased vital capacity Increased tolerance to upright positioning in bed and the wheelchair Independence in transfers or independence directing a caregiver Independence in bed and mat mobility or independence directing a caregiver Independence in wheelchair propulsion on level surfaces Independence in the operation of a motor vehicle (if appropriate) 10. Return to home and school or work 11. Independence in a home exercise and fitness program 12. Patient and family education and instruction Goals regarding ambulation may be appropriate, depending on the patient’s motivation and motor level and the philosophy of the clinic and rehabilitation team. 3000: SO Ie GN Development of the Plan of Care The primary PT is responsible for developing the patient’s plan of care. The treatment interventions selected to achieve patient goals can be separated into two different approaches: compensatory and restorative. The compensatory approach is guided by the premise that the patient will learn new motor skills through the use of compensatory strategies including strengthening intact muscles; using muscle substitution, momentum, and principles, such as the head-hips relationship; and the incorporation of adaptive equipment and environmental modifications. Patients that are classified as AIS A or B (voluntary motor function is absent below the injury site) must utilize a compensatory approach to achieve functional skills. When using the restorative approach to SCI rehabilitation, the focus is on the patient's ability to use normal movement patterns in the acquisiton of functional skills. Relearning previous motor skills and limiting the use of compensatory strategies form the basis of the restorative approach. Functional gains can be achieved through the incorporation of either approach exclusively or in combination (Somers and Bruce, 2014; Somers, 2010). In addition to mastery of functional skills, the PT will want to promote certain behaviors in the patient. Patients who have sustained SCIs must become active problem solvers. The patient needs to determine how to move using his or her remaining innervated muscles. The patient also needs to know what to do in emergency situations. For example, the patient must be able to direct someone if he or she should fall out of the wheelchair and is unable to transfer back into it. During the treatment session, tasks should be broken down into component parts, and the PTA should allow the patient to find solutions to the patient’s movement problems. Patients should practice the activity in its entirety but must also work on the steps leading up to the completed activity. An 799 example is practicing the transition from a supine-on-elbows position to long sitting. Patients should also be taught to work in reverse. Once the patient has achieved the desired end position, the patient should practice moving out of that position and back to the start posture. Patients who have sustained SCIs should experience success during rehabilitation. Activities to be selected should provide the patient with the opportunity to succeed. These tasks should be interspersed with activities that are challenging and difficult. Treatment activities selected should help the patient to develop a balance of skills between different postures and stages of motor control. The patient does not need to perfect movement in one postural set before attempting something more challenging. Finally, interventions within the plan of care should be varied. Examples of some of the different components of the patient’s treatment plan that are possible include pool therapy, mat programs, functional mobility activities, group activities, and strengthening exercises. Early Treatment Interventions Mat Activities Early in treatment, the patient should work on rolling. Learning to do this independently can assist with the prevention of pressure ulcers. As the patient practices rolling, the PTA can also work on the patient’s achievement of the prone position. As stated previously, prone is an excellent position for pressure relief and stretching hip flexors. If the patient is wearing a halo, it will often be necessary for the PTA to help the patient with rolling. Prepositioning a wedge under the patient's chest is desirable when the patient is prone. If the patient does not have a halo, rolling can be facilitated in the following way: Step 1. The patient should flex the head and neck and rotate the head from right to left. Step 2. With both upper extremities extended above the head (in approximately 90 degrees of shoulder flexion), the patient should move the upper extremities together from side to side. Step 3. With momentum and on the count of three, the patient should flex and turn the head in the direction he or she wishes to roll while moving the arms in the same direction. Step 4. To make it easier for the patient, the patient’s ankles can be crossed at the start of the activity. This prepositioning allows the patient’s lower extremities to move more easily. To roll to the left, you would cross the patient's right ankle over the left. Intervention 12-4 illustrates a patient who is completing the rolling sequence. Cuff weights applied to the patient’s wrists can add momentum and can facilitate rolling. Intervention 12-4 Rolling from Supine to Prone 760 c A. Rolling from supine to prone can be facilitated by having the patient flex her head and use upper extremity horizontal adduction for momentum. The patient’s lower extremities should be crossed to unweight the hip to assist with rolling. B and C. With momentum and on the count of three, the patient should flex and turn her head in the direction she wishes to roll while throwing her arms in the same direction. Once the patient has rolled from supine to prone, strengthening exercises for the scapular muscles can also be performed. Shoulder extension, shoulder adduction, and shoulder depression with adduction are three common exercises that can be performed to strengthen the scapular stabilizers. Intervention 12-5 shows a patient performing these types of exercises. Intervention 12-5 Scapular Strengthening Scapular-strengthening exercises can be performed in a prone position. Prone 761 From the prone position, the patient can attempt to assume a prone-on-elbows position. Prone on elbows is a beneficial position because it facilitates head and neck control, as well as requiring proximal stability of the glenohumeral joint and scapular muscles. For the patient to attain the prone-on-elbows position, the PTA may need to help. The PTA can place his or her hands under the patient’s shoulders anteriorly and lift them (Intervention 12-6, A). As the patient's chest is lifted, the PTA should move his or her hands posteriorly to the patient’s shoulder or scapular region. If the patient is to attempt achievement of the position independently, the patient should be instructed to place his or her elbows close to the trunk, hands near his or her shoulders. The patient is then instructed to push the elbows down into the mat while lifting his or her head and upper trunk. To position the elbows under the shoulders, the patient needs to shift weight from one side to the other to move the elbows into correct alignment. This is accomplished by movement of the head to the right or the left. The PTA can facilitate weight shifts in the appropriate direction during these activities (Intervention 12-6, B). Intervention 12-6 Prone to Prone on Elbows 762 B = A. The assistant may need to help the patient achieve the prone on elbows position. B. Weight shifting from one side to the other allows the patient to move her elbows into correct alignment. Prone on Elbows Before beginning activities in the prone-on-elbows position, the patient needs to assume the correct alignment, as shown in Figure 12-10. The patient should also try to keep the scapulae slightly adducted and downwardly rotated to counteract the natural tendency to hang on the shoulder ligaments. The PTA may need to provide the patient with manual cues on the scapulae to maintain the correct position. Downward approximation applied through the shoulders or tapping to the rhomboids is often necessary to increase scapular stability. Approximation promotes tonic holding of the muscles. In the prone-on-elbows position, the patient should practice weight shifting to the right, left, forward, and backward. The patient should be encouraged to maintain good alignment and to avoid shoulder sagging as he or she performs exercises in this position. 763 FIGURE 12-10 The elbows should be positioned directly under the shoulders when the patient is in prone on elbows. The physical therapist assistant is applying a downward force (approximation) through the shoulder to promote tonic holding and stabilization of the shoulder musculature. Once the patient can maintain the position, he or she can progress to other exercises that will increase proximal control and stability. Alternating isometrics and rhythmic stabilization can be performed. To perform alternating isometrics, the patient should be instructed to hold the desired position as the PTA applies manual resistance to the right or left, forward or backward. Intervention 12-7, A, illustrates this exercise. With rhythmic stabilization, the patient performs simultaneous isometric contractions of agonist and antagonist patterns as the therapist provides a rotational force. Intervention 12-7, B, shows a PTA who is performing this activity with a patient. Other activities that can be performed in a prone-on-elbows position include lifting one arm, unilateral reaching activities, and serratus strengthening (Intervention 12-8, A). To strengthen the serratus, the patient is instructed to push her elbows down into the mat and to tuck the chin while lifting and rounding the shoulders. For patients with paraplegia, the PTA can provide instruction on prone push-ups, as depicted in Intervention 12-8, B. Intervention 12-7 Alternating Isometrics and Rhythmic Stabilization 764 A. The physical therapist assistant is performing alternating isometrics with the patient in a prone- on-elbows position. Force is being applied in a posterior direction as the patient is asked to hold the position. B. Rhythmic stabilization performed in a prone-on-elbows position. The physical therapist assistant is applying simultaneous isometric contractions to both agonists and antagonists. As the patient holds the position, a gradual counterrotational force is applied. Intervention 12-8 Other Scapular-Strengthening Exercises A. The patient reaches for a functional object. The physical therapist assistant stabilizes the weight- bearing shoulder to prevent collapse. B. The patient with paraplegia performs a prone press-up. Prone to Supine From a prone-on-elbows position, the patient can transition back to supine. The patient shifts weight onto one elbow and extends and rotates his or her head in the same direction. As he or she does this, the patient “throws” the unweighted upper extremity behind. The momentum created by this maneuver facilitates rolling back to a supine position. Supine on Elbows The purpose of the supine-on-elbows position is to assist the patient with bed mobility and to prepare him or her for the attainment of long sitting. Patients with innervation at the C5 and C6 7695 levels may need assistance to achieve the supine on elbows position. Intervention 12-9 depicts a PTA helping a patient make the transition from supine to the supine-on-elbows position. Several different techniques can be used to assist the patient in learning to achieve this position. A pillow or bolster placed under the upper back can assist the patient with this activity. This technique helps acclimate the patient to the position and assists the patient with stretching the anterior shoulder capsule. As the patient is able to assume more independence with the transition from a supine position to supine on elbows, the PTA can have the patient hook his or her thumbs into his or her pockets or belt loops or position the hands under the buttocks. Intervention 12-10 illustrates this approach. As the patient does this, he or she stabilizes with one arm as he or she pulls back with the other, using the reverse action of the biceps. The PT or PTA may need to position the patient’s arms at the end of the movement. Once the patient is in the supine-on-elbows position, work can begin on strengthening the shoulder extensors and scapular adductors. Activities to accomplish this include weight shifting in the position, transitioning back to prone, and progressing to long sitting. Supine pull-ups can also be practiced. While the patient is in a supine position, the PTA holds the patient’s supinated forearms in front of the body and has the patient pull up into a modified sit-up position. This exercise helps strengthen both the shoulder flexors and the biceps. From supine on elbows, the patient can roll to prone by shifting weight onto one elbow, looking in the same direction, and reaching across the body with the other upper extremity. This maneuver provides the patient with another option to achieve the prone position. Intervention 12-9 Supine to Supine on Elbows A. The patient flexes her head to initiate the activity. B. With her hands on the patient’s shoulders, the physical therapist assistant helps to lift the patient’s upper trunk. 766 C. The head is used to initiate a weight shift to the right so that the left elbow can be unweighted and brought back. D. The final position. Intervention 12-10 Independent Supine to Supine on Elbows A. The patient prepositions her hands under her buttocks. B. The patient flexes her neck. Cand D. Using her head to initiate the weight shift, the patient pulls her elbows back. E. The final position. Long Sitting Long sitting can also be achieved from a supine-on-elbows position. Long sitting is sitting with both upper and lower extremities extended and is a functional posture for patients with tetraplegia. This position allows patients with C7 innervation a position in which they can perform lower extremity dressing, skin inspection, and self-range of motion. It may be necessary for the assistant to help the patient achieve the position initially. The technique to assume long sitting is as follows: Step 1. In the supine-on-elbows position, the patient shifts her weight to one side. The patient’s head should follow the movement (Intervention 12-11, A and B). 767 Intervention 12-11 Supine on Elbows to the Long-Sitting Position A and B. In supine on elbows, the patient shifts her weight to one side by moving the head in that direction. C. With her weight on one elbow, the patient throws her other upper extremity behind her buttocks into extension and external rotation. D. Once the weight is shifted onto the extremity, the elbow is biomechanically locked into extension because of the bony alignment of the joint when it is positioned in shoulder external rotation and then depressed. E. The patient shifts her weight back to the midline. 768 F. Once the patient has the elbow locked on one side, she repeats the motion with the other upper extremity. G. The final position. Step 2. With the weight on one elbow, the patient throws her other upper extremity behind the buttocks into shoulder extension and external rotation (Intervention 12-11, C). Once the hand makes contact with the surface, the shoulder is quickly elevated and then depressed to maintain the elbow in extension. The elbow is locked biomechanically (Intervention 12-11, D and E). Step 3. The patient shifts her weight back to the midline (Intervention 12-11, E). Step 4. Once the patient has the elbow locked on one side, she repeats the motion with the other upper extremity (Intervention 12-11, F and G). Special note The fingers should be maintained in flexion (tenodesis) during performance of functional activities to avoid overstretching the finger flexors. This is illustrated in Intervention 12-11, F and G. Initially, the PTA may need to help the patient with the movement and placement of the upper extremities. Patients who lack the necessary range of motion in their shoulders have difficulty in performing this maneuver. As mentioned earlier, patients who have developed elbow flexion contractures are not able to achieve and maintain this position because of their inability to extend their elbows passively. Patients who do not possess at least 90 to 100 degrees of passive straight leg raising should refrain from performing long-sitting activities. Failure to possess adequate hamstring range of motion causes patients to overstretch the low back and ultimately decrease their functional abilities. Patients with injuries at C7 and below also use the long-sitting position. However, it is easier for these patients because they possess triceps innervation and may be able to maintain active elbow extension. Once the patient has achieved the long-sitting position with the elbows anatomically locked and is comfortable in the position, additional treatment activities can be practiced. Manual resistance can be applied to the shoulders to foster cocontraction around the shoulder joint and to promote scapular stability. Rhythmic stabilization and alternating isometrics are also useful to improve stability. If the patient has triceps innervation, the PTA will want to work with the patient on the ability eventually to sit in a long-sitting position without upper extremity support (Figure 12- 11). The patient moves his or her hands from behind the hips, to the hips, and finally to forward at the knees. Hamstring range is essential for the patient to be able to perform this transition safely. Once the patient can place his or her hands in front of the hips and close to the knees, he or she can try maintaining the position with only one hand for support and eventually with no hands. In this position, the patient learns to perform self-range of motion and self-care activities. The PTA guards the patient carefully during the performance of this activity. In addition, the patient's vital signs should be monitored to minimize the possibility of orthostatic hypotension or autonomic dysreflexia. 769 Peni FIGURE 12-11 Balance activities should always be emphasized in the long-sitting position to prepare the patient for numerous functional activities. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) A goal for the patient with triceps function is to do a push-up with the upper extremities in a long-sitting position (Intervention 12-12). This activity usually requires that the patient have at least fair-plus strength in the triceps. To complete the movement, the patient straightens the elbows and depresses the shoulders to lift the buttocks. The patient flexes the head and upper trunk to facilitate a greater rise of the buttocks. Tightness in the low back also allows this to occur. The patient uses this technique (the head-hips relationship) to move around on the mat. This relationship is a compensatory strategy that patients use to complete functional activities. This phenomenon is illustrated when a patient moves the head in one direction and the hips move directly opposite (Somers, 2010). Upper extremity push-ups are also used for transfers in and out of the wheelchair and as a means for the patient to perform independent pressure relief. Intervention 12-12 Push-Up in the Long-Sitting Position The patient uses the head-hips relationship to assist with lifting the buttocks. 770 Transfers Transfers into and out of the wheelchair are an important skill for the patient with a SCI. Patients with high cervical injuries (C1 through C4 level) are completely dependent in their transfers. A two- person lift, a dependent sit-pivot transfer, or a Hoyer lift must be used. Preparation Phase Before the transfer, the patient and the wheelchair must be positioned in the correct place. The wheelchair should be positioned parallel to the mat or the bed. The brakes must be locked and the wheelchair leg rests removed. A gait belt must be applied to the patient before the PTA begins the activity. Two-Person Lift A two-person lift may be necessary for the patient with high tetraplegia. This type of transfer is illustrated in Intervention 12-13. Intervention 12-13 Two-Person Lift Care must be taken so that the patient’s buttocks clear the wheel during the two-person lift. Good body mechanics are equally important for the individuals assisting with this type of transfer. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) Sit-Pivot Transfer The technique for a dependent sit-pivot transfer is as follows: Step 1. The patient must be forward in the wheelchair to perform the transfer safely. The PTA shifts the patient’s weight from side to side to move the patient forward. Often, placing one’s hands under the patient’s buttocks in the area of the ischial tuberosities is the best way to assist the patient with weight shifting. The PTA must monitor the position of the patient’s trunk carefully as he or she performs this maneuver because the patient does not possess adequate trunk control to maintain the trunk upright. Once the patient is forward in the wheelchair, the armrest closest to the mat or bed should be removed. Step 2. The PTA then flexes the patient’s trunk over the patient’s feet. The PTA brings the patient 771 forward over his or her hip that is farther away from the wheelchair. This maneuver allows the PTA to be close to the area where most individuals carry the greatest amount of body weight. The PTA also guards the patient’s knees between his or her knees. Step 3. A second person should be positioned on the mat table or behind the patient to assist with moving the patient’s posterior hips and trunk. Step 4. On a specified count, the PTA positioned in front of the patient shifts the patient’s weight forward and moves the patient’s hips and buttocks to the transfer surface. The position of the patient’s feet must also be monitored to avoid possible injury. Generally, prepositioning the feet in the direction that the patient will assume at the end of the transfer is beneficial. Step 5. Once the patient is on the mat, the PTA who is in front of the patient aligns the patient to an upright position. The assistant does not, however, take his or her hands off the patient because of the patient’s lack of trunk control. Without necessary physical assistance, a patient with tetraplegia could lose balance and fall. Intervention 12-14 shows a PTA performing a sit-pivot transfer with a patient. Intervention 12-14 Sit-Pivot Transfer 772 A. The physical therapist assistant helps the patient to scoot forward in the wheelchair. B. The patient is flexed forward over the physical therapist assistant’s hip. C. The patient’s hips and buttocks are moved to the transfer surface. Modified Stand-Pivot Transfer A modified stand-pivot transfer can also be used with some patients who have incomplete injuries and lower extremity innervation. Additionally, patients with lower extremity extensor tone may be able to perform a modified stand-pivot transfer. The steps in completion of this transfer are similar to the ones described earlier and the techniques discussed in Chapter 10. Intervention 12-15 illustrates this type of transfer. Intervention 12-15 Modified Stand-Pivot Transfer 773 Leverage principles and good body mechanics facilitate this stand-pivot transfer. The patient may assist with this transfer by holding her arms around the person who is completing the transfer. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) Airlift The airlift transfer is depicted in Intervention 12-16 and may be the preferred type of transfer for patients with significant lower extremity extensor tone. The patient's legs are flexed and rest on the clinician’s thighs. The patient is then rocked out of the wheelchair and moved to the transfer surface. The therapist must maintain proper body mechanics and lift with her legs to avoid possible injury to the low back. This type of transfer is often preferred because it prevents shear forces on the buttocks. Intervention 12-16 Airlift Transfer 774 In the airlift transfer, the patient’s flexed legs rest on or between the therapist's thighs. The patient can be “rocked” out of the chair and lifted onto the bed or mat. The patient’s weight is carried through the therapist’s legs and not the back. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) A sliding board can also be used to assist with transfers. The chair should be prepositioned as close as possible to the transfer surface and at approximately a 30-degree angle. As the patient’s trunk is flexed forward over his or her knees, the PTA can place the sliding board under the patient’s hip that is closer to the mat table. The PTA may need to lift up the patient’s buttocks to assist with board placement. Clinicians must be aware of the patient's active trunk control. Many of these individuals are not able to maintain their trunks in an upright position. Once the board is in the proper position, it helps support the patient’s body weight during the transfer. The board also provides the patient’s skin some protection during the transfer. The patient’s buttocks may be bumped or scraped on various wheelchair parts. This can be dangerous to the patient and can lead 779 to skin breakdown. Intervention 12-17 illustrates a patient who is performing a sliding board transfer with the help of the PTA. Intervention 12-17 Sliding Board Transfer A. The patient's weight is shifted to the side farther away from the transfer surface. B. The patient’s thigh is lifted to position the board. The physical therapist assistant remains in front of the patient, blocking the patient’s lower extremities and trunk. Cand D. The patient is transferred over to the support surface. Special note Although patients with high cervical injuries are not able to physically assist in the transfer, the patient must be able to verbally direct caregivers in the completion of the task. A patient with C6 tetraplegia has the potential to transfer independently using a sliding board. 776 Although the patient has the potential for this type of independence, patients with C6 tetraplegia often use the assistance of a caregiver or a family member because of the time and energy involved with transfers. To be independent with sliding board transfers from the wheelchair, the patient must be able to manipulate the wheelchair parts and position the sliding board. Extensions applied to the wheelchair’s brakes are common and allow the patient to use wrist movements to maneuver these wheelchair parts. Leg rests and armrests may also be equipped with these extensions to provide the patient with a mechanism to negotiate these wheelchair parts independently. In an effort to prevent the development of upper extremity overuse injuries, patients should be instructed to limit the numbers of transfers they perform each day and avoid extremes of joint range (Somers, 2010). To position the board, the patient can use tightness in the finger flexors to move the board to the proper location. The patient can also place his or her wrist at the end of the board and use wrist extension to move the board to the right place. Placement of the sliding board under the buttocks can be facilitated by lifting the leg up. Loops can be sewn onto the patient’s pants to make this easier. Once the board is in position, the patient can reposition the lower extremities (Intervention 12-18). Intervention 12-18 Independent Sliding Board Transfer 777 A. and B. The patient prepares to position the sliding board by moving the leg closest to the mat table over the other leg. C. The patient positions the sliding board under the buttock of the leg closest to the mat table. D. Pushing with the forearm closest to the wheelchair armrest and pushing down against the sliding board, the patient lifts herself off of the wheelchair seat. 778 E. The patient then slides her buttocks down the length of the board until she is on the table. F. Continuing to push off the wheelchair arm and using the other arm on the mat table, the patient scoots off the board and onto the table itself. Several different transfer techniques can be used for the patient with C6 tetraplegia. When working with a patient at this level, one must find the easiest method of transfer for the individual. Trial and error and having the patient engage in active problem solving to complete movement tasks are best. Too often, PTs and PTAs provide patients with all the answers to their movement questions. If a patient is allowed to experiment and try some things on his or her own with supervision, the results are often better. Prone-on-Elbows Transfer The modified prone-on-elbows transfer is one method the patient may employ. The patient with C6 tetraplegia rotates his or her head and trunk to the opposite direction of the transfer while still in the wheelchair. Once the patient is in this position, he or she flexes both elbows and places them on the wheelchair armrest. The patient then flexes his or her trunk forward and pushes down on the upper extremities, thus scooting over onto the mat or bed. Some patients may also use the head to assist with the transfer. The patient can place her forehead on the armrest to provide additional trunk stability while attempting to move from the wheelchair. Once the patient is on the mat table, he or she hooks the arm under the knee and uses the sternal fibers of the pectoralis major to extend the trunk. Rolling Out of the Wheelchair After removing the wheelchair armrest, the patient rotates the trunk to the mat table. The patient then positions the lower extremities onto the support surface. The patient can use the back of his or her hand or Velcro loops attached to his or her pants to lift the lower extremities up and onto the support surface. Once the patient’s lower extremities are up on the bed, the patient actually rolls out of the wheelchair. The patient can move to a side-lying position or can roll all the way over to a prone-on-elbows position. Lateral Push-Up Transfer If the patient possesses triceps function, the potential for independent transfers with and without the sliding board is greatly enhanced. As stated earlier, a patient with a C7 injury and good triceps strength should be able to perform a lateral push-up transfer without a sliding board. Initially, when instructing a patient in this type of transfer, the PTA should use a sliding board. The patient positions the board under the posterior thigh. With both upper extremities in a relatively extended position, the patient pushes down with his or her arms and lifts the buttocks up off the sliding board. The patient’s feet and lower extremities should be prepositioned before the start of the transfer. Both feet should be placed on the floor and rotated away from the direction of the transfer. The patient moves slowly, using the board as a place to rest if necessary. As the strength in the patient’s upper extremities improves, the patient will be able to complete the transfer faster and will not need to use the sliding board. Patients with high-level paraplegia also perform lateral push-up transfers. Not until a patient possesses fair strength in the lower extremities are stand-pivot transfers possible. Intermediate Treatment Interventions Mat Activities A major component of the patient’s plan of care at this stage of rehabilitation includes mat activities. Mat activities are chosen to assist the patient in increasing strength and in improving functional mobility skills. The functional mobility activities previously described, including rolling, supine to prone, supine to long sitting, and prone to supine, continue to be practiced until the patient masters the tasks. Other, more advanced mat activities are now discussed in more detail. Independent Self—Range of Motion A patient with C7 tetraplegia should also be instructed in self-range of motion to the lower 779 extremities. Assuming long sitting without upper extremity support is a prerequisite for becoming independent in self-range of motion. The first exercise that should be addressed is hamstring stretching. Two methods can be employed. The patient can assume a long-sitting position and then can lean forward toward the toes. The patient may rest the elbows on his or her knees to assist in keeping the lower extremities extended. The maintenance of a lumbar lordosis is important in preventing overstretching of the low-back musculature (Intervention 12-19). Intervention 12-19 Hamstring Stretching 780 A. When stretching the hamstrings in the long-sitting position, the patient may rest her elbows on her knees to assist in keeping the lower extremities straight. B to E. Stretching the hamstrings in the supine position. The second method entails having the patient place his or her hands under the knee and pull the knee back as he or she leans backward into a supine position. With one hand at the anterior knee and the other at the ankle, the patient raises the leg while trying to keep the knee as straight as possible. The patient can then pull the lower extremity closer to the chest to achieve a better stretch. If the patient does not possess adequate hand function to grasp, he or she can use the back of the wrist or forearm to complete the activity. Intervention 12-19 shows a patient who is performing hamstring stretching. The gluteus maximus should also be stretched. In a long-sitting position with one upper extremity used for balance, the patient places his or her free hand under the knee on the same side. The patient then pulls the knee up toward his or her chest and holds the position. Once the lower extremity is in the desired position, the patient can bring the volar surface of the forearm to the anterior shin and pull the leg closer. This maneuver gives an added stretch to the gluteus maximus (Intervention 12-20). Intervention 12-20 Gluteus Maximus Stretching 781 A. In the long-sitting position the patient uses one upper extremity for support and his free hand to pull the knee on the same side up toward his chest. B. Once the lower extremity is in position, the patient grasps the knee and shin with both hands and pulls the leg toward his trunk. Patients must also spend a portion of each day stretching their hip flexors. This is especially important for individuals who spend a majority of their day sitting. The most effective way to stretch the hip flexors is for patients to assume a prone position. Patients should be advised to lie prone for at least 20 to 30 minutes every day. Patients can do this in their beds or on the floor if they are able to transfer into and out of their wheelchairs. To stretch the hip abductors, adductors, and internal and external rotators, the patient should assume a long-sitting position as described earlier. The knee is brought up into a flexed position. With the nonsupporting hand, the patient should slowly move the lower extremity medially and laterally. The patient can maintain the arm under the knee or can place his or her hand on the medial or lateral surface of the knee to support the lower extremity (Intervention 12-21). Intervention 12-21 Stretching the Hip Rotators 782 A. Hip lateral rotation. B. Hip medial rotation. Stretching of the ankle plantar flexors is also necessary. The patient supports himself or herself with the same upper extremity as the foot he or she is stretching. With the knee flexed approximately 90 degrees, the patient places either the dorsal or volar surface of the opposite hand on the plantar surface of the foot. Placement of the hand depends on the amount of hand function the patient possesses. Patients with strong wrist extensors can use motion at the wrist to stretch the ankle into dorsiflexion slowly (Intervention 12-22). Patients with paraplegia who possess wrist and finger function are able to complete this activity without difficulty. Stretching the ankle plantar flexors with the knee flexed stretches only the soleus muscle. The patient can stretch the gastrocnemius in a long-sitting position with a folded towel placed along the plantar surface of the foot. The ends of the towel are pulled to provide a prolonged stretch. Intervention 12-22 Ankle Dorsiflexion 783 Ankle dorsiflexion. When completing this stretch, patients with C7 innervation will need to maintain one upper extremity in extension for trunk support. Advanced Treatment Interventions Advanced Mat Activities For the patient with paraplegia, practicing more advanced mat exercises is also appropriate. Ina short- or long-sitting position, the patient can practice maintaining his or her sitting balance and finding his or her center of balance and limits of stability. Use of the upper extremities to maintain sitting balance will be dependent on the patient’s motor level. Weight shifting, reaching, and other functional upper extremity tasks can be performed while the patient attempts to maintain his or her posture and balance. As the patient progresses, the therapist may choose to alter the surface. Other advanced mat activities that can be performed include sitting swing-through, hip swayers, trunk twisting and raising, prone push-ups, forward reaching in quadruped, creeping, and tall kneeling. The techniques used to execute each of these activities are as follows: Sitting Swing-Through: Step 1. The patient assumes a long-sitting position with upper extremity support. The patient’s hands should be approximately 6 inches behind the patient's hips. Step 2. The patient depresses the shoulders and extends the elbows. The buttocks should be lifted off the support surface. Step 3. The patient swings the hips back between his or her hands. Hip Swayer: Step 1. The patient assumes a long-sitting position with upper extremity support. Step 2. The patient places one hand as close to his or her hip as possible; the other hand should be placed approximately 6 inches away from the other hip. Step 3. The patient raises his or her buttocks and moves the hips toward the hand that is farther away. Step 4. The patient travels sideways across the mat. Step 5. The patient should practice moving in both directions. Trunk Twisting and Raising: Step 1. The patient assumes a side-sitting position. Step 2. The patient places both hands near the hip that is closer to the support surface. Step 3. The patient straightens his or her elbows to raise the hips to a semi-quadruped position and then lowers himself or herself to the mat. 784 Step 4. The activity should also be practiced on the opposite side. Prone Push-Ups: In a prone position with the hands positioned next to the shoulders, the patient extends the elbows and lifts the upper body off the support surface. Forward Reaching: Step 1. The patient assumes a four-point position. Some patients may need assistance achieving the position. This can be accomplished by having the patient assume the prone position and facilitating a posterior weight shift at the patient’s pelvis while the patient extends his or her elbows. Assistance may be needed. With a gait belt around the patient’s low waist or hips, the PTA, in a standing position, straddles the patient and pulls the patient’s hips up as the patient pushes with the upper extremities. Step 2. If the patient is having difficulty maintaining the four-point position, a bolster or other object can be placed under the patient’s abdomen to maintain the position. Care must be taken with patients who have increased lower extremity extensor tone; if the patient is unable to flex the hips and knees, the patient’s lower extremities can spasm into extension. Step 3. Once the patient can maintain the quadruped position, the patient can practice anterior, posterior, medial, and lateral weight shifts, as well as alternating isometrics and rhythmic stabilization. Step 4. The patient can also practice forward reaching with one upper extremity while maintaining balance. Step 5. If the patient possesses innervation of the trunk musculature, the patient can practice arching the back and letting it sag. Creeping: A patient’s ability to creep depends on lower extremity muscle innervation. Strength in the hip flexors is also needed to perform this activity. Step 1. The patient assumes a quadruped position. Step 2. The patient alternately advances one upper extremity followed by the opposite lower extremity. Tall Kneeling: Step 1. The patient assumes a quadruped position. Step 2. Using a chair, bench, or bolster, the patient pulls up into a tall-kneeling position. The hips must remain forward while the patient rests on the Y ligaments in the hips. Step 3. Initially, the patient works on maintaining balance in the position. Step 4. Once the patient can maintain balance, the patient can work on alternating isometrics, rhythmic stabilization, and reaching activities. Step 5. The patient can advance to kneeling-height crutches. The patient can balance in the position with the crutches, lift one crutch, advance both crutches forward, or pull both crutches back. The functional significance of these activities is widespread. The sitting swing-through, hip swayer, and prone push-up exercises work to improve upper extremity strength necessary for transfers and assisted ambulation. The trunk twisting exercise helps improve the patient’s trunk control for transfers, including those from the wheelchair to the floor. Unilateral reaching in the quadruped position assists the patient in developing upper extremity strength and coordination and improves the patient's ability to transfer from the floor into the wheelchair. Creeping on all fours helps develop the patient’s trunk and lower extremity muscle control. It is also a useful position for the patient to be able to assume while on the floor. Tall kneeling promotes the development of trunk control. It can be used as a position of transition for patients as they transfer from the floor back into their wheelchairs, and it serves as a preambulation activity. Stages of motor control (mobility, stability, controlled mobility, and skill) must also be considered when implementing these interventions. Transfers Wheelchair-to-Floor Transfers Patients with paraplegia should be instructed how to fall while in their wheelchairs and how to transfer back into the chair if, for some reason, they are displaced. In addition, the floor is a good place to perform hip-flexor stretching. In the clinic, the PT or PTA will initiate practice of this skill by lowering the patient to the floor as shown in Figure 12-12. The patient should be instructed to 785 tuck his or her head and to keep the arms in the wheelchair. The patient must be cautioned against trying to soften the fall by using the arms. Extension of the upper extremities can result in wrist fractures. The patient may also want to place one of his or her upper extremities over the knees to prevent the lower extremities from coming up and hitting the patient in the face. FIGURE 12-12 The physical therapist assistant lowers the patient to the floor. Once the patient is on the floor, he or she has several options for transferring back into the wheelchair. It may be easiest for the patient to right the wheelchair and then to transfer back into it. If the patient can position himself or herself in a supported kneeling position in front of the wheelchair, he or she can pull herself back into the wheelchair, as depicted in Intervention 12-23. If the patient possesses adequate upper extremity strength and range of motion, he or she can back up to the wheelchair in a long-sitting position, depress the shoulders, and lift the buttocks back into the wheelchair. The patient’s hands are positioned near the buttocks. Flexion of the neck while attempting this maneuver aids in elevating the buttocks through the head-hips relationship. Although this type of transfer is possible, many patients do not have adequate strength to complete the transition successfully. In the clinic, one can practice this by using a small step stool or several mats. In a long-sitting position, the patient transfers first to the step stool and then back up into the wheelchair. Intervention 12-24 illustrates a patient who is performing a transfer from the floor back into the wheelchair. The patient rotates the wheelchair casters forward and places one hand on the caster and the other on the wheelchair seat and pushes upward. Intervention 12-23 Transfer to Wheelchair from Tall Kneeling 786 The patient pulls herself into the wheelchair from a tall-kneeling position. The patient must rotate over her hips to assume a sitting position. The sequence can be reversed to transfer out of the wheelchair. Intervention 12-24 Transfer to Wheelchair from Long-Sitting Position 787 Transfers from the floor to the wheelchair can be practiced in the clinic with a small step stool. A to C. The patient first transfers from the floor to the stool. The patient uses the head-hips relationship to lift the buttocks. D and E. From the stool, the patient depresses her shoulders and lifts herself back into the wheelchair. Righting the Wheelchair Individuals with good upper body strength may be able to right a tipped chair while remaining in it. To be successful with this activity, the individual must be able to push down with the arm in contact with the floor, use the head and upper trunk to shift weight, and remember to push down on the hand in contact with the wheelchair instead of pulling on it. Intervention 12-25 shows an individual who is completing this activity. Intervention 12-25 Righting the Wheelchair While Seated 788 Some patients will be able to right their wheelchairs while they remain seated. Patients should be carefully guarded while they practice this skill. Caution A word of caution must be expressed during the performance of these activities. Patients who lack sensation in the lower extremities and buttocks must monitor the position of their lower extremities during activity performance. Patients can accidentally bump themselves on sharp wheelchair parts, and these injuries can cause skin tears during these activities. Although patients with tetraplegia cannot complete wheelchair to floor transfers independently, they should practice the task. These individuals must be able to instruct others in ways to assist should this situation occur in the community. Advanced Wheelchair Skills Patients with innervation and strength in the finger muscles should receive instruction in advanced wheelchair skills. Attaining wheelies and ascending and descending curbs should be taught so that the patient can be as independent in the community as possible. Wheelies Before the patient can learn to perform a wheelie independently, the patient must be able to find her balance point in a tipped wheelchair position (Figure 12-13). The easiest way to do this is to tip the 789 patient gently back onto the rear wheels. The PTA should find the point at which the wheelchair is most perfectly balanced. The patient must keep his or her back against the wheelchair back. The patient then grasps the hand rims. If the wheelchair begins to tip backward, the patient should be instructed to pull back slightly on the hand rims. If the front casters begin to fall forward, the patient should push forward on the handrims. Most patients initially overcompensate while learning to attain a balance point by leaning forward or pulling or pushing too much on the rims. . » 7 1 (a a? Me, - 4 #,. oy! as a =‘ hid FIGURE 12-13 Finding the balance point is a prerequisite to popping and maintaining a wheelie position. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) During these early stages of practice, you must guard the patient carefully. Standing behind the patient with your hands resting near the push handles of the wheelchair and standing near the backrest are the best places to guard the patient. Once the patient is able to maintain a wheelie with your assistance, the patient must learn to achieve the position independently. The patient must master this activity to negotiate curbs independently. To attain the wheelie position, have the patient lean forward in the wheelchair. The patient pulls back on the wheelchair rims and then quickly pushes forward at the same time he moves his or her shoulders posteriorly against the back of the wheelchair. The quick forward movement of the chair, combined with the shifting of the patient’s weight backward, causes the front casters of the wheelchair to pop up. With practice, the patient learns how much force is needed to attain the position. Eventually, the patient is able to achieve the wheelie position from a stationary or rolling position. Ascending Ramps A patient should ascend a ramp while in a forward position. The length and inclination must be considered before the patient attempts to negotiate any ramp. When the patient is going up a ramp, instruct him or her to lean forward in the wheelchair. If the ramp is long, the patient uses long, strong pushes on the hand rims. If the ramp is relatively short and steep, the patient uses short, quick pushes to accelerate forward. A grade aid on the wheelchair may be needed to prevent the chair from rolling backward between pushes. The grade aid serves as a type of braking mechanism to assist the patient to change hand position for the next push without rolling backward. 790 Descending Ramps Patients should be encouraged to descend ramps with their wheelchairs facing forward. The patient is instructed to lean back in the wheelchair. The patient then places both hands on the hand rims or on the rims and wheels themselves. The movement of the wheelchair is controlled by friction applied to the hand rims and wheels by the patient. The patient must let the rims move equally between both hands to guarantee that the wheelchair will move in a straight path. Patients may also elect to apply the wheelchair brakes partially when descending ramps. Although this technique provides added friction to the wheels, it can cause mechanical failure to the braking mechanism of the wheelchair. Ramps can also be descended with the patient in a backward position if the patient feels safer using this technique. The patient is instructed to line the wheelchair up evenly at the top of the ramp. The patient leans forward and grasps the hand rims near the brakes. The rims are then allowed to slide through the patient’s hands during the descent. Patients must be careful at the bottom of the ramp because the casters and footrests can catch on the ramp and cause the chair to tip backward. Figure 12-14 shows two methods for descending a ramp. A ta FIGURE 12-14 A, A person with good wheelchair mobility skills may be able to descend a ramp in a wheelie position. B, The safest method to descend a ramp is backward. The person must remember to lean forward while controlling the rear wheels. Ascending a ramp is performed in a similar manner. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins.) Ramps can also be ascended or descended in a diagonal or zigzag manner. Negotiating the ramp in a diagonal pattern decreases the tendency to roll down the ramp during ascent and decreases speed during descent. Ascending a Curb Going up a curb should always be performed with the patient in a forward direction. If the patient is going to be independent with this activity, he must be able to elevate the front casters of the wheelchair. As the patient approaches the curb, he or she pops the front casters up with a wheelie. Once the casters have cleared the curb, the patient leans forward and pushes on the hand rims. Patients require a great deal of practice to master this activity because the timing of the individual components is extremely important and the completion of the task takes considerable muscle strength. Intervention 12-26, A and B, illustrates this skill. Intervention 12-26 Ascending and Descending a Curb 791 A and B. A person ascends a curb by “popping a wheelie” to place the front casters onto the curb, then pulls the rear wheels upward. Timing and good upper extremity strength are important for this activity. C. Descending a curb may be performed by lowering the rear wheels evenly off the curb and completing the activity by spinning the chair to clear the front casters. D. A person may descend the curb forward in a controlled wheelie position. (From Buchanan LE, Nawoczenski DA: Spinal cord injury and management approaches, Baltimore, 1987, Williams & Wilkins, 1987.) Descending a Curb It is often easiest to instruct patients to descend curbs backward; however, most clinicians agree that it presents more danger to the patient because of the risk from unseen traffic. In this technique, the patient backs the wheelchair down the curb. Again, the patient should lean forward and grasp the wheel rims near the brakes on the chair. The position of the footplates must also be observed during performance of this activity. The footplates may catch on the curb as the chair descends. If this occurs, the patient will need to lean back into the chair to allow the casters to clear the curb (Intervention 12-26, C and D). A second method of descending a curb is for the patient to go down in a forward position. Before the patient attempts this maneuver, he or she must be able to achieve a wheelie and roll forward while in a tilted position. As the patient approaches the curb, he or she pops a wheelie. The rear wheels are allowed to roll or bounce off the curb. Once the rear wheels have cleared the curb, the patient leans forward so that the front casters once again are on the ground. Care must be taken when patients learn this task because incorrect shifting of the patient’s weight either too far backward or too far forward can cause the patient to fall out of the wheelchair. It is often easiest to begin training the patient to ascend and descend low training curbs. A 1- to 2-inch curb should be 792 used initially with patients as they try to perfect these skills. Powered Mobility Patients with high-level tetraplegia need to master powered mobility. Often, equipment vendors will provide power chairs for individuals on a trial basis. A portion of your treatment session should be devoted to assisting the patient with the operation of the power chair. Descriptions of different types of power wheelchairs and the operation of these units are outside the scope of this text. Clinicians are encouraged to work with equipment vendors to become knowledgeable about the different wheelchairs and accessories that are available. Wheelchair Cushions Individuals who will be spending a considerable amount of time each day sitting in a wheelchair should also have some type of wheelchair cushion. Specialized cushions are available that reduce some of the pressure applied to the individual’s buttocks. No cushion completely eliminates pressure, and individuals must continue to perform some type of pressure relief throughout the day in order to minimize the risk of pressure ulcers. Cardiopulmonary Training Cardiopulmonary training should also be included in the patient’s rehabilitation program and must be based on the patient’s exercise capacity as determined by the motor level. Incentive spirometry and diaphragmatic strengthening should be continued to further maximize vital capacity. Endurance training can be incorporated into the patient’s treatment plan and can include activities, such as wheelchair propulsion for extended distances, upper extremity ergometry (arm bikes), swimming, and wheelchair aerobics. Although these activities improve the patient’s endurance, the upper extremity muscles are smaller and are more able to perform at a higher intensity for a shorter duration of time than the muscles in the lower extremities. Therefore, these muscles fatigue more quickly (Decker and Hall, 1986; Morrison, 1994). Patients with SCIs lack normal cardiovascular responses to exercise. Individuals with injuries above T4 will generally exhibit maximal heart rates of 130 beats/min or less with exercise while patients with lower level paraplegia will present with increased heart rate responses comparable to the general public (Jacobs and Nash, 2004). Blood pressure, heart rate, cardiac output, and sweating responses are altered secondary to autonomic sympathetic dysfunction and the resultant disturbed blood flow. Therefore, the use of target heart rate alone may not be an appropriate indicator of exercise intensity for patients with spinal cord injuries. Additional methods of monitoring the patient's exercise response, including blood pressure and the Borg Perceived Exertion Scale (a subjective measure of individual exercise intensity), should be employed (Borello-France et al., 2000). Aerobic training effects are, however, still possible and patients can benefit from exercise programs to decrease the risk of secondary complications including hypertension, diabetes mellitus, and elevated cholesterol. Improvements in overall health and quality of life can also be achieved with regular exercise (Burr et al., 2012; Jacobs and Nash, 2004; Lewthwaite et al., 1994). Exercise recommendations for persons with SCI do not vary drastically from those for the general public. Duration of exercise should be 150 minutes a week of moderate intensity aerobic activity or 75 minutes of vigorous-intensity exercise. If a patient is unable to tolerate 20 to 60 minutes of continuous activity, aerobic activity performed for at least 10 minutes is preferred (Department of Health & Human Services, 2008; Jacobs and Nash, 2004). Evidence suggests that cardiovascular fitness can be achieved through several shorter bouts of exercise instead of one longer session (Lewthwaite et al., 1994). Frequency of aerobic exercise should be at least two times a week and not more than six times a week. Possible activities that may be performed include: leg cycling with electric stimulation, body-weight-supported treadmill ambulation, upper extremity and wheelchair ergometry, circuit training, swimming, and wheelchair sports (SCI Action Canada, 2011; Somers, 2010). A break of 1 to 2 days should be taken between exercise sessions to allow for musculoskeletal recovery (Morrison, 1994). Circuit Training Researchers have also studied the effects of circuit training (weight training with exercise 793 equipment and upper extremity ergometry) in individuals with paraplegia. Significant increases in shoulder strength and endurance were noted in individuals who participated in a training program three times a week for 12 weeks. The results of a study by Jacobs et al. (2001) support the beneficial effects of circuit training on fitness levels in individuals with paraplegia. Additionally, upper extremity strengthening programs which target the serratus, middle and lower trapezius, and shoulder external rotators combined with selective stretching of key areas (the pectoralis muscles, upper trapezius, long head of the biceps, and posterior capsule of the shoulder) have been effective in reducing shoulder pain and improving function in patients with paraplegia (Nawoczenski et al., 2006). Maximal-intensity lower extremity strength training has also been shown to improve strength, gait, and balance outcomes in patients with chronic motor incomplete SCI (Jayaraman et al., 2013). Guidelines from the U.S. Department of Health and Human Services (2008) recommend 8 to 10 repetitions (progressing to three sets) of general whole body muscle-strengthening exercises for 2 or more days a week to achieve maximal health benefits. Aquatic Therapy Pool therapy can be a valuable addition to the patient’s overall treatment plan. Water offers an excellent medium for exercising without the effects of gravity and friction and for practicing ambulation skills. Many facilities have warm-water (92° to 96° F) therapeutic pools for their patients. The warm water provides physiologic effects, including increased circulation, heart rate, and respiration rate and decreased blood pressure. In addition, general relaxation is usually accomplished with warm-water immersion. These effects must be kept in mind as the PT develops a pool program for the patient. When designing a therapeutic pool program for a patient with SCI, the PT should consider the following as therapeutic benefits of this type of treatment intervention. Activities performed in the water will help to: 1. Decrease abnormal muscle tone 2. Increase muscle strength 3. Increase range of motion 4. Improve pulmonary function 5. Provide opportunities for standing and weight bearing 6. Exercise muscles with fair-minus strength more easily 7. Decrease spasticity Although most patients can exercise safely in the water, several situations have been identified as contraindications to aquatic programs. A patient with any of the following medical conditions should not be allowed to participate in the program: fever, infectious diseases, tracheostomy, uncontrolled blood pressure, vital capacities less than 1 liter, urinary or bowel incontinence, and an open wound or sore that cannot be covered by a waterproof dressing. Patients with halo traction devices can be taken into the pool as long as their heads are kept out of the water and components of the device that retain water are replaced. Individuals with catheters may participate in pool programs if the drain tubes are clamped and storage bags are attached to the lower extremity (Giesecke, 1997). Pool Program Several logistic factors must be considered before taking the patient in the water for a treatment session. As stated previously, warm water is desirable. However, to accommodate the many patients who may need to use a therapeutic pool at a given facility, the temperature of water may be cooler. This factor must be considered when one works with patients with SCIs because their temperature regulation is often impaired. Different facilities have specific requirements regarding safety procedures that must be followed when working with the patient in the water. Previous water safety experience may be necessary. A minimum number of people may also be needed in the pool area to ensure safety. To prepare the patient for the treatment session, the PT or PTA must discuss the benefits of the program and describe a typical session. The patient’s previous affinity for water must also be determined. Many individuals profoundly dislike water and may be apprehensive about the experience. Reassuring the patient should help. The patient should arrive for the treatment session in a swimsuit. Catheters should be clamped to avoid the potential for leakage. The patient should also be instructed to wear socks, elbow, and knee pads, depending on the treatment activities to be performed. Because sensory impairments are common, areas that 794, could become scraped during the session must be protected. Transfers into and out of the pool can occur in a number of different ways and depend on the type of equipment and facilities present. Frequently, a lift transfers the patient into the pool, or the pool may have a ramp, and entrance is in some type of wheelchair or shower chair. Once the patient is in the water, the PTA must guard the patient carefully. Patients with tetraplegia and paraplegia have decreased movement, proprioception, and light touch sensation. The patient may have difficulty maintaining position in the water. At times, the lower extremities may float toward the surface of the water, and the PTA may have a difficult time keeping the patient’s feet and lower extremities on the bottom of the pool in a weight-bearing position. Gentle pressure applied to the top of the patient’s foot by the PTA’s foot can help alleviate this problem. Flotation vests are helpful and can be reassuring to the patient. Once the patient is more confident in the water, the vest can be removed if allowed by facility policy. Pool Exercises Many pools have steps into them or an area where the PTA and the patient can sit down. This feature provides an excellent environment to work on upper extremity strengthening. With the upper extremity supported, the patient moves the arm in the water and uses the buoyancy of the water to complete range-of-motion exercises. The patient can also work on lifting the extremity out of the water to provide more challenge to the activity. The anterior, middle, and posterior deltoids, as well as the pectoralis major and rhomboids, can be exercised in this position. Triceps strengthening can also occur in a gravity-neutralized or supported position. In addition to working on upper extremity strengthening, use of the sitting position serves to challenge the patient’s sitting balance and trunk muscles that remain innervated. Alternating isometrics and rhythmic stabilization can be applied at the shoulder region to work on trunk strengthening. Exercises to increase pulmonary function can be practiced while the patient is in the water. Having the patient hold his or her breath or blow bubbles while in the water assists in improving pulmonary capacity. The patient can practice standing at the side of the pool while in the water. The PTA may need to guard the patient at the trunk and to use the lower extremities to maintain proper alignment of the patient’s legs. Approximation can be applied down through the hips to assist with lower extremity weight bearing. Some therapeutic pools possess parallel bars within the water to assist with standing and ambulation activities. If the patient has an incomplete injury with adequate lower extremity innervation, assisted walking can be performed. As stated previously, this is an excellent way to strengthen weak lower extremity muscles and to improve the patient’s endurance. Kickboards can also be used to assist with lower extremity strengthening. Floating and Swimming Patients with tetraplegia or paraplegia can be taught to float on their backs. Floating assists with breathing, as well as general body relaxation. Patients can also be instructed in modified or adaptive swimming strokes. Patients with tetraplegia can be taught a modified backstroke and breaststroke. Performance of these swimming strokes assists the patient with upper extremity strengthening and also improves the patient’s cardiovascular fitness. Patients with paraplegia can be instructed in the front crawl or butterfly stroke, which also increase upper extremity strength and improve the patient’s cardiovascular endurance. Other Advanced Rehabilitation Interventions Other treatment activities may be performed as part of the patient’s treatment plan. Neuromuscular stimulation (NMS) may be used in patients with muscle weakness to increase strength and to decrease muscle fatigue. NMS is often suggested when a patient has muscle innervation and weakness as a consequence of an incomplete injury. Other benefits of NMS include decreasing range-of-motion limitations, decreasing spasticity, minimizing muscle imbalances, and providing positioning support for patients who are attempting ambulation. Clinicians can also apply NMS to the upper or lower extremity musculature to assist with arm and leg ergometry. As stated previously, patients with incomplete injuries often have increased muscle tone that interferes with function. Therefore, a component of the patient’s treatment plan is the management of this problem. Stretching, ice, pool therapy, and functional electrical stimulation may be appropriate forms of intervention. Electrical stimulation can be applied either to the antagonist 795 muscle to promote increased strength or to the agonist to induce fatigue. Patients with excessive amounts of abnormal tone may also be receiving pharmacologic interventions, as mentioned previously in this chapter. Ambulation Training One of the first questions that patients with SCIs often ask is whether they will be able to walk again. This question is frequently posed in the acute-care center immediately following the injury. Early on, it may be difficult to determine the patient’s ambulation potential secondary to spinal shock and the depression of reflex activity; however, once this condition resolves, many patients expect an answer to this question. In a study by van Middendorp et al. (2011), the researchers developed a clinical prediction rule for ambulation based on a patient’s age and his or her results on four neurologic tests (motor scores for the quadriceps and gastrocsoleus and light touch sensation in dermatomes L3 and SI). A patient’s motor scores, sensory status, and age can provide health-care providers with an early prognosis regarding the patient’s ability to walk independently after injury (van Middendorp et al., 2011). Different philosophies regarding gait training are recognized, and much depends on the rehabilitation team with which you work. Some health-care professionals believe that it is best to give patients with the potential to ambulate every opportunity to do so. These individuals believe that most patients, given the opportunity to try walking with orthoses and an assistive device, will not continue to do so after they realize the difficulty encountered. It may be best to allow the patient to come to his or her decision on ambulation independent of the PT or health-care team. Other health-care professionals believe that a patient should possess strength in the hip-flexor musculature before ambulation is attempted because of the high energy costs, time, and financial resources associated with gait training. Most patients with higher-level injuries choose wheelchair mobility as their preferred method of locomotion after trying ambulation with orthoses and assistive devices because of the energy expenditure and decreased speed associated with the activity (Cerny et al., 1980; Decker and Hall, 1986; Somers, 2010). Compensatory versus restorative approaches to the treatment of the patient with SCI are best illustrated in the therapist’s approach to gait training. The use of orthoses, assistive devices, functional electrical stimulation, and robotic exoskeletons are examples of compensatory strategies that can be employed to assist patients with ambulation on level surfaces. Locomotor training through partial body-weight-supported treadmill ambulation provides an excellent example of the restorative approach to patient care. Benefits of Standing and Walking Although functional ambulation may not be possible for all of our patients with SCIs, therapeutic standing has documented benefits. Standing prevents the development of osteoporosis and also helps decrease the patient’s risk for bladder and kidney stones. In addition, improvements in circulation, reflex activity, digestion, muscle spasms, and fatigue levels have been noted in individuals who are able to participate in standing programs (Eng et al., 2001; Nixon, 1985). Guidelines have been established regarding assessment of the patient's likelihood for success with ambulation. Factors to consider include the following: (1) the patient’s motivation to walk and to continue with ambulation once discharged from rehabilitation (given the opportunity to try assisted ambulation with orthoses, some patients decide it is too difficult a task and prefer not to continue with the training); (2) the patient’s weight and body build (the heavier the patient is, the more difficult it will be for the patient to walk, and taller patients usually find it more challenging to ambulate with orthoses); (3) the passive range of motion present at the hips, knees, and ankles (hip, knee, or ankle plantar flexion contractures limit the patient’s ability to ambulate with orthoses and crutches; in addition, patients need approximately 110 degrees of passive hamstring range of motion to be able to don their orthoses and transfer from the floor if they fall); (4) the amount of spasticity present (lower extremity or trunk spasticity can make wearing orthoses difficult); (5) the cardiopulmonary status of the patient (patients with better pulmonary function have an easier time meeting the energy demands of walking); and (6) status of the integumentary system. All of these factors must be considered by the rehabilitation team when discussing ambulation with the patient (Atrice et al., 2013; Basso et al., 2000). Depending on the patient’s motor level, different types of ambulation potential have been 796 described. The literature varies on the specific motor level and the potential for ambulation. For patients with T2 through T11 injuries, therapeutic standing or ambulation may be possible. This means that the patient is able to stand or ambulate in the physical therapy department with assistance. However, functional ambulation is not possible. Therapeutic ambulators require assistance to transfer from sitting to standing and to walk on level surfaces. These patients ambulate for the physiologic and therapeutic benefits it offers. Patients with injuries at the T12 through L2 level have the potential to be household ambulators, whereas patients with innervation at L3 can achieve functional community ambulation (Atrice et al., 2013). Individuals who achieve household or community ambulation are able to ambulate in their homes with orthoses and assistive devices. Patients at this level are able to transfer independently, to ambulate on level surfaces of varying textures, and to negotiate doorways and other minor architectural barriers. The energy cost for ambulation in patients with complete injuries above T12 is above the anaerobic threshold and cannot be maintained for an extended period (Atrice et al., 2013). Cerny et al. (1980) reported that gait velocities for patients with paraplegia were significantly slower than normal walking, and gait required a 50% increase in oxygen consumption and a 28% increase in heart rate. Consequently, individuals with paraplegia discontinue ambulation with their orthoses and assistive devices and use their wheelchairs for environmental negotiation (Cerny et al., 1980). Community ambulation is possible for patients with injuries at L3 or lower. These patients are able to ambulate with or without orthoses and assistive devices. Community ambulators are able to ambulate independently in the community and can negotiate all environmental barriers (Atrice et al., 2013; Decker and Hall, 1986). Orthoses Patients with paraplegia who decide to pursue ambulation training need some type of orthosis. Figure 12-15 depicts the most common lower extremity orthoses prescribed. Knee-ankle-foot orthoses may be recommended for patients with paraplegia. These orthoses typically have a thigh cuff and an external knee joint with a locking mechanism (drop locks or bail locks are the most common). They have a calf band and an adjustable locked ankle joint. Scott-Craig knee-ankle-foot orthoses are frequently prescribed for patients with paraplegia. These orthoses consist of a single thigh and pretibial band, a bail lock at the knee joint, and modified footplates. The design of this orthosis provides built-in stability for the patient while standing. 797 798 FIGURE 12-15 A, Combination plastic and metal knee-ankle-foot orthoses. B, The Scott-Craig knee-ankle-foot orthosis is a special design for spinal cord injury. The orthosis consists of double uprights, offset knee joints with locks and bail control, one posterior thigh band, a hinged anterior tibial band, an ankle joint with anterior and posterior adjustable pin stops, a cushion heel, and specially designed footplates made of steel. C, The reciprocating gait orthosis, although generally used with children, is also used with adults. Its main components are a molded pelvic band, thoracic extensions, bilateral hip and knee joints, polypropylene posterior thigh shells, ankle- foot orthosis sections, and cables connecting the two hip joint mechanisms. (From Umphred DA, editor: Neurological rehabilitation, ed 6. St Louis, 2013, Elsevier). The reciprocating gait orthosis is another type of orthosis that may be prescribed for patients with SCls. This device can be used with patients with little trunk control because of the midthoracic and pelvic support. The reciprocating gait orthosis has an external hip joint that is operated by a cable mechanism. When the patient shifts weight onto one lower extremity, the cable system advances the opposite leg. Individuals using reciprocating gait orthoses often use a walker instead of Lofstrand crutches as their preferred assistive device. The reciprocating gait orthosis is frequently prescribed for children with lower extremity weakness secondary to myelomeningocele. Refer to Chapter 7 for a review. A new type of orthotic system is now available for patients with SCIs. The ReWalk system is similar to the reciprocating gait orthosis, but it has a robotic exoskeleton that is interfaced with a computer and motion sensors and allows patients to transfer from sitting to standing more easily. This system appears to have excellent potential for patients with higher-level thoracic injuries (fda.gov, 2014). Preparation for Ambulation The decision to attempt gait training is made by the patient and the rehabilitation team. As stated previously, the patient’s motor level and other factors must be considered. Patients with motor complete, AIS A and B, do not possess adequate lower extremity motor function to ambulate from a restorative treatment approach but may be able to ambulate using compensatory strategies and appropriate bracing and assistive devices. 799 In general, the patient should be independent in mat mobility, wheelchair-to-mat transfers, and wheelchair mobility on level surfaces before beginning gait training. Many clinics possess training orthoses that allow the patient to practice standing before permanent orthoses are prescribed and manufactured. An orthotist should work with the patient to assist in identifying and fabricating the best orthosis for the patient. Special note Depending on the patient’s length of stay in the rehabilitation facility, gait training may begin at the end of the patient’s inpatient hospitalization, or it may begin in earnest in the outpatient setting. Once the permanent orthoses have been delivered, it is time to begin the first gait training session. If possible, the orthotist should be present for this session. Having the patient don the orthoses is the first step. It is often easiest for the patient to do this on the mat in a long-sitting position. The patient should be encouraged to do as much as possible on this first attempt. He or she should start by placing one foot into the shoe and then locking the knee joint. During the performance of this activity, one realizes the necessity of possessing 110 degrees of hamstring range. Once the knee is in the orthosis, the patient can tighten the thigh pad. From there, the patient should start to put the other foot in the orthosis. Once both orthoses are on, the therapist and the orthotist, if present, will inspect the orthoses and check the fit. The orthoses must not rub the patient's skin. This situation can cause areas of redness and can lead to skin breakdown. If everything looks satisfactory, the patient should then be instructed to transfer back to the wheelchair to begin standing activities in the parallel bars. Upon completion of the gait training session and removal of the orthoses, the patient’s skin should be inspected once again to ensure that there are no areas of pressure or skin breakdown. Standing in the Parallel Bars The first thing the patient needs to do is to transfer to standing. The therapist should initially demonstrate this maneuver for the patient. It is easiest to have the patient hold on to the parallel bars and pull forward. In preparation for this transition, the patient needs to move forward in the wheelchair. Having the patient push up and lift the buttocks forward is best to prevent shearing of the patient’s skin. Once the patient is forward in the chair, the therapist will want to make sure the patient's orthoses are locked. If this is the patient's first time to stand up, it will be safest to have two individuals assist. While the patient is wearing the safety belt, one person is positioned in front of the patient and the other person is at the side or the back of the patient. On the count of three, the patient pulls himself or herself forward on the bars. The individuals assisting the patient also provide the patient with the needed strength and momentum to complete the transfer. Once upright, the patient must work to find his or her balance point. The patient’s lower extremities should be slightly apart; the low back should be in hyperextension; the shoulders are toward the back; and the hands must be forward of the hips and holding on to the parallel bars. Essentially, the patient is resting on the Y ligaments in the hip and pelvic region. The lower extremity orthoses and positioning allow the patient to move his or her center of gravity behind the hip joints. Once the patient is able to find his or her balance point, he or she will eventually be able to stand and maintain balance without the use of the upper extremities. To guard the patient during this activity, the therapist will be behind the patient or off to the side. The therapist holds on to the gait belt and should avoid holding on to the patient’s upper arms. The therapist may place a supporting hand on the patient’s anterior shoulder as long as the therapist does not provide a counterbalancing or rotational force. During practice of achievement of the balance point, the patient should initially have both hands on the parallel bars. The patient should be encouraged to hold the bars lightly and should avoid grabbing or pulling on them. Often, just having the patient rest the hands on the bars may be best. Eventually, you will want the patient to balance with one hand, and finally with no hands. The patient should ultimately be able to stand in the orthoses without any upper extremity support. After the patient feels comfortable finding and maintaining the balance point, he or she can begin to practice push-ups in the bars. With the hands in a forward position, the patient pushes down on the bars by depressing the shoulders and tucking the head. Depending on the type of lower 800 extremity orthosis and the presence or absence of a spreader bar, the therapist will want to note what happens to the patient’s lower extremities during the push-up. Most often, the legs dangle free. If a spreader bar is attached to the orthoses, the legs will move as one unit. Performing a push- up is a prerequisite activity for the patient to ambulate in a forward direction. After the patient practices maintaining the balance point, he or she should also practice jack- knifing. Jack-knife can be described as movement of the patient’s upper body and head forward of the pelvis. Although jack-knifing is an undesirable occurrence, the activity should be practiced in the parallel bars during early gait training sessions. With the hands forward, the patient bends forward at the waist and lowers the trunk down toward the parallel bars. The patient then pushes himself or herself back up to an upright position. Once the patient feels comfortable with this activity, he or she can practice falling into a jack-knife position. The patient can initiate this fall either by moving the hands posterior to the hips or by flexing the head forward. The therapist can also assist the patient with the achievement of the jack-knife position by gently pulling the patient’s hips and pelvis in a posterior direction. To review, the jack-knife position is the position the patient will likely assume if he or she loses balance during ambulation activities. The patient should recognize this position and needs to know what to do if it occurs during gait activities. If this position should occur during gait, the patient will want to straighten his or her elbows while extending the head and trunk. Gait Progression Once the patient can maintain his or her balance point and can perform a push-up to clear his or her feet from the floor, he or she is ready to begin forward ambulation in the parallel bars. You may be wondering how long this typically takes. Normally, you will want to progress the patient to taking a few steps on the first standing and ambulation attempt. However, the clinician has to monitor the patient’s responses closely during standing and ambulation. The effects of fatigue, orthostatic hypotension, decreased cardiopulmonary endurance, and the anxiety associated with standing and walking can easily overwhelm the patient. To monitor physiologic responses during the treatment, the clinician should take baseline pulse, respiration, and blood pressure readings before the patient is standing. Careful monitoring of vital signs during the gait training portion of the treatment session is also indicated. In addition, the patient must be instructed to report any feelings of light- headedness or dizziness immediately. The PTA should instruct the patient to find his or her balance point before advancing forward in the parallel bars. The patient’s head should be held upright, looking forward. The patient then flexes his or her head, pushes down on the hands, depresses the shoulders, and lifts the lower extremities off the ground. As the patient depresses his or her shoulders and straightens the elbows, he or she must extend the head and neck and return it to a neutral position. To maintain balance, the patient needs to move his or her hands forward of the hips immediately. If the patient were to maintain his or her hands in the same place after completing the lift, he or she would jack-knife. After the patient’s feet make contact with the floor, he or she must retract the scapula and move the upper trunk and head posteriorly. This type of gait pattern is known as a swing-to pattern because the patient is moving the feet the same distance as his or her hands. The patient should repeat the steps just described until he or she progresses to the end of the parallel bars. Using the verbal instructions “Lean, lift, and land” can be helpful. At this point, someone can pull the wheelchair up behind the patient, or the patient can be instructed in performing a quarter-turn. If the patient is not too tired, he or she should continue and learn the turning technique at this time. Intervention 12-27 illustrates the correct head and trunk positions for gait-training activities. Intervention 12-27 Gait Progression 801 A. The patient finds his balance point. B. He advances the crutches forward. C. The patient tucks his head and pushes down on the crutches. D. His pelvis and lower extremities swing forward. E. His feet strike the floor. F. The patient lifts his head and resumes a lordotic posture. Quarter-Turns To complete a quarter-turn, the patient depresses his or her shoulders and lifts the legs while changing his or her hand position on the parallel bars. In essence, he or she is completing two quarter-turns to change direction. The patient must practice turning in both directions. Sitting Before transferring back to sitting, the patient should be instructed in the proper technique. The wheelchair should not be pulled up to the back of the patient’s legs. Remember, the patient transfers from standing to sitting with the lower-extremity orthoses locked in extension. For this reason, the chair should be at least 12 inches from the patient so he or she will be able to land in the 802 wheelchair seat. If the chair is too close to the patient, he or she might tip the chair over backward. The PTA should have the patient keep both his or her hands on the parallel bars during the descent. In time, the patient will be instructed in other methods to perform transfers from sitting to standing and from standing to sitting without the use of the parallel bars. Swing-Through Gait Pattern Once the patient feels comfortable with the swing-to gait pattern, the patient can progress to a swing-through pattern. The technique is the same as the swing-to pattern, except the patient advances his or her legs a little farther forward, and instead of stopping between steps, the patient moves his or her hands forward again and takes another step. This gait pattern allows the patient to move forward a little faster and is more energy-efficient. Other Gait Patterns If the patient possesses lower extremity innervation, specifically hip flexion, the patient may have the potential to use a four-point or two-point gait pattern. Both patterns more closely resemble normal reciprocal gait patterns with upper and lower extremity movement. These patterns are described in standard texts and are not discussed here. Backing Up Patients should also be instructed in backing up. This is important when the patient begins to use his or her crutches on level surfaces within the physical therapy department. Initially, backing up should be practiced in the parallel bars. The patient tucks the head, depresses the shoulders, and extends the elbows. This position causes the patient to perform a mini—jack-knife and allows the patient’s legs to move backward by virtue of the head-hips relationship. The patient repeats this sequence several times to move the desired distance backward. Progressing the Patient After the patient has practiced ambulation in the parallel bars several times, it is time to progress to ambulation outside of them. It is advisable to progress out of the bars without delay because patients can become reliant on them and may find it difficult to make the transition to overground ambulation in a less secure environment. To assist with this transition, the clinician may elect to introduce Lofstrand (Canadian or forearm) crutches while the patient is still ambulating in the parallel bars. Care must be exercised when practicing transitions into and out of the wheelchair. These techniques are best practiced with the back of the wheelchair positioned next to a wall for greater safety. In addition, the patient should check to make sure the wheelchair brakes are locked. Standing From the Wheelchair If the patient is to become independent in ambulation activities, he or she must learn to transfer from sitting to standing independently. Several methods are possible for the patient. The first method described is probably the easiest. Step 1. The patient places the wheelchair against the wall and locks the brakes. Step 2. The patient places his or her crutches behind the wheelchair to rest on the push handles. Step 3. The patient moves to the edge of the wheelchair. The patient needs to complete mini-push- ups as he or she does this. Scooting forward can cause unnecessary shearing to the patient’s skin. Step 4. With the orthoses locked, the patient crosses one leg over the other. Step 5. The patient then pivots over the fixed foot and pushes up to standing. Step 6. Holding on to the wheelchair armrest, the patient secures one crutch, positions it, and then secures the second crutch. Step 7. Once the crutches are in place, the patient backs up from the wheelchair, taking two or three steps backward. Intervention 12-28 shows the steps needed to transfer from sitting to standing with lower extremity orthoses and Lofstrand crutches. Intervention 12-28 Sit-to-Stand Transfer with Orthoses 803 The sequence for transferring from sit to stand with lower extremity orthoses. (See text description on steps 1 through 7.) An alternative way of completing this transfer is to unlock one of the orthoses and pivot over the unlocked lower extremity. This technique can be less stressful to the hip joint than the one previously described. The patient completes the transition to upright in the same way as noted earlier, except that the patient needs to lock the knee joint of the bent knee once an upright position has been achieved. The patient can also assume standing from the wheelchair by transferring forward. Step 1. The patient moves forward to the edge of the chair. Step 2. With the arms in the crutches, the patient places the crutches flat on the floor, slightly behind the front wheels. Step 3. The patient flexes his or her head and pushes down on the crutches to propel out of the wheelchair. Step 4. Once standing, the patient must quickly extend the head and trunk to regain the lumbar lordosis necessary for standing stability. 804 Step 5. The patient’s upper extremities remain behind until the patient feels he or she has regained balance. Then he or she can move the arms and crutches forward. Intervention 12-29 shows a patient completing this activity. Intervention 12-29 Coming to Stand From the Wheelchair A. The patient flexes his head and upper trunk. B. The patient uses the head-hips relationship and muscle action from the latissimus dorsi and triceps to push himself upright. C. Upright standing. This method is difficult for many patients because it requires a great deal of strength, balance, and coordination. Once the patient is standing and has regained balance, he or she can begin to ambulate using a swing-through gait pattern, as described previously. The clinician guards the patient from behind, with one hand on the gait belt and the other on the patient’s posterior shoulder, as depicted in Figure 12-16. The clinician must be careful to avoid the tendency to apply excessive tactile cues to the patient. Pulling on the gait belt or impeding the movement of the patient’s upper trunk may, in fact, cause the patient to experience balance disturbances. 805 FIGURE 12-16 Patient with an injury at the T12 level ambulating with crutches and bilateral knee-ankle-foot orthoses for balance and lower extremity advancement. (From Adkins HV, editor: Spinal cord injury, New York, 1985, Churchill Livingstone.) To regain a sitting position after walking, the following is recommended: Step 1. The patient faces the wheelchair initially. Step 2. The patient places the crutches behind the chair. Step 3. The patient unlocks one of the knee joints and rotates over that knee to assume a sitting position. Patients can return to sitting using a straight-back method. This technique is difficult, however, and may be best used when a second person is present to assist with the transition to stabilize the wheelchair. Gait Training with Crutches As the patient begins ambulation training on level surfaces with the crutches, he or she once again needs to find his or her balance point. The patient must maintain the hands forward of the hips to prevent jack-knifing. Initially, the clinician may elect to perform a swing-to gait pattern with the patient. The clinician should guard the patient from behind by holding on to the gait belt as necessary. Some clinicians may find it easier to guard the patient from the side initially by holding on to the gait belt and placing the other hand on the patient’s shoulder. Verbal and tactile cueing may be necessary to assist the patient with head positioning and the hyperlordotic posture. Should the patient lose balance and begin to jack-knife, the clinician will push the patient’s pelvis forward and shoulders back to resume the hyperextended posture. Because the patient will be moving relatively quickly, the clinician will need to take bigger steps. As the patient becomes more proficient, the patient can begin a swing-through gait pattern. Falling 806 All patients who attempt gait training with crutches should also be instructed in proper falling techniques to avoid injury. The first attempts at falling should be completed in a controlled manner. You will want to have the patient fall onto a floor mat. The patient is instructed to let go of the crutches and remove the hands from the hand grips. The patient then reaches toward the ground and flexes the elbows to avoid trauma to the wrist. If the facility has a crash mat (these mats are higher and softer), having the patient fall onto it is an easier starting point for the patient. Getting up From the Floor Once the patient has practiced falling to the floor, the patient must also learn how to get up from the floor. The following steps should be used to assist the patient with this activity. Caution This transfer should be practiced close to a wall so the patient has something to lean against as he or she transitions to upright. Step 1. The patient is instructed to assume a prone position on the floor. Step 2. The patient positions the crutches with the tips pointing toward the head and the hand gripping at the hips. Step 3. The patient pushes up to a plantigrade position. (The patient ensures that both orthoses are locked before attempting this maneuver.) Step 4. The patient reaches for one of his or her crutches and puts the crutch tip on the floor to assist in the transition to an upright position. The patient’s hand is on the crutch handle, and the crutch rests against the shoulder. Step 5. The patient uses the crutch on the floor as a point of stability as he or she reaches for the other crutch and positions it on the forearm. Step 6. The patient turns the opposite crutch around and places the forearm cuff at his or her elbow region. Step 7. The patient regains balance with the crutches. Intervention 12-30 depicts this sequence. Intervention 12-30 Getting Up From the Floor 807 A. Instruct the patient to assume a prone position on the floor. Have the patient position the crutches with the tips pointing toward his head and the hand grips at the patient’s hips. B. The patient pushes up to a plantigrade position. (The patient will want to make sure that both orthoses are locked before attempting this.) Cand D. The patient reaches for one of his crutches, using it for balance. The crutch rests against his shoulder. E and F. The patient uses the crutch on the floor as a point of stability as he reaches for the other crutch and positions it on his forearm. G and H. The patient regains his balance with the crutches. Negotiating Environmental Barriers If the patient is to be independent with ambulation in the community, he or she must be able to negotiate ramps, curbs, and stairs with orthoses and braces. Ascending a Ramp Step 1. The patient uses a swing-to gait pattern to move forward up the ramp. Step 2. To maintain balance, the patient keeps his or her crutches several inches in front of the feet. Step 3. To increase hip stability, the patient’s pelvis must be forward in a lordotic posture. 808 Descending a Ramp The same technique used for ambulation on level surfaces can be employed. A swing-through gait pattern is recommended. Ascending a Curb Step 1. The individual approaches the curb head-on. Step 2. In a balanced position near the edge of the curb, the patient places the crutch tips on the curb. Step 3. The patient leans forward, tucks the head, extends the elbows, and depresses the scapulae (jack-knifes) to elevate his or her lower extremities onto the curb. (The patient’s toes drag up the elevation of the curb.) Step 4. The patient can step to or past the crutches. Step 5. Once the patient’s feet land on the curb, he or she will need to regain the balance point. Descending a Curb Step 1. The individual approaches the curb head-on. Step 2. In a balanced position near the edge of the curb, the patient steps off the curb, tucking the head, straightening the elbows, and depressing the scapulae. Step 3. Once the patient’s lower extremities have swung past the edge of the curb, he or she lowers the legs by eccentrically contracting the elbow and shoulder musculature. Step 4. When the patient’s feet come in contact with the ground, he or she needs to regain the balance point. Although the Americans with Disabilities Act increased the accessibility of many public and private buildings, many homes and community buildings are not accessible to certain individuals. For this reason, we review the techniques for instructing the patient in stair negotiation. Ascending Stairs Patients can ascend stairs using the same techniques described to go up a single curb. In addition, patients can be instructed in an alternative approach to ascend the stairs backward. Step 1. The patient stands with the back to the stairs and in a balanced position. Step 2. With the crutches on the step above, the patient leans into the crutches, straightens the elbows, and depresses the scapulae. This maneuver causes the lower extremities to be lifted onto the step. Step 3. Once the patient’s feet have landed, he or she extends the neck and retracts the scapulae to regain a forward pelvis position. The patient repeats these steps until he or she has successfully ascended all the required steps. Descending Stairs The patient who must descend a series of steps can use the techniques described for going downa curb. However, the patient must be careful because the space in which he or she can land is limited. The patient must accurately gauge the length of his or her step so he or she will not miss a step. 809 Body-weight-supported treadmill Research in the basic sciences has been conducted in an effort to attenuate the deficits caused by SCI. Animal research suggests that cats with complete spinal cord transections can regain the ability to walk on a treadmill after training. This research “suggests that the spinal cord is able to integrate and adapt to sensory information during locomotion” (de Leon et al., 2001). Of particular interest to researchers and clinicians alike is the existence of central pattern generators (CPGs), a network of nerve cells in the spinal cord. CPGs produce locomotion and are facilitated by supraspinal input; however, CPGs can be activated by external stimuli in the absence of cortical influence (Basso, 2000; Hultborn and Nielsen, 2007). Key to our understanding of the recovery of locomotion abilities is the role that sensory feedback plays in stepping (Hultborn and Nielsen, 2007). Locomotor training for patients with incomplete spinal cord injury is based on principles of activity-dependent plasticity and automatic movement patterns. Activity-dependent interventions focusing on limiting compensation while activating the nervous system below the injury level are important components of the plan of care for these patients. Locomotor training provides the nervous system with “appropriate sensory input to stimulate the remaining spinal cord injury networks to facilitate their continued involvement even when supraspinal input is compromised” (Harkema et al., 2012). The use of body-weight-supported treadmill training (BWSTT) with manual or electric stimulation or robotic assistance has provided patients with improved outcomes relative to distance and walking speed (Field-Fote and Roach, 2010; Harkema et al., 2012). The patient is suspended by a harness over a treadmill, which provides for upright posturing and decreased loading of the lower extremities. Approximately 35% to 40% of the patient’s weight is supported. Trainers can assist with movement of the patient’s lower extremities while the treadmill is moving. Intervention 12-31 illustrates this type of locomotor training. The movement of the treadmill pulls the hip into extension and facilitates the swing phase of the gait cycle thus providing patients with the sensory experience of walking. Treadmill speeds of 0.8 to 1.0 m/sec are recommended for training. As the patient progresses, treadmill speed, amount of body weight supported, and length of time the patient spends walking can be increased. To review concepts presented in Chapter 10, BWSTT supports the premise of activity-dependent neuroplasticity and the performance of task- specific activities in the treatment of patients with neurologic impairments. Intervention 12-31 Locomotor Training 810 A patient performs body-weight-supported treadmill ambulation. (From Sisto SA, Druin E, Sliwinski MM: Spinal cord injury: management and rehabilitation, St. Louis, 2009, Mosby.) In some research studies, BWSTT and overground ambulation is combined with electrical stimulation. The electrical stimulation elicits reflex-based movements (a flexor-withdrawal response) in the lower extremities to promote stepping and can be used as an Ss This 1 Baa is belacane to facilitate the spinal circuitry underlying locomotion (F ; ). Robotic-assisted BWSTT is also available, providing the patient with ienematically appropriate lower extremity movements. Proprioceptive input is therefore precise and is thought to improve motor learning as it promotes development of an internal reference of correctness ( , 2011). Although less physically demanding for the therapist, there are some concerns with robotic-assisted gait relative to the passive nature of the lower extremity movement and the fact that movement occurs only in the sagittal plane. vention 12-32 illustrates robotic-assisted ambulation (Somers, 2010). 811 Intervention 12-32 Robotic-Assisted Locomotor Training A patient with spinal cord injury is supported in a harness from above while he uses the Lokomat robotic-assisted gait training device. (From Sisto SA, Druin E, Sliwinski MM: Spinal cord injury: management and rehabilitation, St. Louis, 2009, Mosby.) Harkema et al. (2012a) has described four guiding principles for locomotor training: (1) maximize weight bearing on the lower extremities while limiting upper extremity weight bearing; (2) optimize the sensory experience associated with the activity; (3) promote proper limb kinematics and; (4) maximize independence and limit compensations. To improve the patient’s functional abilities, locomotor training must also be performed overground and in the community. For motor learning to occur, the patient must be able to translate skills from one environment to the next. In recent studies conducted by Field-Fote and Roach (2011) and Harkema et al. (2012b), outcome measures including the 10-meter walk, Berg Balance Scores, and walking speed were improved in patients with incomplete injuries who participated in intensive activity-based locomotor programs. 812 Discharge planning As stated previously, lengths of stay for inpatient rehabilitation continue to decrease. As a consequence, one must begin discharge planning during the patient’s first visit to physical therapy. All members of the patient’s rehabilitation team including the patient, family members, significant others, and caregivers must be included in the process. The combined efforts of all involved parties help the patient make a successful transition from the hospital to his or her previous home and work environments. The discharge planning process ideally includes a number of different activities aimed at improving the patient’s functional outcome and providing an easy transition from health-care facility to home. Activities that should be a part of the discharge planning process include (1) a discharge planning conference; (2) a trial home pass; (3) an assessment of the home environment to ensure accessibility; (4) development of a vocational plan; (5) procurement of all necessary adaptive equipment and supplies; (6) driver’s training (if appropriate); (7) education regarding community resource availability; and (8) recommendations regarding additional rehabilitation services and the need for long-term health and wellness services. Discharge Planning Conference The discharge planning conference should be held approximately 1 to 2 weeks before the patient’s anticipated discharge date. At this time, continued medical and rehabilitation follow-up should be addressed, and a review of resources available to both patient and family should be provided. Ideally, patients will have access to comprehensive follow-up services. Spinal cord clinics that offer routine reassessments at predetermined times are beneficial. At these follow-up appointments, many potential long-term complications are discovered and are successfully managed. Unfortunately, many patients are discharged to areas where medical specialists trained in providing long-term care to this patient population are not available. For this reason, patients must be educated regarding their injuries, possible secondary complications, and potential outcomes for their recovery. During the discharge planning conference, certain issues must be addressed. Areas of concern include the following: 1. The patient’s attitude and discharge plans must be discussed. Is the patient realistic regarding what it will be like at home? Is discharge to home possible? 2. The knowledge base and understanding exhibited by the patient’s primary caregivers regarding SCIs and management should be assessed. Do caregivers understand the patient’s condition and the level of care required? 3. The availability of a physician who can deal with the medical problems and secondary complications encountered by patients with SCIs should be discussed. 4, The amount and degree of professional and attendant care required by the patient must be determined. Does the patient possess the financial means (insurance or income) to pay for personal care? Has the patient received all of the adaptive and ADL equipment necessary to function at home? Equipment, including wheelchairs and seat cushions, should be received before the patient’s discharge, so any necessary training or modifications can be performed in the facility. In addition, a relationship with a durable medical provider is suggested. 5. Transportation issues associated with school, work, leisure activities, and doctors’ appointments must be confirmed. Patients with power wheelchairs need access to vans with hydraulic chair lift capabilities. Patients who want to resume driving need to have adaptive hand controls installed in their automobiles. The timetable to receive these items can be long. Therefore, one is advised to begin this planning process early. 6. The accessibility of the patient’s home, school, or workplace must be addressed. Architectural modifications should be completed in advance of the patient’s discharge. 7. Other issues related to accessibility of community resources and support for the patient and his or her family members must be discussed. Support groups for patients and their family members are available in many communities. These groups can often provide the patient both emotional support and a social outlet. Therapeutic passes are often given to patients close to their discharge and are extremely 813 beneficial to the discharge planning process. When a patient is given a pass, the patient is released from the health-care facility for several hours or, in some cases, overnight in the care of a family member. The pass is used to determine how the patient will function once he or she is discharged from the rehabilitation unit. During the pass, the patient and the family can practice essential skills that will be needed once the patient is at home full time. These passes also offer opportunities for the patient to solve problems that may be encountered at home, such as inaccessibility of various rooms. The passes assist the patient in regaining the confidence needed to function outside the safe confines of the rehabilitation setting. Many patients are often anxious about their discharge from rehabilitation. The rehabilitation hospital or unit is considered a safe environment with 24-hour daily care and the comfort of individuals with similar problems and physical deficits. After the pass, the patient returns to the rehabilitation unit for continued intervention and planning for discharge. The patient and family are expected to share their experiences regarding the pass so that additional training and problem solving can occur. Concomitantly, if additional environmental modifications to the dwelling must be made, the pass provides the information necessary to complete those changes. As a component of discharge planning, the patient and the rehabilitation team need to discuss vocational planning. A referral to a vocational rehabilitation specialist or, in some instances, a psychologist can foster adjustment toward the patient’s disability and can assist the patient in having an optimistic attitude toward the future. Many times, the patient is not ready at this particular point to think about the future, especially his or her place in the work world. However, beginning a vocational evaluation and discussing the patient’s return to school or work is extremely positive and helps to foster the expectation that participation in these activities can be resumed. Unfortunately, data show that only 34.9% of individuals with SCI are employed 20 years after initial injury (The National Spinal Cord Injury Statistical Center, 2013). Procurement of Equipment A detailed discussion about securing equipment that the patient will need before discharge from the facility is beyond the scope of this text. Some of the common items that must be considered are presented here. The occupational therapist and the rehabilitation team should be consulted for more specific information. Items frequently needed by the patient at discharge include the following: 1. Wheelchair: The type and specific requirements are determined by the rehabilitation team. The benefits of power versus manual wheelchairs must be considered. Cost and reimbursement issues may be concerns for some patients. 2. Wheelchair cushion to assist with pressure relief: Although pressure-relieving devices are beneficial, they do not take the place of regularly performed pressure-relief or weight-shifting activities. Selecting the proper wheelchair cushion depends on the patient's ability to transfer on and off the cushion and the degree of support needed. 3. Hospital or pressure-relieving bed: Patients with high tetraplegia who are to be discharged to home may require hospital beds, other specialized beds, or air mattresses. 4. ADL adaptive equipment: Examples of items that may be needed include dressing sticks to assist with donning clothing, loops attached to pants to assist with putting them on, button and zipper hooks to assist with securing these items, Velcro straps and elastic shoelaces to increase the ease of donning shoes, bath brushes, handheld shower attachments, and tub benches. Built-up utensils, toothbrushes, and handles may be needed for patients with tetraplegia. Dorsal wrist supports or universal cuffs may be necessary to assist the patient with feeding activities. 5. Environmental control units: Environmental control units interfaced with personal computers, the telephone, and appliances within the home may be recommended. These electronic systems allow the patient with tetraplegia some control over the environment. By activating the environmental control unit, the patient can turn on the lights, television, or other appliances within the home. Referral to a rehabilitation engineer or other provider with expertise in this area is advisable. Home Exercise Program For some patients, discharge from your facility is the end of their rehabilitation. Not all patients receive follow-up services once they are discharged. Therefore, the supervising PT and PTA must design a home exercise program for the patient that will meet the patient’s immediate and long- 814 term needs. It is not reasonable to expect that once a patient is discharged, he or she will spend hours each day performing a home exercise program. The individual will spend a considerable amount of time each day completing ADLs. Thus, the physical therapy team should select only a few activities that will provide the patient with the greatest functional benefits. Things to Consider When Developing a Home Exercise Program Several factors must be considered when developing a home exercise program for your patient. The following is a list of questions you should ask yourself before you finalize the patient’s home program. 1. What activities will the patient be able to perform when he or she is discharged? Will the patient be able to transfer independently? Is progress likely in other functional skills? 2. What motor and cardiopulmonary capacities will the patient need to possess to complete ADLs? Areas to consider include range of motion, strength, flexibility, balance, and vital capacity. 3. How will the patient maintain his or her skin integrity and respiratory status and prevent possible secondary complications? 4. What skills and capacities can the patient maintain by completing his or her daily routine? For example, getting dressed and bathing assist in maintaining upper and lower extremity range of motion. 5. What areas will require extra attention because they are not addressed during routine performance of ADLs? Areas to consider include the maintenance of hip extension and ankle dorsiflexion and cardiopulmonary endurance. In addition to asking these questions about the patient’s motor and cardiopulmonary function, one should also consider the patient and the role of the family or caregivers in designing the home exercise program (Nixon, 1985). As stated earlier, patients who have SCIs must become active problem solvers and must be able to direct and initiate their care. Patients who become reliant on others for making decisions relative to their care may have difficulty in directing a home exercise program. Failure to understand the possible complications of immobility and contractures may lead to lack of interest in a home exercise program. Stretching activities and active wheelchair propulsion each day will do a great deal to assist the patient in maintaining an optimal level of functional independence. Family Teaching As discussed throughout this chapter, family involvement and training are of the utmost importance. Family teaching should be initiated early during the patient’s rehabilitation stay and should not be deferred until a few days before discharge. Family members or caregivers should assist PIs and PTAs with patient transfers, ADL tasks, skin inspection, wheelchair mobility, equipment usage and maintenance, and range-of-motion exercises. We should be patient with family members as they begin to learn these activities because they are often anxious and afraid of causing the patient pain or additional injury. Not only is it important to teach families how to assist patients physically, but families must also be educated about the injury, potential complications, precautions, safety factors, and probable functional outcome. This instruction is best if given over a period of time to give the family member or caregiver adequate time to digest and assimilate information. If the patient is to be discharged home, all individuals responsible for assisting with the care of the patient should demonstrate a level of competence with techniques before the patient’s release from the facility. Community Reentry As the patient prepares for discharge, a final area that must be considered is the individual’s reentry into the community. The patient should be encouraged to resume previously performed activities as his or her level of functional independence and interests warrant. Significant advances have been made in the areas of employment, recreational activities, sports, and hobbies for patients with disabilities. Approximately 34.9% of individuals with SCI are employed 20 years after their injury (National Spinal Cord Injury Statistical Center, 2013). Factors that positively affect employment following injury include younger age, being a white male, higher educational levels, motivation, and prior employment (DeVivo and Richards, 1992). A thorough review of recreational and sports 815 programs is beyond the scope of this text. Quality of Life Research suggests that most individuals who sustain a SCI report that, in time, they achieve a satisfactory quality of life and psychosocial well-being (Lewthwaite et al., 1994). Evidence suggests that the depression often experienced initially after the injury decreases over time, and the individual gains acceptance of the disability. Despite this, individuals with SCI have a decrease quality of life compared with healthy adults and the most pronounced areas are noted in physical functioning and limitations in the ability to carry out physical roles. An individual's social support systems can positively affect the individual’s adjustment to his or her injury. Neurologic level and extent of the injury must also be studied to determine their impact on quality of life (Boakye et al., 2012). Long-Term Health-Care Needs As the population in the United States ages, so do the survivors with SCIs. Investigators have estimated that 40% of individuals with SCIs are more than 45 years old. Research studies are investigating how the normal aging process affects the preexisting musculoskeletal and cardiopulmonary deficits experienced by individuals who have had an SCI and how cumulative stresses sustained from years of wheelchair propulsion, repetitive upper extremity activities, and assisted ambulation may accelerate problems encountered with aging. As patients age, they can experience declines in function and the need to use greater assistance. Fatigue, weakness, medical complications, shoulder pain, weight gain, and postural changes have been attributed to declines in function. Fortunately, many of these functional limitations are amenable to physical therapy intervention, including the procurement of adaptive equipment, seating systems, and power wheelchairs (Gerhart et al., 1993). An important point for health-care providers working with individuals with SCIs is that many of the problems associated with aging and overuse may be preventable through education, health promotion, and wellness activities. Comprehensive follow-up services are extremely important to these individuals and may enhance fitness and decrease the incidence of secondary complications (Gerhart et al., 1993; Somers and Bruce, 2014). Chapter summary Patients with SCIs benefit from comprehensive rehabilitation services to optimize their functional independence. Physical therapy treatment sessions started shortly after the patient’s injury can help improve the patient’s strength, mobility, and cardiopulmonary function. Treatment should continue with admission to a comprehensive rehabilitation center where additional resources can be devoted to the patient’s optimal recovery. Multiple therapeutic interventions and modalities are available to assist the patient in achieving the highest level of functional independence. Emphasizing the patient’s active participation in the rehabilitation process is essential. In addition, patient and family education must be included from the very start of rehabilitation to ensure a successful transition from health-care facility to home. Early discussions with the patient regarding returning to home and work or school assist the patient with reintegration into the community. Adequate long-term follow-up care remains absolutely essential in order to eliminate or minimize the potential secondary complications that can develop in this patient population. Changes in our approach to physical therapy have developed as our understanding of nervous system plasticity have emerged. Review questions 1. List the four most common causes of SCIs. 2. Differentiate between a complete SCI and an incomplete SCI. 3. What are the characteristics of spinal shock? 4. What is autonomic dysreflexia? Describe the clinical manifestations of a patient experiencing this 816 condition. 5. What is the functional potential of a patient with C7 tetraplegia? 6. List three physical therapy interventions that will improve pulmonary function. 7. List the three primary goals of physical therapy intervention during the acute care phase of rehabilitation. 8. Discuss a typical mat exercise program for a patient with C6 tetraplegia. 9. What is the most functional type of wheelchair-to-mat transfer for a patient with C7 tetraplegia? 10. List the benefits of a therapeutic pool program. 11. Discuss the gait training sequence for a patient with paraplegia who will be using orthoses. 12. Describe important areas for patient and family teaching for a patient with SCI. 817 Case studies Rehabilitation Unit Initial Examination and Evaluation History Chart Review The patient is a 20-year-old man who was transferred to the University of Evansville Medical Center 1 week after diving into a shallow wave and hitting a sandbar while surfing. He sustained a teardrop fracture of C5 resulting in a medical diagnosis of C6 incomplete tetraplegia. He aspirated water and lost consciousness. He was initially taken to a local hospital, placed in Gardner-Wells tongs, and treated for aspiration pneumonia. On admission to the Medical Center the patient was conscious and alert. He had decreased breath sounds with crackles over the lateral bases. Light touch and pinprick were intact to T1 with intact perianal sensation. Proprioception was intact in all extremity joints. Computed tomography showed no blockage and surgery was not indicated. X-ray showed diaphragm movement of two intercostal spaces. Past medical history includes childhood asthma and is otherwise unremarkable. Medications: Tylenol for pain as needed. A halo and vest are to be applied tomorrow to provide immobilization of the fracture and to allow for participation in the rehabilitation process. Physical therapy has been ordered for examination and treatment with possible transfer to rehabilitation unit. Subjective The patient states that he is not in pain but that the tongs are annoying. He is a part-time college student and lives at home with his parents. The home is a one-story house with a one-step entry with a railing. At school, all of the buildings have elevators. The patient's goals are to return home to live with his parents and to learn to get around by himself. He gives consent to participate in examination. Objective Appearance, Rest Posture, Equipment: The patient is lying supine in bed with his head in tongs. His arms are in extension at his sides, and his legs are also in extension. He has a Foley catheter in place. IV present left forearm. He is resting on an air fluid mattress. Systems review Communication/Cognition: The patient is alert and oriented x 3. Communication is intact. Yes-no responses are reliable. He is able to follow complex verbal commands with 100% accuracy. Cardiovascular/Pulmonary: BP = 120/75 mm Hg, HR = 70 bpm, RR = 16 breaths/min. Integumentary: Skin is intact. No redness is noted. He is dependent in pressure relief. Musculoskeletal: Gross strength and range of motion (ROM) are impaired bilaterally. No postural asymmetries are noted. Neuromuscular: Movement is impaired bilaterally. Tests and measures Anthropometrics: Height 5'9", weight 160 lbs, Body Mass Index 24 (20-24 is normal). Ventilation/Respiration: Vital capacity is 1,000 mL taken with spirometer in supine. Breathing pattern is 4-diaphragm. Epigastric rise is 1”. Cough is nonfunctional. Range of Motion: Passive ROM: Upper extremity (UE) passive ROM limited bilaterally at shoulders to 90 degrees flexion and abduction due to cervical instability. Shoulder internal and external passive ROM within functional limits (WFL). Elbow, wrist, and hand passive ROM WEL. Lower extremity (LE) passive ROM WFL except passive straight leg raise limited to 60 degrees bilaterally. Active ROM: UE active ROM limited bilaterally at shoulders to 90 degrees flexion and abduction due to cervical instability. No active ROM of neck, trunk, and shoulders past 90 degrees due to cervical instability. Bilateral elbow flexion WFL. Bilateral wrist extension WEL. All other joints: no active ROM noted. Reflex Integrity: Deep tendon reflexes: biceps: 2 + bilaterally. Triceps, patellar, and Achilles: 0 bilaterally. Babinski present bilaterally. There is a mild increase in tone bilaterally in ankle plantar flexors and hamstrings. Motor Function: The patient is dependent in log rolling and all other motor functions. 818 Neuromotor Development: Unable to assess postural reactions secondary to spinal instability. Muscle Performance: All testing was done in the recumbent position. Neck, trunk, and shoulder girdle muscles limited to trace and humeral active motion only without resistance due to cervical instability. 1/5 3/5 3/5 3/5 Finger abductors Hip flexors Knee extensors Ankle dorsiflexors 0/5 Long toe extensors 0/5 Ankle plantar flexors} 0/5 Gait, Locomotion, Balance: The patient is dependent in gait and locomotion. He is limited to recumbent position due to cervical instability. Sensory Integrity: Light touch and pinprick intact through T1, absent below; perianal sensation intact. Proprioception: intact in all UE and LE joints. Self-Care: Patient is dependent in all self-care activities. Assessment/evaluation The patient is a 20-year-old man. His status 1 week after C5 teardrop fracture shows a neurologic level at C5 with an incomplete lesion and anterior cord syndrome. ASIA Impairment Scale: C Motor Incomplete Functional Independence Measure: transfer—1, walk/wheelchair—1 (wheelchair), stairs—1 Problem list 1. Decreased respiratory function 2. Decreased tolerance to upright 3. Decreased strength all intact muscle groups 4. Decreased passive ROM of hamstrings 5. Dependent in pressure relief and skin inspection 6. Dependent in mobility and ADLs 7. Lack of patient and family education Diagnosis Patient exhibits impaired motor function, peripheral nerve integrity, and sensory integrity associated with nonprogressive disorders of the spinal cord. He exhibits neuromuscular APTA Guide pattern 5H. Prognosis Patient will improve his level of functional independence and functional skills as muscle strength and stability of the cervical spine improve. Rehabilitation potential for stated goals is good. The patient is motivated and has good family support and financial resources. Physical therapy visits in acute care: up to 10 visits with continuation to rehabilitation up to 150 additional visits. Short-term goals (2 Weeks) . Patient will tolerate being upright in wheelchair for 2 consecutive hours. . Patient will increase strength of innervated UE muscles by one muscle grade. . Patient will perform pressure relief and skin inspection with minimal assist of 1. . Patient will perform bed/mat mobility with moderate assist of 1. . Patient will perform a lateral transfer with a sliding board with maximal assist of 1. . Patient will propel wheelchair with rim projections 25 feet with minimal assist of 1. . Patient will maintain balance in short sitting with elbows biomechanically locked for 5 minutes independently. 8. Patient will require moderate assist of 1 to perform assisted cough. ND OB WN Fe Long-term goals (6 Weeks, the Anticipated Discharge to Home with Family) 1. Patient will be independent in diaphragm-strengthening exercises and assisted cough techniques. 2. Patient will tolerate being upright in his wheelchair for 8 consecutive hours. 3. Patient will increase strength of innervated UE muscles to 5/5. 4. Patient will increase passive ROM of hamstrings to at least 90 degrees to allow for long sitting. 819 5. Patient will be independent in pressure relief and skin inspection. 6. Patient will be independent in bed/mat mobility. 7. Patient will perform a modified prone-on-elbows transfer independently. 8. Patient will independently propel wheelchair with rim projections over level surfaces and ramps. 9. Patient will perform ADLs with minimum assist of 1. 10. Patient will be able to direct someone how to help him get back into the wheelchair in case of a fall. 11. Family will demonstrate how to assist patient with ADLs, transfers, home exercise program, and stretching. Plan Treatment Schedule: The PT and PTA will see the patient for 45-minute treatment sessions twice a day 5 days a week, and once on Saturday for the next 6 weeks. Treatment sessions will include improving tolerance to upright, respiratory training, strength training, stretching, pressure relief and skin inspection, functional mobility training, family education, and discharge planning. A home assessment will be recommended. The physical therapy team will reassess the patient weekly. Coordination, Communication, Documentation: The PT and PTA will communicate with the patient and his family on a regular basis. The acute-care PT will communicate with the rehabilitation team on his discharge from this facility. Outcomes of physical therapy interventions will be documented on a daily basis. Patient/Client Instruction: The patient and his family will be instructed in stretching exercises and pressure-relief techniques as his condition stabilizes. In rehabilitation, the patient’s family will participate in family training to learn to assist him with ADLs, transfers, and functional mobility activities. Procedural interventions 1. Improve tolerance to upright: a. Elevate head of bed, monitoring vitals, and gradually increasing length of time in this position b. Sitting in a reclining wheelchair with footrests elevated, monitoring vitals, and gradually increasing length of time and decreasing amount of recline c. Standing on a tilt table, monitoring vitals, and gradually increasing incline and length of time 2. Respiratory training: a. Manual chest wall stretching b. Teach huffing c. Assisted cough techniques in supine progressing to prone, short sitting, and then long sitting d. Inspiratory strengthening with manual resistance progressing to weights 3. Strength training: a. Isometric strengthening of neck, trunk, and shoulder girdle muscles with halo in place after receiving approval from physician b. Active movements of humerus without resistance (limited to 90 degrees of flexion and abduction) c. Biceps strengthening against gravity progressing to using TheraBand or cuff weights 4. Stretching: a. Passive stretching of hamstrings and other lower extremity muscles by therapist b. Prolonged stretching of hamstrings using overhead sling in bed 5. Skin inspection and pressure relief: a. Instruct on the importance of pressure relief and skin inspection b. Implement a turning schedule for when patient is in bed c. Implement prone-positioning program —at least 20 minutes in prone three times a day d. Teach weight-shifting techniques while in wheelchair—1 minute of pressure relief for every 15 to 20 minutes of sitting e. Teach skin inspection techniques using mirror 6. Functional mobility training: a. Mat activities—gradually decreasing amount of assistance while rolling prone over a wedge b. Transition to prone on elbows c. Rhythmic stabilization, alternating isometrics in developmental positions d. Weight shifting in prone-on-elbows transition to supine e. Pull-ups using therapist’s hands 820 f. Transition to supine on elbows g. Rhythmic stabilization, alternating isometrics, and weight shifting in supine on elbows h. Transition to long sitting once hamstring range is sufficient i. Teach elbow locking and rhythmic stabilization, alternating isometrics in long sitting . Transfers— gradually decreasing amount of assist: a. Assisted sliding board transfer with elbow locking initially progressing to prone on elbows independently b. Bed to wheelchair c. Wheelchair to car d. Toilet transfers . Wheelchair mobility gradually decreasing amount of assistance: a. Education about wheelchair parts (armrests, footrests, etc.) and how to use them to propel wheelchair over level surfaces, gradually increasing distance b. Propel wheelchair up and down ramps c. Educate on how to safely fall/tip over in wheelchair d. Educate caregiver in how to assist the patient in getting back into wheelchair after a fall . Family education: a. Educate family members on appropriate ways to assist with transfers b. Have family members assist with transfers c. Educate family on how to assist with ADLs d. Have family demonstrate assistance with ADLs 10. Discharge planning: a. Consult with other members of rehabilitation team, patient, and family regarding discharge to home with assistance of family b. Perform home and school assessment as needed c. Secure equipment such as universal cuff, sliding board, pressure reducing bed d. Obtain lightweight wheelchair with ROHO cushion, projection rims, push handles for pressure relief, swing-away desk arms, and swing-away leg rests with heel loops e. Instruct patient in home exercise program and long-term fitness program to address cardiopulmonary fitness, flexibility, and strengthening 11. Refer patient to driver’s training and vocational rehabilitation Questions to think about m What type of specific upper extremity strengthening exercises should be included in the patient’s plan of care? = How can aerobic conditioning be included in the patient's treatment program? m What types of activities or exercises would be included as part of the patient’s home exercise program? 821 References Alvarez SE. Functional assessment and training. In: Adkins HV, ed. Spinal cord injury. New York: Churchill Livingstone; 1985:131-154. American Spinal Injury Association (ASIA). International standards for neurological classification of spinal cord injury. Atlanta, GA: ASIA; 2013. 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Phys Ther. 2002;82:707-715. Finkbeiner K, Russo SG, eds. Physical therapy management of spinal cord injury: accent on independence. Fishersville, VA: Woodrow Wilson Rehabilitation Center, through Project Scientia, a grant from the Paralyzed Veterans of America; 1990:51-58. Fulk GT, Behrman AL, Schmitz TJ. Traumatic spinal cord injury. In: O’Sullivan SB, Schmitz TJ, Fulk GT, eds. Physical rehabilitation. ed 6 Philadelphia: FA Davis; 2014:889-963. Fuller KS. Traumatic spinal cord injury. In: Goodman CC, Fuller KS, eds. Pathology implications for the physical therapist. ed 3 St Louis: Saunders; 2009:1496-1516. Gerhart KA, Bergstrom E, Charlifue SW, et al. Long-term spinal cord injury: functional 822 changes over time. Arch Phys Med Rehabil. 1993;74:1030-1034. Giesecke C. Aquatic rehabilitation of clients with spinal cord injury. In: Ruoti RG, Morris DM, Cole AJ, eds. Aquatic rehabilitation. Philadelphia: JB Lippincott; 1997:134-150. 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Clinical trial of exercise for shoulder pain in chronic spinal cord injury. Phys Ther. 2006;86(12):1604—1618. Nixon V. Spinal cord injury: a guide to functional outcomes in physical therapy management. Rockville, MD: Aspen Systems; 1985 pp 41-66, 177-188. Scelza W, Shatzer M. Pharmacology of spinal cord injury: basic mechanism of action and side effects of commonly used drugs. J Neurol Phys Ther. 2003;27(3):101-108. SCI Action Canada. Physical activity guidelines for adults with spinal cord injury. 2011. http://sciactioncanada.ca/docs/guidelines/Physical-A ctivity-Guidelines-for-Adults-with-a- Spinal-Cord-Injury-Health-Care-Professional.pdf Accessed September 15, 2014. Somers MF. Spinal cord injury functional rehabilitation. ed 3 Boston, MA: Pearson; 2010 pp 527- 551, 67, 130, 136-153, 194-198, 29-300, 345-346. Somers MF, Bruce J. Spinal cord injury. Clinical Summaries American Physical Therapy Association; 2014. http://www.ptnow.org/Clinicalsummaries.aspx Accessed September 15, 2014. U.S. Food and Drug Administration. FDA allows marketing of first wearable, motorized device that helps people with certain spinal cord injuries to walk. van Middendorp JJ, Hosman AJF, Donders ART, et al. A clinical prediction rule for ambulation outcomes after traumatic spinal cord injury: a longitudinal cohort study. Lancet. 2011;377(Mar):1004—1010. Wetzel J. Respiratory evaluation and treatment. In: Adkins HV, ed. Spinal cord injury. New York: Churchill Livingstone; 1985:75-98. Yarkony GM, Chen D. Rehabilitation of patients with spinal cord injuries. In: Braddom RL, ed. Physical medicine and rehabilitation. Philadelphia: WB Saunders; 1996:1149-1179. 823 CHAPTER 13 824 Other Neurologic Disorders Objectives After reading this chapter, the student will be able to: 1. Describe the incidence, etiology, and clinical manifestations of Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Guillain-Barré syndrome, or postpolio syndrome. 2. Understand the typical medical and surgical management of persons with Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Guillain-Barré syndrome, or postpolio syndrome. 3. Identify specific treatment interventions relative to the stage or degree of progression, activity limitations, and participation restrictions of persons with Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Guillain-Barré syndrome, or postpolio syndrome. 4. Discuss strategies for patient/family education to address functional limitations in persons with Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Guillain-Barré syndrome, or postpolio syndrome. 825 Introduction Many neurologic disorders are chronic in nature such as Parkinson disease (PD) and multiple sclerosis (MS), and some are progressive in nature such as amyotrophic lateral sclerosis (ALS) and Guillain-Barré syndrome (GBS). ALS is a terminal degenerative disease of the upper motor neurons (UMNs) and lower motor neurons (LMNs). Individuals with postpolio syndrome (PPS) experience new symptoms decades after having overcome polio. Recovery is not expected in these neurologic disorders, except for individuals with GBS. GBS is a peripheral as opposed to a central nervous system (CNS) phenomenon, and remyelination of nerves can occur. Parkinson disease and multiple sclerosis are both progressive disorders. Despite that fact, life expectancy in all of the neurologic conditions discussed, except ALS, is not usually seriously diminished. There are a few exceptions such as when the cardiopulmonary system is involved or there is rapid progression of the disease. ALS is a major exception as death usually occurs within 4 years of diagnosis. Regardless of whether the disease is acute or chronic, or whether recovery occurs as part of the pathologic process, physical therapy can assist these individuals and their families to function optimally and participate in their life. Intervention strategies must relate to the level of involvement and stage of disease progression or, in some cases, recovery of abilities. For example, a person diagnosed in the early stages of MS, PD, or even ALS may be able to participate in a moderately intense exercise program while a person in the later stages of PD, MS, or ALS would not. Exercise and other physical therapy interventions must be specific to the type and severity of the movement dysfunction. For example, in a patient with MS who exhibits ataxia (a condition of too much movement), stability is more important than mobility. However, in PD where the body, especially the trunk, exhibits rigidity, mobility is more important than stability. As muscle weakness progresses in ALS, the person is able to do less and interventions move from being restorative or preventative in nature to compensatory and palliative. Fatigue is an ever-present finding or concern in all of the neurologic disorders discussed in this chapter, and its management must be an integral part of any plan of care. Each disorder will be presented with its clinical features, incidence and etiology, physical therapy goals, and sample interventions. 826 Parkinson disease Parkinson disease (PD) was first described in 1817 by James Parkinson in an essay on the shaking palsy. It is a chronic, progressive neurologic condition that affects the motor system. The four primary symptoms are bradykinesia (slowness of movement), rigidity, tremor, and postural instability. These symptoms are caused by a decrease in dopamine (DA), a neurotransmitter, stored in the substantia nigra. The substantia nigra is a component of the basal ganglia (see Chapter 2, Figure 2-6). The basal ganglia are primarily responsible for the regulation of posture and movement. Lesions in the basal ganglia change the character of movement rather than produce weakness or paralysis (Fuller and Winkler, 2009). In actuality, parkinsonism is a group of disorders involving dysfunction of the basal ganglia. The most common type of parkinsonism is primary parkinsonism or PD. It is also known as idiopathic Parkinson disease (IPD) because there is no apparent cause. Other types of parkinsonism include secondary parkinsonism and Parkinson-plus syndromes. Secondary parkinsonism occurs as a result of other conditions and can be associated with encephalitis, alcoholism, exposure to certain toxins, traumatic brain injuries, vascular insults, and use of psychotropic medications. Long-term use of medications used to control mood and behavior can produce Parkinson-like symptoms. Parkinson- plus syndromes include disorders such as multisystem atrophy, progressive supranuclear palsy, and Shy-Drager syndrome. These syndromes produce other neurologic signs of multiple system degeneration such as cerebellar dysfunction and autonomic system dysfunction (dysautonomia) in addition to the classic signs indicative of degeneration of the DA-producing neurons of the substantia nigra. PD is one of the most common movement disorders in the United States (Sutton, 2009). It is the most prevalent degenerative CNS disorder. PD accounts for 85% of the cases of parkinsonism. Further description and discussion will be confined to primary or idiopathic PD with only minimal references to the other types of parkinsonism. Incidence is 20.5 per 100,000 in the United States and between 5 and 24 per 100,000 worldwide. The incidence is rising as the Baby Boomers age because PD becomes more common with advancing age. Individuals over the age of 85 have a 1 in 3 risk of PD (Aminoff, 1994). Currently, at least a million people are living with PD in the United States (Melnick, 2013). The average age of onset is 62.4 years, with the majority of cases occurring between 50 and 79 years. Ten percent of cases occur before the age of 40. The etiology of Parkinson disease is probably multifactorial because many factors contribute to the clinical entity. Risk factors are increasing age and having an affected family member. Although very few cases of PD are solely genetic in origin, there is evidence to support a role for genetic factors. Also, there is evidence to support environmental factors, such as significant use of pesticide and herbicide, as playing a role in causing the disease process. In all likelihood, there is an interaction between genetic and environmental factors that cause Parkinson disease (Singleton et al., 2013). Pathophysiology Parkinson disease is a disorder of the DA-producing neurons of the substantia nigra in the basal ganglia. The substantia nigra is subcortical gray matter that contains pigmented neurons. As these neurons degenerate, they lose their color. A 70% to 80% loss of neurons occurs before symptoms become apparent. The severity of loss of DA correlates well with the amount of movement slowness or bradykinesia exhibited by the patient. Loss of DA neurons and the production of Lewy bodies within the pigmented substantia nigra neurons are hallmarks of idiopathic PD. Lewy bodies contain neurofilaments and hyaline. They are part of the aging process and are seen in certain vulnerable neuronal populations. Lewy bodies are found in smaller numbers in other neurodegenerative disorders, such as Alzheimer disease, but in different brain areas. DA is both an excitatory and inhibitory neurotransmitter. Because of the role of the basal ganglia in movement initiation and in releasing one movement sequence in order for another one to begin, basal ganglia circuitry is altered. As DA is depleted, some pathways are insufficiently activated while other pathways become hyperactive. Insufficient activity slows movement and affects timing. The cholinergic system becomes more active because of the lack of inhibition from dopamine. Acetylcholine is used by the small interconnecting neurons in the basal ganglia. The increased 827 cholinergic activity means more acetylcholine and causes an increase in muscle activity on both sides of a joint. This results in symptoms of rigidity and further slowing of movement or bradykinesia. Clinical Features Clinically, a patient with PD exhibits bradykinesia, rigidity, tremor, and postural instability. Bradykinesia is particularly evident in the performance of activities of daily living (ADLs). Slowing of oral movements can result in poor speech intelligibility and inadequate breath support often manifested as a soft monotone voice. Swallowing may become impaired. Handwriting can be cramped and small; an occurrence known as micrographia. Akinesia is an inability to initiate movement such as rising from a chair, turning in bed, or simply crossing the legs. As movement slows, the patient tends to adopt a fixed forward-flexed posture, and the ability to extend against gravity is lost. Rigidity occurs in the trunk and the extremities. An early sign of this problem occurs when the individual loses the ability to swing the arms during walking. Rigidity is resistance to passive movement regardless of the speed of the movement. Two forms of rigidity, lead-pipe and cogwheel, can be demonstrated in a person with PD. In lead-pipe rigidity, there is constant resistance to passive limb movement in any direction regardless of speed. Cogwheel rigidity is the result of combining lead-pipe rigidity and tremor. The rigidity causes a catch, and the tremor allows the letting go. This type of rigidity results in a jerky, ratchet-like response to passive movement characterized by a tensing and letting go. Rigidity of the trunk impairs breathing and phonation by restricting chest wall motion. Rigidity can increase energy expenditure throughout the day and its presence may be related to the postexercise fatigue experienced by these patients. Tremor is often the first sign of PD. Because it manifests at rest and disappears on voluntary movement, it is classified as a resting tremor as opposed to an intention (on action) tremor. The tremor of the hand has a regular rhythm (4 to 7 beats per second) and is described as “pill-rolling.” Tremors can also occur in the oral area or within postural muscles of the head, neck, and trunk. Tremors may begin unilaterally and progress over time to all four limbs and the neck. Tremors rarely interfere with ADLs. Postural instability is a very serious problem for patients with PD and is a major reason for restriction in a person’s activities and participation in life. Loss of postural extension and the inability to respond to expected and unexpected postural disturbances can cause falls. A person’s fall potential increases the longer the person has the disease. People with PD also have lower confidence in being able to avoid a fall while performing ADLs than healthy controls (Adkins et al., 2003). Whether an increased fear of falling further contributes to a greater risk of falling in this population is yet to be determined. Visuospatial deficits and slow processing of sensory information related to balance do contribute to postural instability (Melnick, 2013). The person with PD does not accurately perceive proprioceptive and kinesthetic input (Konczak et al., 2009). Patients with PD mix hip and ankle strategies, which produces maladaptive balance responses (Horak et al., 1996; Horak et al., 2005). Anticipatory postural responses were found to be poor or absent in several studies (Glatt, 1989; Mancini et al., 2009). Abnormal postural responses result from an inability to distinguish self-movement from movement of the environment. The person with PD is overdependent on vision for movement cues and cannot make use of vestibular information from the inner ear to make appropriate postural responses (Bronstein et al., 1990). Other typical features of PD include a flexed posture, masked facies, dysphagia, festinating gait, freezing episodes, and fatigue. Postural deficits include flexion of the head, neck, and trunk, which create a forward displacement of the center of gravity (Figure 13-1). However, exaggeration of flexion in the hips and knees may assist in bringing weight more posteriorly. Over time these postural changes become fixed because of the rigidity of the trunk and have been described as flexion dystonia. Loss of trunk extension occurs early in the disease, followed by loss of rotation and subsequent loss of arm swing. The face becomes rigid and shows little or no facial expression. As oral structures lose their ability to move and become rigid, swallowing becomes more and more difficult, leading to concerns about the person’s nutritional intake. 828 ( Tremor ——\ { Masklike face posture Arms flexed at elbows and wrists Hips and knees slightly flexed Tremor FIGURE 13-1 Typical posture that results from Parkinson disease. (Modified from Monahan FD, Neighbors M: Medical-surgical nursing: foundations for clinical practice, ed 2, Philadelphia, 1998, WB Saunders. In Copstead LEC, Banasik JL: Pathophysiology, ed 3, St. Louis, 2005, Elsevier Saunders.) The gait of a person with PD is shuffling, punctuated by short steps and a progressive increase in speed as if trying to catch up. This is called festination. If festination occurs while walking forward, it is referred to as propulsion; if it occurs while walking backward, it is referred to as retropulsion. Foot clearance is decreased because of the short, slow shuffling, therefore increasing the person’s risk for falling. Freezing occurs when the person becomes stuck in a posture. This usually occurs while walking and can be triggered by environmental situations, such as a doorway or change of floor surface. Freezing episodes can occur at any time, such as when making arm movements, speaking, or blinking. Festinating gait, postural dysfunction, and freezing of gait (FOG) are three contributing causes of the postural instability seen in patients with PD. Fatigue Fatigue contributes to postural instability because of the difficulty the person with PD experiences while trying to sustain an activity. Fatigue affects 50% of this population and is often one of its most disabling effects (Friedman and Friedman, 2001). People with PD exhibit lethargy as the day progresses. A sedentary lifestyle with decreased activity contributes to general deconditioning. Fatigue is strongly correlated with high emotional distress and low quality of life in patients with PD who are nondemented or depressed (Herlofson and Larsen, 2003). Patients with increased levels of fatigue are more likely to be sedentary and have poorer levels of physical function than those with lower levels of fatigue (Garber and Friedman, 2003). Gait Up to a third of patients with PD initially present with postural instability and gait disturbances (PIGD) that constitutes a group (O’Sullivan and Bezkor, 2014). Gait speed is slow with a narrow 829 base and a characteristic festination or shuffling. Arm swing is lost early in the disease process. Posture becomes more and more forwardly flexed and lower extremity range of motion (ROM) becomes more and more restricted. Heel strike and toe-off are both lost, resulting in decreased foot clearance. Because of an inability to change a motor program once it has begun, the person has difficulty altering gait speed or stride length in response to changes in environmental demands. Bradykinesia and rigidity are the causes of the absent arm swing and trunk rotation seen during typical ambulation and turning. Bond and Morris (2000) demonstrated that the gait dysfunction in persons with PD got worse when they were asked to perform a complex task while walking. Difficulty stopping a motor program, such as when walking or running, predisposes the person with PD to slips, trips, and falls (Morris and Iansek, 1997). Falls Falls are a very common problem in persons with PD. Forty-eight percent of early-stage optimally medicated individuals with PD reported a fall in a study by Kerr et al. (2010). Schrag et al. (2002) found that 64% of their community-based subjects with PD had experienced falls with postural instability. Self-selected gait speed can be used to predict fall risk in individuals with PD (Nemanich et al., 2013). A community-dwelling older adult with PD is twice as likely to experience a fall as is a community-dwelling older adult without PD (Wood et al., 2002). Additionally, it was found that previous falls, disease duration, dementia, and loss of arm swing were predictors of falling. Therefore, people with PD who have fallen previously are more likely to fall again, and individuals with dementia or loss of arm swing are more likely to fall. FOG increases the risk for falling (Bloem et al., 2004). The longer a person has PD, the greater the risk for falling. Systemic Manifestations Half of the individuals with PD exhibit dementia and intellectual changes caused by the neurochemical changes in the basal ganglia (Fuller and Winkler, 2009). Dementia along with bradyphrenia, depression, and dysautonomia are systemic manifestations of the disease. Bradyphrenia is a slowing of thought processes. It is usually accompanied by a lack of ability to attend and concentrate. Low motivation and passivity can also be related to depression or to sensory deprivation from a lack of movement. Depression is common in patients with PD and some researchers think that depression may begin even before the onset of PD (Fuller and Winkler, 2009). Stages The Hoehn and Yahr classification of disability (Hoehn and Yahr, 1967) (Table 13-1) is used to stage the severity of involvement of PD. New stages have been added to better describe the progression of the disease. Stage 0 indicates no signs of the disease. Stage 1 indicates minimal disease and stage 5 indicates that the person is in bed or using a wheelchair all of the time. In addition to stage 0, there are stages 1.5 and 2.5 (Goetz et al., 2004). The average patient shows slow, gradual progression of the disease over a period of 5 to 30 years. Therefore, the life expectancy of someone with PD is only a little shorter than someone without PD of the same age (Weiner et al., 2001). Table 13-1 Hoehn and Yahr Staging Scale for Parkinson Disease Stage Progression of Symptoms 0 No signs of disease 1 Unilateral symptoms onl 15 Unilateral and axial involvement Bilateral symptoms, no impairment of balance Mild bilateral disease with recovery on pull test Balance impairment, mild to moderate disease, Severe disability, but still able to walk or stand unassisted Needing a wheelchair or bedridden unless assisted Modified from Goetz CG, Poewe W, Rascol O, et al: Movement Disorder Society Task Force report of the Hoehn and Yahr staging scale: Status and recommendations. Mov Disord 19:1020—1028, 2004. The Hoehn and Yahr scale is commonly used to describe how the symptoms of Parkinson disease progress. The original scale included stages 1-5. Stage 0 has since been added, and stages 1.5 and 2.5 have been proposed to best indicate the relative level of disability in this population. Diagnosis 830 There is no diagnostic test for Parkinson disease; therefore diagnosis is based on the person’s clinical presentation of signs and symptoms and history. Presence of two of the four cardinal features and exclusion of the Parkinson-plus syndromes is usually employed to make the diagnosis (O'Sullivan and Bezkor, 2014). The Parkinson-plus syndromes do not respond typically to anti- Parkinson medication. Neuroimaging and lab tests are usually normal unless there are coexisting morbidities. Medical Management The mainstay of medical management of patients with Parkinson disease is pharmacologic. Selegine also called deprenyl (Eldepryl) or rasagiline (Azilect) are often used as first medications after diagnosis because they delay the need for giving levodopa (L-dopa). These monoamine oxidase (MAO) inhibitors block the breakdown of dopamine and are thought to slow the progression of PD and delay the need for replacement medication for up to a year (Sutton, 2009). The major mainstay in treatment of Parkinson disease remains L-dopa, which is used to replace the lost DA. It works best to decrease rigidity and make movement easier. Dopamine cannot be given because it cannot cross the blood-brain barrier (BBB). L-dopa can cross the BBB. However, because a lot of the L-dopa gets broken down before it reaches the brain, scientists add carbidopa to the L-dopa to delay its breakdown. This addition allows more L-dopa to reach the basal ganglia and smaller doses of medication can be given. Sinemet is the brand name of a commonly used combination of carbidopa and L-dopa. Anticholinergics are medications that block the increase in acetylcholine that results from the decrease in available DA. Anticholinergics are helpful in reducing the resting tremor but have little or no effect on the other symptoms including postural instability. A list of medications and their intended use is found in Table 13-2. The physical therapist should alert the physical therapist assistant to look for possible side effects of the patient’s medications. Table 13-2 Medications Used for Neurologic Disorders e lerate tremor and dystonia associated with wearing off in PD RRMS Copaxone RRMS, CIS. Cortisone, corticosteroids, prednisone| Shorten acute attack in MS Ditropan Bladder urgency and frequency in MS ra Immunoglobulins Duration and severity of GBS 7 Lioresal Spasticit Novatrone SPMS, PRMS, advanced RRMS, IV delivery Parlodel End-of-dose “wearing off” and dyskinesias in PD Probanthine Bladder urgency and frequency in MS Fatigue in MS RRMS Bradykinesia, rigidity, and motor fluctuations in PD Sinemet IR or CR Bradykinesia and rigidity in PD Symmetrel Bradykinesia and rigidity in PD Fatigue in MS, PPS Tonic spasms in MS RRMS not used initially, IV delive Urecholine Urinary retention in MS Valium Night spasms in MS CIS, clinical isolated syndrome; CR, controlled release; GBS, Guillain-Barré syndrome; /R, immediate release; MS, multiple sclerosis; PD, Parkinson disease; PPS, postpolio syndrome; PRMS, progressive relapsing multiple sclerosis; RRMS, relapsing- remitting multiple sclerosis; SPMS, secondary progressive multiple sclerosis. Unfortunately, with long-term use, L-dopa becomes less effective therapeutically. The medication usually works for only 4 to 6 years before its benefits are no longer evident. As the medication benefits decrease, other movement problems occur such as motor fluctuations, dyskinesias, and dystonia. Motor fluctuations are times when symptoms increase because the L-dopa is no longer able to cause a smooth and even effect. These times are also called “on/off” fluctuations or “on/off” phenomenon. Dyskinesias are involuntary movements involving the face, oral structures, head, trunk, or limbs. The timing of dyskinesias can vary. In some individuals, they may occur at the peak effect of the medication. This is the most common pattern. For other individuals, they occur at the beginning or end of a dose. The medication-induced dyskinesias can be reversed by decreasing the dose of anti-Parkinson medication given; however, the tremors, slowness of movement, and gait difficulties worsen. Therefore, some patients prefer to experience the dyskinesias rather than have 831 more severe PD symptoms. Dystonia is a twisting or torsion of body parts caused by a prolonged involuntary contraction. Patients report toe clawing or cramping of back, neck, face, and calf muscles. Wearing-off phenomenon is the deterioration of movement often noted at the end of the time-frame of medication. The therapist needs to be familiar with all of the medications a patient with PD is taking and their side effects. Balancing medications is very challenging in this patient population. Surgical Management Deep brain stimulation (DBS) has emerged as a viable treatment option for patients with PD. Electrodes are implanted into the brain to stop nerve signals that produce symptoms. DBS is safer than formerly used surgical ablation or destruction of structures because it is reversible. Electrodes are implanted into the subthalamic nucleus (STN) with a stimulation box placed subcutaneously in the subclavicular area much like an implantable cardiac pacemaker. The stimulation can be turned on and off by the patient. The amount of stimulation delivered is determined by the physician. Infection and hemorrhage are potential surgical risks. DBS reduces the need for medication and, therefore, the dyskinesias that accompany long-term use of L-dopa. Benefits of STN-DBS include improvement of all motor symptoms such as tremor, rigidity, and bradykinesia but variable results for gait (Kelly et al., 2006). Recent studies have found selective improvements in daily activities, freezing of gait, and turning performance (Rochester et al., 2012; Nui et al., 2012; Lohnes and Earhart, 2012). Physical Therapy Management Patients may be thought to present in three broad categories: tremor predominant, bradykinesia/akinesia, and rigidity/postural instability/gait difficulty. Goals can be related to the type of presentation on examination, but there is considerable overlap. Physical therapy is a beneficial adjunct to medication for people with PD (de Goede et al., 2001; Melnick, 2013; Morris, 2000). The primary physical therapy goal is to maximize function in the face of progressing pathology. Therefore the focus should be on early intervention. Gait hypokinesia or slowness affects almost everyone with PD. Stride length continues to shorten as the disorder progresses. Therefore teaching the patient strategies to move more easily is of utmost importance (Morris et al., 1998). A second goal is to prevent secondary sequelae, such as deconditioning, musculoskeletal changes related to stiffness, and loss of extension and rotation. Most individuals with PD succumb to respiratory infections (Melnick, 2013). The longer a person with PD is mobile, the less likely he or she is to develop pneumonia. Physical therapy interventions should focus on slowing the onset of predictable changes in posture, locomotion, and general activity level. Gait Interventions The physical therapist needs to ascertain the cause of the gait disturbance to pick the correct strategy for intervention. The physical therapist assistant should also understand the rationale behind the selected gait intervention. One of the assistant’s major roles with this population is to educate the patient and the family members about the importance of good posture and daily walking and the benefits of sustained activity. Using visual and auditory cues to improve attention during a movement task are strategies that appear to be helpful in treating the gait hypokinesia (Frazzitta et al., 2009; Nieuwboer et al., 2009). Walking while holding onto poles can vary the motor program enough to elicit a faster gait. Markers can be placed on the floor and the person directed to step on or over them. Walking toward a mirror allows use of visual feedback to maintain an upright trunk. This strategy can be helpful in the early and middle stages. Attentional strategies can also be used to enhance walking including having the person think about taking long strides, mentally rehearsing the path to be taken before walking, and avoiding any additional mental or secondary motor tasks during walking (Morris et al., 2001). In general, regardless of the task, breaking down the task into its component parts so the person can focus attention on each part separately is a very useful strategy (Morris, 2000). Step hesitation is often the beginning of gait problems for the patient with PD. Anticipatory postural adjustments (APAs) depend on proprioceptive awareness of the changes in weight displacement during step initiation (Mancini et al., 2009). Mancini et al. (2009) found that 832 medial lateral anticipatory adjustments were smaller in individuals with early and untreated PD. An accelerometer on the trunk can be used to measure APA. Proprioceptive deficits may appear before motor deficits in PD (Konczak et al., 2009). Slow gait in PD is characterized by a short stride so a way to document change in response to practice is to measure stride length before and after intervention. A measurable goal could be that the person would increase stride length by a certain amount or take less steps for a given distance. Practice alternative walking patterns, such as side stepping, walking backward, braiding, and marching to various rhythms. Giving the person a mark on the floor to work toward or footprints to try and match or step on can also be helpful. Peripheral movement cues to walk are useful. The assistant would stand slightly to the side of the patient so that the patient could see his or her move as the request to walk is given. Freezing strategies that are often employed include having the person kick a box or pick up a penny. Freezing tends to happen in more confined spaces, such as going through a doorway. However, it can happen in an open environment, so several strategies need to be kept in mind. There are no definitive guidelines regarding the use of assistive devices in persons with gait difficulty secondary to PD (Melnick, 2013). The physical therapist will make a determination of the efficacy of using an assistive device. Use of a cane or a walker will depend on the degree of coordination present in the upper and lower extremities. A rolling wheeled walker with pushdown brakes can be helpful for some people, whereas a reverse-facing walker may assist the person who loses balance in a backward direction. Regardless of the device, it should be adjusted to promote trunk extension not flexion. A U walker projects a laser line for the person with PD to step over. Research is being done on developing glasses that would project lines in the same manner. A cane may be useful during a freezing episode. The person can turn it upside down and use it as a cue to continue walking. To date, no one assistive device has been found to be correct for everyone nor is everyone going to be able to benefit from using a device all of the time. Postural Interventions Because trunk extension and rotation are lost early in the disease process, exercises to strengthen postural extensors are important to emphasize soon after diagnosis (Bridgewater and Sharpe, 1998). Additionally, stretching exercises for tight pectorals are indicated if these muscles are shortened, thus preventing thoracic trunk extension. Stretching heel cords is indicated to maintain a plantigrade foot and normal weight transfer during gait. Rotational exercises of the trunk and limbs, such as those depicted in Intervention 13-1 and 13-2, have routinely been recommended. Rotational exercises were used to decrease the incidence of freezing in a small group of patients with advanced stage PD (Van Vaerenbergh et al., 2003). Rhythmic initiation, a PNF technique, can be used to assist the person to begin a movement or increase the ROM through which the movement occurs (see Chapter 9). This technique is most helpful when the patient is performing functional patterns of movement such as rolling and coming to sit or stand. Intervention 13-1 Rotational Activities in Supine 833 834 Rotational exercise sequence in supine can be used to increase range of motion (ROM) of the neck and trunk. Any combination of motions can be used. A. The head is rotated slowly side to side within the available ROM while lower extremities are rotated side to side in the opposite direction. B. The upper extremities are positioned in 45 degrees of shoulder abduction with 90 degrees of elbow flexion. One shoulder is externally rotated while the other shoulder is internally rotated. From this initial position, the shoulders are slowly rotated back and forth from an internally to an externally rotated position. C. Advanced exercise: The head, shoulders, and lower extremities are rotated simultaneously from one position to the other. The head rotates opposite to the hips providing for counterrotation within the trunk. The upper extremity on the face side is externally rotated while the other arm is internally rotated. (Modified from Turnbull GI, editor: Physical therapy management of Parkinson's disease, New York, 1992, Churchill Livingstone, Fig. j 9-11, p. 177.) Intervention 13-2 Rotational Activities in Side-Lying 835 Side-lying is also a good position to obtain a stretch of the trunk. In side-lying, the thorax is slowly rotated forward and backward relative to the position of the pelvis while the upper extremity is protracted and retracted relative to the thorax. A. Forward view of this movement. B. Posterior view. C. Advanced exercise: The patient rotates the pelvis backward as the thorax is rotated forward. The patient then rotates the pelvis forward as the thorax is rotated backward. These two combinations result in counterrotation of the trunk. 836 (Modified from Turnbull GI, editor: Physical therapy management of Parkinson's disease, New York, 1992, Churchill Livingstone, Fig. 9-11, p. 178.) Relaxation techniques are used to treat rigidity and fatigue (Melnick, 2013; O’Sullivan and Bezkor, 2014). Gentle, slow rocking of the trunk and rotation of the extremities can decrease rigidity. These techniques are best used while the person is sitting because in a supine position rigidity may be increased. Also, rhythmical rotation should be started proximally and then applied distally as proximal muscles are often stiffer than distal ones. After a decrease in rigidity, movement is often easier and less fatiguing. Large movements are especially helpful and need to encompass the entire range and should emphasize extension. Bilateral symmetrical movements are easier than reciprocal ones. The person can then be progressed to the use of diagonal patterns of movement, such as chops and lifts (see Chapter 9). Deep breathing can be done to promote relaxation. The person can be in a comfortable supported position in supine and be taught to take slow deep breaths using the diaphragm. Progress the patient to sitting and standing while still concentrating on using the diaphragm and lateral chest expansion. Complete chest wall expansion is difficult for the patient to obtain because the trunk is often rigid. Therefore, chest wall stiffness and any postural malalignment need to be addressed using visual feedback, stretching, and strengthening exercises. For example, the individual can perform bilateral D, flexion proprioceptive neuromuscular facilitation (PNF) patterns while taking a deep breath, and expiration can be carried out during D, extension. Stretching and flexibility exercises should be performed daily if possible but at a minimum of 2 to 3 days per week. Holding each stretch for 15 to 60 seconds for at least 4 repetitions is recommended (Protas et al., 2009). As the loss of extension is predictable, stretching of cervical, shoulder, trunk, hip, knee, and ankle joints is a must. If the person can lie flat in supine or get into a prone position for any amount of time, it can be beneficial. When implementing a stretching program, it is important to recognize when a deformity is fixed versus flexible. Some patients with PD require multiple pillows to support a permanently kyphotic spinal deformity. Such persons will not be able to regain normal postural alignment and compensations in sitting and lying need to be made. Before the development of fixed contractures, wall and corner stretches for the pectorals and lying over a bolster or towel roll placed along the length of the spine to stretch the axial skeleton are all appropriate interventions. Make automatic postural adjustments throughout the day to perform movement transitions of sit to stand, changing directions while walking, turning, talking and walking, carrying books, and going through a cafeteria line. Postural instability may be a major problem for someone who is moving slowly or for someone with advanced disease and is rigid. People with PD lose the ability to perform simple automatic postural adjustments like standing up straight and rising from a chair. Cognitive coaching can be a powerful tool to give the person with PD to think about a way on performing an activity that used to be done automatically. Telling a person to move his head forward and upward may be all that is necessary to help him rise to standing after many unsuccessful attempts. The exact cognitive strategy may differ from person to person, depending on the movement task and where the sequence is breaking down. Motor learning theory would indicate that practice of specific task is needed in an appropriate environmental context. It is very important to teach family members or caregivers the cognitive strategies that have been successful in therapy. Lee Silverman Voice Treatment (LSVT®) BIG Training BIG is the application of motor training principles used with the voice to train individuals with PD to move more. The premise is that the person with PD perceives that he or she is moving normally and does not recognize how small the movements are being done. By encouraging BIG movements, the person resets kinesthetic awareness of self-generated movements. The individual who uses LSVT BIG undergoes a certification program to be allowed to use this treatment approach. The person must maintain certification by retaking courses at certain intervals. Exercise is a therapeutic medium that has the potential to modify the manifestations of disease in the case of PD (Farley et al., 2008). Eighteen people with PD participated in an intervention program of four times a week using big movements and big stretches. The program lasted 4 weeks. Disease severity based on the Hoen and Yahr classification ranged from stage 1 to 3 with a relatively equal number of participants in each stage. Results of the study showed that subjects increased gait speed and reaching. Those with less severe disease showed greater change. 837 As the tremors usually do not interfere with ADL function, those individuals are not as likely to be seen in physical therapy unless they also have problems with slowness of movement, postural instability, or gait difficulties. The patient and family can be taught strategies to deal with freezing episodes and the slowness in movement transitions, such as coming to stand, turning over in bed, or changing directions while walking. Dyskinesias are the least amenable to therapeutic intervention (Morris et al., 2001). Fatigue is an important determinant of the physical function of persons with PD (Garber and Friedman, 2003). Fatigue can be the cause or result of inactivity; therefore, aerobic conditioning should be begun as soon as the diagnosis of PD is made. The greater the level of fatigue, the less a person with PD participates in leisure activities and in moving around during the day. Additionally, people with PD show a greater decline in activity than age-matched peers (Fertl et al., 1993). However, Canning et al. (1997) believe that with regular aerobic exercise, people with mild to moderate PD have the potential to maintain normal exercise capacity. Therefore, incorporating an aerobic element into movement interventions is strongly suggested (Dean and Frownfelter, 2012). Not only does aerobic exercise provide musculoskeletal benefits but also can keep airway secretions mobilized while maximizing ventilation. Exercise Strategy and Results Exercise is a cornerstone of the intervention strategies used for people with PD. Exercise promotes physical activity, maintains flexibility, improves initiation and fluidity of movement, and decreases postural instability and fatigue. Exercise must be designed within the context of ADLs and should represent the range from practicing writing on lined paper to turning over and getting out of bed. Functional improvement has been seen after 3 months of twice-a-week physical therapy (Yekutiel et al., 1991). Clients were able to demonstrate a decrease in the amount of time it took to stand from a seated position. Teaching strategies for coping with functional problems is a large part of the basic training routine. Strategies used to enhance performance of daily tasks, such as walking, turning around, standing up and sitting down, turning over, and getting out of bed, are clearly described in Table 13-3. Morris (2000) also recommends exercises for upper extremity function, which are depicted in Table 13-4. Table 13-3 Strategies to Enhance Daily Tasks Task Strateg Walking Instruct to walk with long steps Swing arms Place lines on the floor spaced at appropriate step lengths for person’s age and height Turning around Instruct patient to use a large arc of movement Standing up and sitting down Use mental rehearsal before moving Use gentle rocking back and forth before moving Ensure sufficient forward lean to get weight over the feet Increase height of seat or use armrests Turning over and getting out of bed Use a night light Use a lightweight bedcover Use mental rehearsal before moving Use verbal cues to trigger each part of the sequence Sufficient bed height to stand easily Reaching, grasping, manipulating objects, and writing] Mentally rehearse before moving Use the object as a visual cue Break down the task into component parts Use verbal cues for each part of the sequence Avoid distractions or secondary tasks at the same time From Morris ME: Movement disorders in people with Parkinson disease: A model for physical therapy. Phys Ther 80:578-597, 2000. Table 13-4 Exercises for Upper Extremity Function Task Exercises Buttoning Button clothing, practicing with buttons of different sizes and shapes. Handwriting Practice handwriting by doing crossword. puzzles, writing on lined paper, signing name, and filling in forms with multiple boxes, Reaching/grasping} Reach, grasp, and drink from cups of different sizes, shapes, and weights. Lift jars and boxes of different weights onto and off of pantry shelves of different heights. Fine-motor skills__| Pick up grains of rice with the thumb and forefinger and place them in a teacup. + Pick up a straw between the thumb and forefinger and place it in a soda can. Dressin; Practice dressing, such as putting on a coat or sweater using verbal cues, such as “left arm,” “right arm,” and “pull.” Pressing/pushing Practice pushing the correct sequence of telephone buttons to call family, friends, and local businesses while sitting or standing. Folding Fold napkins and place folded paper into envelopes. Pouring Pour water from one cup to another. Opening/closin, Open and close food jars of different sizes. 838 Modified from Morris ME: Movement disorders in people with Parkinson disease: A model for physical therapy. Phys Ther 80:578— 597, 2000, p. 588. 839 Multiple sclerosis MS is a chronic debilitating demyelinating disease of the CNS. It is a disease of young adults between the ages of 20 and 40. The incidence for females is two times higher than for males. The disease is aptly named because sclerotic plaques form throughout the brain and spinal cord. Charcot’s triad of intention tremor, scanning speech, and nystagmus were described as early as 1869. Today, visual problems, such as optic neuritis, are often part of the initial event. However, presentation of symptoms is not always consistent within an individual or from one attack to another. Before the availability of magnetic resonance imaging (MRI), it was more difficult to diagnose a person with MS because the person might present with only one symptom, or symptoms might be mild or remit after a time. MS affects more than a 400,000 people in the United States (Hassan-Smith and Douglas, 2011). The incidence has been reported to be 4.2 per 100,000 (Hirtz et al., 2007). Rates are higher in the United States, Canada, and northern Europe, possibly because people of northern European heritage are more likely to be affected than other racial groups. Incidence is very low in Asians, Eskimos, and North- and South-American Indians (Sutton, 2009). A U.S. study found that black women have a higher risk for MS than black men whose risk is similar to whites (Langer-Gould et al., 2013). MS does, however, have a worldwide distribution. More cases of MS are found in temperate climates with fewer cases closer to the equator. Although the etiology is still as yet unknown, viral infections and autoimmune dysfunction have been implicated. Viral infections can trigger an MS attack, and immune cells are present in acute MS lesions (Fuller and Winkler, 2009). Susceptibility to immune system dysfunction may be inherited but not the disease of MS. Pathophysiology Patches of demyelination occur in the white matter of the brain and spinal cord. Areas of the nervous system with a high concentration of myelin appear white because it is partially composed of fat. In the CNS, myelin is produced by oligodendrocytes. Their destruction leaves the axon unprotected and vulnerable to possible damage. Inflammation accompanies the destruction of the myelin sheath and can lead to axon damage and plaque formation. Plaques are replaced by scar tissue produced by glial cells, and the trapped axons degenerate (Fitzgerald and Folan-Curran, 2002). Glial cells constitute the connective tissue of the nervous system. Because the immune-system response in the brain of a patient with MS is more robust than normal, it may also play a role in plaque formation. Plaques are part of acute or chronic lesions that may be evident on MRI. The areas of the nervous system more likely to be involved include the optic nerve, periventricular white matter, corticospinal tracts, posterior columns, and cerebellar peduncles. Clinical Features Sensory symptoms are often the first signs of MS. The person may complain of “pins and needles” (paresthesias) or abnormal burning or aching (dysesthesias). Visual symptoms occur in 80% of individuals with the disease and can present as decreased visual acuity, inflammation of the optic nerve (neuritis) that causes graying or blurring of the vision, or double vision (diplopia). Nystagmus, also a common symptom, is caused by a lesion of the cerebellum or central vestibular pathways. Nystagmus is an oscillating movement of an eye at rest. The type of nystagmus depends on the direction the eye is moving. Horizontal nystagmus is the most common type although the person may exhibit vertical or rotatory eye movements. Nystagmus is named for the direction of the fast component of the oscillating movement. Motor pathways are involved, as well as sensory pathways in MS. Motor weakness, typically in one or both legs, indicates involvement of the corticospinal tract. Clumsiness in reaching is often seen with the person overshooting the target. Coordination of alternating movements like flexion and extension are impaired resulting in walking difficulty. Gait is often characterized by poor balance and lurching. Ataxia or general incoordination is evident when there is involvement of the white matter of the cerebellum. A postural tremor of an extremity or the trunk may be evident in sitting or standing. Difficulty coordinating oral movements may interfere with speaking and swallowing. Scanning speech is slow with long pauses and lacks fluidity. There is an increased risk 840 for aspiration in a person who cannot adequately coordinate breathing and eating. Fatigue Fatigue is a major problem in people with MS. It is the most frequently reported symptom, slightly ahead of walking difficulty as cited in one study of almost 700 patients with MS (Aronson et al., 1996). Although fatigue is a major symptom of the disease, its relationship to disease severity is weak. In other words, someone does not have to have a severe case of the disease to be severely fatigued. In fact, the fatigue is often out of proportion to the extent of the disease. Despite a decade of research, the underlying pathophysiologic process of fatigue in MS remains obscure. There is no laboratory or physiologic marker of fatigue in patients with MS. Fatigue is worsened by heat. This fact distinguishes it from fatigue seen in healthy individuals or those with other progressive neurologic diseases. Uhthoff phenomenon is the heat-related onset of blurred vision, increased paresthesias, or overwhelming fatigue. It is considered a pseudoattack that is resolved when the body temperature returns to normal. Fatigue has a profound effect on the individual's ability to complete ADLs and to continue to be employed. It is very important to understand the patient’s perception of fatigue, because MS fatigue is closely linked to how the person perceives his quality of life (QOL) and general and mental health (Bakshi, 2003). In a meta-analysis, exercise was found to modify behavior and positively affect the QOL in individuals with MS (Motl and Gosney, 2008). Cakit et al. (2010) found that exercise decreased depression, and Dalgas et al. (2010) saw an improvement in mood, fatigue, and QOL. Cognitive Impairment Half of the patients with MS will experience some degree of cognitive deficit (O’Sullivan and Schreyer, 2014). These deficits range from mild to moderate in severity and may involve problem solving, short-term memory, visual-spatial perception, and conceptual reasoning. Fortunately, only 10% have problems severe enough to interfere with ADLs. Although persons with MS often associate higher levels of fatigue with poorer cognitive performance, a recent study showed that level of fatigue did not affect cognitive performance (Parmenter et al., 2003). Lesions in the frontal lobe can affect executive brain functions such as judgment and reasoning, making the patient cognitively inflexible. Global deterioration of intelligence or dementia is rare but may occur if the disease is the rapidly progressive type. People who have chronic diseases are more prone to depression, and individuals with MS have more bouts of depression than the general population (Patton et al., 2000; Berg et al., 2000). The rates reported in these studies range from 14% to 54%. Higher levels of helplessness were associated with more fatigue and depressive mood in one study (van der Werf et al., 2003). It appears that the experience of fatigue and depression may be mediated by similar factors. Additionally, depression is also related to emotional stability. Patients with MS can demonstrate emotional lability, being euphoric one minute and crying uncontrollably the next. Autonomic Dysfunction Bowel and bladder problems in patients with MS are indicative of involvement of the autonomic nervous system. The bladder can fail to empty completely, leading to urinary retention, and thus setting up a perfect culture medium for bacterial growth. The reflex control of the bowel and bladder can be impaired and lead to constipation or inadequate emptying, urinary frequency, and nocturia (frequency at night). Complete loss of bowel and bladder control, as well as sexual dysfunction, are possible in the later stages of the disease. Some medications used to treat these bladder problems can be found in Table 13-2. Disease Course The course of the disease is unpredictable because its presentation is highly variable. The majority of cases of MS are the relapsing-remitting multiple sclerosis (RRMS) in which there are definable periods of exacerbations and remissions. Exacerbations occur when symptoms worsen acutely and then remit or recover with a time of symptom stability. Symptoms may completely resolve or there may be residual neurologic deficits. The amount of time that passes between attacks or relapses can be as long as a year at the beginning of the disease. The time between attacks may shorten as the disease progresses. Despite the relapsing-remitting course, there is evidence that the disease is 841 active even when symptoms appear stable (Miller et al., 1988). Many individuals with RRMS go on to develop secondary progressive multiple sclerosis. The other three types of MS are primary progressive, secondary progressive, and progressive relapsing. Primary progressive (PPMS) is characterized by a relentless progression without any relapses. This form is rare, affecting only about 10% of those with MS. Secondary progressive (SPMS) begins with relapses and remissions but then becomes progressive with only occasional relapses and minor remissions. Progressive relapsing (PRMS) is progressive from the onset but has clear, acute exacerbations with and without full recovery. Diagnosis The diagnosis of MS continues to be based on clinical evidence of multiple lesions in the CNS white matter, distinct time (temporal) intervals, and occurrence in an individual between the ages of 10 and 50 years old. The cerebrospinal fluid is usually examined for the presence of higher amounts of myelin protein and oligoclonal bands. The former would be elevated during an acute episode and be indicative of immune system involvement. Presence of oligoclonal bands is not specific to MS. If sensory pathways are involved, recording evoked sensory potentials may provide further evidence of demyelination. As vision is often affected, assessing visual evoked potentials can be helpful part of the diagnostic process. MRI is the best tool to assist in confirming the diagnosis of MS. An MRI can visualize small and large lesions. With the proper enhancement, it is possible to tell if the lesions are new and active. McDonald criteria for MS are used to make the diagnosis easier (Polman et al., 2011). Medical Management Medications are the mainstay in the management of MS. The majority of these disease-modifying agents (DMAs) are synthetic immune system modulators developed for the most common form of MS, which is relapsing remitting. They are approved by the Food and Drug Administration for that form but are used off-label for other forms of MS. The purpose of a DMA is to modify the disease and reduce the frequency and severity of attacks. Avonex, Betaseron, and Copaxone modify the disease. Copaxone has been shown to reduce the frequency of attacks. All of the drugs are injected. Avonex is taken weekly, Betaseron every other day, and Copaxone daily. These medications are currently recognized as standard treatment for patients with RRMS. Newer medications such as Tysabri and Novantrone have to be delivered by IV while the person is in a medical center, because constant monitoring is indicated. Individuals may need to try several DMAs to find one that is best tolerated. A person with MS may exhibit myriad symptoms that reflect the diverse areas of the nervous system that are involved. Common symptoms that are treated pharmacologically include muscle spasms, spasticity, weakness, fatigue, visual symptoms, urinary symptoms, pain, and depression. Refer to Table 13-2 for a partial list of medications that might be prescribed for a patient with MS. Symptoms related to muscle spasms or spasticity can be managed by using physical therapy interventions in addition to medication. Physical Therapy Management The goals of rehabilitation in the patient with MS are to: 1. minimize progression; 2. maintain an optimum level of functional independence; 3. prevent or decrease secondary complications; 4. maintain respiratory function; 5. conserve energy/manage fatigue; and 6. educate the patient and their family. These goals are met by managing the symptoms that the patient presents with in such a way that the impact on function is minimized. Weakness The most common neurologic symptoms of MS are weakness, spasticity, and ataxia. Weakness can result directly from lesions involving the corticospinal tract or cerebellum. Weakness also develops 842 secondary to inactivity and generalized deconditioning. Therefore, strengthening is an important goal of physical therapy, and exercise should be initiated early before secondary impairments develop (O’Sullivan and Schreyer, 2014). Many types of exercise can be used, but only low to moderate intensities are tolerated. Frequent repetitions are needed to obtain a training effect. Because of fatigue, a delicate balance must be achieved between rest and exercise. Shorter bouts of exercise with 1 to 5 minute rests between exercises may be indicated. Overwork and overheating must be avoided. It is possible to increase strength and endurance in patients with MS (Cakit et al., 2010; Dalgas et al., 2010). Resistance training can use isokinetic or progressive resistive modes or water. Exercises can be made more functional by having the person perform PNF patterns because functional movements almost always have some rotational component. Additionally, the rotation may help to reduce tone. Resistance within the PNF diagonals should be graded to match the patient's abilities. Energy consumption can be decreased during functional activities by placing an emphasis on strengthening proximal muscle groups. Exercise for this population should also have an aerobic component as a means of preventing or treating deconditioning. Individuals with MS have been shown to have a normal cardiovascular response to exercise. Even a short-term exercise program had a positive effect on aerobic fitness, health perception, fatigue, and activity level in individuals with MS (Mostert and Kesselring, 2002). These researchers recommended that regular aerobic training be part of any rehabilitation program. A low-level graded exercise test is indicated before having the person take part in an aerobic training program, because as the disease progresses, the potential for autonomic cardiovascular dysfunction increases. A low-level graded exercise test consists of using established protocols, as in cardiac rehabilitation, to assess a person’s ability to respond to increasing working loads using either a treadmill or a cycle ergometer. Increases in core body temperature in patients with MS can result in a temporary increase in clinical symptoms. Precooling (lowering the body temperature) was found to be effective in preventing increases in core temperature during exercise (White et al., 2000). To avoid any adverse effects of heat, exercise should be performed in cool environments. Additional cooling sources, such as fans, and even personal cooling suits can be used. Heat sensitivity is related to MS fatigue. Exercise in a cool pool that is between 80° F and 85° F is recommended for patients with MS. The water provides challenges and support to balance and can be an effective medium for exercise in this population (Roehrs and Karst, 2004). Patients with MS can experience fatigue related to the disease process. Secondarily, fatigue is related to deconditioning and respiratory muscle weakness and overuse. Exercising to fatigue is contraindicated. Submaximal levels of exercise appear to be the safest with a discontinuous schedule of training. Submaximal levels are less than 85% of the person’s age-predicted heart rate (220 minus age) or less than 85% of the maximum heart rate achieved on a graded exercise test. For deconditioned patients, starting at 50% to 60% of their maximum heart rate may produce aerobic conditioning. A discontinuous schedule builds in sufficient rest times to prevent or lessen fatigue. The person’s heart rate, blood pressure, and perceived exertion using the Borg scale should be used as a Way to monitor exercise response. Nonfatiguing exercise protocols are discussed under postpolio syndrome. Spasticity Stretching should always precede an exercise session. Stretching is an integral part of preparation for exercise, especially in muscles that exhibit increased tone. Individuals with MS have spasticity secondary to the UMN lesions and decreased flexibility secondary to decreased movement and activity. Slow static stretching is indicated with no bouncing. The patient and family should be taught self-stretching with particular attention to stretching the cervical region, hamstrings, and heel cords. Self-stretching combined with slow rhythmical rotation can be an effective means to gain range. The new stretched position should be held for 30 to 60 seconds to allow the muscle to adjust to the new length. PNF techniques, such as hold relax and contract relax, can be used to gain ROM. Refer to Chapter 9 for more information on PNF techniques. The muscle groups exhibiting spasticity vary from patient to patient. However, the plantar flexors, adductors, and quadriceps are often involved in the lower extremity. Stretching the hamstrings can be accomplished several different ways, as seen in Intervention 13-3. Methods include static stretching in supine and in sitting. Hip flexors and hamstrings can also be kept 843 flexible by using a program that consists of lying in a prone position on a firm surface several times a day for at least 20 to 30 minutes. A tilt table can be used if the person is unable to get into a prone position, but straps are necessary to maintain hips and knees in extension. Some benefit is derived from weight bearing in an upright position for tone management. Heel cords can be stretched passively using the tilt table. If the ankles are plantar flexed, a wedge may be used to ensure weight is borne through the entire foot. Over time, the size of the wedge may be decreased. Intervention 13-3 Stretching Activities 844 845 847 Supine static stretch of the heel cords and hamstrings using a towel: A. The patient lies on a firm surface in the hook-lying position. Then while one leg is bent, the other leg is raised. A towel is placed around the foot. The free ends are grasped and pulled gently to stretch the ankle into dorsiflexion. The stretch is held for 30 to 60 seconds. B. To stretch the hamstrings, the patient slowly straightens the raised leg as far as possible and holds the stretch for 30 to 60 seconds. The stretch is repeated with the other leg. Supine static stretch of the hamstrings using another person: C. The patient lies on a firm surface. The clinician raises one leg keeping the knee straight as ina straight-leg raise. The end position is held for 30 to 60 seconds. The other leg may be bent or straight, as pictured. If a pull is felt in the low back, the patient should bend the leg that is not being stretched to avoid lumbar strain. The clinician may use the proprioceptive neuromuscular facilitation (PNF) technique hold relax in this position to gain additional range of motion (see Chapter 9 for an explanation of the technique). Sitting stretch of the hamstrings using a stool: D. The patient sits with the heel of one leg resting on a stool or other stable raised object. The trunk is kept erect and the patient leans forward while maintaining a lumbar lordosis as much as possible. The patient reaches with one or both hands toward the ankle of the raised leg and tries to keep the knee as straight as possible to maximize the stretch of the hamstrings. The stretch is held for 30 to 60 seconds and repeated several times. The stretch is then repeated with the other leg. When stretching the heel cords in this position, the patient uses a towel around the foot as in Intervention 13-3A and pulls the foot gently into dorsiflexion while keeping the knee as straight as possible. Sitting stretch of the hamstrings on a low mat: E. The patient sits on a low mat with one leg on the floor and one leg on the mat table. The trunk is kept erect and the patient leans forward at the hips to ensure that the stretch occurs in the hamstrings and not the low back. The patient may reach with one or both hands toward the ankle. Again, the heel cord can be stretched by using a towel (as in Intervention 13-3A) in this 848 position. The stretch is held for 30 to 60 seconds and then repeated with the other leg. Wall stretch of the hamstrings and hip adductors: F. The patient lies on the floor on her back with the legs supported by the wall. The hips should be as close to the wall as possible to obtain the greatest stretch of the hamstrings. The patient may need assistance to get into and out of this position. The patient should not lift the pelvis or arch the back. When the patient slides the legs out to either side, the hip adductors are stretched. Depending on the patient’s ability, the legs can be moved one at a time or together. The legs are slowly separated and the stretched position held for 30 to 60 seconds. Hamstring stretch against a wall: G. The patient lies on the floor on her back (preferably in a doorway). One of the patient’s legs protrudes through the doorway; it can be bent at the knee, as pictured, or straight. The leg to be stretched is propped up against the wall or door frame with its knee straight. The patient brings her hips as close to the wall/door frame as possible to obtain the best possible stretch. Lower trunk rotation is quite effective in reducing tone in the trunk and proximal pelvic girdle muscles. Use of a ball in modified hook lying is shown in Intervention 13-4. The ball supports the weight of the legs, keeping them in flexion as the assistant guides the ball and the patient’s limbs to either side, producing trunk rotation. A person can also practice trunk rotation when moving from a hands-and-knees position to side sitting, as seen in Intervention 13-5. The person may need assistance to attain the four-point position and may need to be guarded while moving through the available range. If the person cannot get all the way to side sitting, pillows or a wedge can be used to allow the person to go through as much range as possible. Hand position can be varied. Hands can be on the support surface or on a raised bench. In the case of the latter, the person can move from kneeling to side sitting. Intervention 13-4 Rhythmical Rotation of the Lower Trunk 849 850 851 852 The patient lies supine on a firm surface. A therapy ball is used to support the lower extremities. The ball should be large enough to support the lower legs but small enough to keep the hips and knees in a flexed position. This technique is used as a preparation for functional movements, such as rolling and coming to sit. A. The clinician places the patient’s knees and lower legs on the ball and uses manual hand contact on the outside of the patient’s knees. B. The clinician gently rotates the patient’s lower extremities, supported by the ball to one side. C. The clinician moves the patient’s lower extremities back to center. D. Then the clinician gently rotates the patient’s lower extremities, which are still supported by the ball to the other side. Trunk rotation will occur with greater amounts of rotation. Intervention 13-5 Movement Transition from Four-Point to Side Sitting 853 854 C Movement transitions, such as from four-point to side sitting, can be used to practice trunk rotation. The clinician’s hand placement provides manual cues for either moving into side sitting or back into four-point. A. The patient begins in a hands-and-knees or four-point position. The clinician uses manual hand contacts on the sides of the hips to guide the patient. B. The clinician guides the patient to rotate diagonally backward from four-point into a side-sitting position. C. The clinician then guides the patient’s return from side sitting to the four-point position. The movements can be assisted at first and then resisted. Ataxia Control of static postures or postural stability is difficult for the patient with MS exhibiting ataxia. Postures that enable the person to load the trunk and other extremities not involved in movement 856 are helpful in providing stability. Unilateral limb holding in mid ranges and weight bearing, especially in antigravity postures, with slow controlled weight shifting can be beneficial. The limits of stability of these individuals can be quite precarious. The developmental sequence, especially the prone progression, can provide a wealth of treatment ideas. PNF techniques that are helpful with this problem include alternating isometrics, rhythmic stabilization, and slow reversal hold in an ever-decreasing range. Functional movement transitions are very important to focus on for the patient with MS to ensure safety. Should the patient have the upper extremities loaded when moving from sit to stand to give more stability to the upper trunk? Does the person reach more smoothly if the nonreaching arm is in weight bearing (loaded)? Does the person have more distal control if the elbow is loaded? Can the person benefit from the use of weights around the waist or trunk? Weight belts and vests are available that may increase proprioceptive awareness and enhance stability in sitting, standing, and walking. Light distal weights have been used to improve coordination of the upper extremities during reaching and of the lower extremities during walking. Although such weights can provide some improved awareness, they can also produce a rebound phenomenon when removed. Dysmetric movements (overshooting) may appear to worsen after weights are removed so caution must be practiced when deciding to weight a limb distally. Using the least amount of weight to achieve the desired effect, and loading the axial skeleton (trunk) rather than the extremities is preferable. TheraBand wrapped around a limb can provide resistance to movement in both directions, such as reaching out and returning the arm to the lap. Of course, graded manual resistance can do the same thing but that requires having an assistant or caregiver available any time the person wants to reach, which is not practical. Balance training incorporates dynamic as well as static interventions. However, movable surfaces are more challenging for the patient and the assistant. The patient must be safe at all times, which may necessitate the need of additional support staff. Use of a tilt board, a biomechanical ankle platform system (BAPS) board, a ball, or a balance master may all be indicated but safety must always be the first consideration. If the person is not safe when trying to control movement on a movable surface, anonmovable surface may be indicated. Another modification that can be used would be to have the person seated while an extremity or extremities are placed on a movable surface. For example, the person could be seated on a low mat table with hand support and the feet could be placed on a tilt board or a BAPS board. Another modification would be to use a DynaDisc or an inflatable disc for the person to sit on while the feet are supported on the floor and the hands are on the support surface. As the person is better able to deal with a disturbance of balance at the pelvis, hand support could be decreased. Frenkel exercises are classic coordination exercises that can be done in four standard positions: lying, sitting, standing, and walking. Although described for the lower extremities, similar ones can be developed for the upper extremities. These exercises are intended to be done slowly with even timing. The patient may initially need to have a limb supported so that the exercises can be progressed from assisted to independent and from unilateral to bilateral. See Table 13-5 for a complete list of these exercises. Table 13-5 Frenkel Exercises 857 Position Movements Supine . Flex and extend one leg, heel sliding down a straight line on a table. . Abduct and adduct hip smoothly with knee bent, heel ona table. . Abduct and adduct leg with knee and hip extended, leg sliding ona table. . Flex and extend hip and knee with heel off a table. . Place one heel on knee of opposite leg and slide heel smoothly down shin toward ankle and back to knee. . Flex and extend both legs together, heels sliding on table. . Flex one leg while extending other leg. . Flex and extend one leg while abducting and adducting other leg. 1 2 3 + 5 6 7. 8 1. Place foot in therapist's hand, which will change position on each trial. 2. Raise leg and put foot on traced footprint on floor. 3. Sit steady for a few minutes. Modified from Umphred DA: Neurological rehabilitation, ed 5. St. Louis, 2001, Mosby, p. 735. Ambulation is challenging for a person with ataxia. As an immediate compensation, the base of support is widened and the knees are often stiffened to increase stability. Some individuals may compensate by bending the knees, thereby lowering the body’s center of gravity. The arms are also used to counteract the increased postural sway. The increased postural sway is also exhibited in sitting and often necessitates that the person lean on outstretched arms to provide stability. Despite difficulties, a majority of patients with MS are still able to walk after 20 years (Schapiro, 2003). Mobility options are many and varied. For persons with ataxia, a weighted walker may be the best option as it affords stability and mobility. A wheeled walker with hand brakes and a seat can provide for frequent rest periods. A motorized scooter or other forms of power mobility may be indicated when fatigue is the overriding problem or tremors and weakness make propulsion of a standard wheelchair difficult. Wheelchairs should be prescribed using typical seating guidelines with a seatbelt for safety. A cushion should always be used to provide extra protection from pressure when an individual becomes wheelchair-dependent. Using a three-wheeled scooter may have less social stigma than using a wheelchair. There are also many types of orthotic options. Probably the most typical type of orthosis used by someone with MS is an ankle-foot orthosis (AFO). Indications for use of an AFO include saving energy, improving foot/toe clearance, providing greater ankle stability, controlling knee hyperextension, and improving overall gait pattern. Guidelines for use of an AFO can be found in Table 13-6. The rehabilitation team consisting of the PT and the orthotist will make a final recommendation. Rocker clogs have also been found to be helpful in accommodating for loss of ankle mobility (Perry et al.,1981). Some have reported use of a reciprocal gait orthosis (RGO), a type of hip-knee-ankle-foot orthosis (HKAFO) for patients with MS. Table 13-6 Guidelines for use of Ankle-Foot Orthoses (AFO) Relative Contraindications Standard poly propylene Saves energy Impedes tibial advancement during sit | Moderate or severe spasticity Improves toe and foot clearance Severe edema in the foot Improves safety Severe weakness (2/5 or less at Improved knee control during midstance the hips Avoid knee hyperextension Greater ankle stability Polypropylene with articulating ankle All of the above Same as above joint Tibial advancement during sit to stand More normal ankle movement during gait Able to squat May have a plantar flexion stop or a dorsiflexion assist Double upright metal with articulating All of the above Weight ankle joint May have straps to correct valgus or varus Poor cosmesis May accommodate significant fluctuations in limb volume 858 (Data from Schapiro R: Multiple Sclerosis: A Rehabilitation Approach to Management. New York, 1991, Demos Publications; Edelstein JE, Wong CK: Orthotics. In O’Sullivan SB, Schmitz TJ, Fulk GD, editors: Physical Rehabilitation, ed 6. Philadelphia, 2014, FA Davis, pp. 1325-1363; and Lusardi MM, Bowers DM: Orthotic decision making in neurological and neuromuscular disorders. In Lusardi MM, Jorge M, Nielsen CC: Orthotics and Prosthetics in Rehabilitation, ed 3. Philadelphia, 2013, Saunders, pp. 266-307.) Additional Concerns Some patients with MS exhibit emotional lability. They demonstrate rather volatile swings in mood, ranging from euphoria to crying. These abrupt changes in behavior need to be managed with calmness and firm direction in order for them to not totally disrupt a treatment session. In some cases, the patient can benefit from psychologic intervention. Another challenging situation occurs when a patient continuously exhibits nystagmus. The patient extends the head to minimize the amount of movement of the eyes. The tilted head posture should not be corrected as that will remove the compensation and may negatively affect the patient’s balance. Other patients may experience vertigo with sudden head movements. In this situation, the person needs to move the head more slowly or actually fix the head in a position before attempting a movement so as to not produce a loss of balance. Summary Exercise is a crucial part of the physical therapy intervention for a person with MS. Exercise balanced with rest can improve the quality of life of an individual dealing with this chronic disease. Although symptoms vary depending on the sites in the nervous system that are involved, fatigue is a pervasive problem. Whether the fatigue is stress-related or heat-related, it can produce immobility, which may all too quickly become part of a cycle of disuse and deconditioning. Therefore, regular exercise is essential to preserving function in this population. 859 Amyotrophic lateral sclerosis ALS is a terminal progressive disease involving both UMNs and LMNs. It is commonly known as Lou Gehrig disease. UMNs degenerate in the cortex and corticospinal tract, LMNs degenerate in the brainstem (cranial nerve nuclei) and anterior horn cells in the spinal cord. Therefore, signs of both UMN and LMN involvement will be evident. The loss of LMNs results in muscle atrophy and weakness (amyotrophy) and the destruction of the corticospinal and corticobulbar tracts, which results in the lateral sclerosis (UMN symptoms) (Hallum and Allen, 2013). Muscle weakness is the cardinal sign of ALS (Dal Bello-Haas, 2014). Incidence and Etiology ALS is the most common motor neuron disease in adults, with an incidence of 3 to 5 per 100,000 individuals. There are an estimated 30,000 people with ALS in the United States, with a prevalence of between 4 and 10 per 100,000 (Dal Bello-Haas, 2014). ALS usually occurs between middle and late sixth decade of age. Men are slightly more likely to be affected than women. The cause of ALS is unknown, with the exception of an inherited form. In about 20% of inherited cases, the person has a mutation of a gene involved in producing enzymes that eliminate free radicals. The majority of people with ALS have no prior family history. Theories as to the cause of ALS include protein- folding errors, neurotoxicity, programmed cell death (apoptosis), and autoimmune reactions (Hallum and Allen, 2013; Dal Bello-Haas, 2014). Clinical Presentation ALS can present with limb loss onset or bulbar loss onset. The majority of people with ALS (70% to 80%) present asymmetric weakness in an arm or a leg. A smaller percentage (20% to 30%) presents difficulty swallowing or speaking. Fasciculation (twitching of muscle fibers) may be seen in the tongue. Earliest signs of ALS include muscle cramps, weakness, atrophy, and fatigue. Involvement spreads regionally with distal symptoms occurring before proximal ones. Bulbar signs commonly occur later in the disease progression, unless the initial presentation of loss is in the cranial nerves, which are responsible for tongue movements, chewing, and swallowing. There is no one definitive laboratory test for ALS. However, elevation of creatine phosphokinase levels is present in 70% of cases (Ilzecka and Stelmasiak, 2003). Diagnosis is based on the combination of signs and symptoms in the UMNs and LMNs, supplemented by electromyography, nerve conduction velocity tests, neuroimaging, and nerve and muscle biopsies. According to the revised El Escorial criteria, a “definite” diagnosis of ALS requires LMN + UMN findings in 3 regions (Brooks et al., 2000). Regions include bulbar, cervical, thoracic, or lumbosacral. There is no sensory involvement or eye muscle involvement in typical ALS. Spinocerebellar and sensory systems are sparred. Previously, the presence of cognitive deficits would exclude a diagnosis of ALS. However, the prevailing thought is that mild to extreme cognitive problems are part of the disease (Lomen-Hoerth et al., 2003). More than half of patients with ALS have cognitive impairments (Woolley and Jonathan, 2008). A therapist should be suspicious of cognitive involvement in a patient with ALS who exhibits delays in executive function, such as not following through on exercise or medication recommendations and verbal fluency (Abrahams et al., 2000). A small group of people with ALS coincidentally exhibit a frontotemporal dementia (FTD) characterized by behavioral and personality changes as well as decline in executive function. FTD can present before the ALS or with the ALS or develop after the ALS. The overlap of these two diseases is being studied to gain insight into their neuropathology (Giordano et al., 2011). The diagnosis of FTD, along with ALS, decreases median survival time (Olney et al., 2005). Because of the relentless progression of ALS, staging is best thought of as early, middle, and late. More in-depth staging has been devised for drug research, but to provide a framework for intervention, three stages works well. Early on, the person has mild to moderate weakness in specific muscle groups. Realize that a person may have lost 80% of motor neurons before reporting weakness (Hallum and Allen, 2013), so there may not be an extreme impact on gait, ADLs, or speech. By the end of the early stage, the person is experiencing difficulty with ADLs and mobility. During the middle stage, mobility continues to decrease with a wheelchair needed for long 860 distances. ADLs continue to decline. Pain is manifested because of decreased ROM, faulty posture, or spasticity. Late stage is marked by total dependence in mobility and ADLs, dysarthria and dysphagia, respiratory compromise, and pain. The patient may be restricted to bed. Death results from respiratory failure as muscles of ventilation, the diaphragm, intercostals, and accessory muscles become weak. Medical Management There is no cure for ALS, and medical management focuses on symptom management. A multidisciplinary clinic is best equipped to provide the most optimal and comprehensive care for individuals with ALS and their families. Riluzole (Rilutek) is the only disease-modifying medication presently approved for the treatment of ALS. Other medications may be prescribed for muscle cramping, spasticity, sialorrhea, and depression. With bulbar involvement, swallowing and nutrition issues are best addressed by a speech-language pathologist and a nutritionist or registered dietitian. The need for augmented feeding via a percutaneous endoscopic gastrostomy (PEG) tube may be considered in the middle stage of the disease. Some individuals choose invasive mechanical ventilation during the later stage of the disease. Physical Therapy Management During the early stages of the disease, individuals may participate in preventive exercise programs to forestall activity limitations. Exercise involving moderate loads and moderate resistance was found to improve function in a group of patients with early-stage ALS compared with a matched control group doing stretching (Dal Bello-Haas et al., 2007). Research from other patients with progressive neuromuscular disorders has resulted in several suggestions or guidelines for exercise in the ALS population. These general suggestions include: (1) avoid heavy eccentric exercise; (2) moderate resistance can increase strength in muscles with a manual muscle testing (MMT) grade of 3 or higher out of 5; (3) overuse is not an issue if the muscles exhibit an MMT grade of 3 or better out of 5. As the disease progresses, mobility concomitantly decreases so the strategy becomes one of support for weak muscles and modification of the home and workplace. Some individuals are helped by a custom orthosis to support the neck and upper thoracic spine. It is appropriate to assess the person’s need for pressure-relieving devices, such as a mattress or a wheelchair cushion. As with all the diseases discussed so far, the balance between rest and activity is essential. Pulmonary care in the patient with ALS must be geared to prevention and education regarding potential for aspiration and difficulty with airway clearance as the respiratory muscle weaken. The physical therapist can play a very important role in assisting the patient with ALS and the family to cope with this devastating disease. 861 Guillain-barré syndrome GBS is the most frequent cause of acute generalized weakness now that polio is all but eradicated. It is referred to as a syndrome because it represents a broad group of demyelinating inflammatory polyradiculoneuropathies. There are many forms of GBS. Two major subgroups can be distinguished based on pathologic and electrophysiologic findings: acquired inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). Cranial nerves, which are a part of the peripheral nervous system, may also be involved. Seventy percent of patients with GBS exhibit facial nerve palsy (van Doorn et al., 2008). Another common variant of GBS involving cranial nerves is Miller-Fisher syndrome, consisting of ophthalmoplegia, ataxia, and areflexia. GBS is a classic LMN disorder because nerve roots (radiculopathy) and peripheral nerves (polyneuropathy) are affected, resulting in flaccid paralysis. Incidence and Etiology GBS is rare with an incidence of about 1.2 to 2.3 cases per 100,000 people (Hughes and Cornblath, 2005). It occurs in all age groups, both children and adults. The majority of individuals who acquire GBS experience a respiratory or gastrointestinal illness before the onset of weakness and sensory changes. It is a postinfectious disorder. Campylobacter jejuni, a common cause of gastroenteritis, is the most frequent infectious agent. Although certain viruses, bacteria, surgery, and vaccinations have been linked to GBS, there is no one causal agent. It is a reactive, self-limited autoimmune disease with a good overall prognosis. Pathophysiology The pathophysiology of GBS is complex because it involves autoimmune reactions. The infection- induced immune responses cause a cross-reaction with neural tissue. When myelin is destroyed, destruction is accompanied by inflammation. These acute inflammatory lesions are present within several days of the onset of symptoms. Nerve conduction is slowed and may be blocked completely. Even though the Schwann cells, which produce myelin in the peripheral nervous system, are destroyed, the axons are left intact in all but the most severe cases. Two to three weeks after the original demyelination, the Schwann cells begin to proliferate, inflammation subsides, and remyelination begins. Although GBS is the most common cause of acute paralysis, the exact pathogenesis is as yet unclear. The progression of the demyelination appears to be different in the AMAN type of GBS versus the AIDP type. Patients with the AMAN GBS have a more rapid progression and reach nadir earlier. Nadir is the point of greatest severity. The only way to classify a patient with GBS as having axonal or nonaxonal type is electrodiagnostically (Hiraga et al., 2003). Clinical Features GBS is characterized by a symmetrical ascending progressive loss of motor function that begins distally and progresses proximally. Distal sensory impairments often present as paresthesias (burning and tingling) of the toes or hypesthesias (an abnormal sensitivity to touch). The sensory involvement varies and is usually not as significant as the motor involvement. The progression of motor and sensory changes may be limited to the limbs, or the progression of weakness can impair the diaphragm and cranial nerves. The diaphragm is the major muscle of ventilation. Weakness of shoulder elevators and neck flexion parallels diaphragmatic weakness. The diaphragm is innervated by cervical nerve roots 3, 4, and 5. If the diaphragm becomes involved, the person will need to be placed on mechanical ventilation. Additionally, 50% of the people with GBS experience changes in the autonomic nervous system such as fluctuations of blood pressure and pooling of blood with poor venous return, tachycardia, and arrhythmias. Pain is reported by patients as being muscular in nature, which is myalgia. Pain can be an early symptom and requires constant intervention. Hypesthesias may cause using a bed sheet uncomfortable. Pain can be difficult to manage and can add to the person’s fear and anxiety. The cause of pain is often unclear but it may come from spontaneous transmissions from demyelinated nerves (Sulton, 2002). 862 Half of the patients with GBS have oral-motor involvement in the form of weakness that causes difficulty speaking (dysarthria) and swallowing (dysphagia). Alternative means of communication may need to be explored as well as measures taken to prevent aspiration. The facial nerve (cranial nerve VII) is frequently involved and bilateral facial weakness is common. Double vision (diplopia) can result from eye muscle weakness secondary to cranial nerves III, IV, and VI involvement. Paralysis of cranial nerves is termed bulbar palsy. Cranial nerve involvement is referred to as bulbar because the majority of cranial nerves exit the bulb or brainstem. Deep tendon reflexes are absent because of the demyelination of the peripheral nerves, therefore making areflexia a core feature of this LMN disorder. Medical Management Plasmapheresis, or plasma exchange (PE), or infusion of intravenous immunoglobulins (IVIGs) has been found to be equally effective in treating GBS (Van Doorn et al., 2008; Van Koningsveldt et al., 2007). However, IVIG is the preferred treatment because of availability and greater convenience (Hughes et al., 2006). Either of these interventions needs to be initiated within the first or second week of symptom onset to shorten the course of the disease (Van Doorn et al., 2008). Despite the use of either PE or IVIG treatment, 20% of severely affected patients are unable to ambulate after 6 months (Hughes et al., 2007). There are three phases of GBS: acute, plateau, and recovery. The first stage lasts up to 4 weeks. During this time, symptoms appear; 80% of individuals present with paresthesias, 70% with areflexia, and 60% with weakness in all limbs. In time, the percentages of patients exhibiting the core symptoms increase to close to 100%. The plateau phase is defined by the stabilization of symptoms. Although symptoms are present, they are not progressing or worsening. This phase can also last up to 4 weeks. Lastly, the recovery phase is evident when the patient begins to improve. Eighty percent of patients recover within a year but may have some neurologic sequela or residual deficits. The recovery phase can last a few months to a couple of years. Patients who tend to have a poorer outcome are those who needed ventilatory support, had a rapid progression of demyelination, and demonstrated low distal motor amplitudes on electromyography (EMG) (Ropper et al., 1991). The latter finding is reflective of the amount of axonal damage incurred. Physical Therapy Management Acute Phase Supportive care during the acute stage is a necessity. Because of the possibility of respiratory involvement, people with GBS are hospitalized and may spend a long time in intensive care. During the acute phase, it is most appropriate for the physical therapist to treat the patient as symptoms are usually progressing. If a patient’s respiratory musculature becomes involved, he or she will likely require ventilatory support and be in an intensive care unit (ICU). Physical therapy goals during the acute stage include minimizing the acute signs and symptoms; supporting pulmonary function, preventing skin breakdown and contracture formation; and managing pain. Exercise is limited to those movements that can be made without pain or excessive fatigue (Hallum and Allen, 2013). If the physical therapist assistant is providing passive ROM and positioning under the supervision of the physical therapist, the therapist needs to provide information about oxygen saturation and vital capacity parameters in order for the assistant to be alert to the changes in the patient's respiratory status. The physical therapist assistant may also provide postural drainage with percussion to maintain airway clearance. Gentle stretching of the chest wall and trunk rotation may be done while the patient is still on a ventilator. The person is positioned to decrease potential contractures with hand and foot splints. Extra care should be taken when performing ROM as denervated muscles can easily be damaged. The assistant should be careful to support the limb to prevent overstretching. Always ensure that the ankle is in a subtalar neutral position before stretching the heel cord. Subtalar neutral is the position in which the talus is equally prominent when palpated anteriorly, as seen in Figure 13-2. ROM should be performed at least twice a day. The schedule of positioning, splinting, and the ROM program should be posted at the patient's bedside (Hallum and Allen, 2013). 863 y Calcaneus Tarsals Cuboid Lateral cuneiform Talus Navicular Intermediate Tarsals cuneiform Phal alanges Medial cuneiform Metatarsals FIGURE 13-2 Finding subtalar neutral before stretching heel cords. With the patient supine, hold the heel of the foot with one hand. Grasp the foot over the fourth and fifth metatarsal heads using the thumb, index, and ring fingers of the other hand. Palpate both sides of the talus on the dorsum of the foot (refer to the frontal view and skeletal structure). Passively dorsiflex the foot until resistance is felt. In this position, supinate and pronate the foot; the talus will bulge laterally and medially, respectively. Positioning the foot so that there is no bulge is subtalar neutral. Pain is one of the most difficult symptoms to treat in patients with GBS. Medications are not always effective. Passive ROM, massage and transcutaneous electrical nerve stimulation (TENS) may be helpful. If the patient demonstrates an increased sensitivity to light touch, a cradle can be used to keep the bed sheet away from the skin. Low-pressure wrapping or a snug-fitting garment may provide a way to avoid light moving touch on the limbs. Pain may be heightened by the patient’s fear as to what has happened. Reassurance and an explanation about what to expect may help alleviate anxiety that could compound the pain. Plateau Phase When respiratory and autonomic functions stabilize, a program to increase tolerance to upright can be begun. This must be initiated gradually as the patient may still be on a ventilator. Physical therapy goals during the plateau phase include acclimation to upright posture, maintenance of ROM, improvement in pulmonary function, and avoidance of fatigue and overexertion. The patient is acclimated to sitting upright with appropriate postural alignment and truncal support because it may still have minimal innervation. Pressure relief is still provided by changing positions on a regular basis. If the patient continues to experience pain, it may lead to holding limbs in potentially contracture-prone positions. Heat may be used before stretching if there is no sensory loss. Return of oral musculature may signal the need for additional team members to work on the movement patterns needed for swallowing, eating, and speaking. The physical therapist assistant may provide postural support for the patient during these sessions. At the very least, the assistant needs to be aware of any precautions regarding potential aspiration and any requirement for maintaining an upright upper body posture after any oral intake of food or fluids. Recovery Phase Muscle strength is gradually recovered 2 to 4 weeks after the condition has reached a plateau. The 864 muscles return in the reverse order or descending pattern. This is opposite from the ascending order of loss. As the neck and trunk muscles recover, the patient may begin to use a tilt table for continued acclimation to upright and weight bearing on the lower extremities. Positioning splints may be needed for the lower extremities as well as TED stockings to decrease venous pooling. Muscles of respiration can be weak if the person required ventilatory assistance and this weakness may limit tolerance to upright. Physical therapy goals at this time now encompass strengthening and maximizing functional abilities in addition to carrying over any goals from the previous phases. Strengthening activities and exercise prescription for these individuals is challenging. Depending on the number of intact motor units present in any given muscle, the same amount of exercise can be harmful or beneficial. If there are too few motor units, working the muscle may be detrimental to its recovery. Unfortunately, there is no easy way to ascertain how many motor units are present in a patient recovering from GBS. Once the patient has stabilized or reached a plateau, active exercise can begin. Each patient must be progressed individually based on his or her response to exercise. Rehabilitation should begin as soon as improvement starts (Van Doorn et al., 2008). Gupta et al. (2010) found that patients continued to improve over a one-year period following initial hospitalization. Patients were transferred from the hospital to a neurorehabilitation unit on average of 29.5 days after initial hospital admission. The mean length of stay in the unit was 32.9 days. Longer stays were associated with autonomic dysfunction but not with cranial nerve involvement of need for ventilator assistance. In a recent systematic review by Kahn and Amatya (2012), “satisfactory” evidence was found for both inpatient rehabilitation and physical therapy/exercise to produce positive functional gains in patients with GBS. There was “good” evidence for outpatient high intensity rehabilitation to produce long-term gains even 6.5 years after initial diagnosis with GBS. The authors did point out that there is still a need for more high-quality randomized controlled trials (RCTs) to determine effectiveness of timing, intensity, and progression of rehabilitation programs for this very challenging and complex condition. Bensman’s recommendations in 1970 are still useful guidelines for exercise in this population: 1. Use short periods of nonfatiguing exercise matched to the patient’s strength. 2. Increase the difficulty of an activity or level of exercise only if the patient improves or if there is no deterioration in status after a week. 3. Return the patient to bed rest if a decrease in strength or function occurs. 4. Direct the strengthening exercises at improving function not merely at improving strength. Overworking a partially denervated muscle produces a profound decrease in that muscle’s ability to demonstrate strength and endurance. Signs of overuse weakness are delayed onset of muscle soreness, which gets worse 1 to 5 days after exercising, and a reduction in the maximum amount of force the muscle is able to generate (Faulkner et al., 1993). Bassile (1996) recommends training muscles that are at a 2/5 muscle strength in a gravity-eliminated plane using only the weight of the limb. Once the person can move the limb against a resistance equal to the mass of the limb, the person can perform antigravity exercise. Exercise progression in this population must be taken slowly. Care must be taken to avoid straining weaker muscles while increasing resistance to those showing good recovery. The distal muscles of the hands and feet are often the ones most likely to not recover fully. Use of lightweight orthoses can be helpful to support muscles around the ankle from overuse. Regardless of the terminology, everyone agrees that it is best to start with low repetitions and short, frequent bouts of exercise matched to the patient’s muscular abilities, that is, muscle strength. For example, someone who has poor (2/5) deltoid muscle strength could exercise in a pool, or with an overhead sling apparatus or a powder board. All of these situations are gravity-eliminated. Facilitation techniques, such as stroking, brushing, vibration, and tapping of the muscle, can be combined with gravity-eliminated exercise. The patient is restricted from moving against gravity until the deltoid muscles’ strength is a 3/5. The lower extremities are going to recover after the upper extremities. Most people walk within 6 months of the onset of symptoms (Van Doorn et al., 2008) but 20% of the severely involved do not achieve this milestone. The dilemma comes as to whether to attempt ambulation with a patient before the muscles of the lower extremities have at least a fair grade (3/5) (Bassile, 1996). To date, there are no valid outcome measures to use to evaluate functional progress. Adaptive equipment needs change as the patient recovers. Once acclimated to upright, mobility may initially be limited to a wheelchair. When ambulation is achieved, a walker, forearm crutches, 865 or a cane may be needed as an assistive device. Orthotic assistance needs to be lightweight. A plastic AFO or even an air stirrup splint can provide support for weak ankles. Residual weakness is most often apparent in the distal muscles of the hands and feet such as the wrist extensors, finger intrinsics, ankle dorsiflexors, and foot intrinsics. The gluteal and quadriceps may also remain weak. Endurance is often lacking and may be a major obstacle even if the person is strong enough to return to work. Endurance training should be included in the patient’s home exercise program; otherwise the patient may continue to be minimally active despite adequate strength. Pitetti et al. (1993) studied a 54-year-old man who has been 3 years post-GBS. He was able to improve leg strength and total work capacity after a thrice-a-week aerobic exercise program using a bike ergometer. He was even able to return to gardening. A recent case study of a highly trained athlete with GBS (Fisher and Stevens, 2008) was reported in the literature. The individual recovered within 3 weeks using a combined treatment with IVIG, PE, and corticosteroids. Summary The prognosis for a person with Guillain-Barré syndrome is usually very good. Fortunately, the muscle weakness is reversed as the peripheral nervous system recovers. However, patients with GBS are often immobilized for lengthy periods of time because of the slow nature of the recovery process. The health-care team’s role during that time is to safeguard the musculoskeletal and cardiopulmonary systems so that when recovery occurs, the patient is able to make the most of the changes. The role of exercise in this neuromuscular disease is to improve function without causing overuse damage. The use of nonfatiguing exercise protocols is indicated. These protocols will be further discussed in the next section. 866 Postpolio syndrome PPS is the name given to the late effects of poliomyelitis. Polio is a viral infection that attacks some of the anterior horn cells in the spinal cord and results in muscular paralysis. Polio was epidemic in the United States from 1910 to 1959. Decades after having survived polio, 25% to 40% of these individuals experience fatigue, new muscle weakness, and loss of functional abilities (National Institute of Neurological Disorders and Stroke [NINDS], 2012). PPS was first described and recognized as a clinical entity in 1972, when Mulder et al. published criteria for its diagnosis. The latest criteria consist of: (1) having had polio based on history; (2) a positive neurologic exam or EMG; (3) a period of relative stability lasting at least 15 years; and (4) development of new neurologic weakness and abnormal fatigue, which persists for at least a year and is unexplained by any other pathology (NINDS, 2012). Because records are not as accurate as one might expect, we only have an estimate of the number of people who actually experienced polio. According to Post-Polio Health International (PHI), the estimates on which people may experience PPS range from 12 million to 20 million people worldwide. The National Institute of Neurological Disorders and Stroke (NINDS) (2012) report that more than 443,000 individuals in the United States may be at risk for PPS. The severity of PPS is related to the severity of the original polio infection. If a person had a mild case of polio, the PPS is also going to be mild. Conversely, if a person had a severe case, which required use of an iron lung (Figure 13-3), the PPS may be just as severe. Postpolio syndrome shows a slow progression over a long period of time and is rarely life-threatening. 867 FIGURE 13-3 A, A hospital respiratory ward in Los Angeles in 1952. B, A patient in an iron lung during the Rhode Island polio epidemic of 1960. (Courtesy Centers for Disease Control and Prevention.) Etiology Most sources accept the theory that postpolio syndrome is caused by decades of increased metabolic demand made on the body by giant motor units (Gonzalez et al., 2010; Trojan and Cashman, 2005). These giant motor units were formed during the recovery process from the original viral infection. After the poliovirus destroys anterior horn cells, muscle fibers innervated by those anterior horn cells are orphaned. During recovery, the anterior horn cells not destroyed by the virus reinnervate some of these orphaned fibers, creating giant motor units. The repair process involves branching and cutting back of neural processes. This repair process continued after the original infection, but as time passed, the ability of the body to keep up with the necessary changes diminished. Stress and overuse of the large motor units is hypothesized to lead to distal degeneration of axons (Wiechers and Hubbell, 1981). The body’s response to the original pathology is compounded by age-related changes in the nervous system. Because there is a loss of motor units during normal aging, a person who had polio may lose some giant motor units. The end result is a subsequent loss of function in the person with PPS. Clinical Features Fatigue One of the most commonly reported and debilitating problems in patients with PPS is fatigue (Gonzalez et al., 2010). In fact, fatigue is one of a triad of symptoms, which include pain and a decline in strength. This fatigue goes beyond the typical fatigue everyone has felt after working hard. This fatigue is described as an overwhelming tiredness or exhaustion occurring with only minimal effort. It can be so severe that the person’s ability to concentrate is affected. The fatigue may occur at the same time of day and be accompanied by signs of autonomic distress, such as sweating or headaches. Some people have described the feeling of fatigue as “hitting the wall.” Defects in neuromuscular transmission caused by the degeneration of the distal motor unit in PPS may contribute to muscular fatigue (Trojan and Cashman, 2005). Fatigue is multidimensional. Muscular factors, such as overuse, high-energy cost of even submaximal workloads, and decreased cardiopulmonary deconditioning, can contribute to physical fatigue. Mental fatigue may impact psychosocial function and lead to a decreased QOL. Modifiable risk factors for fatigue, such as stress and physical activity, must be considered in the management of patients with PPS (Trojan et al., 2009). New Weakness 868 New muscle weakness is a hallmark of postpolio syndrome. It occurs in muscles already involved and in muscles that did not clinically show any effects of the original polio infection. There is evidence that these “new muscles” may actually have been involved subclinically, based on EMG results. The weakness is asymmetric, usually proximal and slowly progressive in nature. As mentioned previously, overuse has been associated with the new muscle weakness seen in individuals with PPS. If fatigue is a contributing factor, the weakness may be transient. Motor units normally break down with increasing age, and in the case of individuals with PPS, these may be giant motor units. After years of increased metabolic effort, these giant motor units break down and cause new weakness, which is permanent. Because of increased muscle weakness, patients with PPS may experience impaired balance and, therefore, be at greater risk for falls. Assistive devices for ambulation including use of a wheelchair may need to be considered. Pain Muscle and joint pain are common manifestations of PPS. Muscle pain is related to overuse of weak muscles. The pain and fatigue in these muscles occurs 1 to 2 days after an activity. It is lessened by rest and responds well to pacing of activities to avoid excessive fatigue. Muscle pain is diffuse and takes a long time to recover from, as evidenced by research on patient’s adherence to recommendations regarding pacing and lifestyle changes (Peach and Olejnik, 1991). Those subjects that followed the recommendations had a higher percentage of resolution or improvement in muscular pain. Joints can become unstable when muscles are weak or when excessive daily physical activity overstresses these muscles and their surrounding soft tissues. Mobility is often curtailed in the presence of joint or muscle pain, which then leads to muscular atrophy. Pain is usually the result of repetitive microtrauma from years of moving joints that are misaligned or malaligned, secondary to weakness or frank postural deformity. Joint pain is a result of wear and tear on joints, of poor posture, and of deterioration of soft-tissue or orthopedic surgical procedures done to treat the residual effects of polio. Reports of joint and muscle pain are more likely from women with PPS than men with PPS (Vasiliadis et al., 2002). Other Symptoms Cold Intolerance Because of sympathetic involvement, the person with PPS is intolerant of cold. The limbs are often cold and require extra clothing to minimize heat loss. Because of this intolerance, use of cold as a modality is usually met with resistance. If the person has difficulty with edema, heat is often not the modality of choice. Therefore, extensive patient education may be required to convince a person with PPS to use local cold as a treatment for edema. Decreased Function Fatigue, pain, and weakness conspire to produce a cycle of inactivity in the person with PPS. When asking a person with PPS what he or she does on a regular basis, his or her reply is “not much.” However, with probing, you may realize that the person used to be very active and do a lot but has curtailed his own activity level because of a combination of fatigue, pain, and weakness. With less activity comes deconditioning of the cardiopulmonary systems. The deconditioning further exacerbates fatigue and weakness, leading to less activity and an even lower level of social engagement. Any one of the triad of symptoms, fatigue, pain, or weakness, can trigger the cycle of decreased activity and function. Vital functions, such as eating and breathing, can be affected if the person originally had bulbar involvement. Cranial nerves exiting from the brain stem or bulb support oral motor and cardiorespiratory function. If the poliovirus attacked the brain stem, the central control of breathing could have been compromised in addition to the muscles of ventilation, such as the diaphragm and intercostals. Subsequently, after years of working, the person with PPS may be so exhausted at the end of the day that he or she collapses at night. Shortness of breath is a common complaint. Sleep may be interrupted by periods of apnea or pain and, thus, further compounds the problems with fatigue, pain, and weakness encountered during waking hours. The individual with oral-motor, a significant pulmonary involvement, or sleep disturbances will be more appropriately treated by a team member with expertise in that area, such as an occupational therapist or a speech therapist. A 869 pulmonologist may recommend use of a positive-pressure breathing device at night to ensure adequate oxygenation. Having walked for years with significant gait deviations, people with PPS are at risk for falls and loss of bone density. These individuals have prided themselves on using assistive devices only when absolutely necessary, although others have walked with knee-ankle-foot orthoses (KAFOs) and forearm crutches. Many have established compensatory movements with or without orthoses and assistive devices that allowed them functional movement. With the onset of fatigue and new weakness, these compensations may no longer be adequate and may put them at high risk for falls and other musculoskeletal injuries. These risks interfere with the accomplishment of tasks of daily living. Many postural abnormalities are seen in patients with PPS including a forward head, forward-leaning trunk, an absent lumbar curve, uneven pelvic base, and scoliosis. People with PPS have a greater chance of having osteoarthritis than the general population. Medical Management Medications for fatigue have not been proven effective. High dose of prednisone and amantadine have not been shown to improve strength or treat fatigue (NINDS, 2012). Management of patients with PPS is based on physical activity and an individualized muscle training program. Additionally, healthy diet, positive-pressure ventilation, treatment for sleep apnea, and staying warm are all recommendations that might be made to an individual with PPS. The medical focus has been on managing the signs and symptoms of the syndrome for these individuals to improve their QOL. In a recent review, Gonzalez et al. (2010) recommended that physical therapy be emphasized as part of a multidisciplinary and multiprofessional approach to rehabilitation for patients with PPS. Physical Therapy Management Goals for physical therapy management of the individual with PPS are to: 1. Decrease work load on muscles; 2. Avoid fatigue; 3. Ambulate safely; 4, Achieve an optimal level of functional independence; and 5. Educate the patient and the family. Physical Activity/Exercise Individuals with PPS benefit from physical activity. Individuals who engage in regular physical activity reported a higher level of functioning and fewer symptoms than those who are not as active (Fillyaw et al., 1991; Willen et al., 2001). Every exercise program needs to be tailored to the person’s presentation, as most people with PPS exhibit asymmetrical muscle weakness. General guidelines include avoiding overuse and disuse and modifying the level of physical activity to decrease pain. Heart rate, blood pressure, and rate of perceived exertion should all be monitored. Trojan and Finch (1997) recommended a Borg rating of 14, which equates to “hard.” The original Borg scale is preferred over the newer 10-point one. In keeping with a nonfatiguing protocol, the duration of the exercise should be short and use a submaximal workload. Customized exercise programs have been shown in multiple studies to be effective in improving mild to moderate weakness without causing muscle overuse (Bertelson et al., 2009; Farbu, et al., 2006; Jubelt and Agre, 2000). Short intervals of exercise are recommended with rests in between to recover. Nonfatiguing protocols consist of submaximal and maximal strengthening exercises combined with short duration repetitions. An every-other-day schedule of exercise is used to avoid overuse and to provide for full recovery. Exercise should be supervised by a physical therapist or physical therapist assistant to ensure that correct techniques are being used and to monitor that the patient avoids increasing muscle or joint pain and producing excessive muscle fatigue. Studies have found exercise and lifestyle modifications to positively contribute to reducing signs of overuse, improving fatigue, and improving function (Cup et al., 2007; Klein et al., 2002; Oncu et al., 2009). For examples of nonfatiguing protocols, see Table 13-7. Table 13-7 870 Nonfatiguing Exercise Protocols Nonfatiguing Nonfatiguing Aerobic Interval Training Strengthening Exercise Resistance Target heart rate—low range, 60%-70% 60%-80% of one repetition maximum Repetitions NA Goal of 5-10 Duration 15-30 minutes NA Contract NA 5 seconds/10 seconds time/rest time Intervals Start with 2- or 3-minute exercise bouts interspersed with 1-minute rests for a session of 15 minutes; when able to do this comfortably for a total | NA of 20 minutes for 2 weeks, increase each exercise bout by 1 minute. Goal: 4 minutes each exercise bout, 1-minute rest interval, total session: 30 minutes total of exercise bouts. Kinds of Walking, swimming, pool walking, stationary bicycling, arm ergometer—selection is based on strongest muscle group to achieve heart rate goals | Concentric exercise and avoid joint trauma. Measurable and] Pretest, then 2 and 4 months. Pretest, then at 1, 3, 6 reproducible months, and yearly testing intervals. Data from Owen RP: Postpolio syndrome and cardiopulmonary conditioning, in rehabilitation medicine—adding life to years, special issue. West J Med 154:557-558, 1991; McNelis A: Physical therapy management of post-polio syndrome. Rehab Manag 38-43, 1989; Dean E, Ross J: Modified aerobic walking program: Effect on patients with postpolio syndrome. Arch Phys Med Rehabil 69:1033-1038, 1988; and Jones DR, Speier J, Canine K, Owen R, Stull GA: Cardiorespiratory responses to aerobic training by patients with postpoliomyelitis sequelae. JAMA 261:3255-3258, 1989. Exercise plays a pivotal role in managing PPS. To date, no prospective data has linked increased physical activity to muscle weakness (Farbu et al., 2006). Exercises must strengthen muscles, not induce muscle fatigue. A relaxed pace is best for any exercise routine. Teach your patients with PPS to avoid overdoing it in a workout and to not go beyond the point of pain or fatigue. They must learn that if it takes several days to regain their strength, what was done was too much. Aerobic exercise, such as walking on a treadmill, bicycle ergometry, and swimming, are recommended. Aquatic exercise can be very beneficial because water decreases the stress on the joints, bones, and muscles. Studies have shown improvement in flexibility, strength, and cardiorespiratory fitness in patients with PPS who participated in aquatic exercise programs (Willen et al., 2001). Tiffreau et al. (2010) also found that aquatic physical therapy had a positive impact on muscle function and pain. Stretching Stretching overworked muscles may not be indicated because of the potential for increasing joint instability. The person with PPS may have already achieved a delicate balance of ligamentous and muscular tightness that has substituted for weak or absent musculature. A mild shortening of the plantar flexors may increase knee stability when there is quadriceps weakness. In such a case, stretching the heel cord could impair function. Any increase in ROM must be able to be supported by adequate muscle strength, which may not be possible in this population. Gentle stretching may be indicated as a strategy to combat pain or cramping from occasional overuse (Gawne et al., 1993). Pain Management Pain management depends on the type of pain that the patient with PPS is experiencing. There are three types of pain that have been described in the literature: cramping, musculoskeletal, and biomechanical (Gawne et al., 1993). Gentle stretching after application of heat is indicated in the presence of cramping. This is very similar to the way people with polio were initially treated. As musculoskeletal pain often results from overuse; the structure involved, such as the tendon, bursa, fascia, or muscle, must be identified before an appropriate treatment can be determined. Treatment for inflammation or strains should incorporate use of an antiinflammatory medication and appropriate modalities and changes in patterns of use of the involved extremities. By far, the most frequent type of pain comes from biomechanical changes, resulting from degenerative joint disease, low back pain, and nerve compression. Posture education and recommending the use of an assistive device are the best strategies to use in this instance. Orthoses may be indicated to provide better biomechanical alignment of the feet and lower extremities. In PPS, the individual usually has a combination of biomechanical malalignment and muscle imbalance. An orthosis may only be able to support better joint alignment, not accomplish a complete correction. The most frequently prescribed orthoses include shoe lifts, AFOs and KAFOs. These orthoses often improve gait quality and gait safety and reduce knee and general pain. Kelly and DiBello (2007) provide a useful classification system for making decisions about orthoses for people with PPS. Use of assistive devices may also need to be considered. Lifestyle Modification 871 People with PPS must change their lifestyle. Although this is easy for us to say, it is very difficult for them to do. Having survived polio and not let it get the best of them, these individuals often resist seeing the need for and implementing change. Mobility is freedom and independence, which is something they fought for and achieved a long time ago. Change is going to come slowly. The adage of working through pain was used successfully before and so they might think that this strategy will work again. Slowing down seems a poor option when it is equated in their mind to give in. A recent review by Gonzalez et al. (2010) suggests reducing physical and emotional stress, joint protection, modification of work and home environments, and the use of mobility aids to reduce fatigue and preserve function. Others recommend energy conservation, weight loss, and use of an assistive device as lifestyle changes to combat fatigue and musculoskeletal pain (joint and muscle pain). Energy Conservation Because of the far-reaching effects of fatigue and the danger of overuse, energy conservation must be an integral part of the management of a patient with PPS, and may be the most important aspect of management. Energy conservation is a means of modifying a person’s lifestyle to conserve energy. It can incorporate changes in the environment, the task, or the way the mover performs the task. One person with PPS may need to use an assistive device when none was used before to conserve energy relative to ambulation. Someone else may require the use of an electric scooter. When performing ADLs, the person has to ask if the task can be done in one trip rather than three. For example, can all the dishes be unloaded from the dishwasher onto a cart and the cart moved to a location where all the dishes can be put away rather than making multiple trips to and from the dishwasher to various locations. Can the person sit rather than stand to perform filing (if that is part of the person’s job)? Analysis of activities that constitute a person’s day can be helpful in determining where changes can easily be made. Activity pacing is part of energy conservation and, therefore, of lifestyle modification. Pacing requires a balance between rest and activity. Does the person have more energy in the morning or in the afternoon? Taking advantage of planning activities according to when energy is available makes good sense. Taking more frequent rest breaks may allow someone to continue to work as well as perform daily household activities. Adequate rest may be different for every individual with PPS. Daytime naps may be needed. Continuing to do our “jobs” whatever that entails leads to having a better sense of self and quality of life. Therefore, the assistant should council the person with PPS to increase the amount of rest while reducing stress (Halbritter, 2001). Balance Between Activity and Rest Physical therapy management of the patient with PPS is aimed at decreasing the workload of muscles used on a daily basis. Nonfatiguing exercise protocols, energy conservation, activity pacing, breathing exercises, and coordination of breathing with activity are all strategies that are used at some point with a person experiencing PPS. The biggest challenge comes not in identifying intervention strategies but in helping the person find the most beneficial balance between activity and rest. How much exercise can the person do while conserving energy throughout the daily routine? This is a real balancing act. More is not better in this case, less is best. Chapter summary The neurologic disorders reviewed in this chapter have several things in common. They all significantly impact the ability of a person to function. Mobility, daily living activities, job performance, and participation in leisure activities may all be seriously compromised as a result of these disorders. All of these disorders produce fatigue and create the potential for deconditioning regardless of the underlying pathologic process involved. Exercise is beneficial for the individual with any of these neurologic disorders, even in the case of an individual with amyotrophic lateral sclerosis. Exercise is the central strategy and the most crucial part of the overall therapeutic management plan. Precautions regarding overuse are applicable to all patients with these types of neurologic disorders. Regardless of specific disorder, interventions require all individuals to find a balance between the amount of rest and activity that can be tolerated while continuing to optimize function. Early intervention, which in this context means “soon after diagnosis,” provides the person the best possible plan of care. This initial plan of care may contain many episodes and 872 allows for continual modification of the intervention strategies based on disease progression or recovery. The plan is instituted and carried out by a team of health-care practitioners. The physical therapist and physical therapist assistant are part of the team that play an important role in managing individuals with Parkinson disease, multiple sclerosis, amyotrophic lateral sclerosis, Guillain-Barré syndrome, and postpolio syndrome. Case Studies Rehabilitation Unit Initial Evaluation: JB History Chart Review: JB was transferred to a regional medical center from a rural county hospital for severe progressive weakness 3 weeks ago. The patient was admitted through the emergency room on the day before the transfer, complaining of weakness in all extremities. He had a viral infection a few days earlier, with diarrhea, fever, and chills. No previous history of diabetes, chronic obstructive pulmonary disease (COPD), heart disease, or hypertension. Patient had previous hospitalization via the emergency room for kidney stones. He has no allergies and is on no medications. He recently completed a course of IV gammaglobulin. PT order for examination and treatment received upon transfer to the rehabilitation unit. Subjective JB states that he is married and is a high school math teacher. He reports having a viral illness lasting 3 days from which he fully recovered. Three weeks ago, he noticed that he had difficulty writing because of arm weakness. On admission to the rural hospital, he had partial paralysis of his arms and total paralysis of his legs. He had no pain. He and his wife were anxious about the reason for his transfer to a regional medical center, but following diagnosis and treatment of Guillain- Barré syndrome (GBS), they are looking forward to his recovery. He grows tomatoes as a hobby. He lives in a one-story house with two steps to enter. He gives consent for the examination. Objective Appearance/Equipment: Patient is supine in bed on an egg-crate mattress. A Foley catheter in place. Systems Review Communication/Cognition: Speech is normal. He understands multiple step directions, is alert and cooperative. Cardiovascular/Pulmonary: HR 82 b/min; BP 130/90 mm Hg; RR 20 b/min; Integumentary: Skin intact, no redness or edema Musculoskeletal: PROM intact; AROM impaired Neuromuscular: Gait, locomotion, and balance impaired. UE and LE paralysis; sensation intact proximally, impaired distally. Psychosocial: Wife is at bedside. Tests and Measures Anthropometric: Height, 6' 3", weight, 190 lbs. Arousal, Attention, and Cognition: Oriented x 3, mental status intact. Circulation: Skin is warm to touch, pedal pulses present bilaterally, strong radial pulse Ventilation/Respiration: Breathing pattern is 2-neck, 2-diaphragm. No chest wall expansion noted. Epigastric rise is 1/2". Vital capacity is 3 L, 50% of normal. Cranial Nerve Integrity: Cranial nerves intact. Reflex Integrity: Biceps 2 +, patellar, Achilles 0 bilaterally; Babinski absent bilaterally; muscle tone is flaccid in the lower extremities, trunk, and below the elbows; tone in the arms, shoulders, and neck appears normal. Range of Motion: PROM WEL; active shoulder flexion/abduction in sitting to 60 degrees bilaterally, active elbow flexion to 90 degrees bilaterally, elbow extension lacks 15 degrees from complete extension, neck motion WEL, no other active movement. Motor Function: Patient requires max assist 1 for rolling and coming to sit. Patient can sit up supported in bed for 20 minutes at a time. He is dependent in sitting and standing. Patient requires max assist of 2 for bed «-— W/C transfer. Muscle Performance: Tested per Berryman Reese manual muscle testing procedures. Patient is 873 in supported sitting with appropriate stabilization. Muscles of facial expression are intact bilaterally. t] co Upper trapezius| Wrist extensors Finger flexors ip flexors [0 | [0 | [0 | Lo | [0 | elelelelele tele [Upper rapeziug [Wrist extensors| [Finger flexors _| [Anterior tibialis Anterior tibialis] 0 [0 | [Gastrocsoleus [0 [0 | Sensory Integrity: Pinprick intact throughout the upper extremities except diminished below the wrists; intact on the trunk and lower extremities to the knees, absent below. Pain: 0 ona scale of 0-10. Posture: At rest, the patient is in supine on an ege-crate mattress with a Foley catheter in place. His upper limbs are flexed across his lower trunk. His lower limbs are externally rotated at the hips, extended at the knees, and plantar flexed at the feet. Gait, Locomotion, and Balance: Dependent in gait and locomotion. Patient is unable to take any challenges in a supported sitting position. Self-Care: Dependent in feeding, dressing, personal hygiene. Assessment/evaluation JB is a 53-year-old married, male teacher who, after experiencing a viral illness, was hospitalized with paralysis of his arms and legs. On day 2, he was transferred from a local hospital to a regional medical center for continued evaluation and treatment. The diagnosis of GBS was made and he underwent IV infusion with gammaglobulin. He is dependent in transfers and locomotion. Functional Independence Measure: transfers 1, locomotion 1. He is being transferred to the rehabilitation unit at the medical center. Problem List 1. Dependent in mobility 2. Dependent in activities of daily living (ADLs) and transfers 3. Decreased strength and endurance 4. Dependent in pressure relief 5. Lacks knowledge of disease course and rehabilitation Diagnosis: JB exhibits impaired motor function and sensory integrity associated with an acute polyneuropathy which is guide pattern 5G. This pattern includes Guillain-Barré syndrome. Prognosis: Over the course of 2 months, JB will improve his level of functional independence and functional skills. Changes will be limited by the degree and rapidity of recovery of muscle function and strength and any residual musculoskeletal or neuromuscular deficits. Short-Term Goals (2 weeks) 1. JB will maintain passive range of motion of all joints within functional limits for ADL. 2. JB will increase vital capacity to 100% to improve cough effectiveness. 3. JB will demonstrate a 2-chest, 2-diaphragm breathing pattern to increase tolerance to upright. 4. JB will increase strength in all innervated muscles to 3 + to improve sitting and standing balance. 5. JB will increase tolerance to upright sitting in a wheelchair to 4 hours a day with no loss of skin integrity. 6. JB will roll supine — prone and back with min assist of 1 for pressure relief. 7. JB will transfer from bed to wheelchair with min assist of 1 using stand pivot. Long Term Goals (6 weeks at Discharge from Rehabilitation Unit) 1. JB will ambulate 150 feet x 3 independently with or without and assistive device. 2. JB will negotiate a set of 4 stairs with handrails. 3. JB will stand for 45 consecutive minutes (class period) without a break. 4. JB will drive his car from home to school. 5. JB will plant 5 tomato plants without a rest break. Plan Patient will be seen twice a day 5 days a week and once on Saturday and Sunday for 45-minute treatment sessions. Treatment sessions are to include positioning, range of motion, pulmonary rehabilitation, functional mobility training, patient/family education, and discharge planning. 874 Patient will be reassessed weekly. Coordination, Communication, and Documentation: The physical therapist and physical therapist assistant will be in constant contact. The physical therapist will also be communicating with the occupational therapist, the respiratory therapist, the physician, the nursing staff, and the nutritionist. Patient/Client Instruction: JB and his wife will be educated regarding the pathologic process involved in GBS, the importance of range of motion, monitoring for changes in muscle function, and avoiding overuse. Procedural Interventions 1. Passive range of motion to all extremities that lack voluntary movement. 2. Positioning program to prevent contractures including low top tennis shoes. 3. Turning schedule for pressure relief. 4. Chest wall stretching. 5. Diaphragm strengthening and incentive spirometry. 6. Transfer training progressing from sit pivot—stand pivot to and from the bed to commode, bed to wheelchair (W/C); W/C to car. Tilt table for standing. . Strengthening exercises as muscle function returns. 9. Endurance training using a nonfatiguing protocol. 10. W/C mobility training. 11. Gait training progressing from parallel bars to level ground to elevations. 12. ADL training with upper extremity support and hand over hand progressing to independent feeding, dressing, and toileting. 13. Monitor muscle and sensory return. goN Discharge Planning JB will be discharged to home with spouse. A home and school assessment will be performed if needed and equipment secured as necessary. Vocational rehabilitation will be contacted. Questions to think about a What procedural interventions are appropriate for the physical therapist assistant to perform? a When would transfers to sitting and standing be initiated? a What signs and symptoms should the physical therapist assistant use to indicate a negative change in status? Review questions 1. What is the most common cause of acute paralysis in adults? 2. What is one of the three most common movement disorders seen in the United States? 3. 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A Abduction splint, simple, myelomeningocele and 180f Abnormal positioning, following cerebrovascular accidents 309-310 Abstract thought 60 Academic skills, hemispheric specialization and 15t Acclimation, to upright position, of spinal cord injury patients 414-415, 415f Acetylcholine 11, 462 Acquired brain injuries 370 Acquired cerebral palsy 131 Acquired inflammatory demyelinating polyradiculoneuropathy 479 Acquired scoliosis, myelomeningocele and 175 Activities of daily living cerebral palsy and 161-162, 162t myelomeningocele and 192-193 Activity-dependent plasticity 51 Activity limitations 2 Acute care setting 310 Acute motor axonal neuropathy 479 Adams' closed-loop theory, of motor learning 47 Adaptation, as developmental process 66 Adapted tricycle 209f Adaptive equipment See also Assistive devices age appropriate 126t Duchenne muscular dystrophy and 228 goals for 119, 119b for Guillain-Barré syndrome 482-483 positioning and mobility and 117-126 side lyer 124b for standing 125b Adaptive seating 122-123 devices for 123f Adolescence 58, 162-164, 216 Adulthood 58, 216 882 Advanced balance exercises 357 Aerobic training, for spinal cord injury patients 440-441 Afferent tracts 12-13 Aggressive behaviors, traumatic brain injuries and 388-389 Agnosia, visual 303 Agonistic reversal technique 275-277, 278b Air splints 101f, 319 Airlift transfer 425, 428b Akinesia 462 Alcohol exposure 171-173 Allergy, latex, myelomeningocele and 178 Alpha-fetoprotein 173 Alternating isometric technique 267-273, 276b, 285b, 289b, 419b Alzheimer disease, Down syndrome and 205 Ambulation arthrogryposis multiplex congenita and 210 cerebral palsy and 144, 152-153, 153t cerebrovascular accidents and 302, 342-349, 343-344b Duchenne muscular dystrophy and 228 level of 191, 191b for multiple sclerosis 477 myelomeningocele and preparation for 182-183 reevaluation of 193-194 progression of 344t resisted progression technique and 296 spina bifida and 186t therapeutic 408-409 training, for spinal cord injury patients 442-452, 445b backing up in 446 environmental barriers in 450-452 falling in 448, 451b gait progression in 446, 447b gait training with crutches in 448-450, 448b orthoses for 443-444, 444f preparation for 445 progressing in 446-448 quarter-turns in 446 sitting in 446 swing-through gait pattern in 446 American Physical Therapy Association (APTA) 2, 310-311, 369 883 Amnesia, concussion and 368-369 Amyotrophic lateral sclerosis (ALS) 478-479 Anencephaly 171 Aneurysms, cerebrovascular accidents and 301 Angelman syndrome 206 Ankle 356 Ankle dorsiflexion promoting of 314-315, 317b spinal cord injury patients and 428, 434b Ankle-foot orthoses (AFOs) cerebral palsy and 156-157 cerebrovascular accidents and 347-349, 347-349f for multiple sclerosis 477, 478t myelomeningocele and 186-187, 188f Ankle splinting 158¢ Anoxia 132 Anoxic injuries 371 Anterior and posterior weight shifts, in tilt board 359-360 Anterior cerebral artery occlusion 302 Anterior cord syndrome 401, 401f, 401¢ Anterior depression, scapular 261b Anterior elevation pelvic 270b scapular 260b Anterior hiking 306 Anterior horn cells 21 postpolio syndrome and 483-484 Anterior tilting 306 Anterograde amnesia, concussion and 368-369 Anticholinergics, for Parkinson disease 464-465 Anticipatory postural adjustments, for Parkinson disease 466 Anticipatory preparation 43 Antigravity extension 64 Antigravity neck flexion 72, 109 Aphasia, cerebrovascular accidents and 306 Approximation 103-104, 103-105b, 313 proprioceptive neuromuscular facilitation and 251 Apraxia 306 Aquatic exercise postpolio syndrome and 485 Prader-Willi syndrome and 206, 207t 884 Aquatic therapy, for spinal cord injury patients 441-442 Arachnoid layer 13 Arnold-Chiari malformation 176, 176f Arousal 372 Arteries 301 Arteriovenous malformations (AVMs) 301 Artery occlusion 302 Arthrogryposis multiplex congenita 206-210, 208f, 209t, 210-211f Articulated ankle-foot orthoses 348-349, 348-349f Ashworth Scale 304, 304+ ASIA International Standards for Neurological Classification of Spinal Cord Injury 396, 397f Aspen collar 399f Asphyxia, cerebral palsy and 132 Aspiration, cerebrovascular accidents and 307 Assisted cough techniques, for spinal cord injury patients 410, 412b Assistive devices See also specific devices cerebrovascular accident and 344-346 Parkinson disease and 466 postpolio syndrome and 485 for spinal cord injury patients 454-455 tone reduction and head lifting and 109b Associated reactions, cerebrovascular accidents and 308, 308t Association cortex 15 Astrocytes 10, 12f Asymmetrical tonic neck reflex 142-143, 143f, 144t, 308t Asymmetry, cane use and 346 Ataxia cerebral palsy and 135-137, 136f, 144-146 cerebrovascular accidents and 303 multiple sclerosis and 470, 474-477 Atherosclerosis, thrombotic CVAs and 300 Athetoid cerebral palsy 134, 136f Athetosis, cerebral palsy and 135, 136f, 137, 144-146, 145¢ Atlantoaxial instability (AAI), Down syndrome and 202-203, 203b Atonic cerebral palsy 134 Attention deficits, traumatic brain injuries and 387 Attentional strategies, for Parkinson disease 466 Autogenic drainage, cystic fibrosis and 219 Autoimmune dysfunction, multiple sclerosis and 470 Autonomic dysreflexia, in patients with spinal cord injuries 402-403, 422-423 Autonomic nervous system (ANS) 25-26, 28-30f 885 multiple sclerosis and 471 Autosomal dominant inheritance 202 Autosomal dominant trait 202 Autosomal recessive inheritance 202 Autosomes 201-202 Avonex, for multiple sclerosis 471 Awareness, traumatic brain injuries and 372, 375, 379-380 Axonal sprouting, peripheral nerve injuries and 30 Axons 11, 470 B Babinski sign 20, 20f Backing up, of spinal cord injury patients 446 Baclofen 158-161, 161f, 405 Balance cerebral palsy and 156b cerebrovascular accidents and 331-332, 339, 340f, 356-360 changes in, with aging 86-87 strategies, in sitting 45 Bands See Elastic bands Basal ganglia 461 Basal nuclei 16-17 Base of support 252 Basilar breathing, for spinal cord injury patients 410 Bear walking, motor development and 77, 77f Becker muscular dystrophy 229 Bedside activities, cerebrovascular activities and 314-315, 315-317b Behavior 15t Behavioral deficits, traumatic brain injuries and 373, 388 Behavioral phenotype 201 Berry aneurysm, cerebrovascular accidents and 301 Betaseron, for multiple sclerosis 471 Biomechanics, proprioceptive neuromuscular facilitation and 252 Birth weight, cerebral palsy and 132-133 Bladder dysfunction cerebrovascular accidents and 308 multiple sclerosis and 471 myelomeningocele and 178 spinal cord injuries and 404 Blocked practice, motor learning and 49 Blood pressure, of spinal cord injury patients 402 886 Bobath, Karel and Berta 322, 375 Body jacket 399f Body mechanics, proprioceptive neuromuscular facilitation and 250 Body position, proprioceptive neuromuscular facilitation and 250 Body-weight support treadmill training (BWSTT) 153-154, 154f, 383, 452-453, 452-453b Down syndrome and 205 Bones See Skeletal system Borg Perceived Exertion Scale 439-440 Botulinum toxin 159, 405 type A, for abnormal posturing and 309 Bowel dysfunction cerebrovascular accidents and 308 multiple sclerosis and 471 myelomeningocele and 178 spinal cord injuries and 404 Brachial plexus 23, 24f Bradykinesia 462 Bradyphrenia 464 Brain 14f, 131 See also Traumatic brain injuries Brain attack 302 Brain Injury Association of America 368 Brain stem 17-18, 18f reflexes, cerebrovascular accidents and 308, 308t, 318-319 Breath support, for cerebrovascular accidents 311 Breathing cystic fibrosis and 216-217 diaphragmatic 219, 221b, 409f exercises, for cerebrovascular accidents 311 inefficiency, cerebral palsy and 138-139 spinal cord injuries and 404 Breathlessness positions, cystic fibrosis and 219, 220b Breech presentation, cerebral palsy and 132 Bridging 277, 278b, 280, 313, 313-314b Broca aphasia 306 Broca’s area 14—15 Bronchial hygiene, of spinal cord injury patients 410 Bronchiectasis, cystic fibrosis and 216-217 Brown-Séquard syndrome 400-401, 401f, 401 Brunnstrom, Signe 304 Brunnstrom stages of motor recovery 304-305, 305t Bulbar palsy, Guillain-Barré syndrome and 480 887 Cc C1 through C3, injuries at, functional potentials of patients with 406-408 C4, injuries at, functional potentials of patients with 408 C5, injuries at, functional potentials of patients with 408 C6, injuries at, functional potentials of patients with 408 C7, injuries at, functional potentials of patients with 408 C8, injuries at, functional potentials of patients with 408 Calcaneovalgus foot 175f Campylobacter jejuni 479-480 Canes, cerebrovascular accident and 345-346, 346f Carbamazepine (Tegretol), for seizures 371 Cardiopulmonary retraining, cerebrovascular accidents and 311-313 Cardiopulmonary system, Guillain-Barré syndrome and 483 Cardiopulmonary training, for spinal cord injury patients 439-441 Cardiovascular system, multiple sclerosis and 472 Carotid arteries, common 26-28 Carrying positions cerebral palsy and 148 head control and 111 interventions for 100b Cat-cry syndrome 205 Catching, motor development and 82, 82f, 84f Cauda equina, injuries to 395-396, 401, 401¢ Caudate nucleus 16-17 Cell body, defined 10-11 Center of gravity 252 Central cord syndrome 401, 401f, 401¢ Central nervous system (CNS) 10 deterioration 176-177 Cephalocaudal development 63, 63f Cerebellum 17, 18f Cerebral circulation 26-29, 302, 302t anterior 26-28, 31f posterior 28-29 Cerebral cortex 18f motor areas of 15 Cerebral hemispheres 13, 13f, 17f Cerebral infarct 300 Cerebral palsy 131-170, 164b case studies on 165b causes of 131 888 perinatal 132-133, 133f prenatal 131-132 classification of 133-136 deficits associated with 137-141, 139b diagnosis of 137 early intervention for 147-154 etiology of 131-133, 132t functional classification of 136-137, 137t, 138f incidence of 131 interventions for adulthood 164 preschool period 154-162 school age and adolescence 162-164 pathophysiology of 137, 139t physical therapy for examination 141-145 intervention 145-165 risk factors associated with 132t Cerebrospinal fluid (CSF) circulation 171, 176f Cerebrovascular accidents (CVAs) 300-367, 362b abnormal tone management and 360-361 acute care setting and 310 acute medical management of 301 ambulation and 342-343 balance exercises and 356-360 cardiopulmonary activities and 311 case studies on 363b complications following 309-310 coordination exercises and 356 definition of 300 developmental sequence and 349-353 diagnosis of 301 directing interventions to physical therapist assistant 310-311 discharge preparation and 361 early functional mobility tasks and 313-322 environmental barrier negotiation and 354-356 etiology of 300-301 facilitation and inhibition techniques and 317-322 fine motor skills and 356 functional activities and 323-325 functional limitations after 308 889 gait and 341 home environment and 361-362, 362b impairments from 304-308 leaving items within reach and 313 medical intervention for 301 midrecovery to late recovery of 353-362 movement assessment and 316-317 movement transitions and 324-325 neglect and abnormal tone and 312-313 neurodevelopmental treatment approach and 322 orthoses and 347-349 physical therapy intervention for 311-353 positioning and 311 prevention of 302 recovery from 301-302 reflex and 307, 307-308t sitting and 325-334, 328f standing and 334-344 syndromes of 302-304, 302t treatment planning and 308-309 functional assessments of 309 goals and expectations of 309 upper extremity activities and 317, 318b Cerebrovascular anatomy 26 Cerebrum 17f lobes of 14-15 Cervical plexus 22-23, 24f Cervical spine 395 Chest physical therapy, cystic fibrosis and 217 Chest wall stretching, for spinal cord injury patients 410, 411b Child abuse, traumatic brain injuries and 368 Childhood, as developmental time period 57-58 Children, with neurologic deficits 91, 92 Child's impairments cri-du-chat syndrome and 205-206 cystic fibrosis and 217-222 Down syndrome and 205 Duchenne muscular dystrophy and 225-229 intellectual disability and 233-241 osteogenesis imperfecta and 211-216 Prader-Willi syndrome and 206-210, 207t, 208b 890 Chin cup, for patients with spinal cord injuries 406-408 Cholinergic activity, Parkinson disease and 462 Chopping pattern 262, 273b Chops See Lifts and chops Chromosomes arthrogryposis multiplex congenita and 206-207 cat-cry syndrome and 205 cri-du-chat syndrome and 205 cystic fibrosis and 216 Down syndrome and 202 fragile-X syndrome and 229-230 genetic transmission and 201-202 Prader-Willi syndrome and 206 Circuit training, for spinal cord injury patients 441 Classification of cerebral palsy 133-136 of intellectual disability 233 of Parkinson disease 464, 464t of spinal cord injuries 396 of traumatic brain injuries 368-372 Clonazepam 158-159 Clonus 30-32, 307-308 Closed injuries 368 Closed skills, motor learning and 49 Clouding of consciousness 372 Clubfoot 175f, 210 Cocktail party speech 190 Cocontraction 36-37 Cognition adolescence and 58 hemispheric specialization and 15t level of 376, 377t motor development and 59-62 myelomeningocele and 189-190, 193, 193b traumatic brain injuries and 376 Cognitive deficits fragile-X syndrome and 230-231 multiple sclerosis and 470-471 traumatic brain injuries and 372, 387-389 Cogwheel rigidity, Parkinson disease and 462 Cold intolerance, postpolio syndrome and 484 891 Collagen 203-204 Coma, traumatic brain injuries and 372 “Commando crawling,” 93, 144 Commission on Accreditation in Physical Therapy Education (CAPTE) 4 Communication cerebral palsy and 138-139 cerebrovascular accidents and 306 Guillain-Barré syndrome and 480 traumatic brain injuries and 373 Community integration, cerebral palsy and 164 Community reentry, of spinal cord injury patients 455 Compensation, traumatic brain injuries and 383 Compensatory approach, to spinal cord injuries 415-416 Complete injuries, of spinal cord 400 Complex regional pain syndrome (CRPS) 310 Complications, cerebrovascular accidents and 309-310 Compression 103-104, 103b, 313 injuries, in spinal cord 398, 398f Concentration, Parkinson disease and 464 Concrete operations 57-58, 60 Concussion 368-369 Confabulation 372 Conference, discharge planning 453-454 Congenital cerebral palsy 131 Congenital heart disease 206 Congenital scoliosis, myelomeningocele and 175 Conjugate eye gaze, cerebrovascular accidents and 303 Consciousness, traumatic brain injuries and 372 Constant practice, motor learning and 49 Constraint-induced movement therapy (CIMT) 150 Contract relax technique 267 Contractures arthrogryposis multiplex congenita and 206-207 cat-cry syndrome and 206 cerebrovascular accidents and 309 genetic disorders and 237-238 myelomeningocele and 186 spinal cord injuries and 403 traumatic brain injuries and 374, 379 Contrecoup lesions 369, 369f Control See Motor control See also Postural control 892 Controlled mobility See also Mobility agonistic reversal technique and 275-277 bridging and 280-281 kneeling and 284, 290b pregait activities and 292-296 prone progression and 283 quadruped position and 287b sitting and 327 slow reversal technique and 275 standing position and 292 supine progression and 280 Contusion 369-370 Conus medullaris syndrome 401, 401t Coordination 356 See also Ataxia; Motor coordination multiple sclerosis and 470 Copaxone, for multiple sclerosis 471 Corner chair 97f Cortical blindness, cerebrovascular accidents and 303 Coughs 219, 410 Coup lesion 369, 369f Cranial nerves 21, 22t, 303, 307, 479 Creeping 278, 432 cerebrovascular accidents and 350 as milestone of motor development 67, 68f motor development and 77 quadruped position and 93 as skill movement 38 Cri-du-chat syndrome 205-206 Crisis, traumatic brain injuries and 388 Critical periods, neural plasticity and 50-51 Cross extension reflex 307 Crouching 151b Cruising 67, 68f, 78, 79f Crutches 189 gait training with 448-450 Curbs 356, 438-439, 440b, 451 Cystic fibrosis (CF) 216-222 diagnosis of 216 pathophysiology and natural history of 216-217 Cysts See Myelomeningocele 893 D Dantrium 158-159, 309 Dantrolene 158-159 Dantrolene sodium, for abnormal posturing and positioning 309 Deafness, cerebrovascular accidents and 303 Decerebrate rigidity 372-373 Decorticate rigidity 372-373 Deep brain stimulation, for Parkinson disease 465 Deep tendon reflexes (DTRs) 223, 307-308, 480 Deep vein thrombosis, spinal cord injuries and 403-404 Deformities genetic disorders and 237-238 prevention of, myelomeningocele and 179 Degrees of freedom 44-45 Delayed postural reactions 233-234, 233f Deletions defined 202 partial, chromosome abnormalities and 202 Delirium 372 Dementia amyotrophic lateral sclerosis and 479 Parkinson disease and 464 Dendrites 10-11 Deprenyl, for Parkinson disease 464-465 Depression 310 multiple sclerosis and 471 Parkinson disease and 464 Dermatomes 21, 177, 396 Developmental intervention 93-95 Developmental sequence 279-297, 349-353 Diabetes, cerebral palsy and 132, 132t Diagnosis of cerebral palsy 137 of cerebrovascular accidents 301 of multiple sclerosis 471 of Parkinson disease 464 in patient/client management 3-4 Diagonal movement patterns 252 lower extremity 254-257, 263f, 264t, 265-266b, 267t, 268-269b, 282b scapula and pelvic 254, 262f upper extremity 253f, 254t, 255-256b, 257t, 258-259b 894 Diaphragmatic breathing 219, 221b, 409f Diaphragmatic strengthening, cerebrovascular accidents and 311 Diazepam 158-159 Diencephalon 16, 17f Diffusion weighted imaging, cerebrovascular accidents diagnosis and 301 Diplegia 133-134, 133f Diplopia 303, 470, 480 Disability, as Nagi Disablement Model component 1 Discharge planning for spinal cord injury patients 453-456 traumatic brain injuries and 390 Disease, as Nagi Disablement Model component 1 Disorientation, traumatic brain injuries and 387 Dissociation 63, 72-73 Distributed control 44 Distributed practice, motor learning and 49 Dizziness, cerebrovascular accidents and 303 Dopamine 11, 461 Dorsal columns 400-401 Dorsal column syndrome 401, 401f, 401¢ Double-arm elevation 317, 318b with splint 322b Down syndrome 202-205, 203-204f, 234f Drag crawling, defined 93 Draw sheet, to assist bridging 314b Driver education, myelomeningocele and 194 Dual-channeled air splints 319 Dual task training 357 Duchenne muscular dystrophy medical management of 227-228 pathophysiology and natural history of 225 Duchenne muscular dystrophy (DMD) 224-229, 228b, 229f, 230t Dura mater 13 Dynamic balance activities 357 Dynamic postural control See Controlled mobility Dysarthria 303, 306, 480 Dysautonomia 461-462 Dysesthesias, multiple sclerosis and 470 Dyskinesias 135, 465, 468-469 Dysphagia 303, 307, 480 Dyspnea scale 222t 895 Dysreflexia 402-403 Dystonia 465 cerebral palsy and 135 Dystrophin 225 E Early adulthood transition 58 Ecological plasticity 51 Edema, spinal cord injuries and 399 Efferent fiber tracts 12-13 Elastic bands See also TheraBand as sling 346 for strengthening exercises 413-414 Elbow splint 319-321 Electric stimulation, for spinal cord injury patients 452-453 Embolic origin, CVAs of 300 Emotional lability 306 Emotions 15t, 306, 477-478 Encephalopathy 131 Endurance training Guillain-Barré syndrome and 482-483 myelomeningocele and 192, 194-195 spinal cord injury and 439 Energy conservation, postpolio syndrome and 486-487 Environmental accessibility, myelomeningocele and 194 Environmental adaptation, motor control 43 Environmental barriers, negotiation of 354-356 Environmental control units, for spinal cord injury patients 455 Environmental factors, in Parkinson disease 462 Ependymal cells 10, 12f Epidural hematomas 370, 370f Epidural space 13 Epigastric rise 409-410 Epigenesis, motor development and 62, 62f Epiphyses, maturation and 64-66 Equilibrium reactions cerebrovascular accidents and 340f motor control and 38t, 39 motor development and 78, 78f myelomeningocele and 182, 183b Equinovarus foot 175f 896 Equipment See Adaptive equipment See also Assistive devices Erikson's theory of development See Maslow and Erikson's theory of development Erythroblastosis, cerebral palsy and 137 Esotropia, cerebral palsy and 140 Evaluation, in patient/client management 3-4 Examination, in patient/client management 3-4 Exercises cerebral palsy and 149 cystic fibrosis and 219-222 Duchenne muscular dystrophy and 226 multiple sclerosis and 472 nonfatiguing 485, 486 Parkinson disease and 469, 469t postpolio syndrome and 485 spinal cord injury and breathing 409-410, 409f pool 442 range of motion 411-413 Exotropia, cerebral palsy and 140 Experience-dependent neural plasticity 51, 51t Experience-expectant neural plasticity 51 Expressive aphasia, cerebrovascular accidents and 306 Extension antigravity 64 diagonal movement patterns and 252, 253f lower extremity 254-257, 264t, 266b, 267t, 269b upper extremity 253f, 254, 254t, 256b, 257t, 259b trunk, interventions for 124b Extremity See also Lower extremities; Upper extremities usage of 144 Eye-head stabilization 42 F Face washing 381b Facial muscles, cerebrovascular accidents and 307 Facilitation techniques, for cerebrovascular accidents 317-319 Falling 448, 451b, 464 Family education cerebral palsy management and 147 myelomeningocele and 184-185 for spinal cord injury patients 455 897 traumatic brain injuries and 376, 380 Family participation, cerebrovascular accidents and 356 Family systems 58-59 Fasciculation, amyotrophic lateral sclerosis and 478 Fatigue cerebrovascular accidents and 307 multiple sclerosis and 470 Parkinson disease and 463, 469 postpolio syndrome and 484 Feedback 40 role of 34-35 Feedforward processing 43 Feeding cerebral palsy and 137-138, 148-149, 149b Down syndrome and 202-203 Prader-Willi syndrome and 206 Feet, myelomeningocele and 180-181 Festination, Parkinson disease and 463 Fine-motor activities 189 Fire hydrant position 257 Fitness 163-164, 204 Fitts’ stages, of motor learning 48, 48 Flaccid bladder, spinal cord injuries and 404 Flaccid muscles 304 Flexibility 192, 194-195 Flexion antigravity neck 64, 72, 109 cerebrovascular accidents and 309, 314-315, 316-317b diagonal movement patterns and 252 lower extremity 257, 264t, 265b, 267t, 268b upper extremity 253f, 254, 254t, 255b, 257t, 258b, 267 Parkinson disease and 463 physiologic 64, 64f spinal cord injuries and 397-398, 398f Flexor withdrawal reflex 307t motor control and 35 Floating, for spinal cord injury patient 442 Flutter valves, cystic fibrosis and 220f Focal seizures, cerebral palsy and 140, 140 Folic acid, myelomeningocele and 171-173 Foot splints 158t, 321-322 898 Forced expiration technique, cystic fibrosis and 219 Formal operations stage 58, 60 Forward reaching 432 Four-point activities 350, 350b to tall-kneeling 350 Fractures 216 Fragile-X syndrome (FXS) 229-231, 230f Framingham Heart Study 301-302 Free radical theory 59 Freezing, Parkinson disease and 463 Frenkel exercises 477, 477t Frontal lobe 14-15 Frontotemporal dementia, amyotrophic lateral sclerosis and 479 Fugl-Meyer Assessment, cerebrovascular accidents and 309 Function defined 2 Parkinson disease and 469t postpolio syndrome and 484-485 related to posture 92-93, 92f three domains of 3f Functional activities arthrogryposis multiplex congenita and 209-210 cerebrovascular accidents and 323-325 osteogenesis imperfecta and 213, 214b, 214f Functional coughs, spinal cord injuries and 410 Functional Independence Measure (FIM) 309 Functional limitations, as Nagi Disablement Model component 1 Functional mobility tasks cerebrovascular accidents and 313-322 traumatic brain injuries and 380-381, 381b Functional movement cerebral palsy and 161 myelomeningocele and 173 Functional performance, defined 2 Functional potentials, spinal cord injuries and 406-409, 406t Fundamental movement patterns, motor development and 81-85 G G-aminobutyric acid (GABA) 11 Gait arthrogryposis multiplex congenita and 209-210 899 cerebral palsy and 155-156, 155-156b, 156f, 160 cerebrovascular accidents and 302, 341, 344, 345t Duchenne muscular dystrophy and 227 motor development and 85 multiple sclerosis and 470 myelomeningocele and 190-191, 190b normal components of 341-342 in older adult, changes in, with aging 87-88 osteogenesis imperfecta and 213, 214b, 214f Parkinson disease and 463-466 progression, spinal cord injury patients and 446, 447b proprioceptive neuromuscular facilitation and 292-297, 297b spinal muscular atrophy and 224 Gastroenteritis, Guillain-Barré syndrome and 479-480 Gene therapy, Duchenne muscular dystrophy and 227-228 Generalized seizures, cerebral palsy and 140, 140t Genetic disorders 201-248 Angelman syndrome 206 arthrogryposis multiplex congenita 206-210, 209t, 210-211f autism spectrum disorder 232 Becker muscular dystrophy and 229 case studies on 241b, 243b cri-du-chat syndrome 205-206 cystic fibrosis 216-222 Down syndrome 202-205, 203-204f Duchenne muscular dystrophy 224-229, 228b, 229f, 230t fragile-X syndrome 229-231, 230f intellectual disability and 232-241 myelomeningocele and 171-173 osteogenesis imperfecta 211-216, 211b phenylketonuria 224 Prader-Willi syndrome 206 Rett syndrome 231-232 spinal muscular atrophy 222-224 Genetic transmission 201-202 Genomic imprinting 206 Genu recurvatum, myelomeningocele and 179 Giant motor units, postpolio syndrome and 483-484 Glasgow Coma Scale (GCS) 371, 371 Glial cells, multiple sclerosis and 470 Global aphasia, cerebrovascular accidents and 306 900 Globus pallidus 16-17 Glossopharyngeal breathing 410 Glutamate 11, 300-301 Gluteus maximus, stretching of, for spinal cord injury patients 428, 433b Gower maneuver 224-225, 225f Grasp reflex 307 Grasping, as milestone of motor development 67 Gravity 111f, 179 Gray matter, spinal cord and 399 Gross Motor Function Classification System 136-137, 138f Growth, as developmental process 64, 65f Guide to Physical Therapist Practice 1-2 Guillain-Barré syndrome 479-483 clinical features of 480 medical management of 480 pathophysiology of 480 physical therapy management of 480-483 Gyri 13 H Half-kneeling 284 activities 352-353, 352b Halo vest 399f, 411 Hammock 103, 103f Hamstrings spinal cord injuries and 412-413, 428, 431b stretching of, multiple sclerosis and 472, 473b Hand-over-hand guiding 381b Hand regard, as milestone of motor development 67-68, 69f Hand splint 319-321 Handling See Positioning and handling Handshake grasp 107f Head control cerebral palsy and 141 interventions for 108-111, 110b, 113b as milestone of motor development 66, 66f, 71f myelomeningocele and 181-182, 181b, 181f positioning for encouragement of 108-109 sitting position and 112f traumatic brain injuries and 380 Head lifting 901 ball use for 109b interventions for 109b, 119b Head positioning, sitting position and 328-329 Head stabilization in space strategy (HSSS) 42 Health-care needs, long-term, of spinal cord injury patients 456 Hearing 104, 141, 203 Heart disease, cerebrovascular accidents and 302 Heel cords, stretching of, multiple sclerosis and 472, 473b Hematomas 370, 370f Hemiplegia 133f, 137, 322 supine positioning for 311-312, 312b Hemispheric specialization 15-16, 15t Hemiwalkers, cerebrovascular accidents and 344-345 Hemorrhage 1321, 137, 370, 399 Hemorrhagic cerebrovascular accidents 301 Hemorrhagic strokes 301 Heterotopic ossification 375, 403 Heterozygous, defined 202 Hierarchical theories, of motor control 35-39 development of 36, 37f equilibrium reactions and 38t, 39 postural control and 38-39 protective reactions and 39 righting reactions and 38-39, 38t stages of 36-38, 38f Hip extension 315b Hip flexion 314-315, 316b Hip-knee-ankle-foot orthoses 184 for multiple sclerosis 477 for myelomeningocele 184, 185f for osteogenesis imperfecta 213-215 Hip rotators, stretching of, for spinal cord injury patients 428, 433b Hip swayer 432 Hitching 77 Hoehn and Yahr classification of disability 464, 464t Hold relax active movement technique 264-266, 293b Hold relax technique 267 Home environment 361-362 Home exercise program, for spinal cord injury patients 455 Home program 94-95 Homeostasis 21 902 Homolateral limb synkinesis 308 Homonymous hemianopia 140-141, 303 Homozygous, defined 202 Hook lying position 264, 279, 280b Hopping, motor development and 81 Horn cells 222-223 postpolio syndrome and 483-484 Hydrocephalus, myelomeningocele and 176, 177f Hydromyelia, myelomeningocele and 177 Hygiene, myelomeningocele and 195 Hyperextension, spinal cord injuries and 398, 398f Hyperflexion, spinal cord injuries and 398, 398f Hyperreflexia, peripheral nerve injuries and 30-32 Hyperreflexic bladder, spinal cord injuries and 404 Hypersensitivity, to touch 102 Hypertension 302, 402 Hypertonia cerebral palsy and 134, 157 holding and carrying and 98-99 Hypesthesias, in Guillain-Barré syndrome 480 Hypokinesia, Parkinson disease and 465 Hypotension 403, 414-415 Hypothalamus 16 Hypotonia cerebral palsy and 134, 134f, 157 cri-du-chat syndrome and 205 Down syndrome and 203-204 genetic disorders and 233-234, 233f holding and carrying and 98-99 Prader-Willi syndrome and 206 spinal muscular atrophy and 222-223 Hypoxia 137, 300 I Ice application, cerebrovascular accidents and 319 Idiopathic Parkinson disease (IPD) 461-462 Immune responses, in Guillain-Barré syndrome 480 Immune system, after spinal cord injuries 399 Immunoglobulins, for Guillain-Barré syndrome 480 Impairments 304-308 myelomeningocele and 173 903 as Nagi Disablement Model component 1 Incentive spirometry, for spinal cord injury patients 410 Incidence of arthrogryposis multiplex congenita 206-207 of Becker muscular dystrophy 229 of cerebral palsy 131 of cri-du-chat syndrome 205 of cystic fibrosis 216 of Down syndrome 202-203 of Guillain-Barré syndrome 479-480 of multiple sclerosis 469 of myelomeningocele 171 of Parkinson disease 462 of Prader-Willi syndrome 206 of spinal muscular atrophy 223 of traumatic brain injuries 368 Incomplete injuries, of spinal cord 400-401, 401f, 401 Incontinence 308 of bowel and bladder 195 spinal cord injuries and 404 Independent living, myelomeningocele and 195 Independent mobility 154-158 Infancy, as developmental time period 57 Infantile spinal muscular atrophy 223 Infants, typical motor development of 70-78 Infections, cerebral palsy and 131-132, 132t Inflammation, Guillain-Barré syndrome and 480 Inflatable air splints 319 Inheritance, autosomal dominant 202 Inhibition techniques, for cerebrovascular accidents 319 Inspiration, deeper, cerebral palsy and 150b Intellectual changes, in Parkinson disease 464 Intellectual disability of Becker muscular dystrophy 229 cerebral palsy and 139-140 classification of 233t of fragile-X syndrome 229-230 of Rett syndrome 231-232 Intelligence development of 59 Down syndrome and 203 904 fragile-X syndrome and 231 multiple sclerosis and 470 myelomeningocele and 189-190 osteogenesis imperfecta and 211 Piaget's theory and 60 spinal muscular atrophy and 222-223 Intelligence quotients (IQs) 203 Internal capsule 16 International Classification of Functioning, Disability, and Health (ICF) 2, 2-3f Interneurons 10 Interventions, in patient/client management 3-4 Intracerebral hemorrhage 301 Intracranial injury 368 Intracranial pressure (ICP) 370-371 Intrathecal baclofen pumps for abnormal posturing and 309 spinal cord injuries and 405 Iron lung, postpolio syndrome and 483, 483f Irradiation, proprioceptive neuromuscular facilitation and 251 Ischemia 137, 300 Ischemic cerebrovascular accidents 300 Ischemic penumbra 300 Isometric stabilizing reversals 267-273, 277b, 285b Isometrics 267-273, 276b J Jack-knife position, spinal cord injury patients and 446 Joints arthrogryposis multiplex congenita and 207 cri-du-chat syndrome and 206 facilitation of 251 hypermobility, Down syndrome and 202-203 postpolio syndrome and 484 proximal 103b, 105 Jumping, motor development and 81, 81f K Kabat, Herman 249 Kernicterus 132 Klonopin 158-159 Knee-ankle-foot orthoses 187f, 443, 444f, 485 905 Knee control, ambulation after cerebrovascular accident and 336-337, 339b Knee flexion 316b Kneeling position advantages and disadvantages of 106t cerebral palsy and 146 four-point to 115 to half-kneeling 115, 117b prone to 116b proprioceptive neuromuscular facilitation and 283-284, 288—290b to side sitting 115 Knott, Margaret 249 Kugelberg-Welander syndrome 223 Kyphosis 175, 215-216 L L3 through LS, injuries at, functional potentials of patients with 409 Lacunar infarcts, cerebrovascular accidents and 303 Landau reflex 73 Language impairments 141 Lateral basal chest expansion 222b Lateral expansion, for spinal cord injury patients 410 Lateral push-up transfer 427 Latex allergy, myelomeningocele and 178 L-dopa, for Parkinson disease 464465 Lead arm 257-262 Lead-pipe rigidity, Parkinson disease and 462 Learning See Motor learning Lee Silverman voice treatment (LSVT®) BIG, for Parkinson disease 468-469 Left cerebral hemisphere, functions of 15-16, 15t Lentiform nucleus 16-17 Lesion function related to level of 174t level of 186 Leukemia, Down syndrome and 205 Leukomalacia, cerebral palsy and 132 Lever arm 252 Levodopa, for Parkinson disease 464-465 Lewy bodies, Parkinson disease and 462 Life expectancy, Down syndrome and 205 Life span concept 56, 57f Lifestyle modification, for postpolio syndrome 486 906 Lifting pattern 257-262, 271b Lifts and chops 351, 351b Limbic system 17 Limits of stability, motor control and 40-41, 42f Lioresal 158-159, 405 Lobes, of cerebrum 14—15 Locked-in syndrome 303, 370 Locomotor training, for spinal cord injury patients 452-453 Lofstrand crutches 191 Long arm splint 319, 320-322b, 321 if Long leg splint 321 Long sitting 120f, 421-424, 422b, 423f push-up in 423-424, 424b Lordosis 175 Lou Gehrig disease 478 Lower extremities advanced exercises for 356 deformities, common 175f proprioceptive neuromuscular facilitation and 254-257, 263f, 264t, 265-266b, 267t, 268-269b Lower trunk rotation 314-315, 316b Lumbar spine, injuries to 395-396 Lumbosacral plexus 23-25, 25-26f Lumbrical grip 250f Lungs cystic fibrosis and 216 expansion, cerebrovascular accidents and 307 M Manual chest stretching, for spinal cord injury patients 410, 411b Manual contacts 99-101, 101f, 105, 250, 250f Manual resistance 250-251, 267-270, 414 Maslow and Erikson's theory of development 60-61, 60f, 61¢ Mass to specific motor development 63 Massed practice, motor learning and 49 Mat activities 416, 428, 431-434 Mat mobility 183-184, 184b Maturation, as developmental process 64-66 Medical intervention See also Physical therapy interventions cerebrovascular accidents and 301 for spinal cord injuries 398, 399f Medical management 907 of amyotrophic lateral sclerosis 479 of Duchenne muscular dystrophy 227-228 of Guillain-Barré syndrome 480 of multiple sclerosis 471 of Parkinson disease 464—465, 465t of postpolio syndrome 485 Medications for cerebral palsy 158-159 for traumatic brain injuries 371 Medulla 17-18 Memory 387 Meninges 13, 13f Meningocele 171, 172t Mental retardation, fragile-X syndrome and 229-230 Methylprednisolone 398 Microcephaly 205 Microglia 10, 12f Micrographia, Parkinson disease and 462 Midbrain 17-18 Middle adulthood 58 Middle cerebral artery occlusion, cerebrovascular accidents and 303 Milestones, motor 66-69, 66t Miller-Fisher syndrome 479 Minimally conscious state 372 Mobility adaptive equipment for 117-126 arthrogryposis multiplex congenita and 207 bridging and 280-281 cerebral palsy and 144, 150-152 Duchenne muscular dystrophy and 226-227 genetic disorders and 234, 237-238b hold relax active movement and 264 hold relax technique and 267 kneeling and 284 motor control and 36-38 prone progression and 283 quadruped position and 283, 288b rhythmic initiation technique and 264 rhythmic rotation and 264 slow reversal hold technique and 275 slow reversal technique and 275 908 standing and 291-292 supine progression and 279 Modified Ashworth Scale 304, 304t Modified plantigrade position, cerebrovascular accident recovery and 353, 353b Modified pull-to-sit maneuver 109, 111f, 112b Modified stand-pivot transfer 425, 427b Monoamine oxidase (MAO) inhibitors, for Parkinson disease 464-465 Motivation 59-62, 162-163 Motor control 33-55, 34f, 53b age-related changes in 45 cerebrovascular accidents and 304, 327 constraints to 50 Down syndrome and 202-203 hierarchical theories of 35-39, 36f interventions based on 51-53 issues related to 44-46 program model of 39-40 reflex and 35-39 role of sensation in 34, 35f systems models of 40-44, 41f theories of 35-44 time frame of 34 traumatic brain injuries and 380 Motor coordination, motor control and 43 Motor deficits, traumatic brain injuries and 372-373, 389 Motor development 56-90, 88b at age eight months 77 at age five months 72-73, 72-73f at age five years 84 at age four months 71-72, 71f at age four years 81-84 at age nine months 77-78 age related differences in 85-86, 86f at age seven months 76-77 at age six months 73-76, 73f at age six years 84-85, 85f at age three years 81 at age twelve months 78-79 at age two years 81 at ages birth to three months 70-71, 70-71f at ages sixteen and eighteen months 80-81, 80f 909 biomechanical considerations in 64 cognition and motivation and 59-62 constraints to 50 developmental concepts and 62-64 developmental processes and 64-66 directional concepts of 63 Down syndrome and 203-204, 204 fragile-X syndrome and 231 general concepts of 63 life span approach 56-57, 57f concept and 56, 57f view of 57 motor learning and 46 motor milestones and 62, 66-69, 66t osteogenesis imperfecta and 211 stages of 69-86, 70t theories of 61-62, 62f time periods of 57-59, 57 Motor function, positioning and handling to foster 91-130 Motor impairments, cerebrovascular accidents and 304-306 Motor learning 33-55 age-related changes in 50 constraints to 50 definition of 46 interventions based on 51-53 proprioceptive neuromuscular facilitation and 298 stages of 47-53, 48t theories of 46-47 time frame of 46 Motor milestones 145 Motor neurons See Neurons Motor paralysis 171 Motor performance, hemispheric specialization and 15t Motor planning deficits, cerebrovascular accidents and 306 Motor program 40, 47 model, of motor control 39-40 theory 40 Motor skills acquisition, cerebral palsy and 149-150 cerebrovascular accidents and 356 910 Motor vehicle accidents (MV As) 368, 370 Motor weakness, multiple sclerosis and 470 Movable surfaces, dynamic sitting and standing balance exercises using 357-360 Movement assessment of cerebrovascular accidents 316-317 cerebral palsy and 161 functional 126-128, 126-1271, 128b general physical therapy goals and 92 handling techniques for 99-102 multiple sclerosis and 474—477, 476b positioning for 95 preparation for 105-108 spinal muscular atrophy and 223 timing of 251 Mucus, cystic fibrosis and 216 Multiple sclerosis 469-478 autonomic dysfunction in 471 clinical features of 470-471 course of 471 medical management of 471 pathophysiology of 470 physical therapy management of 471-478 Multisystem atrophy, Parkinson disease and 461-462 Muscle spindles 21 Muscle tone 42 Muscles See also Spasticity cerebrovascular accidents and 304, 307, 312-313 Duchenne muscular dystrophy and 225, 227 Guillain-Barré syndrome and 482 segmental innervation of 406, 406¢ spasticity of, spinal cord injuries and 400 spinal cord injuries and 396-397, 397t spinal muscular atrophy and 223 stretching of, multiple sclerosis and 472, 473b tone and movement of, cerebral palsy and 134-136 traumatic brain injuries and 373 Muscular dystrophy 227-228 Musculoskeletal system Down syndrome and 202-203 Guillain-Barré syndrome and 483 impairments, myelomeningocele and 173-174 911 motor control and 42 problems in, cri-du-chat syndrome 206 Myalgia, Guillain-Barré syndrome and 480 Myelin 11 Myelin sheaths after spinal cord injuries 399 multiple sclerosis and 470 Myelodysplastic defects 172t Myelomeningocele 171-200, 172t, 196b case studies on 197b clinical features of 173-178 defined 172t etiology of 171-173 incidence of 171 mobility options for children with 191b overview of 171 physical therapy intervention of 178-196 first stage of 178-185 second stage of 185-193, 186b third stage of 193-196 positions to be avoided in children with 179b prenatal diagnosis of 173 responsibilities and challenges in the care of child with total management of, collaboration for 193 Myelotomy 405 Myoblast transplantation, Duchenne muscular dystrophy and 227-228 Myotomes 21, 396 N Nadir, Guillain-Barré syndrome and 480 Nagi Disablement Model 1, 2f and International Classification of Functioning, Disability, and Health (ICF) 2 Nashner's model of postural control, in standing 43-44 Nebulin, Duchenne muscular dystrophy and 225 Necrosis, spinal cord injuries and 399 “Neo-Bernsteinian” model, of motor learning 48-49, 48t Nerve cells 10 types of 10 Nervous system anterior horn cells of 21 association cortex and 15 autonomic 25-26, 28-30f 912 axons and 11 brain and 13-18 brain stem and 17-18 cerebellum and 17 cerebral circulation and 26-29 cerebral cortex and 15 cerebrum lobes and 14-15 components of 10-29, 11f deeper brain structures and 16-17 fibers and pathways and 12-13 gray matter and 12 hemispheric connections and 16 hemispheric specialization and 15-16, 15t muscle spindles of 21 nerve cells of 10 neuron structures and 10-11 neurotransmitters and 11 peripheral 21-26, 22f principal anatomic parts of 18f reaction to injury and 30-32 somatic 21-25, 23f spinal cord and 18-21, 18f supportive and protective structures of 13 synapses and 11 white matter and 11-12 Neural plasticity 50-51 interventions based on 51-53 Neurectomy 159, 405 Neuritis, multiple sclerosis and 470 Neuroanatomy 10-32, 32b Neurodevelopmental treatment (NDT) approach, cerebrovascular accident and 322 Neuroglia 10, 12f Neuroimaging, cerebrovascular accidents diagnosis and 301 Neurologic deficits, children with 91, 92t Neurological disorders 461-492, 487b case studies on 487b Neurological level, of spinal cord injury 396 Neuromuscular stimulation, for spinal cord injury patient 442 Neurons 10, 12 structures of 10-11 Neuropathic fractures, myelomeningocele and 174-175 913 Neuroplasticity 360-361, 361f Neuroprotective agents, for cerebrovascular accidents 301 Neurosurgery, for cerebral palsy 160-161 Neurotransmitters 11 acetylcholine 11 cerebrovascular accidents and 300 dopamine as 11, 461 g-aminobutyric acid (GABA) 11 glutamate 11, 300 norepinephrine 11 serotonin 11 Neutral pelvis 329b Nocturia, multiple sclerosis and 471 Nodes of Ranvier 11 Nondisjunction, chromosomal abnormalities and 202 Nonfunctional coughs, spinal cord injuries and 410 Nonreflexive bladder, spinal cord injuries and 404 Norepinephrine 11 Noxious stimuli 375-376 Nystagmus 140-141, 470, 477-478 O Obesity, Prader-Willi syndrome and 206 Obtundity 372 Occipital lobe 15 Older adulthood 58-59 Oligodendrocytes 10, 12f Open and closed tasks 49 Open injuries 368 Open skills, motor learning and 49 Optimization principles, motor control and 45 Orofacial deficits, cerebrovascular accidents and 307 Orthoses See also specific orthoses arthrogryposis multiplex congenita and 207 cerebral palsy and 156-157, 157f cerebrovascular accidents and 347-349 donning and doffing of 189 Down syndrome and 205 Duchenne muscular dystrophy and 228 multiple sclerosis and 477, 478t myelomeningocele and 179-180, 180f 914 osteogenesis imperfecta and 215 postpolio syndrome and 486 spinal cord injury patients and 443-444, 444f types of 187-189 wearing time of 189 Orthostatic hypotension, spinal cord injury patients and 414-415, 422-423 Orthotic management Duchenne muscular dystrophy and 228 myelomeningocele and 186-189 Orthotic Research and Locomotor Assessment Unit (ORLAU) 189f Ossification 375 heterotopic 403 Osteogenesis imperfecta 211-216, 211b classification of 211 medical management of 215 overview of 211 prone positioning and 213f therapeutic management of 212t Osteoporosis 174, 310, 404 Outcomes, in patient/client management 3-4 Overstimulation, traumatic brain injuries and 375 Oxidative damage hypothesis 59 Oxygen consumption, cerebrovascular accidents and 307 Oxygen saturation 219-222, 371, 481 P Pacing, postpolio syndrome and 487 Pain Guillain-Barré syndrome and 480 postpolio syndrome and 484, 486 spinal cord injuries and 403 Palmar grasp reflexes 68, 313 Pancreas, cystic fibrosis and 216 Parallel bars, for spinal cord injury patient 445-446 Paralysis Guillain-Barré syndrome and 479 spastic 135 Paralytic strabismus, cerebral palsy and 140 Paraplegia 395-396, 431 Parapodium 186, 187f, 188-189 Paresthesias 915 in Guillain-Barré syndrome 480 multiple sclerosis and 470 Parietal lobe 15 Parkinson disease 461-469 classification of 464, 464t clinical features of 462-464 exercise strategy for 469 medical management of 464-465, 465t pathophysiology of 462 physical therapy management of 465-469 stages of 464 surgical management of 465 systemic manifestations of 464 typical posture and 463f Parkinson-plus syndromes 461-462 Part task training, motor learning and 49-50 Partial seizures, cerebral palsy and 140 Partial tendon release, cerebral palsy and 159 Participation restrictions 2 Passive range of motion exercises, for cerebrovascular accidents 317 of spinal cord injury patients 412-413, 413b Patient education, traumatic brain injuries and 376 Patient management, role of physical therapist in 3-4, 3f Patterns of movement 251 Peer interaction, cerebral palsy and 161-162 Pelvic patterns 257, 262f, 270b Pelvic pressure, interventions for 107b Pelvic rocking 108b Pelvic support 94f Pelvic tilts 328 Pelvis, positioning of 328, 328f, 329b Perceived exertion scale 222 Perception 15t, 193 problems in, myelomeningocele and 193 Percussion 217-219, 217f, 410 Peripheral nerves 25, 27f Peripheral nervous system (PNS) 10, 21-26, 22f Guillain-Barré syndrome and 479 Periventricular leukomalacia, cerebral palsy and 132 Perseveration 303 916 Persistent vegetative state 372 Phenylalanine 224 Phenylketonuria 224 Phenytoin, for seizures 371 Philadelphia collar 399f Phrenic nerve pacing 406-408 Physical environment, traumatic brain injuries and 383-386 Physical therapist assistant 1 cerebral palsy and 147-148 cerebrovascular accidents and 310-311 as member of the health-care team 8, 8b role of, in treating patients with neurologic deficits 4-8, 5-7f Physical therapy interventions See also Medical intervention cerebral palsy and 145-165 cri-du-chat syndrome and 205-206 cystic fibrosis and 217-222 Down syndrome and 205 Duchenne muscular dystrophy and 225-229 genetic disorder and 233-241 osteogenesis imperfecta and 211-216 Prader-Willi syndrome and 206-210, 207t, 208b Physiologic changes, in cerebral palsy 163 Physiologic flexion, motor development and 64, 64f Pia mater 13 Piaget's stages of cognitive development 60, 60 Pincer grasps 69, 69f Placenta, inflammation of, cerebral palsy and 131-132 Plan of care, in patient/client management 3-4 Plantigrade position, cerebrovascular accidents and 353, 353b Plaques, multiple sclerosis and 470 Plasmapheresis, Guillain-Barré syndrome and 480 Plasticity, cerebral palsy and 147 Play complexity of 128b development of 127t Plegia, cerebral palsy and 133-134 Polar brain damage 369-370 Polio 483 Pons 17-18 Pool exercise 213 Pool program, for spinal cord injury patients 441 917 Poor head control, spinal muscular atrophy and 223 Positioning and handling 91-130, 128b adaptive equipment for 117-126 arthrogryposis multiplex congenita and 209 case studies on 129f, 129b cerebral palsy and 148, 148f cerebrovascular accidents and 311, 337, 339b function and 95-97, 96-97f handling techniques for movement and 99-102 head control and 108-111 holding and carrying positions 98-99, 100b at home 97-98, 97-99b manual contacts and 99-101, 101f osteogenesis imperfecta and 211-213, 212b, 213f preparation for movement and 105-108 sensory input and 102-104 spinal muscular atrophy and 223 tips for 101-102 traumatic brain injuries and 373-374, 374b, 376-379, 379b trunk control and 111-117 Posterior artery occlusion, cerebrovascular accidents and 303 Posterior columns 401 Posterior cord syndrome 401, 401t Posterior depression pelvic 270b scapular 260b Posterior elevation, scapular 261b Posterior leaf splints, cerebrovascular accidents and 347 Postoperative positioning, myelomeningocele and 179 Postpolio syndrome 483-487 Posttraumatic amnesia, concussion and 368-369 Posttraumatic seizure disorder, traumatic brain injuries and 371 Postural alignment, movement and 105 Postural control age-related changes in 45 components of 40-43, 41f cri-du-chat syndrome and 206 genetic disorders and 234-237, 239-240b motor control and 38-39 multiple sclerosis and 474 Nashner's model of 43-44 918 static 36 traumatic brain injuries and 380 Postural drainage 217-219, 217b, 217-219f, 410 Postural hypotension, spinal cord injuries and 403 Postural readiness 43, 105, 106t Posture See also Postural control changes in, with aging 86, 87f dynamic 95-97 function related to 92-93, 92f Parkinson disease and 462-463, 463f, 466-468 pyramid of 92f Posture walker 126f Posturing, abnormal 309-310 Power mobility 154, 158, 439 Prader-Willi syndrome 206, 207t natural history of 207 pathophysiology of 207 Precooling, multiple sclerosis and 472 Predictive central set 43 Prednisolone 227-228 Prednisone, for postpolio syndrome 485 Pregait activities, proprioceptive neuromuscular facilitation and 292-297, 297b Prehension 67 Prematurity, cerebral palsy and 132-134, 132t Prenatal diagnosis, of myelomeningocele 173 Preoperational stage of intelligence 60 Preoperational thinking 57-58 Prepositioning, rolling and 281 Pressure, intracranial 370-371 Pressure relief, independence in, myelomeningocele and 192 Pressure ulcers 177-178, 402 Prevention, of cerebrovascular accidents 302 Primary progressive multiple sclerosis 471 Primitive reflexes 35, 36t, 318 Problem-solving, traumatic brain injuries and 387-388 Prognosis, in patient/client management 3-4 Progressive relapsing multiple sclerosis 471 Progressive supranuclear palsy 461-462 Pronated reaching, motor development and 75-76 Prone-on-elbows transfer 427 Prone positioning 919 advantages and disadvantages of 106t arthrogryposis multiplex congenita and 209 cerebral palsy and 146 cerebrovascular accidents and 349 in elbows to four-point 349-350 coming to sit from 114 equipment for 119-120, 119b to four-point 115, 116b head control and 108-109, 110b, 111 interventions for 99b, 108b, 114b, 116b myelomeningocele and 179, 179b as postural level 93 spinal cord injury patients in 416, 417-418b, 418f traumatic brain injuries and 374b trunk control and 113-114, 114b Prone progression, proprioceptive neuromuscular facilitation and 283 Prone push-ups 432 Prone stander 124f Propped sitting 96f Proprioception 306 Proprioceptive neuromuscular facilitation 249-299, 298b basic principles of 250-252, 250t application of 252 biomechanical considerations for 252 cerebrovascular accidents and 317, 333b checklist for clinical use of 252t developmental sequence in 279-297 kneeling 283-284, 289-290b pregait activities 292-297, 297b prone progression in 283 quadruped position 283, 284—287b rolling in 281-283, 281-282b scooting 287-288 sit to stand 288-291, 294b sitting 284-286, 292b standing 291-292, 295b supine progression in 279-281, 280b extremity patterns in 252-257 lower 254-257, 263f, 264t, 265-266b, 267t, 268-269b upper 252-254, 253f, 254t, 255-256b, 257t, 258-259b history of 249 920 motor learning and 298 pelvic patterns and 257, 262f, 270b scapular patterns and 254, 260-261b, 262f techniques for 262-279, 275t agonistic reversals 275-277, 278b alternating isometrics in 267-273, 276b applications of 278-279 contract relax 267 hold relax 267 hold relax active movement 264-266 resisted progression in 278 rhythmic initiation 264 rhythmic rotation 264 rhythmic stabilization 267, 277b slow reversal 275, 286b slow reversal hold 275 trunk patterns in 257-262 upper 257-262, 271-274b use of, to treat impairments 279t Proprioceptive Neuromuscular Facilitation: Patterns and Techniques 298 Propulsion, Parkinson disease and 463 Protective reactions 36, 332-333, 332-333b Proximal joints 103b Proximal muscle groups, development of spasticity in 305-306, 305f Proximal to distal motor development 63 Pseudohypertrophy 225, 226f Psychomotor development 233 Pull-to-sit maneuver as milestone of motor development 75, 75f modified 112b Pulling 77 Push-up, in long-sitting position, for spinal cord injury patients 423-424, 424b Pusher syndrome, cerebrovascular accidents and 303, 346-347 Pushing 77 Putamen 16-17 Q Quadriplegia 133-134, 133f Quadriplegic cerebral palsy 133-134, 133f Quadruped position advantages and disadvantages of 106t 921 arthrogryposis multiplex congenita and 209 cerebral palsy and 146 in developmental sequence 350b as postural level 93 proprioceptive neuromuscular facilitation and 278, 283, 284—287b Quality of life myelomeningocele and 195-196 of spinal cord injury patients 455-456 Quality of movement, versus function 344 Quarter-turns, spinal cord injury patients and 446 R Raimiste phenomenon 308t Ramps 356, 438, 439f, 450 Rancho Los Amigos Scale of Cognitive Functioning 376, 388 Random practice, motor learning and 49 Range of motion arthrogryposis multiplex congenita and 209 Duchenne muscular dystrophy and 225, 227, 227b Guillain-Barré syndrome and 481 multiple sclerosis and 472 myelomeningocele and 181 osteogenesis imperfecta and 213 Parkinson disease and 463-464 spinal cord injuries and 411-413, 413t traumatic brain injuries and 379 Rappaport Coma/Near-Coma Scale (CNC) 379-380 Rasagiline, for Parkinson disease 464-465 Reaching 332b as milestone of motor development 67 Readiness, postural 105, 106t Recall schema 47 Receptive aphasia 306 Recessive inheritance, autosomal 202 Reciprocal, defined 67 Reciprocal creeping 68f Reciprocal interweaving, motor development and 63-64 Reciprocating gait orthosis 187f, 188-189, 443, 444f, 477 Recognition schema 47 Recurrent traumatic brain injury See Sudden impact syndrome Reflex-inhibiting postures, traumatic brain injuries and 375 922 Reflex sympathetic dystrophy 310 Reflexes See also Tonic neck reflex asymmetrical tonic neck 142-143, 143f, 144t autonomic dysreflexia and 402 brain stem 308, 308¢, 318-319 cerebrovascular accidents and 307, 307—308t deep tendon 223, 307-308, 480 Landau 73, 74f motor control and 35-39 palmar grasp 68, 313 peripheral nerve injuries and 30-32 primitive 35, 36t, 318 spinal 307-308, 307t, 318 stretch 250 tendon 307-308 tonic 318-319 traumatic brain injuries and 375 Reflexive motor response 34 Relapsing-remitting multiple sclerosis (RRMS) 471 Relaxation techniques, for Parkinson disease 466 Release, as milestone of motor development 67 Replication technique 264 Resisted progression technique 278 Respiration, cerebral palsy and 148-149 Respiratory compromise, spinal cord injuries and 404 Respiratory function Duchenne muscular dystrophy and 228-229, 229b genetic disorders and 238-241 Respiratory impairments, cerebrovascular accidents and 307 Rest, postpolio syndrome and 487 Resting hand splint 313 Restorative approach, to spinal cord injuries 415-416 Retardation See Mental retardation Retrograde amnesia, concussion and 368-369 Retropulsion, Parkinson disease and 463 Rett syndrome 231-232 Reverse chop 262, 274b Reverse lifts 262, 272b Rhizotomy 160, 160f, 405 RhoGAM 132 Rhythmic initiation technique 264, 466 923 Rhythmic rotation technique 264, 475b Rhythmic stabilization technique 267-273, 277b, 285b, 419b Rib flare 123f Rifton gait trainer 158f Right cerebral hemisphere, functions of 15t, 16 Righting of wheelchair 434-438, 437b Righting reaction 73 Righting reactions, myelomeningocele and 182, 183b Rigidity 135, 372-373, 462 Riluzole, for amyotrophic lateral sclerosis 479 Ring sitting 120f Risk factors for cerebral palsy 132t for cerebrovascular accidents 302 for Parkinson disease 462 Robotic assistance, for spinal cord injury patients 452-453, 453b Rocker clogs, for multiple sclerosis 477 Rolling cerebrovascular accidents and 323-324 to involved side 323 to uninvolved side 323-324, 323b interventions for 108), 114b proprioceptive neuromuscular facilitation and 281-283, 281-282b rhythmic initiation and 264 spinal cord injury patients and 416, 417b Root escape 402 Rotation 105-108, 107f, 107-109b multiple sclerosis and 472-474, 475b Parkinson disease and 466, 467—468b spinal cord injuries and 397-398, 398f Routines, daily 94, 94-95f Running, motor development and 81 S Sacral sitting 121f Sacral sparing 400 Safety, positioning for 95 Saltatory conduction 11, 13f Scanning speech, multiple sclerosis and 470 Scapular depressors 305 Scapular mobilization, cerebrovascular accidents and 317, 318b 924 Scapular patterns 254, 260-261b, 262f Scapular protraction, with splint 321b Scapular strengthening, for spinal cord injury patients 417b, 419b Schemas 60 Schmidt's schema theory, of motor learning 47 School age 216 Schwann cells, Guillain-Barré syndrome and 480 Sclerotic plaques, multiple sclerosis and 469 Scoliosis cerebral palsy and 142-143 myelomeningocele and 175 osteogenesis imperfecta and 215-216 spinal muscular atrophy and 224 Scooting 324 proprioceptive neuromuscular facilitation and 281, 287-288 Scott-Craig knee-ankle-foot orthoses 443, 444f Secondary brain damage 369-370 Secondary parkinsonism 461-462 Secondary progressive multiple sclerosis 471 Segmental rolling, as milestone of motor development 66-67, 73-74, 74f Seizures 140, 140t, 371 Selective dorsal rhizotomy, cerebral palsy and 160 Selegiline, for Parkinson disease 464-465 Self-calming 102b Self-care, independence in, myelomeningocele and 192-193 Self-range-of-motion 428-430 Self-responsibility 162-163, 162f Sensation 177 Sensorimotor development, age-appropriate, promotion of, myelomeningocele and 181-184 Sensorimotor stage of intelligence 60 Sensory deficits, traumatic brain injuries and 373 Sensory impairments cerebrovascular accidents and 306 myelomeningocele and 177-178 Sensory information, slow processing of, Parkinson disease and 462-463 Sensory input, positioning and handling and 102-104 Sensory integration, fragile-X syndrome and 231 Sensory organization, motor control and 41-42 Sensory precautions, myelomeningocele and 180-181 Sensory stimulation, traumatic brain injuries and 375-376 Sensory systems, Down syndrome and 203 925 Serotonin 11 Sex chromosomes 201-202 abnormalities 201-202 Sex-linked inheritance 202 Sex-linked trait 202 Sexual dysfunction multiple sclerosis and 471 spinal cord injuries patients and 404—405 Shoulder, subluxations of 330, 330f Shoulder/hand syndrome 310 Shoulder pain, cerebrovascular accidents and 310 Shunts 176, 177t Shy-Drager syndrome 461-462 Side lyer 124b Side-lying position advantages and disadvantages of 106t cerebral palsy and 146 cerebrovascular accidents and 312, 312b coming to sit from 114 interventions for 98b Parkinson disease and 468) positioning and handling and 123-124, 124b proprioceptive neuromuscular facilitation and 281 traumatic brain injuries and 374b Side sitting 121f four-point to 115 kneeling to 115 with no hand support 113 propped on one arm 112-113 Sip-and-puff wheelchair, for patients with spinal cord injuries 406-408 Sit-pivot transfer 383, 385b, 424-425, 426b Sit-to-stand proprioceptive neuromuscular facilitation and 288-291, 294b transition 334-336, 334-338b Sitting position See also Supported sitting advantages and disadvantages of 106t cerebral palsy and 134f, 142f, 146, 150, 151f, 152b cerebrovascular accidents and 325-334, 328f equipment for 95f, 97f forward on both arms 111-112 926 on one arm 112 interventions for 97-99b lateral, on one arm 112, 113f as milestone of motor development 67, 67f motor development and 75-77f, 76 multiple sclerosis and 473b myelomeningocele and 182 osteogenesis imperfecta and 213 as postural level 93 postures of 96f, 120-123, 120-121f progression of 113b to prone position 114-115 propped on bolster 113f proprioceptive neuromuscular facilitation and 284-286, 292b spinal cord injury patients and 414-415, 421-424, 422b, 423f traumatic brain injuries and 381-383, 382b, 384-385b trunk control and 111-113 without hand support 112 Sitting swing-through 431-432 Skeletal system motor control and 50 myelomeningocele and 174 osteogenesis imperfecta and 211 Skeletal traction, for spinal cord injuries 398 Skill kneeling and 284 prone progression and 283 resisted progression technique and 278 scooting and 281 slow reversal technique and 275 Skilled activities, sitting and 327 Skin breakdown, prevention of 180 care Duchenne muscular dystrophy and 227 myelomeningocele and 192 cerebrovascular accidents and 347-348 Skull 13, 13f Sliding board transfers 425, 425b, 429-430b Slow reversal hold technique 275 Slow reversal technique 275, 286b 927 Social-emotional growth, myelomeningocele and 193, 193b Socialization, myelomeningocele and 195 Soma 10-11 Somatic nervous system 21-25, 23f Somatosensation 42 Souques phenomenon, cerebrovascular accidents and 308t Spastic bladder, spinal cord injuries and 404 Spastic cerebral palsy 133-134, 133f, 141-144 Spastic diplegia 155 928 Spastic hemiplegia, cerebral palsy and 137 Spastic paralysis, cerebral palsy and 135 Spasticity Ashworth Scale and 304, 304t botulinum toxin and 159 Brunnstrom stages of motor recovery and 305t cerebral palsy and 135, 145-146, 159 cerebrovascular accidents and 304-306, 305f, 309-310 impairments, activity limitations, participation restrictions, and focus of treatment in 142t multiple sclerosis and 472-474 oral medications for 159t peripheral nerve injuries and 30-32 spinal cord injuries and 400, 405 Speech 137-138, 141, 470 See also Communication Spina bifida 171, 172f, 172 Spina Bifida Association of America 184-185 Spina bifida cystica 171, 172t Spina bifida occulta 171, 172t Spinal cord 18-21, 18f afferent (sensory) tracts of 20, 20f descending tracts of 20-21 efferent (motor) tract of 20, 20f internal anatomy of 19, 19f levels of 396f myelomeningocele and 171 Spinal cord injuries 395-460, 456b acute care for 409-415 advanced treatment interventions for 431-442 ambulation training for 442-452, 445b body-weight support treadmill for 452-453, 452-453b case studies on 457b clinical manifestations of 402 complications of 402-405 discharge planning for 453-456 early treatment interventions for 416-427 etiology of 395, 396f functional outcomes following 405-409 functional potential for patients with 406-409, 406t inpatient rehabilitation for 415-452 intermediate treatment interventions for 428-430 929 lesion types of 400-402, 400 mechanisms of 397-398 medical intervention for 398, 399f naming level of 395-397 orthoses and 443-444, 444f pathologic changes after 399-400 physical therapy goals for 415 plan of care development for 415-416 spinal shock resolution and 402 types of 398f Spinal deformities 175-176 Spinal muscular atrophy 222-224 type I 223, 223f type II 223-224 type III 224 Spinal nerves 21 Spinal reflexes, cerebrovascular accidents and 307-308, 307t, 318 Spinal shock 400, 402 Spirometry 410 Splints cerebrovascular accidents and 319, 320-322b, 321f, 347 myelomeningocele and 179-180, 180f Sports, cystic fibrosis and 222 Squatting 151b Stability See also Limits of stability alternating isometrics and 267, 276b bridging and 280-281 cerebral palsy and 144-145 Down syndrome and 203-204 genetic disorders and 234, 235—236b kneeling and 284 motor control and 36 prone progression and 283 quadruped position and 283 rhythmic stabilization technique and 273, 277b sitting and 327 slow reversal hold technique and 275 standing and 291-292, 295b supine progression and 280 Stairs 354-356, 354-355b, 452 Stand-pivot transfer 327b 930 Standing frames See Vertical standers Standing position advantages and disadvantages of 106t cerebral palsy and 136f, 144f, 146, 150, 152-153b, 156f cerebrovascular accidents and 334-344, 339-340b, 342b motor control and 45-46 positioning and handling and 124-126, 124f, 125b, 125t, 126f as postural level 93 proprioceptive neuromuscular facilitation and 291-292, 295b spinal cord injury patients and 443 traumatic brain injuries and 383, 386b Startle reflex 307t Static encephalopathy 131 Static postural control See Stability Strabismus, cerebral palsy and 140 Straight leg raising 315b Strengthening exercise cerebral palsy and 163 Duchenne muscular dystrophy (DMD) and 226 myelomeningocele and 192, 194-195 osteogenesis imperfecta and 213 Prader-Willi syndrome and 206, 207t spinal cord injury patients and 413-414, 414b Stretch reflex, proprioceptive neuromuscular facilitation and 250 Stretching Guillain-Barré syndrome and 481, 481f multiple sclerosis and 472, 473b Parkinson disease and 466 postpolio syndrome and 485 spinal cord injury patients and 428, 431b, 433b Striking, motor development and 83 Stroke syndromes 302-304, 302 Strokes See Cerebrovascular accidents (CVAs) Stupor 372 Subarachnoid hemorrhages 301 Subarachnoid space 13 Subdural hematoma 370, 370f Subluxations 330, 330f Substantia nigra 16-17, 462 Subthalamic nuclei 16-17 Sudden impact syndrome 370 931 Sulci 13 Supinated reaching, motor development and 75-76, 76f Supine position advantages and disadvantages of 106t cerebral palsy and 134f, 146 cerebrovascular accidents and 311-312, 312b coming to sit from 114 equipment for 119-120 head control and 109, 110b interventions for 98b, 108b, 114b multiple sclerosis and 473b Parkinson disease and 467b as postural level 93 to sitting position 98b spinal cord injury patients and 418, 420-421b trunk control and 113-114, 114b Supine progression, proprioceptive neuromuscular facilitation and 279-281, 280b Supine-to-sit transfer 324-325, 324b, 326b, 382b Support, positioning for 95 Supported sitting 109-110, 112f, 112b Supported standing, optimal dosages for 125t Supramalleolar orthosis, cerebral palsy and 157 Surgical management of cerebral palsy 159-161, 159-160f of Duchenne muscular dystrophy 228 of osteogenesis imperfecta 215-216, 215f of Parkinson disease 465 Swallowing Guillain-Barré syndrome and 480 Parkinson disease and 462 Sway strategies 43, 43f Sweat chloride test, cystic fibrosis and 216 Swimming postpolio syndrome and 485 spinal cord injury patients and 442 “Swimming” posture 72-73, 73f Swing, head control and 111 Swiss ball, cerebrovascular accidents and 357-358, 357b Swivel walkers 189, 189f Symmetric tonic neck reflex 143, 143f, 144t, 308t Synapses 11 932 Syndromes, stroke 302-304, 302t Synergies, cerebrovascular accidents and 304-305, 305t Systems models, of motor control 40-44, 41f T T1 through T9, injuries at, functional potentials of patients with 408-409 T10 through L2, injuries at, functional potentials of patients with 409 Tactile cues, to assist bridging 314b Tactile defensiveness 102, 231, 231t Tactile stimuli 375-376 Tailor sitting 120f Tall-kneeling activities 351-352, 351-352b, 432-434 to half-kneeling 352 Task performance, hemispheric specialization and 15t physical and cognitive components of 389-390 Task-specific movements 33 Task-specific practice 49 Techniques, for proprioceptive neuromuscular facilitation 262-279, 275t Tegretol See Carbamazepine (Tegretol) Temperature regulation 180-181, 211-213, 441 Temporal lobe 15 Tendon, cerebral palsy and 159 Tendon reflexes 307-308, 480 Tenodesis 411, 413f, 422b Tenotomy 159, 405 Teratogen exposure 132 Tethered spinal cord 177 Tetraplegia 395-396, 441-442 Thalamic pain syndrome 303 TheraBand, for multiple sclerosis 474-477 Therapeutic ambulation 408-409 Therapeutic exercise See Exercises Thoracic spine, injuries to 395-396 Three-jaw chuck grasp 69, 69f Thrombolytic medications 301 Thrombosis 300, 403-404 Thrombotic cerebrovascular accidents 300 Throwing, motor development and 82, 83t, 83f Tilt boards 358-360, 358f, 359b Tilt reactions 39 933 Tilt table 414-415, 415f Time frame, of motor control 34, 34f Tiptoe standing 144f Tissue plasminogen activator (tPA) 301 Toddler, typical motor development of 78-81 Toe flexion, inhibition of 314-315, 317b Tone, assessment of 304 Tone reduction 109b Tonic holding 36 Tonic labyrinthine reflex 105, 142, 143f, 308t Tonic neck reflex cerebral palsy and 143, 143f, 144t motor control and 35-36, 70, 71f Tonic reflexes cerebral palsy and 142-143, 143f, 144t cerebrovascular accidents and 318-319 motor control and 35-36 positioning and handling and 105 Tonic thumb reflex 308¢ Top down control 44 Toronto parapodium 187f, 188 Total body splint 180f Touch, positioning and handling and 102-103, 102b Toxemia, cerebral palsy and 132, 132t Traction, proprioceptive neuromuscular facilitation and 251 Transfers sit-pivot 383, 385b spinal cord injury patients and airlift 425, 428b aquatic therapy and 441-442 lateral push-up 427 modified stand-pivot 425, 427b prone-on-elbows 427 rolling out 427 sit-pivot 424-425, 426b sliding board 425, 425b, 429-430b to wheelchair 424-427, 425b, 434-438, 434—436b, 434f supine-to-sit 324-325, 324b, 326b, 382b traumatic brain injuries and 383, 385b wheelchair-to-bed/mat 325, 327b Transient ischemic attacks (TIAs), cerebrovascular accidents and 301 934 Transition to standing, osteogenesis imperfecta and 213-215 Transitional movements cerebral palsy and 144 cerebrovascular accidents and 324-325 coming to stand 115-117, 118f defined 92 motor development and 73-74 for multiple sclerosis 476b trunk control and 113-117 Transitional zone 300 Translocation, chromosomal abnormalities and 202 Trauma, spinal cord injuries and 397 Traumatic brain injuries (TBIs) 368-394, 390b acute care for 373-376 classifications of 368-372 discharge planning and 390 examination and evaluation of 371-372 inpatient rehabilitation and 376-386 physical and cognitive treatment integration and 387-390 problem associated with 372-373 secondary problems associated with 370-371 subtypes of 368-370 Treadmill 153, 161, 161f, 452-453, 452b Treatment planning, traumatic brain injuries and 383 Treatments, aging and 88 Tremor, Parkinson disease and 462, 468—469 Trendelenburg signs, spinal muscular atrophy and 224 Triceps strengthening, for spinal cord injury patients 414) Trisomies, chromosomal abnormalities and 202 Trunk control alignment and 105 cerebral palsy and 141-142 Down syndrome and 203-204 genetic disorders and 234 interventions for 111-117 movement transitions for encouragement of 113-117 myelomeningocele and 182 positioning for independent sitting and 111-113 sitting position after cerebrovascular accidents and 328 traumatic brain injuries and 380 Trunk extension, interventions for 124b 935 Trunk flexion, in sitting 384b Trunk patterns, proprioceptive neuromuscular facilitation and 257-262, 271-274b Trunk rotation 141-142, 182, 314-315, 316b interventions for 107-108b Trunk twisting and raising 432 Two-person lift 424, 425b hthoff phenomenon 470 Icers 194-195, 402 nclassified seizures 140, 140t niform Data System for Medical Rehabilitation (UDSMR) 309 nilateral reach, motor development and 76, 77f p-and-down movement, cerebrovascular accidents and 306 C2 se (SS oa Se Ss pper extremities activities, cerebrovascular accidents and 317, 318b, 342-343 preparation of, for weight bearing 104b proprioceptive neuromuscular facilitation and 252-254, 253f, 254t, 255-256b, 257t, 258-259b strengthening, myelomeningocele and 183 Upper limb function, myelomeningocele and 189 Vv Valium 158-159 Valued life outcomes, cerebral palsy and 146-147 Variable practice, motor learning and 49 Vegetative state 372 Verbal input, proprioceptive neuromuscular facilitation 251 Vertebrobasilar artery occlusion, cerebrovascular accidents and 303 Vertical standers arthrogryposis multiplex congenita and 209-210, 210f myelomeningocele and 184, 184f osteogenesis imperfecta and 213-215 positioning and handling and 125b Vertical talus foot 175f Vertigo 303 Vestibular system 103-104, 103f Vibration 216, 410 Viral infections, multiple sclerosis and 470 Vision cerebral palsy and 140 cerebrovascular accidents and 303 936 Down syndrome and 203 Guillain-Barré syndrome and 480 multiple sclerosis and 471 myelomeningocele and 190 Parkinson disease and 462-463 positioning and handling and 104 traumatic brain injuries and 381 Visual cues, proprioceptive neuromuscular facilitation and 251 Visual impairments cerebral palsy and 140-141 Down syndrome and 203 Visual learning, fragile-X syndrome and 231 Visual perception, myelomeningocele and 190 Vital capacity, of spinal cord injury patients 410 Voluntary grasp, as milestone of motor development 69, 69f Voluntary movement, motor control and 34 Voss, Dorothy 249 Ww W sitting 74, 96f, 142f Walkable LiteGait 156f Walkers cerebral palsy and 155-156, 157f cerebrovascular accidents and 344-345 for multiple sclerosis 477 posture 126f swivel 189, 189f Walking cerebrovascular accident recovery and 339-344, 346 Down syndrome and 205 as milestone of motor development 67, 68f motor development and 77-78, 79f spinal cord injury patients and 443 Wallerian degeneration 30, 31f Weak functional coughs, spinal cord injuries and 410 Weakness 226-227 multiple sclerosis and 472 postpolio syndrome and 484 Weight bearing and acceptance interventions for 104b, 119b, 122b in involved hand 329-330, 330b 937 myelomeningocele and 182-183 preparation for 105b spinal cord injury patients and 415 Weight-bearing joints 252 Weight-shifting activities, cerebrovascular accidents and 330-331, 331b, 331f, 337-338 Werdnig-Hoffman syndrome 223 Wernicke aphasia, cerebrovascular accidents and 306 Wheelchairs cerebral palsy and 154, 158 Duchenne muscular dystrophy and 224 mobility, myelomeningocele and 191—-192b, 194 multiple sclerosis and 477 spinal cord injury patients and 406-408, 454 advanced skills for 438-439 curb and 438-439, 440b cushions for 439 powered mobility of 439 ramps and 438, 439f righting of 437b standing from 448, 449-450b, 450f transfer to 424-427, 425b, 434-438, 434-436), 434f traumatic brain injuries and 376-379, 379b Wheelchair-to-bed/mat transfers, cerebrovascular accidents and 325, 327b Wheelies 438, 438f White matter 11-12, 470 Whole task training, motor learning and 49-50 Wide abducted long sitting 96f Wolfe's law, adaptation and 66 X X-linked recessive inheritance 202 Z Zanaflex 158-159 Zone of partial preservation 400 938