Pediatric Rehabilitation Principles and Practice 4th Edition

Pediatric Rehabilitation

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FOURTH EDITION Pediatric Rehabilitation Principles and Practice EDITORS Michael A. Alexander, MD Professor, Pediatrics, Physical Medicine and Rehabilitation Thomas Jefferson University Philadelphia, Pennsylvania Chief of Pediatric Rehabilitation Alfred I. duPont Hospital for Children Wilmington, Delaware Dennis J. Matthews, MD Fischahs Chair Pediatric Rehabilitation Medicine Professor and Chair Department of Physical Medicine and Rehabilitation University of Colorado Denver, School of Medicine Aurora, Colorado New York

Acquisitions Editor: Beth Barry Cover Design: Steve Pisano Compositor: NewGen North America Printer: Bang Printing Visit our website at www.demosmedpub.com © 2010 Demos Medical Publishing, LLC. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher. Medicine is an ever-changing science. Research and clinical experience are continually expanding our knowledge, in particular our understanding of proper treatment and drug therapy. The authors, editors, and publisher have made every effort to ensure that all infor- mation in this book is in accordance with the state of knowledge at the time of production of the book. Nevertheless, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, express or implied, with respect to the contents of the publication. Every reader should examine carefully the package inserts accompanying each drug and should carefully check whether the dosage schedules mentioned therein or the contraindications stated by the manufacturer differ from the statements made in this book. Such examination is particularly important with drugs that are either rarely used or have been newly released on the market. Library of Congress Cataloging-in-Publication Data 2009024706 Pediatric rehabilitation : principles and practice / [edited by] Michael A. Alexander, Dennis J. Matthews.—4th ed. p. ; cm. Includes bibliographical references and index. ISBN 978–1–933864–37–2 1. Children with disabilities—Rehabilitation. I. Alexander, Michael A. (Michael Allen), 1947– II. Matthews, Dennis J. [DNLM: 1. Disabled Children—rehabilitation. WS 368 P37125 2009] RJ138.P38 2009 617Ј.03—dc22 Special discounts on bulk quantities of Demos Medical Publishing books are available to cor- porations, professional associations, pharmaceutical companies, health care organizations, and other qualifying groups. For details, please contact: Special Sales Department Demos Medical Publishing 11 W. 42nd Street, 15th Floor New York, NY 10036 Phone: 800–532–8663 or 212–683–0072 Fax: 212–941–7842 E-mail: [email protected] Made in the United States of America 09 10 11 12 54321

Dedication To Dr. Gabriella Molnar, a recognized founder of our Medicine Service. Concluding her career at Children’s field of pediatric rehabilitation medicine. Dr. Molnar Hospital and Research Center in Oakland, California, created our first textbook, edited the subsequent two where she created a new Department of Pediatric editions, and wrote numerous state-of-the-art text- Rehabilitation Medicine, she finished training her book reviews for the Child with Physical Disability. last of over 50 domestic and international fellows. After escaping from Hungary in 1956 from the Russian Her speaking career has included invitations from all occupation and communist regime, Dr. Molnar dis- over the world, including Australia, Europe, Asia, and played much foresight and courage throughout her England. She has served on the editorial boards for professional career. Her guiding principle has always the Archives of Physical Medicine and Rehabilitation been that children are not miniature adults, but indi- from 1976 to 1994 and Developmental Medicine and viduals with changing physical, intellectual, and Child Neurology from 1992 to 1997. She is a recipient emotional abilities and needs. At every age, there- of the Krusen Award from the American Academy of fore, the principles of rehabilitation medicine have Physical Medicine and Rehabilitation (AAPMR), the to be adapted to these changing aptitudes. Beginning highest honor obtainable for proven performance in as a resident at Albert Einstein College of Medicine, clinical expertise, contributions to the literature, and Dr. Molnar quickly rose through the ranks from administration in the field of rehabilitation medicine. faculty instructor to full tenured professor, while Simply stated, Dr. Molnar defines the standard for the developing and running the Pediatric Rehabilitation rest of us to follow. v

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Contents Chapter 1 Preface...................................................................................................................................................... ix Chapter 2 Contributors .............................................................................................................................................. xi Chapter 3 Chapter 4 History and Examination.................................................................................................... 1 Chapter 5 Chapter 6 Michael A. Alexander and Gabriella E. Molnar Chapter 7 Medical Care of Children with Disabilities ........................................................................ 13 Chapter 8 Chapter 9 Susan D. Apkon and Deirdre Arnholz Chapter 10 Psychological Assessment in Pediatric Rehabilitation ....................................................... 21 Jane A. Crowley and Kayla White-Waters Language Development in Disorders of Communication and Oral Motor Function ............. 53 Lynn Driver, Rita Ayyangar, and Marie Van Tubbergen Adaptive Sports and Recreation....................................................................................... 79 Ellen S. Kaitz and Michelle Miller Orthotic and Assistive Devices....................................................................................... 103 Elizabeth L. Koczur, Carrie E. Strine, Denise Peischl, Richard Lytton, Tariq Rahman, and Michael A. Alexander Electrodiagnosis in Pediatrics ........................................................................................ 127 Craig M. McDonald Cerebral Palsy............................................................................................................... 165 Mary McMahon, David Pruitt, and Jilda Vargus-Adams Spina Bifida .................................................................................................................. 199 Elaine L. Pico, Pamela E. Wilson, and Rochelle Haas Traumatic Brain Injury ................................................................................................... 231 Linda E. Krach, Mark E. Gormley, Jr., and Marcie Ward vii

viii Contents Chapter 11 Spinal Cord Injuries ....................................................................................................... 261 Virginia Simson Nelson and Joseph E. Hornyak Chapter 12 Neuromuscular Diseases............................................................................................... 277 Craig M. McDonald Chapter 13 Pediatric Limb Deficiencies............................................................................................ 335 Deborah Gaebler-Spira and Robert D. Lipschutz Chapter 14 Orthopedics and Musculoskeletal Conditions ................................................................. 361 Kevin P. Murphy, Colleen A. Wunderlich, Elaine L. Pico, Sherilyn Whateley Driscoll, Elizabeth Moberg-Wolff, Melanie Rak, and Maureen R. Nelson Chapter 15 Aging With Pediatric Onset Disability and Diseases ........................................................ 425 Margaret A. Turk, Lynne Romeiser Logan, and David Kanter Chapter 16 The Assessment of Human Gait, Motion, and Motor Function......................................... 461 James J. Carollo and Dennis J. Matthews Chapter 17 Psychosocial Aspects of Pediatric Rehabilitation ............................................................ 493 Lee Renee Lucas Index ........................................................................................................................................................ 501

Preface This is the first edition of Pediatric Rehabilitation that following the children serially. Our options for spas- has not been under the leadership of Dr. Gabriella ticity management are different and more widely used Molnar, who is happily retired and living with her than in the last edition of this book. “cowboy” in Oklahoma. She has passed the torch to me and Dennis Matthews and we have been stim- We are constantly challenged by children who ulated by the challenge. For the most part we have are surviving cancers to assist in their rehabilitation. kept the basic structure of past editions while mak- Patients with solid organ transplants benefit from our ing changes that reflect the advancement of pediatric services. Our patients seem sicker, spend less time on rehabilitation. our inpatient units, and are now managed in day pro- grams. The chapter authors have diligently incorpo- Our field is changing and so we have streamlined rated these issues and many more. some topics and added new chapters on gait labs, as many of us are actively involved in this exciting A new feature of this edition is “Pearls and Perils” adjunct to our practice of medicine. We have added a of caring for different types of patients. These pearls new chapter on aging with pediatric onset disability and perils are important take-home points some of the that will be of great interest to physiatrists. authors have for you. Medicine and rehabilitation are changing. Many You will notice that some chapter authors have of us see very few arthritis patients. Our rheumatology returned and we have asked them to incorporate new colleagues are doing a fantastic job with these children pediatric rehabilitation specialists, as it is our hope and so the need for rehabilitations has decreased. Leg that these new coauthors will become the senior lengthening, limb salvage procedures, limb reattach- authors of future chapters and perhaps editors of ment, and improved safety of farm machinery have future editions. decreased the number of children who need prosthe- sis. Genetic testing has altered the referral patterns for We are happy to present to you this compiled wis- children who need electromyography. Bladder conti- dom of the brightest and most enthusiastic clinicians nence and bowel irrigation surgeries have changed in our tightly knit group of pediatric rehabilitation how we manage patients with spinal cord injuries. specialists. More of us are seeing children with concussions Michael A. Alexander as the literature proves the value of screening and Dennis J. Matthews ix

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Contributors Michael A. Alexander, MD President, Commission for Motion Laboratory Accreditation, Inc. Professor Denver, Colorado Pediatrics and Physical Medicine and Rehabilitation Thomas Jefferson University Jane A. Crowley, Psy D Philadelphia, Pennsylvania Chief of Pediatric Rehabilitation Psychologist Alfred I. duPont Hospital for Children Division of Rehabilitation Medicine Wilmington, Delaware Alfred I. duPont Hospital Wilmington, Delaware Susan D. Apkon, MD Lynn Driver Associate Professor Department of Rehabilitation Speech-Language Pathologist University of Washington University of Michigan Health Systems Director Rehabilitation Medicine C.S. Mott Children’s Hospital Seattle Children’s Hospital Ann Arbor, Michigan Seattle, Washington Deborah Gaebler-Spira Deirdre Arnholz, MD Attending Physician Kaiser Permanente Pediatric and Adolescent Rehabilitation Program Colorado Permanente Medical Group Professor Aurora, Colorado Physical Medicine and Rehabilitation Department of Pediatrics Rita Ayyangar, MD Feinberg School of Medicine Northwestern University Associate Professor Chicago, Illinois Department of Physical Medicine and Rehabilitation University of Michigan Mark E. Gormley, Jr., MD Pediatric Physiatrist C.S. Mott Children’s Hospital Clinical Assistant Professor Ann Arbor, Michigan Department of Physical Medicine and Rehabilitation James J. Carollo, PhD, PE University of Minnesota Minneapolis, Minnesota Director Pediatric Rehabilitation Medicine Center for Gait and Movement Analysis Gillette Children’s Specialty Healthcare Director St. Paul, Minnesota Musculoskeletal Research Center The Children’s Hospital Rochelle Haas, MD Associate Professor Physical Medicine and Rehabilitation Director Department of Orthopaedics Spinal Dysfunction Clinic School of Medicine Department of Pediatric and Physical Medicine and University of Colorado Rehabilitation xi

xii Contributors Alfred I. duPont Hospital for Children Northwestern University Wilmington, Delaware Chicago, Illinois Joseph E. Hornyak, MD, PhD Lynne Romeiser Logan, PT, PCS Associate Professor Program Manager Physical Medicine and Rehabilitation and Kinesiology Tone Management and Mobility Program Co-director SUNY Upstate Medical University Muscular Dystrophy Association Pediatric Syracuse, New York Neuromuscular Clinic Medical Director Lee Renee Lucas, LCSW Physical Activity and Exercise Intervention Laboratory Department of Patient and Family Services Department of Physical Medicine and Rehabilitation Alfred I. duPont Hospital for Children University of Michigan Medical School Wilmington, Delaware Attending Physician Division of Pediatric Rehabilitation Richard Lytton, MA, CCC-SLP C.S. Mott Children’s Hospital University of Michigan Speech Language Pathologist and Augmentative and Ann Arbor, Michigan Alternative Communication Specialist Alfred I. duPont Hospital for Children Ellen S. Kaitz, MD, FAAPMR Wilmington, Delaware Assistant Professor Dennis J. Matthews, MD Physical Medicine and Rehabilitation Ohio State University Director Fischahs Chair Pediatric Rehabilitation Medicine Fellowship Pediatric Rehabilitation Medicine Nationwide Children’s Hospital Professor and Chair Columbus, Ohio Department of Physical Medicine and Rehabilitation University of Colorado Denver, School of Medicine David Kanter, MD Aurora, Colorado Assistant Professor Craig M. McDonald, MD Physical Medicine and Rehabilitation SUNY Upstate Medical University Professor Syracuse, New York Pediatrics and Physical Medicine and Rehabilitation Director Elizabeth L. Koczur, MPT, PCS Muscular Dystrophy Association Neuromuscular Disease Clinics Physical Therapist Davis Medical Center Alfred I. duPont Hospital for Children University of California Wilmington, Delaware Medical Director Spinal Cord Injury Program Linda E. Krach, MD Director Electrodiagostic Laboratory Associate Professor Shriners Hospitals for Children Department of Physical Medicine and Rehabilitation Sacramento, California University of Minnesota Minneapolis, Minnesota Mary McMahon, MD Director of Research Administration Gillette Children’s Specialty Healthcare Associate Professor St. Paul, Minnesota Program Director Physical Medicine and Rehabilitation Robert D. Lipschutz, CP Cincinnati Children’s Medical Center Cincinnati, Ohio Director Prosthetics and Orthotics Education Michelle Miller, MD Prosthetics and Orthotics Clinical Center Rehabilitation Institute of Chicago Section Chief Chicago, Illinois Department of Physical Medicine and Rehabilitation Clinical Instructor Assistant Professor Northwestern University Prosthetic-Orthotic Center Department of Clinical Physical Medicine and Rehabilitation Ohio State University College of Medicine Columbus, Ohio

Contributors xiii Elizabeth Moberg-Wolff, MD Elaine L. Pico, MD, FAAP, FAAPM&R Associate Professor Board Certified in Pediatrics Pediatric Physical Medicine and Rehabilitation Board Certified in Physical Medicine and Medical College of Wisconsin Rehabilitation Program Director Board Certified in Pediatric Rehabilitation Tone Management Oakland, California Director Pediatric Physical Medicine and Rehabilitation David Pruitt, MD Fellowship Children’s Hospital of Wisconsin Assistant Professor Milwaukee, Wisconsin Departments of Physical Medicine and Rehabilitation and Pediatrics Gabriella E. Molnar, MD Cincinnati Children’s Hospital Medical Center University of Cincinnati College of Medicine Retired Cincinnati, Ohio Tulsa, Oklahoma Tariq Rahman, PhD Kevin P. Murphy, MD Director Medical Director Department of Biomedical Research Medcenter One Pediatric Rehabilitation Center Center for Orthopaedics Bismarck, North Dakota Alfred I. duPont Hospital for Children Medical Director Wilmington, Delaware Northern Minnesota Clinics Gillette Children’s Specialty Healthcare Melanie Rak, MD Associate Professor Department of Physical Medicine and Pediatric Physiatrist Rehabilitation Department of Physical Medicine and Rehabilitation Duluth School of Medicine Northwestern University University of Minnesota Rehabilitation Institute of Chicago Colonel, MNARNG Medical Corps Chicago, Illinois Duluth, Minnesota Carrie E. Strine, OTR/L Maureen R. Nelson, MD Senior Staff Therapist Adjunct Associate Professor Alfred I. duPont Hospital for Children Physical Medicine and Rehabilitation Wilmington, Delaware University of North Carolina Chapel Hill Director, Pediatric Rehabilitation Services Margaret A. Turk, MD Carolinas Rehabilitation Levine Children’s Hospital Professor Charlotte, North Carolina Physical Medicine and Rehabilitation Pediatrics Director Virginia Simson Nelson, MD, MPH Pediatric Rehabilitation Medicine Program SUNY Upstate Medical University Professor Syracuse, New York Physical Medicine and Rehabilitation Department of Physical Medicine and Marie Van Tubbergen, PhD Rehabilitation University of Michigan Medical School Clinical Lecturer Chief Department of Physical Medicine and Division of Pediatric and Adolescent Rehabilitation Rehabilitation C.S. Mott Children’s Hospital University of Michigan University of Michigan Rehabilitation Psychologist Ann Arbor, Michigan MedRehab Milestones Pediatric NeuroRehabilitation Program Denise Peischl, BSBME Ann Arbor, Michigan Rehabilitation Engineer Jilda Vargus-Adams, MD, MSc Alfred I. duPont Hospital for Children Wilmington, Delaware Assistant Professor Departments of Physical Medicine and Rehabilitation and Pediatrics

xiv Contributors Pamela E. Wilson, MD Cincinnati Children’s Hospital Medical Center Associate Professor University of Cincinnati College of Medicine Department of Physical Medicine and Rehabilitation Cincinnati, Ohio University of Colorado at Denver and Health Sciences Center Marcie Ward, MD Denver, Colorado Department of Rehabilitation Medicine Adjunct Instructor The Children’s Hospital Department of Physical Medicine and Aurora, Colorado Rehabilitation University of Minnesota Colleen A. Wunderlich, MD, MSc Minneapolis, Minnesota Pediatric Rehabilitation Medicine Associate Director Gillette Children’s Specialty Healthcare Department of Pediatric Rehabilitation St. Paul, Minnesota Levine Children’s Hospital and Carolinas Rehabilitation Sherilyn Whateley Driscoll, MD Charlotte, North Carolina Consultant and Director Department of Pediatric Physical Medicine and Rehabilitation Assistant Professor Department Physical Medicine and Rehabilitation Mayo Clinic College of Medicine Rochester, Minnesota Kayla White-Waters, PhD Assistant Professor Department of Human Services Washburn University Topeka, Kansas

Pediatric Rehabilitation

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1 History and Examination Michael A. Alexander and Gabriella E. Molnar The physiatric history and examination of a child during the visit, and for how long, and then asking the require a blend of medical diagnostic skills to estab- parents to tell in their own words why they came and lish or confirm the diagnosis as well as a knowledge what specific questions they have for you. of child development and behavior to evaluate func- tional assets and difficulties for the intervention phase Concerns stated by the referral source should be of rehabilitation. shared with the parents. Many parents are unsure about what information the visit can provide. This SETTING THE TONE is the opportunity to explain what pediatric rehabil- itation is and what it can offer the child and family. To ensure the best cooperation, especially in the pre- The examiner also should explain that it is part of the school age, the environment should be child-friendly. examination to watch the child so that the parents will Exposure to crying and upset children should be not feel offended by the examiner’s wandering gaze. avoided in the waiting room or other areas. If the fam- Because observation of spontaneous behavior is one ily brings the child’s siblings, someone should take of the most informative aspects of evaluating young- care of them during the appointment so that the par- sters with a disability, examination begins from the ents can focus on the interview without distraction. moment the child is in the physician’s view. Questions The examination room should have a small table and about history and illnesses should be asked in sim- chair with an assortment of toys for different ages to ple terms so that the family can understand them make the child comfortable and relaxed. The examin- and provide proper information. It is also important er’s attire also influences the child. A good rule is to to clarify insurance coverage and whether additional “lose the white coat.” The child is not impressed by tests can be performed on the same day or must await it and, in fact, may be intimidated from past medical approval. visits. Pictures of cartoon characters or animals on the wall, small toys, and decals on instruments help to HISTORY create a playful atmosphere and alleviate the child’s fears. Prenatal and Perinatal History Start the visit by introducing yourself, which The prenatal and perinatal history includes the pre- should include telling the patient and parents some- conceptual period and the parents’ ages and health thing about yourself, and what will be happening before and since the birth of the child. Maternal

2 Pediatric Rehabilitation factors during gestation may lead to fetal malforma- or regression noted at a particular point. However, the tions. Examples of these associations include febrile relatively fast pace of early motor development may illnesses (1) anticonvulsants (2) with spina bifida; mask slow deterioration due to progressive neurologic maternal diabetes with caudal regression syndrome disease for a while. and sacral agenesis; and rubella, thalidomide, or fetal alcohol syndromes. Feeble or eventually lost Developmental history and subsequent assess- fetal movements may be the earliest sign of a motor ment must take into consideration the interactive disability of prenatal origin. Prenatal care, unusual effect of coexistent deficits. A significant cognitive weight gain or loss, hypertension, or any other ges- dysfunction by itself may delay gross and fine motor tational problems should be explored. Mode and development (4). It also tends to enhance the func- duration of delivery, use of anesthesia, induction, tional consequences of a neuromuscular disability. intrapartum complications, and expected and actual Slow development in personal and adaptive tasks that date of birth should be noted. History of previous require both motor and cognitive abilities may be pregnancies, deliveries, and fetal loss is necessary. related to impairment in either area. A combination of Prenatal cerebral damage seems to be increased both can create the impression that the motor deficit is in infants of mothers with previous spontaneous more severe than it actually is. abortions (3). A detailed neonatal history is essen- tial, including birth weight, Apgar scores, onset A history of delay in communication develop- and success of breastfeeding, and the infant’s age ment raises several differential diagnostic possibili- at discharge. Weak lip seal and sucking force and ties: (a) true language dysfunction affecting receptive inadequate feeding may be preliminary signs of oral or expressive domains or both, (b) oral motor dys- motor dysfunction. If the infant needed admission to function interfering with speech production, and (c) the neonatal intensive care unit (NICU), what were significant hearing loss. In a child with motor dis- the problems, medications, and supportive mea- ability, language dysfunction may result from dif- sures? Neonatal seizures may signal pre- or perina- fuse or focal cerebral lesions, such as head injury or tal brain damage. Prematurity, particularly very low cerebral palsy, particularly when cognitive function birth weight, is a frequent cause of cerebral palsy (2). is also affected. Large birth weight may lead to intrapartum trauma, brachial plexus palsy, or, on rare occasions, spinal The ability to follow simple and, at a later point, cord injury, particularly with breech or other fetal complex commands indicates preserved receptive lan- malposition. When extended hospitalization was guage even in the absence of verbalization. Parents required, one should note the infant’s age, weight, report a variety of responses, such as smiling, coo- and condition on discharge, including means of ing, crying, pointing, or vocalization with inflection feeding and need for ventilatory or other supportive as a substitute for speech. Oral motor dysfunction is measures at home, which may predict subsequent, also associated with cerebral palsy, most often with persistent, or recurrent problems. spastic quadriparesis or dyskinetic disorders due to suprabulbar or pseudobulbar palsy. Bulbar palsy in Developmental History medullary involvement affects speech production, for example, in spinal muscular atrophy or spina The developmental history should cover all major bifida with syringobulbia. There is a close associa- aspects of function and behavior. For details of devel- tion between anatomical structures and neurologic opmental milestones and testing, the reader is referred control for speech and oral feeding. Concurrent oral to Chapter 2. This discussion presents only guide- motor dysfunction with feeding difficulties is an add- lines for the purpose of diagnostic interpretations. itional sign of bulbar or pseudobulbar pathology and Discrepancies between different areas of functioning confirms the suspicion of speech production deficit. In provide clues about the nature of medical diagnosis such cases, history of early feeding is most relevant. and developmental disability. For example, was there a good lip seal and strong suction on breastfeeding? When bottlefed, the infant Delayed accomplishments, primarily in motor can handle 4 ounces in about 10 minutes, and feed- function, suggest a neuromuscular deficit. One of the ings every three to four hours are generally adequate. earliest signs that parents report is a lack of sponta- The need for longer and more frequent feeding to neous movements when the infant is held or placed maintain weight gain, especially during the first few in the crib. They may add that the baby feels limp or months; coughing; nasal regurgitation of liquids; dif- stiff, suggesting hypotonia or spasticity. In all cases of ficulty with drinking from a cup; and difficulty with motor dysfunction, it is important to clarify whether introduction of solid food due to chewing problems the dysfunction was a steady, continuing delay from are early symptoms of oral motor dysfunction and an early age, suggesting a static disease, or an arrest a possible subsequent deficit in speech production. Augmentative communication training should be ini- tiated early in such cases.

Chapter 1 History and Examination 3 Hearing is an essential factor for speech devel- Nutrition, with special consideration for the opment. Early cooing and babbling are innate char- child’s disability, should be reviewed. Feeding diffi- acteristics of infants and involve the same vocal culties or behavior problems may lead to inadequate components, regardless of the language spoken in consumption of calories and essential nutrients. their environment. Infants with hearing loss start Dietary intake may be lower than required for the to fall behind after six to eight months of age when increased energy expenditure on physical activities learning of auditory-dependent vocalization begins. in children with motor disability. In contrast, calo- Parents may notice a decrease even in spontaneous ric intake may be excessive when physical activity babbling at that age. All neonates and infants at high level is restricted and lead to obesity, most often risk for developmental disability or recurrent ear in wheelchair users with spina bifida (6) or mus- infections should have an initial and, if warranted, cular dystrophy. Dietary information and guidance repeat hearing evaluations. Correction of a hearing are fundamental for regulation of neurogenic bowel deficit should be provided as soon as possible after it incontinence. Family eating patterns should be is detected (5). taken into consideration. Injuries, burns, fractures, and spinal cord and head trauma are followed by For infants and young children, the history is a catabolic state. Monitoring of weight, nutrition, obtained from parents or caretakers. While gathering and fluid intake is essential during inpatient reha- information from one person about another, the exam- bilitation for major injuries. Caloric requirements iner gains an understanding of both and establishes for children are calculated from age-appropriate rapport with parents and child. Early school-aged chil- standards, which take into consideration growth. In dren can provide some information about themselves children with motor disability, upward or downward and should be encouraged to do so. Preadolescents adjustment in height and weight may be needed, and particularly adolescents generally prefer to give depending on their level of physical activity and an account of their problems and achievements. individual growth trend. Specific recommendations Adolescents often wish to have privacy without the are available for children with spina bifida to avoid parent present, at least for part of the visit. obesity (7,8). General Health History History of respiratory complications, past or present, should be explored in certain disabilities. Central ven- The examiner should determine whether the patient tilatory dysfunction (CVD) is a complication of Arnold- is an essentially well child with impairment or a sick Chiari malformation in spina bifida (9). Syringobulbia child who has been hospitalized several times. In may cause similar symptoms. Nightmares, insom- the latter case, one should explore in detail the fre- nia, and night sweating are complaints associated quency, reasons, tests, and treatments. Even if one with hypercapnia, and may be reported in advanced has access to records, the parents should be asked stages of muscular dystrophy or atrophy. Hypercapnia to tell the child’s history in their own words. Their and sleep apnea may occur in diseases of the central account provides an insight into their knowledge and nervous system. Intercostal muscle paralysis in high participation in the child’s care. One should ask how thoracic paraplegia with spinal cord injury or spina many visits they make to medical centers and thera- bifida, spinal muscular atrophy, or advanced muscle pists and how much time is spent in transit for the diseases leads to inefficient pulmonary ventilation and child’s care. handling of secretions. With severe spastic or dyski- netic cerebral palsy, the respiratory musculature may History of allergies to medications or other sub- lack coordination. Such children are prone to recur- stances should be noted. An early history of allergies rent bouts of pulmonary infections. Coexistent feed- to different and often inconsistent formulas may indi- ing difficulties with minor aspirations, or restrictive cate that the child in fact had feeding difficulties that pulmonary disease due to spinal deformities are add- were attributed to allergy. Multiple exposures to latex itional adverse factors. and any signs of allergy should be determined, partic- ularly in spina bifida or after repeated surgeries. Any Restricted mobility of the spine and thoracic medications that the child takes regularly, including cage may be present in ankylosing spondylitis or dietary supplements and homeopathic or alternative severe systemic-onset juvenile rheumatoid arthritis. medications or aerosols, should be recorded with dos- Detailed information about home management and age and schedule. use and frequency of equipment must be included in the history. Exercise dyspnea may be a sign of pulmo- The risk and incidence of seizures are higher in nary compromise or deconditioning due to the high static and progressive diseases of the central nervous energy cost of physical activities in children with a system. Overt or suspicious signs, type and frequency motor disability. Cardiac decompensation with right- of seizures, anticonvulsants, and their effectiveness sided failure, a potential complication of pulmonary and possible side effects should be recorded.

4 Pediatric Rehabilitation dysfunction, is more likely to occur in older children In other cases, parents may report excessive crying or young adults with the previously mentioned disabil- and restlessness while the child is awake and dur- ities. Myopathic conduction defects and arrhythmias ing sleep. Some children may show excessive mood are often symptom-free in the absence of heart fail- swings from lethargy to hyperactivity, whereas oth- ure. Consultation with pulmonary and/or cardiology ers are even-tempered and react appropriately. One specialists should be arranged when history reveals should ask the parents whether the child is friendly, suspicious symptoms. outgoing, and sociable or shy and withdrawn, par- ticularly in group situations. Parental guidance may Visual and hearing impairments are more fre- be needed to encourage interactive behavior by the quent in childhood disabilities. Inquiry about these child. Compliance or problems with obedience, daily aspects of function should not be overlooked in taking activity level, attention span, sleeping and eating hab- the history. The necessity of regular hearing assess- its, and special interests and dislikes are revealing ment was mentioned earlier. The same applies to information. Separation from the parents may be a visual function. Prenatal infections, anoxic or infec- problem for children with disability. The parents may tious encephalopathy, metabolic diseases, meningitis, be uncomfortable to leave the child with relatives or hydrocephalus, and head injury warrant exploration other caretakers. In this context, it is important to of visual and auditory function. With the develop- point out the need and methods to foster the child’s ment of new antibiotics, acquired hearing deficit due independence. to antibiotic use is not a significant concern. Like all children, handicapped youngsters are prone to a Educational and Social History variety of childhood illnesses. In some cases, how- ever, acute symptoms and febrile illnesses may be Very young children may be enrolled in an early inter- directly related to complications of a specific disabil- vention program, home- or center-based. Frequency, ity. Vomiting, headache, irritability, or lethargy may length of sessions, components of training, the child’s be prodromal signs of decompensating hydrocepha- tolerance and cooperation in the program, and its effec- lus in spina bifida, cerebral palsy (2), or an intercur- tiveness, as perceived by the parents, should be clar- rent unrelated illness. Recurrent headaches are also a ified. The same applies when the slightly older child manifestation of autonomic dysreflexia in spinal cord attends a preschool program. In school-aged children, injury, along with bowel or bladder distention. Fever information about the type of class—mainstream, inte- may represent central hyperpyrexia in severe head grated, or special education—is important. Academic injury or hyperthermia due to pseudomotor paraly- expectations are different in each of these educational sis in high thoracic spinal cord injury. However, such pathways and should be taken into consideration when conclusions can be reached only after other causes report card grades are interpreted. Individualized edu- of fever have been excluded. In neurogenic bladder, cation program (IEP) meetings and environmental urinary tract infection should always be investigated accommodations are other pertinent details. The child as a possible cause of febrile illness. A history of the may have special interests and strengths that should usual pattern of the amount and frequency of void- be further developed or difficulties in certain subjects, ing is essential in neurogenic bladder dysfunction. which may require additional help and adjustment Systematic daily recording is a guide for bladder train- of the IEP. Review of educational status is a consis- ing. Fluid intake, in accordance with pediatric norms, tent part of follow-up visits, and assistance should be needs to be monitored at home, and records of both offered when problems arise. bladder and bowel dysfunction should be available on the medical visit. Opportunities to meet and play with other chil- dren in addition to school or home contacts, visits Immunization history is part of all pediatric visits. and sleepovers with friends, and participation in var- Often, a disabled child in good health has not received ious recreational activities are formative experiences the recommended vaccinations because of excessive that prepare all youngsters for social functioning and concern on the part of the family or pediatrician. But adulthood. Asking the parents to describe the child’s it also may mean that the child has always looked ill daily schedule, including regular and occasional activ- when scheduled for immunization. ities on weekdays and weekends, yields a valuable insight into these aspects of the entire family’s life- History of Behavior style. Time spent in school, therapy, homework, play, and leisure activities with family members, friends, or The examiner should ask about the child’s behavior alone should be noted. Housing, employment of the in terms of temperament and personality. The par- parents, siblings and their ages, and social support of ents may state that the child was always a good baby, the family provide further understanding of the phys- but this report may mean that the youngster never ical and social environment. Some families with a cried and slept more than expected for his or her age.

Chapter 1 History and Examination 5 disabled child experience social isolation. Information spontaneous exploration and interest in toys, games, about or referral to community resources is helpful in or books in the room; style, concentration, atten- all cases. tion span, or distractibility during play; level and manner of motor activities; attempts to engage the Family History parents and the examiner in conversation, vocabu- lary, complexity of language, and quality of speech; In motor or other developmental disabilities, a detailed and interaction with parents or examiner (appropri- family history must be obtained to rule out the pos- ate, shy, or demanding). Observations of the par- sibility of an inheritable genetic disease. Health and ents’ response and their way of handling the child’s function of the parents, siblings, and other family behavior are also revealing. members on the maternal and paternal sides should be explored through several preceding generations. Examination by Age One should ask specifically whether there are other children in the family with developmental delay or For infants and young children, the examiner must adults with known motor disability, limb deficiency, create an atmosphere of trust. Friendly advances dur- or other malformations. Historical information is at ing history-taking or while the child is at play allay times incomplete until further questioning brings to initial fears and anxiety. At this age, most, if not all, light additional facts. Family albums and pictures of of the examination can be accomplished with the relatives may be helpful to detect dysmorphic facial child in the parent’s lap if the child remains fearful. or other features. Consanguinity is an increased risk Interactive play in this phase of the examination can for genetic disease, including diseases with a recessive incorporate developmental testing by offering toys autosomal inheritance pattern. In some autosomal- for grasping or raisins to test pincer grasp. Hearing, dominant conditions, mild variants of a disease may vision, cranial nerves, and postural abnormalities also be missed until a thorough investigation of suspected can be observed. family members is carried out. Congenital myotonic dystrophy and facioscapulohumeral dystrophy are As the parent gradually undresses the child, gen- examples. Affected males with familial history on tle touch and tickling or funny sounds with a smile the maternal side are typical of X-linked conditions. help to maintain relaxation and to facilitate hands-on Multifactorial inheritance, such as spina bifida, creates examination. Inspection and palpation of body parts a complex situation, with or without known familial and gentle movements to examine tone are performed history (2,7). Referral for genetic workup is neces- at this point. The examiner should be prepared to sary whenever a genetic condition is known or sus- improvise if the child shows increasing anxiety. pected. Pregnant mothers of affected children should be referred for genetic counseling; prenatal diagnostic The actual hands-on examination, consisting of tests for detection are also available. bodily handling and manipulation, is the last stage; anxiety-provoking or painful tests are deferred to the EXAMINATION end. If the examination requires placement of the child on a table, the mother can sit at the end and This chapter provides only general guidelines for the let the child’s head rest in her lap. With anxious chil- format and structure of the pediatric rehabilitation dren, performance of gross motor activities, such as examination at different ages. Specific details of diag- sitting, crawling, standing, or walking, also can be nostic signs and interpretation of findings are discussed conducted through the parent. One should note the in subsequent chapters about different disabilities. quality of movements, postures, weakness, incoordi- nation, asymmetry, or reflex abnormalities that reflect Observation a motor deficit. Range of motion, deep tendon reflexes, or primitive reflexes that need physical manipulation As emphasized previously, the examination begins should be examined after evaluation of active mobility. as soon as the family and child enter the examina- Tests that require instrumentation, such as sensation, tion room, before the child is actually touched or fundoscopy, otoscopy, and oral function, conclude the asked to perform. Sometimes, it may be the most examination. informative phase of the examination. Specific behaviors to observe and note include reaction to Giving choices involves the preschool child in the separation from the parents (in young children); examination. For example, the examiner may ask, apparent visual and auditory awareness; tempera- “Should we look at your arm or leg now?” On the other ment (calm or hyperactive, compliant, or difficult); hand, questions such as “Can I look at your arm?” should be avoided because if the child says “no,” con- frontation results. Parents can often bring out many capabilities of their children without the examiner touching them.

6 Pediatric Rehabilitation School-aged and Adolescent Patients and children. A bald spot or area of short, thinning hair over the posterior skull is a sign of weak neck The customary method of systematic medical exam- muscles, most likely associated with generalized ination is applicable. Children with cognitive defi- weakness. Extraocular, facial, and tongue muscle cit need to be approached according to their mental weakness may represent cranial nerve dysfunction, rather than chronologic age. Children in this age myopathy, or other neurologic disease. Involuntary group, particularly adolescents, are usually embar- eye movements and nystagmus are noted in cerebel- rassed about walking in underwear in front of their lar or other CNS disorders. parents. Shorts or a bathing suit is more acceptable. Adolescents need to be seen with and without their The skin should be inspected for telangiec- parents. Their concerns may be different from those tasias, nevi, or other lesions. Cafe-au-lait spots or of the family and should be addressed with respect for pigmented skin areas are seen in neurofibromato- their privacy. sis. In children with ataxia, telangiectasias are usu- ally present over the flexor surface of the knees and The scope of the examination is expanded to elbows. Malar rash suggests a rheumatic disease. reflect the growing child’s increasing functional needs Adenomatous rash, seizures, and hemiplegia are pre- in activities of daily living (ADLs) and other areas of sent in tuberous sclerosis. Hairy patches, dimples, or competence. A comprehensive examination includes other skin lesions over the spine are frequent signs screening in educational achievements, reading, writ- of spina bifida occulta (7). A small sinus, dermal ing, and arithmetic. Formal psychological or psycho- tract, or pylonidal cyst in the gluteal crease also may educational testing follows in case of deficits. accompany occult spina bifida. Sudden weakness in such cases may indicate an infection penetrating Growth into the spinal canal or a neurologic complication related to underlying malformation in or around Parameters of physical growth should be routinely the spinal cord. In children with sensory deficit, measured on each visit and plotted on the standard the entire area must be routinely examined for skin growth chart. Height and weight are obtained at all lesions, pressure abrasions, ulcerations, and infec- ages, and head circumference is measured in children tions. Foot deformities, varus or valgus deformity, under three years and thereafter in children with devi- or claw toes lead to abnormal weight distribution ations. Serial monitoring is necessary in hydrocepha- and callus formation consistent with the pathologic lus, regardless of etiology, and microcephaly, which posture. Calluses over the dorsum of the feet and reflects defective brain growth. In spina bifida and knees, the so-called “housemaid’s knee,” develop in other disabilities that require full-time wheelchair use, older children whose preferred mode of locomotion arm span measurement is recommended instead of is crawling. Multiple scars, bruises, and abrasions in height (7). Extremity length and girth are recorded in various stages of healing may indicate frequent falls children with localized growth disturbance due to neu- or child abuse. rogenic weakness, epiphyseal fracture, or arthritis. In growth disturbances that involve one side of the body, Asymmetry in the size of skeletal muscles should one must determine whether the condition represents be noted in terms of location and distribution. hemihypertrophy or hemiatrophy. Hemihypertrophy Anterior axillary and upper chest muscle atrophy unrelated to neurologic causes requires investigation may represent absent pectoralis muscle or wasting for renal tumor. due to an old brachial plexus injury. Congenital club- feet or multiple joint deformities are manifestations Inspection of prenatal muscle weakness due to spina bifida, arthrogryposis, or myotonic dystrophy, or may be General appearance and special features may help idiopathic. A hypertrophic, “muscle-bound” appear- to establish a diagnostic entity. Dysmorphic facial ance is a sign of myotonic dystrophy. Deformed, features, epicanthal folds, increased intercanthal fusiform, dimpled joints are seen in arthrogryposis. distance, external ear anomalies, and malforma- Lower extremity joint positions reflect the distribu- tions of the toes or fingers suggest a prenatal disor- tion of muscle weakness in newborns with spina der, possibly teratogenic or genetic, and at times, an bifida. Hypertrophy of the calf muscles is an early identifiable syndrome (l0). Blue sclerae are a sign of sign of Duchenne muscular dystrophy. Hypertrophic osteogenesis imperfecta. Asymmetric facial and pal- musculature of the shoulder girdles and upper pebral fissures and pupils may indicate facial palsy extremities is a convincing indication of functional or Horner’s syndrome, whereas craniofacial asym- crutch walking or effective wheelchair locomotion. metry and vertical strabismus develop in torticol- An enlarged limb with bruit detectable by palpation lis. Dolichocephaly is typical in premature infants or auscultation may signal an arteriovenous shunt and increased blood flow in the extremity.

Chapter 1 History and Examination 7 Flaring of the ribs, or the so-called bell-shaped the pediatric physiatrist should perform a selective chest, suggests ineffective intercostal muscle func- general physical examination. The emphasis is placed tion in children with motor unit disease or high spi- on organ systems that are at increased risk in certain nal cord dysfunction. In scoliosis, the thoracic cage is handicaps and may affect both overall health and suc- asymmetric. cessful rehabilitation. Palpation Vital signs, including blood pressure and heart rate, are obtained in all patients. In myopathies and In infants and young children, the fontanelles and cra- collagen diseases, cardiac auscultation should be per- nial sutures should be palpated for patency, tension, formed because of the possibility of associated heart and size with the child in sitting position and while disease. In a child with developmental delay, the the child is quiet and not crying. A tense fontanel in presence of a heart murmur may suggest an undiag- a vigorously crying child does not necessarily mean nosed syndrome. Blood pressure monitoring is par- increased intracranial pressure. In case of ventricu- ticularly important in spinal cord injury, neurogenic loperitoneal shunt, the reservoir should be located bladder, Guillain-Barré syndrome, and residual polio- and checked for ease of emptying and speed of refill. myelitis, as well as in children receiving stimulant The skin should be felt for texture, temperature, and medications. absent or excessive perspiration. Pseudomotor paraly- sis in spinal cord injury eliminates sweating below In disabilities that cause ineffective ventilation the level of the lesion, and compensatory excessive and involve the risk of minor aspirations, auscultation perspiration occurs above the level of the lesion with of the lungs must be a routine procedure. Myopathies, high environmental temperature. Vasomotor dysfunc- thoracic spinal cord dysfunction due to injury or mal- tion with coldness to touch and paleness or slight cya- formation, severe spastic quadriparetic cerebral palsy, nosis of the skin may be present in severe upper motor and any disability with oral motor dysfunction are neuron impairment. It is seen in the lower extremities such indications. of some children with cerebral palsy. Subcutaneous abnormalities may be palpable, such as hard cal- Abdominal and rectal examinations are essential cific deposits in dermatomyositis or neurofibroma- in children with neurogenic bladder and bowel dys- tous nodules along the course of peripheral nerves. function to evaluate bladder distention, bowel or rectal When arthritis is suspected, each joint should be felt impaction, and anal sphincter tone. Stool consistency, for the cardinal signs of inflammation, warmth, dis- intermittent or continuous bladder incontinence, and comfort, and swelling due to synovial thickening and gross appearance and microscopic examination of effusion. the urine should be noted. Umbilical movements in response to eliciting superficial abdominal reflexes Much can be learned from palpation of muscles. help to delineate the spinal cord level in thoracic Tone and bulk are reduced in lower motor neuron lesions. Absent abdominal muscles result in loose skin paralysis; in longstanding denervation, the muscle folds resembling a prune; hence, the name prune-belly tissue feels less resilient and fibrotic. The pseudo- syndrome. hypertrophic calf muscles in Duchenne muscular dystrophy have a typical rubbery, doughy, hard con- Neuromuscular System sistency. A fibrotic nodule is usually palpable in the sternocleidomastoid muscle in congenital torticollis. Examination of neuromuscular function consists of In an infant who has an isolated knee extension con- testing reflexes, tone, active motion, strength, and tracture, a palpable nodule in the quadriceps indicates coordination. Limited understanding and coopera- fibrotic muscle changes at the site of previous repeated tion in infants and young children requires adapta- intramuscular injections. Localized pain and swelling tion of traditional methods of testing. After four to five accompany injuries to soft tissue or bone. Osteoporotic years of age, the standard examination is generally fractures in lower motor neuron lesions with sensory applicable. deficit show swelling but are painless. Tenderness in many muscle groups with weakness, fatigue, or skin In infancy, reflex testing includes age-appropriate rash is suspicious for myositis due to collagen disease responses that reflect early immaturity and subsequent or parasitic or viral infections. maturation of the central nervous system. In new- borns and young infants, state of alertness, activity, Organ Systems and comfort influence muscle tone (11–14). If the baby is anxious, upset, restless, or crying, this part of the Although the primary health care of children with dis- examination should be postponed. Valid assessment abilities remains the responsibility of the pediatrician, may require several attempts. In the first few months of life, flexor tone predominates. Hypotonia or hyper- tonicity signals neurologic abnormalities. Increased tone is the symptom of corticospinal or basal ganglion

8 Pediatric Rehabilitation damage. Myopathy, cerebellar dysfunction, and lower muscles acting on the joint. The deformity or deviation motor neuron lesions due to anterior horn disease, is in the direction of over-pull. Such imbalance may be neuropathy, or spina bifida all can result in hypoto- spastic or paralytic. nia. However, a hypotonic stage usually precedes the appearance of increased tone in perinatal anoxic Visual observation during performance of func- brain damage (l5). This stage of hypotonicity tends to tional activities to detect muscle weakness should con- last longer in dyskinetic cerebral palsy than in spas- sider the child’s age and the achievements expected tic types. On passive motion of hypotonic muscles or for the child’s developmental stage. Walking on tip- extremities, no resistance is felt. The infant with gen- toes, squatting and rising without using the arms for eralized hypotonia is limp and floppy with handling assistance, and straight sitting up from the supine and, in severe cases, may feel like a “rag doll”—a position without rolling to the prone position or to the descriptive term for this finding. In hypotonia related side are mastered by children around three years of to motor unit disease or lower motor neuron lesion, age (19). Thus, inability of younger children to per- deep tendon reflexes are diminished or absent. In con- form these activities in a mature pattern should not trast, they are present or increased in floppy infants be interpreted as weakness of the plantar flexors, hip during the transient hypotonic phase of central ner- and knee extensors, or abdominal muscles. Testing for vous system damage (l5). Trendelenburg’s sign and grading the triceps surae by having the child rise on the toes of one leg must be Spastic hypertonicity and related postures are deferred until four years of age, when children develop influenced by position in space and the effect of grav- adequate balance. ity. The child should be examined in supine, prone, and vertical positions to elicit typical postures. Examples The standard technique of manual muscle testing include increased scissoring, extension, and plantar can be used after school age, except in children who flexion of the legs when a child with spastic cere- have serious behavioral problems or mental retarda- bral palsy is suddenly lifted into vertical suspension. tion (20–23). The customary grading system of scores Resistance to both slow and fast stretching of muscle from 0–5 or zero to normal is used. Above fair grade, should be tested to differentiate rigidity from spastic- the wide range of normal variations in growth pat- ity (l6). In infants and young children, one may use a terns should be considered in judging good versus number of developmental reflexes to examine active normal strength. Because children are adept in using movements and strength (l7). The Moro reflex includes substitution movements, the examiner must pay spe- shoulder abduction followed by forward flexion of the cial attention and adhere to precise technical conduct arm. Eliciting palmar or plantar grasp reflexes dem- of testing individual muscles. Side-to-side comparison onstrates finger or toe flexor function. Asymmetric may detect even mild neurologic weakness, although responses in the upper extremities may suggest Erb’s disuse atrophy or mild bilateral neurologic weak- or Klumpke’s paralysis or hemiplegia. Unilateral or ness may escape detection. Quantitative strength bilateral absence of protective extension response determination with comparison of both sides is help- is likewise suggestive of weakness in the respective ful to demonstrate unilateral disuse atrophy in such extremity. A four-month-old infant elevates the head strong muscles as the quadriceps. This determina- and trunk on extended arms in the prone position. tion is particularly advisable in teenage athletes after Scapular winging during this activity is a sign of a knee injury. Resumption of training for competition weak serratus anterior muscle (18). In older children, before virtually equal bilateral quadriceps strength is the wheelbarrow maneuver demonstrates the same regained predisposes to recurrent injuries. Testing of finding (18). Lifting up under the axilla elicits sponta- strength in upper motor neuron lesions requires the neous active shoulder depression. When these muscles well-known considerations for position in space and are weak, the shoulders slide upward, virtually touch- orientation of head and major joints, which may affect ing the ears. These signs suggest myopathy with prox- recruitment of motor units and produce synergistic imal weakness. movement patterns. Young children often adopt ingenious substitu- A common sign of central movement disorders is tions or vicarious movements to cope with weakness impaired coordination. Proprioceptive sensory loss or of particular muscles. With weakness of the deltoid, parietal lobe syndrome may contribute to incoordina- they may fling the arm forward by momentum or sub- tion. Movement abnormalities associated with cere- stitute the long head of the biceps for shoulder flexion. bellar dysfunction, basal ganglion disease, dyskinetic In advanced shoulder and elbow weakness, they may disorders, or spastic incoordination present with spe- “walk up” the arm on the torso, using their fingers to cific distinguishing signs. Detection of coordination get the hand to the mouth. Combat crawl is a usual way deficit is based mostly on observation of gross and fine of crawling in lower extremity paralysis. Deformities motor function in children less than two to three years around a joint reflect an imbalance of strength in of age. Concurrent mild delay of motor development is not unusual. After three years of age, the examination

Chapter 1 History and Examination 9 becomes more specific for testing the quality of perfor- neonates is consistent with prenatal coxa valga and mance in complex and more advanced developmental increased anteversion. Femoral inclination is 160 skills. Around three years of age, the child can walk degrees, and the angle of anteversion is 60 degrees. along a straight line, unsteadily placing one foot in Respective adult measurements of 125 and 10 to 20 front of the other. In comparison, facility at tandem degrees develop postnatally and are accelerated by walking at five years of age is a good illustration of weight bearing. continuing refinement of motor skills with age. The pediatric physiatrist may be asked to evaluate the Persistent fetal configuration in nonambulatory appropriateness of coordination in children without children with physical disabilities enhances the effect an overt physical disability (24). Clumsiness of hand- of neurogenic muscle imbalance on the hip joint and writing and drawing, difficulties in physical education contributes to acquired hip dislocation in spina bifida or sports, and other subtle signs may be present. Such and cerebral palsy. The popliteal angle is 180 degrees children may have a motor incompetence of apraxic in the hypotonic preterm infant, compared with nature, sometimes related to visuomotor perceptual 90 degrees in full-term neonates. A combination of deficit (25). It also may be associated with learning increased flexor tone and retroversion of the proximal and behavioral dysfunction. A number of tests are tibia causes this limitation of knee extension in mature available for examining motor proficiency and dex- newborns. By 10 years, tibial retroversion resolves terity in children without physical disability (26,27). spontaneously. An early varus configuration of the Tasks to evaluate youngsters with minor neurologic tibia contributes to the physiologic bowleg appearance dysfunction include imitation of gestures (28), hopping in infancy and corrects itself by two to three years of (29), hand-clapping (30), and pegboard performance age. A systematic review of skeletal development, with (31,32). examination of the spine and extremities, is presented in Chapter 14. Musculoskeletal System Normal variations of stance and gait should not be Examination of the musculoskeletal system includes mistaken for pathology in the growing child (35,40,41). inspection and palpation of bones and soft tissues, Gait abnormalities evident on clinical observation measurement of active and passive joint range of include asymmetric stride length and stance phase in motion, and assessment of stance and gait (33–36). It hemiparesis; toe walking and scissoring with lower is complementary to neuromuscular assessment. As extremity spasticity; crouch posture and gait in diple- in previous parts of this chapter, only developmental gic cerebral palsy; Trendelenburg’s gait in motor unit variations are discussed. diseases and hip dislocation; gastrocnemius limp with lack of push-off in L4–L5 weakness due to spina Bone configuration and joint mobility change bifida; and various types of gait deviations associated during the growing years (37,38). Full-term infants with involuntary movements, such as ataxia, tremor, may lack as much as 25 degrees of elbow extension or dyskinesias, in dysfunction of the central nervous because of predominant flexor tone. In contrast, joint system. hyperextensibility and hypotonia allow increased passive motion in preterm infants. The scarf sign is a Sensory Examination good illustration of excessive joint mobility in prema- ture babies. Holding the infant’s hand, the examiner A complete examination of all peripheral sensory draws one arm across the chest, like a scarf, toward modalities is possible only in older children(42). the contralateral shoulder. In premature infants, the Nevertheless, some modalities can be tested in infants elbow crosses the midline, indicating hypotonic lax- and young children, and provide significant informa- ity of the shoulder and elbow joints. Full-term neo- tion. An infant who cries and squirms to move away nates have incomplete hip extension with an average from pinprick obviously perceives pain(43). A sleepy limitation of 30 degrees as a result of early flexor infant may be slow to respond and requires repeated tone predominance (37,38). The limitation decreases stimuli. Withdrawal of the leg from painful stimuli to less than 10 degrees by three to six months. At may represent the triple flexion spinal withdrawal birth and during early infancy, hip external rotation reflex in thoracic spinal cord lesion and should not be exceeds internal rotation (37,39). With the resolution mistaken for active movement and presence of sen- of early hip flexion attitude, internal rotation grad- sation. Comparing the infant’s reaction to pinprick ually increases. Differences between bilateral hip on the arms or face differentiates actual sensory per- abduction, apparent shortening of one leg, and asym- ception in such cases. Older infants respond to touch metric gluteal and upper thigh skin folds are highly and vibration by turning toward or moving away from suggestive of congenital or acquired hip dysplasia or the stimulus. Presence of superficial reflexes signals dislocation (38). Alignment of the femoral neck in an intact afferent and efferent reflex arc. The neuro- segmental levels are T8–T12 for abdominal reflexes,

10 Pediatric Rehabilitation L1–L2 for the cremasteric reflex, and S4–S5 for the Functional Evaluation anocutaneous reflex. In spina bifida, absence of these reflexes generally coincides with sensory deficit in The pediatric rehabilitation examination is meaning- the respective dermatomes. In young children who less if the physiatrist does not construct from it a coher- cannot be tested for proprioceptive function, ataxia ent picture of the child’s functional achievements. and incoordination may suggest absence of this sen- This evaluation both complements and integrates the sation. Testing of position sense is usually reliable by variety of information derived from all phases of the school age. examination. Cortical sensory function is impaired in parietal lobe The developmental diagnostic evaluation is a damage (42,44). The most frequent childhood example convenient, functionally oriented assessment tool for is hemiparetic cerebral palsy. Disproportionately poor infants and preschool children (19,52). Language, fine spontaneous function, neglect, and visual monitor- motor and adaptive skills, gross motor abilities, and ing during use of the arm and hand are suspicious personal–social behavior are the four major areas of signs. Objective evaluation is generally feasible after function in the organizational framework of devel- five to six years of age, using the same technique as in opmental testing. The same functional domains are adults for stereognosis, two-point discrimination (45). considered in the evaluation of older children and and topognosia with single or double sensory stimu- adolescents. However, in these age groups, the exami- lation. Testing for graphesthesia may be attempted by nation includes a wider range of developmental expec- using a circle or square. Around eight years of age, the tations and abilities to function in school and society. traditional number identification gives more accurate ADLs and gross mobility skills need to be assessed in information. Cutaneous sensation and proprioception this context. In addition to speech, testing of language must be intact, and adequate cognitive ability is a pre- function includes other modes of communication: requisite for testing cortical sensory function. reading, writing, spelling, and, if indicated, augmen- tative communication. Drawing, design construction, The child’s age and ability to cooperate need to arithmetic problems, and questions about handling be considered in the examination of special senses. hypothetical situations in daily life offer a brief, pre- Moving a bright light or attractive object across the liminary insight into cognitive and learning abilities. visual field is used to test vision in infants. At one A number of specific assessment instruments were month, the infant will follow to midline and at three designed for various childhood disabilities (53–56). months, from side to side through a 180-degree arc. These instruments are useful functional assessment The Stycar test and the illiterate E chart are used for tools for their designated conditions and appropriately screening preschool children at risk for visual deficit complement the customary developmental evaluation. (46,47). At an early age, unilateral impairment or loss of vision and visual field defects, such as hemianop- INFORMING INTERVIEW sia, are more likely to remain undetected than bilateral deficits. A child with strabismus or suspicion of dimin- Informing the family about the findings of the ished vision should see an ophthalmologist as soon as examination and their implications is an important the problems are discovered. Early treatment with eye responsibility of the physician. Factual information patching or corrective lenses is necessary to prevent must be imparted with a caring attitude. Informing amblyopia ex anopsia (48,49). Central dysfunction the parents about a newly established diagnosis of visual attentiveness, discrimination, and informa- should be considered as crisis intervention. A diag- tion processing may be misinterpreted as diminished nostic label is insufficient without explanation of its vision and require both ophthalmologic and neuropsy- meaning. The parents need to know the estimated chologic investigation. prognosis, including the uncertainties of early prog- nostication, particularly in central nervous system Screening of auditory function is a routine pro- dysfunction, with the possibility of multiple handi- cedure in the neonatal nursery, pediatric office, and caps. Future needs in care and functional rehabil- school. The examination of handicapped infants and itation should be outlined. One should emphasize children also should include simple screening of hear- the need to avoid focusing on the physical disability ing, eliciting the blink or startle reflex. Responses by alone and to consider the child’s developmental and hand clapping to speech of conversational loudness or social needs. Effective counseling and communica- whisper; perception of finger rubbing near the ear; and tion skills are essential for establishing a partner- reaction to tuning fork, bell, or cricket toy are methods ship between the physician and family to ensure the of testing. Absent, lost, or delayed speech, articulation successful outcome of a comprehensive rehabilita- deficits, inattentiveness to sound, a history of recur- tion program. rent otitis media, head injury, or failure to pass the screening test indicates a need for complete evaluation of auditory function (43,48,50,51).

Chapter 1 History and Examination 11 REFERENCES 27. Broadhead GD, Bruininks RH. Factor structure consistency of the Bruininks-Oseretsky Test-Short Form. Rehabil Let. 1. Milunski A, Ulcickas M, Rothman, K, Willet, W, Jick, S, and 1983;44:13. Jick, H. Maternal heat exposure and neural tube defects. JAMA. 1992;268:882. 28. Berges J, Lezine I. The imitation of gestures. Clin Dev Med. 1965;1812. 2. Aicardi J. Diseases of the nervous system in childhood. Clin Dev Med. 1992;11:118. 29. Denckla MB. Development of coordination in normal chil- dren. Dev Med Child Neuro. 1974;16:729. 3. Nelson KB, Ellenberg JH. Antecedent of cerebral palsy: Multivariant analysis of risk. N Engl J Med. 1986;315:81. 30. Denckla MB. Development of speed in repetition and suc- cessive finger movements in normal children. Dev Med 4. Molnar GE. Motor deficit in retarded infants and young Child Neurol. 1973;15:635. children. Arch Phys Med Rehab. 1974;55:393. 31. Gardner RA. Normative Data (Revised) Examiner Manual 5. Northern J, Downs M. Hearing in Children. 4th ed. for the Purdue Pegboard Test. Chicago: Science Research Baltimore: Lippincott, Williams & Wilkins; 1991. Associates; 1978. 6. Mita K, Akataki K, Ito, K, Ono, Y, Ishida, N and Oki, T. 32. Wilson BC, Iacovillo JM, Wilson JJ, Risucci D. Purdue Assessment of obesity in children with spina bifida. Dev Pegboard performance in normal preschool children. J Clin Med Child Neurol. 1993;35:305. Neuropsychol. 1982;4:125. 7. Shurtleff DB, ed. Myelodysplasias and Extrophies: Significance, 33. Inman VT, Ralston JH, Todd F. Human Walking. Baltimore: Prevention and Treatment. New York: Grune and Stratton; Lippincott, Williams & Wilkins; 1981. 1986. 34. Lehman JF, DeLateur BJ. Gait analysis, diagnosis and man- 8. Roberts D, Sheperd RW, and Sheperd, K. Anthropometry agement. In Kottke JF, Lehman JF, eds. Krusen’s Handbook and obesity in myelomeningocele. J Pediatr Child Health. of Physical Medicine and Rehabilitation, 4th ed. Philadelphia: 1991;27:83. WE Saunders; 1990. 9. Hays R, Jordan R, McLaughlin, J and Nickel, R. Central 35. Sutherland DM, Olshen R, Cooper L, Woo-Sam J. The devel- respiratory dysfunction in myelodysplasia: An indepen- opment of mature gait. J Bone Joint Surg. 1980;62A:336. dent determination of survival. Dev Med Child Neurol. 1989;31:366. 36. Broughton NS. A Textbook of Pediatric Orthopedics. Philadelphia: WE Saunders; 1997. 10. Jones KL. Smith’s Recognizable Patterns of Human Malformation, 5th ed. Philadelphia: WE Saunders; 1997. 37. Steindler A. Kinesiology of the Human Body. Springfield, IL: Charles C Thomas; 1955. 11. Baird HW, Gordon EC. Neurologic evaluation of infants and children. Clin Dev Med. 1983;84–85. 38. Tachdjian MO. Pediatric Orthopedics, 2nd ed. Philadelphia: WE Saunders; 1990. 12. Brazelton TB. Neonatal behavioral assessment scale. Clin Dev Med. 1984;88. 39. Forero N, Okamura LA, Larson, MA. Normal ranges of hip motion in neonates. J Pediatr Orthop. 1086;9:391. 13. Dubowitz V, Dubowitz L. The neurological assessment of the preterm and full-term infant. Clin Dev Med. 1981;79. 40. Staheli LT. In-toeing and out-toeing in children. J Fam Pract. 1983;16;1005. 14. Fenichel GM. Neonatal Neurology. New York: Churchill Livingstone; 1980. 41. Sutherland DM. Gait Disorders in Childhood and Adolescence. Baltimore: Lippincott, Williams & Wilkins; 1984. 15. Dubowitz V. The floppy infant. Clin Dev Med. 1980;76. 16. Young RR, Delwaide PJ. Spasticity. Parts I and II. N Engl J 42. Brown SB. Neurologic examination of the older child. In Swaiman YF, Wright FS, eds. The Practice of Pediatric Med. 1981;304:28,96. Neurology. St. Louis: Mosby; 1982. 17. Johnson EW. Examination for muscle weakness in infants 43. Brown SB. Neurologic examination during the first 2 years and small children. JAMA. 1958;168:1306. of life. In Swalman KF, Wright FS, eds. The Practice of 18. Swaiman KF. Pediatric Neurology: Principles and Practice, Pediatric Neurology. St. Louis: Mosby; 1982. 2nd ed. St. Louis: Mosby; 1994. 44. Rapin I. Children with Brain Dysfunction: Neurology, Cognition, 19. Gesell AL, Halverson HM, Thompson H, et al. The First Five Language and Behavior. New York: Raven Press; 1982. Years of Life. New York: Harper and Row; 1946. 45. Hermann RP, Novak CB, MacKinnon SE. Establishing nor- 20. Cole TM, Barry DT, Tobis JS. Measurement of musculo- mal values of moving two-point discrimination in children and adolescents. Dev Med Child Neurol. 1996; 38:255. skeletal function. In: Kottke FJ, Lehman JF, eds. Krusen’s Handbook of Physical Medicine and Rehabilitation. 4th ed. 46. Savitz R, Valadian I, Reed R. Vision Screening of the Preschool Philadelphia: WE Saunders; 1990. Child. Washington DC: U. S. Department of Health and 21. Daniels L, Worthingham C. Muscle Testing Techniques of Manual Human Services, Children’s Bureau, 1965. Examination, 5th ed. Philadelphia: WE Saunders; 1986. 22. Kendall FP, McCreary EK. Muscles, Testing and Function, 47. Sheridan MD. Manual for the STYCAR Vision Tests, 3rd ed. 3rd ed. Baltimore: Lippincott, Williams & Wilkins; 1983. Windsor: NFER, 1976. 23. Pact V, Sirotkin-Roses M, Beatus J. The Muscle Testing Handbook. Boston: Little, Brown; 1984. 48. Lewis M, Taft LT, eds. Developmental Disabilities. Theory, 24. Touwen BCL. Examination of the child with minor neuro- Assessment and Intervention. New York: SP Medical and logic dysfunction. Clin Dev Med 1980;71. Scientific Books; 1982. 25. Rapin I. Children with Brain Dysfunction: Neurology, Cognition, Language and Behavior. New York: Raven Press; 49. Martin LJ. Pediatric ophthalmology. In Behnnan RE, Vaughan 1982. VC, eds. Nelson’s Textbook of Pediatrics, 12th ed. Philadelphia: 26. Lin JP, Brown JK, Walsh EG. The maturation of motor dex- WE Saunders; 1983. terity, or why Johnny can’t go any faster. Dev Med Child Neurol. 1996;38:244. 50. Milstein JM. Abnormalities of hearing. In Swaiman KF, Wright FS, eds. The Practice of Pediatric Neurology, 2nd ed. St. Louis: Mosby; 1982. 51. Rapin I. Children with hearing impairment. In Swaiman KF, Wright FS, eds. The Practice of Pediatric Neurology, 2nd ed. St. Louis: Mosby; 1982.

12 Pediatric Rehabilitation 52. Gesell AL, Ilg FL. The Child from Five to Ten. New York: 55. Sousa YC, Tezrow RW, Holmir, R, McCartin, R and Harper and Row; 1946. Shurtleff, D. Developmental guidelines for children with myelodysplasia. Phys Ther. 1983;63:21. 53. Gross Motor Measures Group. Hamilton, Ontario, Chedoke- McMasters Hospital, 1993. 56. Pruitt S, Varni YW. Functional states in limb deficiency: Development of outcome measures for preschool children. 54. Hally SM, Coster B, Ludlow, L, Haltiwanger, J, and Arch Phys Med Rehabil. 1998;79:405. Andrellos, P. Pediatric Evaluation of Disability Inventory (PEDI): Development, Standardization and Administration Manual: New England Medical Center Hospital. Boston: PEDI Research Group; 1992.

2 Medical Care of Children with Disabilities Susan D. Apkon and Deirdre Arnholz Children with special health care needs (CSHCN) MEDICAL HOME are those children who have been or are at risk for a chronic physical, developmental, behavioral, or The concept of a medical home has long been endorsed emotional condition and who also require health by the American Academy of Pediatrics as the optimal and related services of a type or amount beyond model for the provision of primary care for all children. that required by children generally(1). An estimated As defined in 1992, a medical home should provide 12.8% of children in the United States had a spe- care that is “accessible, continuous, comprehensive, cial health care need in 2001(2). Due to the large family-centered, coordinated, and compassionate. It number of CSHCN, primary care providers (PCP) should be delivered or directed by well-trained physi- and pediatric subspecialists care for this compli- cians who are able to manage or facilitate essentially cated group of children on a regular basis. Routine all aspects of patient care. The physician should be health maintenance visits are frequently omitted in known to the child and family, and should be able to favor of visits for acute illnesses, which can result develop a relationship of mutual responsibility and in a failure to discuss routine health care issues, trust with them” (3,4). The provision of “culturally such as growth and development, immunizations, effective” care is an additional mandate of a medical vision, hearing, and dental care. Evaluation of chil- home. dren during an acute illness can pose unique chal- lenges to care providers relating to many factors, Given the multiplicity of the needs of CSHCN, including extensive past medical and surgical his- access to a medical home, as defined previously, is tories, lengthy lists of medications, and the lack of of critical importance. Beyond the provision of acute typical signs and symptoms normally present dur- and routine medical care, the medical home can pro- ing a typical childhood illness. This chapter will vide both “vertical links” within the medical commu- focus on a discussion of the provision of medical nity and “horizontal links” to the wider community. care to CSHCN using a medical home model, the Within such a network, families should feel that they routine health maintenance issues for children with have a supportive, effective, informed, and caring net- disabilities, and the common acute medical issues work to rely on to help them meet the acute, chronic, that a PCP may be asked to evaluate in this group of and often unanticipated problems of a child with spe- children. Finally, this chapter will discuss the issue cial health care needs. of palliative care for children with special health care needs. Within the medical community, families rely on the primary care physician to make appropriate referrals to and communicate with the multiple subspecialists

14 Pediatric Rehabilitation who also provide care to many of these children. It can having parents hold the child while stepping on the be of enormous benefit to have a designated individual scale themselves. This is more effective in a smaller in the office or clinic who is able to coordinate multi- child, but is more difficult in a larger child or one with ple appointments on the same day, thus lessening the severe spasticity or hypotonia. It is recommended that burden of travel for these families. Having translator an office who cares for large numbers of children who services available, as well as written materials in the are nonambulatory obtain a wheelchair scale, which family’s primary language, is an additional benefit. allows the child to be weighed easily in his or her own wheelchair. Assessment of the length of a child is also Children with special health care needs often problematic when the ability to stand is limited. Use require therapeutic as well as supportive services. of arm span as a substitution for height may be an Examples of therapeutic services include home nurs- acceptable option. Alternatives to standing height also ing; physical, occupational, or speech therapy; and in include individual measurements of lower extremity some cases, mental health services. Supportive ser- segments when significant joint contractures are pre- vices may include the provision of letters of medical sent. To obtain segmental measurements, the child is necessity, assistance with transportation, acquisition placed in the supine position on the examination table of durable medical equipment, provision of informa- and the assessment is done by adding all of the mea- tion regarding financial entitlements and respite care, surements obtained from the head to pelvis, the pelvis connections to community support groups, and com- to knees, and knees to feet. Use of knee height has also munication with schools. Care coordination can and been used as another means of monitoring a child’s should be facilitated by knowledgeable individuals growth (6–8). within the medical home, typically experienced regis- tered nurses (RNs) and social workers. The provision of Plotting a child’s anthropometric data on a growth such services can play a pivotal role in decreasing the chart will allow the PCP to track a child’s nutritional care burden on the family, promoting maximal inde- status. A weight-to-length ratio below the fifth percen- pendence of the child and enabling full participation tile may represent failure to thrive. However, growth in community life. The medical home has additionally velocity is the more important piece of information. been described as an effective model for implementing Many children with disabilities will be below the fifth a successful transition to adult medical care as chil- percentile for their age, but as long as their weight and dren with special health care needs age. length increase in parallel to a normal curve, growth may be appropriate. A child’s age should be corrected ROUTINE HEALTH MAINTENANCE for prematurity until 2 years of age. One must remem- ber that some children with special health care needs Children with special health care needs see their have short stature as part of their disease process or PCP more frequently than a typical child. (5) Many syndrome. Special growth charts are available for of the visits are for routine care, such as well-child children with Down’s syndrome and Turner syndrome checks, immunizations, and school and sports physi- (9,10). cals. Routine visits, however, can often be redirected quickly toward a disease-specific focused discussion. A nutritional assessment should be completed dur- Although important, the basic well-child visit is no ing routine health maintenance visits. When there is longer the focus. It is important for the PCP to ensure concern about a child’s growth, a more careful inves- that routine health care needs are being addressed, tigation into the food intake is necessary. The amount, even if it means that a second appointment is sched- variety, and consistency of food eaten may provide the uled. The following are examples of health care topics examiner with information regarding the caloric intake that should be addressed during routine visits. of the child. The amount of food eaten is important, but the amount of time it takes a child to complete a Growth and Nutrition meal also is essential. It is not unusual for a child with severe cerebral palsy or an infant with spinal muscu- Assessment of growth is a basic element of the rou- lar atrophy to eat a meal over a prolonged period. The tine physical examination of children. Routine care amount of energy that is expended during this lengthy in a typical child becomes a challenge when deal- meal may cost more in energy expenditure than is ing with a child with a disability. Length, weight, actually gained in caloric intake. Additional informa- and head circumference should be obtained at each tion that is important to obtain includes how safe the health maintenance visit in the very young child, and child appears while eating. This is best achieved by length and weight should be obtained for the older watching an infant or child eat or drink during their child. Obtaining a weight on a child who is unable routine visit. Signs and symptoms of feeding problems to stand on the office scale is often accomplished by include coughing or choking while eating, a wet vocal quality during or after the meal, poor sucking, gag- ging easily, and vomiting after a meal. A referral to

Chapter 2 Medical Care of Children with Disabilities 15 a comprehensive feeding clinic should be considered child weighs more than 10 kg. In the case of Duchenne if there is a concern about the weight of the child or muscular dystrophy, it is recommended that children his or her safety while eating. An interdisciplinary receive all of their immunizations prior to the initia- clinic may include an occupational and speech ther- tion of corticosteroids (13). apist, nutritionist, physiatrist, gastroenterologist, and/ or developmental or rehabilitation physician. Immunization against influenza of CSHCN, fami- lies, and medical providers on a yearly basis is critical Immunizations to decrease the potential devastating morbidity and mortality associated with this virus. Chemoprophylaxis Routine immunization against childhood diseases during an influenza outbreak is also recommended to should be recommended for all children with dis- decrease the ongoing spread. Influenza immunization abilities. The most current schedule can be obtained of all high-risk children older than 6 months of age through the Centers for Disease Control and Prevention and their close contacts should be strongly encour- (CDC) and is approved by the American Academy aged each fall (14). High-risk children being seen in of Pediatrics and American Academy of Family the rehabilitation clinics should include those with Physicians. (11) Special consideration must be given to recurrent pneumonias or upper respiratory infections children with special health care needs. Although chil- and those with neuromuscular diseases such as spi- dren with disabilities are not necessarily at higher risk nal muscular atrophy (SMA), congenital myopathies, for contracting childhood infections, they may have and muscular dystrophies. Children who may have greater morbidity when ill with one of these infections. increased risk from complications due to pneumococ- One of the more controversial subjects is administra- cal disease should receive the pneumococcal conju- tion of the diphtheria and tetanus toxoids and acel- gate and/or polysaccharide vaccine (12). lular pertussis (DTaP) or measles, mumps, rubella (MMR) vaccine to children with a personal or family Dental history of seizures. Administration of these vaccines can increase the risk of seizures in this group of chil- Tooth decay is one of the most common diseases of dren (12). The seizures are typically short in duration, childhood (15). Tooth decay and poor dental hygiene generalized, self-limited, and associated with a fever. in children with disabilities is related to swallow- Because the pertussis immunization is given during ing problems, drooling, and gastroesophageal reflux. infancy, the onset of a seizure after the vaccine can be The administration of medications with sweeteners confusing. Frequently, parents implicate the vaccine to make the taste more palatable or those that cause as the cause of a new-onset seizure disorder, such as gingival hyperplasia such as phenytoin also contribute infantile spasms, when in fact, the association is coin- to tooth decay. Routine dental care of a child or ado- cidental. It is recommended that the DTaP be delayed lescent with severe developmental disabilities may be until a complete neurologic evaluation is completed challenging for parents and caregivers due to an oral and the cause of the seizure determined. The MMR, aversion, a tonic bite reflex, or the inability of the child on the other hand, is not recommended to be with- to follow instructions to open his or her mouth. Other held, even with a recent history of seizure, because daily care activities, such as administration of multi- it is typically first given after the onset of infantile ple medications or respiratory treatments, may make seizures and the etiology of the seizure is generally dental hygiene less of a priority. Once a child takes already known. over the care of his or her own teeth, the quality of cleaning may not be optimal because of cognitive and Special attention should be given to children who physical limitations. are immunocompromised. Children with physical dis- abilities, such as those with rheumatologic diseases Dental health of children with cerebral palsy (CP) and Duchenne muscular dystrophy who are on chronic compared to children with other disabilities is most corticosteroids, are included in this special popula- frequently described in the literature. The incidence of tion. In general, it is not recommended that children dental caries in children with CP is similar to the gen- who are immunocompromised from corticosteroid use eral population, although the quality of the caries is receive live bacterial or viral vaccines. Although defin- different. The size of the carious lesions is greater than itive guidelines do not exist, the current Red Book rec- what is seen in typical children (16–18). Periodontal ommendation is that children receiving high doses of disease is more prevalent in children with CP com- systemic corticosteroids given daily or on alternative pared to their typical peers, likely due to the presence days for more than 14 days not receive live-virus vac- of gingival hyperplasia from phenytoin administration cines until 1 month after the discontinuation of the (19). Malocclusion and developmental enamel defects medications. High-dose corticosteroids are defined were also more common in children with CP (20–24). by receiving >2 mg/kg per day or >20 mg/day if the Erosion of primary and permanent teeth has been attributed to chronic gastroesophageal (GE) reflux.

16 Pediatric Rehabilitation The severity of erosion has been correlated with the association of visual disorders with neurologic dis- duration of the GE reflux disease, frequency of vomit- eases. The eye evaluation from birth to 3 years should ing, pH of the acid, and the quality and quantity of include a vision assessment, which is accomplished by saliva (25–28). Despite the fact that children with CP having the infant or young child fix on an object. The don’t participate in high-risk activities as frequently as examiner assesses the child’s ability to maintain the their able-bodied peers, dental trauma is more com- fixation and follow the object into different gaze posi- mon (29,30). These injuries, most commonly to the tions, a skill that by 3 months of age is developmentally maxillary incisors, are related to trauma during trans- appropriate. Further evaluations of the young child fers or falls. should also include external inspection of the eye and lids, pupillary and red reflex examination, and ocular There is little information about dental problems alignment. Assessment of the child older than 3 years for children with spina bifida. An important issue that should also include age-appropriate visual acuity mea- must be addressed at each visit is to ensure that the surements and an attempt at ophthalmoscopy. dental office or operating room provide a latex-free environment (31). Families may need to remind the Ophthalmologic disorders frequently seen in chil- dentist and hygienist of the child’s risk for an aller- dren with cerebral palsy require very close follow-up gic reaction to latex. Latex-free gloves must be avail- with an ophthalmologist (38). Annual evaluation for able to reduce the risk of an allergic reaction. Boys cataracts should be completed in children with myo- with Duchenne muscular dystrophy (DMD) can have tonic dystrophy or those on chronic corticosteroids, malocclusion with anterior and posterior open bites, such as boys with Duchenne muscular dystrophy or which are associated with lip incompetence, mouth a child with a juvenile rheumatoid arthritis (13,39). breathing, and macroglossia. Deteriorating oral mus- Detailed and accurate documentation of the ophthal- cle function as the child gets older is associated with mologic examination of a child with spina bifida can increased plaque and calculus formation and gingival be helpful when assessing possible ventriculoperito- inflammation, but not necessarily with the presence neal (VP) shunt malfunctions. For example, a malfunc- of dental caries (32,33). Boys with Duchenne muscular tioning VP shunt may cause papilledema or changes dystrophy have a greater risk of malignant hyperther- in extraocular movements. These are early indications mia when anesthesia is used for dental care (34,35). that may manifest prior to more obvious signs, such as headaches or lethargy. Routine examinations and cleaning to maintain optimal dental hygiene should be performed by a den- The eye examination of a child with a disability tist comfortable in the care of children with special is best performed by a pediatric ophthalmologist due needs. Some of the dental care may need to be accom- to the child’s high risk for ophthalmologic problems. plished under anesthesia in order to obtain the maxi- The ophthalmologists have the skill needed to obtain mum benefit. Combining dental procedures with other a thorough assessment. A referral to a pediatric oph- necessary procedures, such as a brainstem auditory thalmologist for specialized tests, such as an electro- evoked response (BAER), local intramuscular injec- retinogram (ERG), may be useful in assisting with the tions with phenol or botulinum toxin, or certain ortho- diagnosis of rare neurologic conditions, such as mito- pedic procedures, may limit the exposure to anesthetic chondrial diseases. agents. The American Academy of Pediatrics Policy Statement on oral heath care states that children with Hearing special health care needs be referred to a dentist as early as 6 months of age and no later than 6 months Newborn hearing screening is standard of care in after the eruption of their first tooth, or 12 months of the United States. In 1999, the American Academy of age (whichever comes first) (36). Visits will provide the Pediatrics endorsed the implementation of a univer- dentist with the opportunity to provide specific educa- sal newborn hearing screening program (40). Two tion to the family to allow for optimal dental care. technologies are used for newborn hearing screening: brainstem auditory evoked response (BAER) and otoa- Vision coustic emissions (OAEs). It is important that all new- borns be screened and is particularly imperative for Vision screening and eye examination should be a com- children with disabilities. Periodic reassessments of ponent of all routine health care visits. The American children with disabilities are important, since a hear- Academy of Pediatrics recommends that the evalua- ing impairment can significantly affect their develop- tion begin in the newborn period and then at all sub- mental skills. sequent visits, with the goal of identifying conditions that might result in visual impairments or represent Primary care providers should pay special atten- serious systemic diseases (37). In the child with a dis- tion to children with specific disabilities, as they are at ability, this is especially important, given the frequent greater risk for developing hearing loss. For example, children with Down’s syndrome are at increased risk

Chapter 2 Medical Care of Children with Disabilities 17 of otitis media and concomitant transient conductive used when out of the home setting. Use of medica- hearing loss (41). Children with congenital cytomega- tions such as glycopyrolate or the scopolamine patch lovirus (CMV), both symptomatic and asymptomatic can decrease the volume of secretions. The use of bot- at birth, are at risk for progressive and late-onset hear- ulinum toxin injections into the submandibular and ing loss (42). Children with athetoid cerebral palsy due parotid glands is being recommended more frequently to kernicterus have a high incidence of hearing loss, for children with cerebral palsy (47). Surgical ligation as do children who have been treated with ototoxic of the glands is typically reserved for cases that are antibiotics for systemic infections (43,44). unresponsive to medications. ACUTE ILLNESS IN THE PRIMARY Drooling may indicate that a child is having diffi- CARE OFFICE culty with eating, drinking, or swallowing. In a child with a degenerative neuromuscular disease, such as Children with disabilities present to primary care spinal muscular atrophy, the development of increased providers with the same childhood illnesses of their drooling or difficulty managing oral secretions should typical peers, but the presenting signs and symptoms prompt a further investigation into his or her feeding may be quite different. Medical personnel who care status. A referral to a feeding team should be made for for these children need to be acutely aware of these consideration of performing a modified barium swal- differences in order to accurately and efficiently diag- low. Alternative feeding modalities, such as a nasogas- nosis and treat the acute illness. It is important that tric tube or a gastrostomy tube, may be necessary. Use the primary care providers understand the disease- of medications to dry secretions in a child with muscle specific complications and how they may present. weakness may be counterproductive, as thicker secre- Referring to previous medical records can be helpful tions may be more difficult to clear. in determining the unique issues for a particular child. The American Academy of Pediatrics (AAP) provides Respiratory Distress specialized forms for families and medical personnel to maintain an up-to-date record of a child’s medical Children with upper respiratory infections commonly history, current medications, past medical complica- present to their primary care provider with fever, tions and how they typically present, and a treatment increased work of breathing, and tachypnea. The plan based on presenting signs and symptoms (45). evaluation and treatment of a child with a disability An up-to-date form can provide medical care providers who presents with these symptoms should be simi- information in a critical situation. The following sec- lar to a typical child. However, the deterioration may tion reviews specific acute and chronic complications be accelerated, requiring a rapid diagnosis and ini- and strategies for the primary care provider when tiation of treatment. The assessment should begin approaching a child with special health care needs in with a review of vital signs, including pulse oxim- order to facilitate an appropriate diagnosis and treat- etry. The physical examination focuses on assess- ment plan. ing the child’s level of alertness, his or her work of breathing, and a chest examination. Children with Respiratory Complications neuromuscular diseases will frequently increase their respiratory rate in order to maintain oxygen Drooling saturation. Unfortunately, a child can decompen- sate quickly in this situation as a result of significant Difficulty in managing oral secretions in children fatigue. Oxygen saturations can be falsely reassuring with disabilities results from poor oral motor control. in the face of hypoventilation. Parents may express concern over their child’s drool- ing, frequent cough, or increased upper airway conges- Diagnostic testing may include pulse oximetry, tion. Management of drooling can be pharmacologic chest x-ray, venous or arterial blood gas, sputum cul- or surgical. Treatment is recommended when drool- ture looking for a bacterial etiology, and viral studies ing causes significant skin irritation, social problems, for identification of common viruses such as influenza or the child is having recurrent respiratory infections and respiratory syncytial virus (RSV). Viral etiolo- secondary to poor secretion management (46). gies are the most common causes of upper respiratory infections in both disabled and typical children. When oral secretions are copious, use of a suc- tion catheter by caretakers can keep the oral cavity Use of antiviral medications should be consid- and upper airway clear. Families should be instructed ered in children with disabilities because of their in the appropriate technique of oral cavity suction- high risk for significant morbidity. Enteral or par- ing and have a portable suction machine that can be enteral antibiotics should be reserved for suspected bacterial etiologies. Coverage for anaerobic bacteria should be initiated when aspiration pneumonia is suspected.

18 Pediatric Rehabilitation A child with a neuromuscular disease, such as spi- Spasticity nal muscular atrophy or Duchenne muscular dystro- phy, may need assistance with secretion mobilization The majority of children with cerebral palsy have spas- and airway clearance. Secretion mobilization can be ticity as a component of their upper motor neuron dis- addressed with chest percussion or a vibratory vest, order. Primary care providers are frequently asked to skills that a family should be comfortable perform- evaluate a child with increasing tone. The acute onset ing. Airway mobilization can be accomplished with of increased tone may represent an intercurrent illness, the use of a cough-assist machine. The In-Exsufflator, such as an otitis media or a urinary tract infection, a commercially available device that provides a pos- causing pain in a child, which is manifested as spas- itive pressure breath followed by a large exhalation, ticity. The sole presenting signs of an acute fracture improves peak cough expiratory flow rates (48,49). of an extremity in a child who is nonverbal may be Children and caregivers should be familiar with the increased tone. A careful assessment of all extremities different techniques and initiating their use at the is necessary when the diagnosis is unclear. Treatment first signs of a respiratory illness. When symptoms of the increased spasticity should focus on treatment increase and evidence of hypoventilation is present, of the underlying illness. Use of antispasticity agents use of noninvasive and invasive respiratory support such as diazepam and baclofen may be a necessary may be necessary. Noninvasive support may include adjunct when tone is markedly increased. Acute with- negative pressure ventilation or positive pressure ven- drawal from a malfunctioning intrathecal baclofen tilation with bilevel positive airway pressure (BiPaP) pump (see chapter on cerebral palsy) may present with (50). It is important for primary care providers to have increased tone, diaphoresis, tachycardia, hyperten- knowledge of the various options for respiratory sup- sion, and irritability. The irritability may be related to port and to understand the family’s wishes on the the pruritis that is an idiosyncratic reaction not associ- extent of treatment the family wants in the case of ated with a rash. Primary care providers who care for acute decompensation. Acute events are less stress- children who have intrathecal baclofen pumps should ful when families and their primary care providers be familiar with these common presenting symptoms have discussed their wishes while the child is well and management of the withdrawal from baclofen. and prior to the event. Immediate administration of oral baclofen or intrave- nous (IV) diazepam will help decrease the symptoms, Neurological Complications but referral to a center that can evaluate and treat the malfunction is needed to resolve the problem (52). Seizure Activity Orthopedic Complications Children with cerebral palsy are at increased risk of having seizures (51). The primary care provider is Fractures frequently asked to evaluate a child who is having increased seizure activity. Identification of an inter- The incidence of fractures associated with minimal current illness, which may lower the seizure threshold, trauma is increased in children with cerebral palsy, and a review of adherence to the current medication spina bifida, and Duchenne muscular dystrophy regimen are critical questions that must be asked. (53). This is related to reduced bone mineral den- Obtaining levels of the antiepileptic medication is use- sity secondary to immobilization or limited mobility. ful in determining whether suboptimal levels are the Children with cerebral palsy or Duchenne muscular etiology of the increased seizure activity and there is dystrophy with an acute fracture typically present a subsequent need to increase the medication dose if with pain and/or irritability. However, children with levels are low. A recent increase in weight might trig- spina bifida or a spinal cord injury may only present ger the need to adjust the current dose. A referral for with swelling of the limb due to their lack of sensa- an electroencephalogram (EEG) and consultation with tion. Radiographs should be utilized when swelling a pediatric neurologist may be generated if the pattern of a limb is present, even when no trauma history is of seizures is determined to be changing. Empowering elicited. a family to treat seizure activity with fast acting ben- zodiazepine in the home setting is an important way Treatment of fractures in children with disabilities to decrease the need for emergency room visits. varies, depending on the diagnosis, type, and location of the fracture. Casting of a limb in a child may depend New-onset seizures in a child with a disability on his or her degree of mobility. In other words, in a should be evaluated thoroughly. For example, a new child who is wheelchair-dependent, a bulky splint may seizure in a child with spina bifida and shunted hydro- be applied. This is especially true for a child who is cephalus may represent a shunt malfunction with sub- insensate, since a plaster or fiberglass cast may lead to sequent worsening hydrocephalus. pressure sores. Prophylactic treatment of reduced bone mineral density in children with special health care

Chapter 2 Medical Care of Children with Disabilities 19 needs is controversial and should be addressed by a 2. van Dyck PC, Kogan MD, McPherson MG, Weissman GR, specialist in disorders of bone metabolism (54). Newacheck PW. Prevalence and characteristics of children with special health care needs. Arch Pediatr Adolesc Med. PALLIATIVE CARE 2004;158(9):884890. Primary care for children with special health care needs 3. American Academy of Pediatrics Ad Hoc Task Force may include consideration for palliative care services. on Definition of the Medical Home: The medical home. Children who should be referred for palliative care Pediatrics. 1992;90(5):774. are those with potentially life-threatening diseases. This diverse group includes children with diagnoses 4. Medical Home Initiatives for Children With Special of advanced or progressive cancer, neuromuscular Needs Project Advisory C. The Medical Home. Pediatrics. diseases, severe cerebral palsy, acquired brain inju- 2002;110(1):184–186. ries, severe central nervous system (CNS) malforma- tions, complex and severe cardiac abnormalities, and 5. Newacheck PW, Taylor WR. Childhood chronic illness: chromosomal or metabolic abnormalities. Children prevalence, severity, and impact. Am J Public Health. with HIV infection, severe immunodeficiency, cystic 1992;82(3):364–371. fibrosis, and severe epidermolysis bullosa also meet the criteria for palliative care. Palliative care for chil- 6. Bell KL, Davies PS. Prediction of height from knee height dren can and often does include life-prolonging treat- in children with cerebral palsy and non-disabled children. ments, such as a tracheostomy placement for a boy Ann Hum Biol. 2006;33(4):493–499. with Duchenne muscular dystrophy, as well as poten- tially curative treatments, such as chemotherapy for a 7. Hogan SE. Knee height as a predictor of recumbent length child with advanced cancer. for individuals with mobility-impaired cerebral palsy. J Am Coll Nutr. 1999;18(2):201–205. In a policy statement on palliative care, the American Academy of Pediatrics states, “Palliative 8. Stevenson RD. Use of segmental measures to estimate stat- treatments focus on the relief of symptoms (eg, pain, ure in children with cerebral palsy. Arch Pediatr Adolesc dyspnea) and conditions (eg, loneliness) that cause Med. 1995;149(6):658–662. distress and detract from the child’s enjoyment of life. It also seeks to ensure that bereaved families are 9. Cronk C, Crocker AC, Pueschel SM, Shea AM, Zackai E, able to remain functionally intact.”(55) Palliative care Pickens G, et al. Growth charts for children with focuses on the quality of the life remaining to the Down syndrome: 1 month to 18 years of age. Pediatrics. child. Palliative care addresses not only the physical 1988;81(1):102–110. symptoms, but also the psychological, social, and spir- itual issues of a child who lives with life-threatening 10. Lyon AJ, Preece MA, Grant DB. Growth curve for girls with or terminal conditions. Turner syndrome. Arch Dis Child. 1985;60(10):932–935. CONCLUSION 11. Centers for Disease Control and Prevention. Practices ACoI. Recommended Immunization Schedule for Persons Aged In summary, children with special health care needs 0–6 Years. Available from. http://aapredbook.aappublica- account for a large percent of children in a primary care tions.org/. practice. It is imperative that the primary care provider be familiar with the associated medical conditions of 12. Diseases AAoPCoI. Red Book. 27th ed. Pickering L, ed. Elk each child and the need to provide routine health care. Grove Village, IL: American Academy of Pediatrics; 2006. Furthermore, it is critical for the primary care provider to be familiar with the frequently occurring acute med- 13. Moxley RT, III, Ashwal S, Pandya S, Connolly A, Florence J, ical illnesses in children with disabilities and the com- Mathews K, et al. Practice Parameter: Corticosteroid mon manner in which they may present. treatment of Duchenne dystrophy: Report of the Quality Standards Subcommittee of the American Academy REFERENCES of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2005 ;64(1):13–20. 1. McPherson M, Arango P, Fox H, Lauver C, McManus M, Newacheck PW, et al. A new definition of children 14. Committee on Infectious D. Prevention of Influenza: with special health care needs. Pediatrics. 1998 Jul;102 Recommendations for Influenza Immunization of Children, (1 Pt 1):137–140. 2007–2008. Pediatrics. 2008;121(4):e1016–31. 15. Pierce KM, Rozier RG, Vann WF, Jr. Accuracy of Pediatric Primary Care Providers’ Screening and Referral for Early Childhood Caries. Pediatrics. 2002;109(5):e82–. 16. Costello PJ. Dental health status of mentally and physically handicapped children and adults in the Galway Community Care Area of the Western Health Board. J Ir Dent Assoc. 1990;36(3):99–101. 17. Pope JE, Curzon ME. The dental status of cerebral palsied children. Pediatr Dent. 1991;13(3):156–162. 18. Guare Rde O, Ciamponi AL. Dental caries prevalence in the primary dentition of cerebral-palsied children. J Clin Pediatr Dent. 2003;27(3):287–292. 19. Guare Rde O, Ciampioni AL. Prevalence of periodontal disease in the primary dentition of children with cerebral palsy. J Dent Child (Chic). 2004;71(1):27–32. 20. Franklin DL, Luther F, Curzon ME. The prevalence of mal- occlusion in children with cerebral palsy. Eur J Orthod. 1996;18(6):637–643. 21. Carmagnani FG, Goncalves GK, Correa MS, dos Santos MT. Occlusal characteristics in cerebral palsy patients. J Dent Child (Chic). 2007;74(1):41–45.

20 Pediatric Rehabilitation 22. Bhat M, Nelson KB, Cummins SK, Grether JK. Prevalence with Cerebral Palsy: Gross Motor Function Classification of developmental enamel defects in children with cerebral Scale. Invest Ophthalmol Vis Sci. 2008;49(2):572–580. palsy. J Oral Pathol Med. 1992;21(6):241–244. 39. Machuca-Tzili L, Brook D, Hilton-Jones D. Clinical and molecular aspects of the myotonic dystrophies: a review. 23. Bhat M, Nelson KB. Developmental enamel defects in Muscle Nerve. 2005;32(1):1–18. primary teeth in children with cerebral palsy, mental 40. Task Force on Newborn and Infant Hearing Loss: Detection retardation, or hearing defects: a review. Adv Dent Res. and Intervention. Pediatrics. 1999;103(2):527–530. 1989;3(2):132–142. 41. Mitchell RB, Call E, Kelly J. Ear, nose and throat disor- ders in children with Down syndrome. Laryngoscope. 24. Bhat M, Nelson KB, Swango PA. Lack of stability in enamel 2003;113(2):259–263. defects in primary teeth of children with cerebral palsy or 42. Fowler KB, McCollister FP, Dahle AJ, Boppana S, mental retardation. Pediatr Dent. 1989;11(2):118–120. Britt WJ, Pass RF. Progressive and fluctuating sen- sorineural hearing loss in children with asymptom- 25. Linnett V, Seow WK, Connor F, Shepherd R. Oral health atic congenital cytomegalovirus infection. J Pediatr. of children with gastro-esophageal reflux disease: a con- 1997;130(4):624–630. trolled study. Aust Dent J. 2002;47(2):156–162. 43. Cunningham M, Cox EO, Committee on Practice and Ambulatory Medicine, Section on Otolaryngology, 26. Milosevic A. Gastro-oesophageal reflux and dental erosion. Bronchoesophagology. Hearing Assessment in Infants and Evid Based Dent. 2008;9(2):54. Children: Recommendations Beyond Neonatal Screening. Pediatrics. 2003;111(2):436–440. 27. Shaw L, Weatherill S, Smith A. Tooth wear in children: an 44. Shapiro SM, Nakamura H. Bilirubin and the auditory investigation of etiological factors in children with cere- system. J Perinatol. 2001 Dec;21 Suppl 1:S52–S555; discus- bral palsy and gastroesophageal reflux. ASDC J Dent Child. sion S9–S62. 1998;65(6):484–486. 45. Cooley WC and Committee on Children with Disabilities. Providing a Primary Care Medical Home 28. Su JM, Tsamtsouris A, Laskou M. Gastroesophageal reflux for Children and Youth With Cerebral Palsy. Pediatrics. in children with cerebral palsy and its relationship to ero- 2004;114(4):1106–1113. sion of primary and permanent teeth. J Mass Dent Soc. 46. Lal D, Hotaling AJ. Drooling. Curr Opin Otolaryngol Head 2003;52(2):20–24. Neck Surg. 2006;14(6):381–386. 47. Reid SM, Johnstone BR, Westbury C, Rawicki B, 29. Costa MM, Afonso RL, Ruviere DB, Aguiar SM. Prevalence Reddihough DS. Randomized trial of botulinum toxin injec- of dental trauma in patients with cerebral palsy. Spec Care tions into the salivary glands to reduce drooling in chil- Dentist. 2008;28(2):61–64. dren with neurological disorders. Dev Med Child Neurol. 2008;50(2):123–128. 30. Holan G, Peretz B, Efrat J, Shapira Y. Traumatic injuries to 48. Homnick DN. Mechanical insufflation-exsufflation for air- the teeth in young individuals with cerebral palsy. Dent way mucus clearance. Respir Care. 2007;52(10):1296–1305; Traumatol. 2005;21(2):65–69. discussion 306–307. 49. Miske LJ, Hickey EM, Kolb SM, Weiner DJ, Panitch HB. 31. Hamann CP, Rodgers PA, Sullivan K. Management of dental Use of the mechanical In-Exsufflator in pediatric patients patients with allergies to natural rubber latex. Gen Dent. with neuromuscular disease and impaired cough. Chest. 2002;50(6):526–36. 2004;125(4):1406–1412. 50. Bach JR, Niranjan V, Weaver B. Spinal muscular atrophy 32. Morel-Verdebout C, Botteron S, Kiliaridis S. Dentofacial type 1: A noninvasive respiratory management approach. characteristics of growing patients with Duchenne mus- Chest. 2000;117(4):1100–1105. cular dystrophy: a morphological study. Eur J Orthod. 51. Singhi P, Jagirdar S, Khandelwal N, Malhi P. Epilepsy in 2007;29(5):500–507. children with cerebral palsy. J Child Neurol. 2003;18(3): 174–179. 33. Symons AL, Townsend GC, Hughes TE. Dental characteris- 52. Zuckerbraun NS, Ferson SS, Albright AL, Vogeley E. tics of patients with Duchenne muscular dystrophy. ASDC Intrathecal baclofen withdrawal: emergent recognition and J Dent Child. 2002;69(3):277–283. management. Pediatr Emerg Care. 2004;20(11):759–764. 53. Apkon SD. Osteoporosis in children who have disabilities. 34. Birnkrant DJ, Panitch HB, Benditt JO, Boitano LJ, Carter ER, Phys Med Rehabil Clin N Am. 2002;13(4):839–855. Cwik VA, et al. American College of Chest Physicians con- 54. Ward L, Tricco AC, Phuong P, Cranney A, Barrowman N, sensus statement on the respiratory and related management Gaboury I, et al. Bisphosphonate therapy for children of patients with Duchenne muscular dystrophy undergoing and adolescents with secondary osteoporosis. Cochrane anesthesia or sedation. Chest. 2007;132(6):1977–1986. Database Syst Rev. 2007(4):CD005324. 55. Committee on Bioethics and Committee on Hospital Care. 35. Driessen JJ. Neuromuscular and mitochondrial disor- Palliative Care for Children. Pediatrics. 2000;106(2):351–357. ders: what is relevant to the anaesthesiologist? Curr Opin Anaesthesiol. 2008;21(3):350–355. 36. dela Cruz GG, Rozier RG, Slade G. Dental Screening and Referral of Young Children by Pediatric Primary Care Providers. Pediatrics. 2004;114(5):e642–e652. 37. Committee on Practice and Ambulatory Medicine, Section on Ophthalmology, American Association of Certified Orthoptists, American Association for Pediatric Ophthalmology and Strabismus, and American Academy of Ophthalmology. Eye Examination in Infants, Children, and Young Adults by Pediatricians. Pediatrics. 2003;111(4):902–907. 38. Ghasia F, Brunstrom J, Gordon M, Tychsen L. Frequency and Severity of Visual Sensory and Motor Deficits in Children

3 Psychological Assessment in Pediatric Rehabilitation Jane A. Crowley and Kayla White-Waters The goal in pediatric rehabilitation is not an end point as larger world, the children and teens we work with do in the adult world. Rather, it is a process toward the con- not have the standard equipment or inter-relationships tinued development of ever-changing abilities and emo- among skills. For example, a child’s motor disability can tional, behavioral, and cognitive structures. The goal of easily alter the basic emotional developmental tasks. any pediatric rehabilitation process is to foster the contin- The protraction of physical dependence that is a reality uing work of childhood. This additional distinguishing for a child with a congenital disability like spina bifida, dimension of pediatric rehabilitation relates to the central at the very least, risks altering the psychological mile- imperative of the pediatric population—development. stones of separation/individuation. Cognitive sequelae There is a dual goal: rehabilitation to prior levels and of that central nervous system (CNS) disorder can also habilitation for the remaining development in that child’s result in academic and adaptive behavior deficits. In or teen’s life. An important tool for establishing current these cases, standard developmental schema often do levels, setting future goals, and tracking progress over not apply (2). Not only because of deficits, but because time is psychological assessment. there are unique tasks to be mastered with a disabil- ity. Functional use of a wheelchair, doing activities of The rehabilitation physician and the team will treat daily living (ADLs) with one arm, self-catheterization, a wide array of medical conditions among their patients. and visual competence with a field cut are but a few Rehabilitation medicine departments will encounter specific “milestones” our patients face. In the case of a requests for treatment for those with congenital dis- traumatic injury, the disruption of a normal life, with ability, acquired disability from illness or injury, and typical developmental progress and engagement in the chronic medical conditions. A recent estimate of the world, is an emotional maelstrom for the patient and his incidence of severe chronic illness seen in rehabilita- or her family (3). tion is more than 1 million children in the United States (1). These children, and those who survive catastrophic Potential distortions in many aspects of the nurturing illness or injury, are a growing population due to med- and individuating demands of competent development ical advances that reduce mortality, covering the full abound in children with disabling conditions (4). The age range from infancy to young adulthood. barrage of medical technology and interventions is vast in variety and effectiveness. Yet, the psychological In acknowledging that normal development assumes cost of these necessities can be high. The challenges an intact sensory, motor, and overall neurologic system of hospitalization, a disruption of familiar routine, the for interaction with the environments of family and the

22 Pediatric Rehabilitation therapy demands of rehabilitation, absence of parents, is indeed adaptive to the conditions and situations of and intrusive or painful medical procedures are addi- a child’s medical condition. Though some behaviors tional tasks against which to bulwark the patient (5). In may be unusual in the healthy child, they may be a broader context, there is prejudice against those with adaptive to this population (8). The concept of adjust- disability, and children must face the extra demands of ment encompasses the variability that these patients bridging ignorance and misconceptions. encounter. It can express the unique trajectory that these children’s lives will take, and recognizes it as In line with the centrality of development, the objects adaptive in that it is age-appropriate for those condi- of assessment constitute a “moving target.” Environmental tions and oriented ultimately toward healthy adult demands change, as does the child’s or teen’s abilities to functioning. meet them. At school age, the child must now function competently in the ever-increasing demands for indepen- A wide body of literature addresses the adjustment dence reflected in the school setting. Furthermore, the of children with chronic physical conditions. This medical condition can itself change over a child’s devel- group was twice as likely to have adjustment problems opment. A disease process can progress (for example, as healthy children in a meta-analysis of 87 articles by juvenile rheumatoid arthritis), or increasing body size Lavigne and Faier-Routman (9). Though the specific can change the nature of mobility (for example, spina prevalence rates were higher yet among these chil- bifida), and prior function can be lost. The task is to have dren, only a minority showed maladjustment. Such these experiences remain challenges to development and children, then, are more vulnerable than those who not become barriers. This argues for continued moni- are healthy. Newer assessment instruments have been toring throughout a child’s development as a vital fac- developed that utilize the concept of quality of life (10) tor and the importance of psychological assessment as a and will be discussed here in the section “Population- vital part of that monitoring to be utilized throughout the Specific Assessments.” With this approach, the nature pediatric course of a patient’s life. of a child’s or teen’s adjustment and the reflection of the uncontrollable factors in his or her situation are The relationship of family functioning to outcome captured for a wider rubric than the inadequate dichot- in pediatric disability has been widely demonstrated omy of normal versus abnormal. (6,7). The challenge to a family is to walk an unfamiliar path, as few families have direct experience with child- However, the use of psychiatric diagnoses can be hood disability. The effects may be bidirectional (4), appropriate in both this population and those with with the deficits from the medical condition interact- neurodevelopmental disabilities. The latter group ing with parental features or the child’s status result- showed a rate six times that of the general popula- ing in disrupted parenting approaches. Parents often tion for significant emotional and behavioral problems must assume an additional role as case manager and (drawn from an outpatient clinic population) (11). The advocate in the medical and educational systems. In identified problems run the gamut, encompassing a addition, they have to “translate” their child’s issues to breadth of disorders, and are more likely to persist into other family members at the nuclear and extended fam- adulthood. There is the primary impairment of the ily levels. The family becomes a vital arena of interven- neurologic disorder and a secondary impairment of tion. The family is the first-order site of development psychosocial support problems (4). With the primary and stimulation as well as a filter for the larger world. disturbance in the brain, these findings are not sur- prising. Other factors exist as well to either exacerbate ADJUSTMENT VERSUS or ameliorate the brain’s abnormality, but the overall PSYCHIATRIC DIAGNOSIS picture is one of significant neurologic and psycholog- ical morbidity, with the source of disability either con- It is important to recognize the distinction between genital or traumatic. psychiatric disturbance and adjustment problems as conversant concepts in assessment for a pediatric reha- The most common disabling injury of childhood bilitation population. Indeed, psychiatric disturbance is, in fact, traumatic brain injury (12), which carries is not common in children with chronic conditions, a substantial risk for long-term cognitive disability as some studies show that their functioning is better as well as behavioral deficits (13). Expressed differ- than children in the mental health clinic population. ently, incidence figures are such that by the tenth Taken together, however, children and teens seen in grade, 1 in 30 students would have had a traumatic rehabilitation medicine settings do have a greater risk brain injury (across severity ranges). This popula- for adjustment problems (1). Their medical condition tion will be a substantial part of a pediatric rehabili- acts as a life stressor not encountered by their healthy tation medicine practice. The causative link between peers. To use psychiatric diagnoses in this population cognitive and behavioral functioning represents the belies the reality of behavioral symptomatology that juncture of thinking and adaptive behavior that can be devastating to the ongoing development of a child survivor (14). There is a particular danger in the

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 23 misattribution that easily occurs. Behavioral deficits child population of the United States. The comparison are attributed to more common etiologies, as opposed is carried out by converting the raw score into some to the organic brain disorder from the injury. With mis- relative measure. These are derived scores and indicate attribution comes inappropriate treatment. Cognitive the standing of a patient relative to the norm group. limitations are not accounted for in treatment efforts, These scores also allow for comparison of the child’s or the wrong premise (for example, antecedent versus performance on different tests. Stanines, standard contingent programming) used, and failure occurs or scores, age- and grade-equivalent scores, and percen- even exacerbation of the original problems. Awareness tile ranks are the most common tests. and consideration of the brain damage from an injury means assessment must encompass a wide focus. A central concept in the expression of individual Neuropsychological testing is the centerpiece in these performance as compared to a norm group is the nor- children and teens, representing a subtype of general mal curve. The normal curve (Fig 3.1) is a bell-shaped psychological assessment that will be an important curve. It represents the distribution of many psycholog- aspect of many rehabilitation cases. ical traits, with the greatest proportion at the “middle” of the curve, where it is the largest, and the abnormal NATURE OF MEASUREMENT levels—both below- and above-average—at the two “tails.” All derived scores have a distinct placement on The essence of psychological assessment lies in the the normal curve and are varying expressions of the construction of the instruments used to explore vari- location of an individual’s performance on that curve. ous concepts of adjustment, personality functioning, behavior, and cognition. This construction always has Stanines are expressed as whole numbers from 1 at its core the notion of standardization through its ref- to 9. The mean is 5, with a standard deviation of 2. erence to a norm group, whose performance is charac- Substandard performance would be judged with sta- terized by a transformation of the raw score earned by nines in the range of 1–3 and above average at 7–9. In an individual. Even the most skilled observer could not this transformation, the shape of the original distribu- provide the richness of the information gleaned from a tion of raw scores is changed into the normal curve. psychometrically sound test. Such a test allows for the comparison of that subject to the typical performance Standard scores are generally the preferred derived of his or her peers in a fair and objective way. The value score (15). Their transformation of raw scores yields a of standardized assessment depends on some core con- mean for the normative group and a standard devia- cepts, elucidated in the following sections. tion. This places a given score across the normal curve, and the scores express the distance from the mean of Norm-Referenced Measurement that patient’s performance. Norm-referenced tests are standardized on a clearly T scores, z scores, and the well-known IQ of the defined group, referred to as the norm group, and scaled Wechsler scales are all standard scores. Like all stan- so that each individual score reflects a rank within the dard scores, the z score derives a constant mean and norm group. The examinee’s performance is compared standard deviation across all age ranges The z score to the group, generally a sample that represents the has a mean of 0 and a standard deviation of 1. It expresses below-normal performances with the minus sign and above-average with the plus sign, with scores in a range of –3 to +3. These scores are often trans- formed into other standard scores to eliminate the positive and negative signs (see Figure 3.1). T scores 13.59% 13.59% 2.14% 2.14% 34.13% 34.13% −3σ −2σ −1σ Mean +1σ +2σ Z-scores −3.0 −2.0 −1.0 0.0 1.0 2.0 3.0 Percentile 0.13 2.5 16 50 84 97.5 99.87 T-score 20 40 50 60 70 80 IQ (SD=15) 55 30 85 100 115 130 145 CEEB or 70 200 400 500 600 700 800 SAT score 1 300 8 9 Stanine 2 34 56 7 Figure 3.1 The Normal Curve.

24 Pediatric Rehabilitation and the IQ scores are drawn from the z score, with way a typical 8-year-old child would, and certainly different numerical rubrics that eliminate the plus or should not be treated like an 8-year-old for most issues minus sign associated with z score. in rehabilitation programming. Multiplying by 10 and adding a constant of Finally, as is the case with percentiles, age- and 50 yields a T score ranging from 20 to 80, with an grade-equivalents cannot be used in statistical tests, average of 50. Another transformation occurs by mul- as there is an unequal distribution of scores. Both tiplying the standard score by 15 and adding 100. require conversion to another scale before they can be This provides a range from 55 to 145, with a mean used in data analysis. of 100 and a standard deviation of 15 or 16, depend- ing on the test used. This is the method that produces Reliability the Deviation IQ, the form of derived score used on the Wechsler intelligence batteries. The alternative This concept of reliability refers to the ability of a test to the Deviation IQ is the Ratio IQ, which is the ratio to yield stable (ie, reliable) results. There needs to of mental age to chronological age multiplied by 100, be a consistency and stability of test scores, and the used in the Stanford–Binet tests. The statistical prop- nonsystematic variation reduced as much as possible. erties of this are poor, and it is not generally used or Psychometric theory holds that any score is composed well regarded. of the measurement of the actual trait that a child pos- sesses as well as an error score, which represents the What appear more understandable, but are not as variation or error of measurement. The reliability coef- psychometrically sound as standard scores, are per- ficient is the conversant statistic to express this prop- centile ranks and age- and grade-equivalent scores. erty. It can vary from 0.00, indicating no reliability, to Percentile ranks offer easy interpretation, with the 1.00, indicating perfect reliability. High-reliability coef- rank reflecting the point in a distribution at or below ficients are considered particularly important for tests which the scores of a given percentage of individu- used for individual assessment. In the case of cogni- als fall. To a lay audience, this is often confused with tive and special ability tests, a reliability coefficient of percentages, which are not referenced to a normative 0.80 or higher is required for sufficient stability to be a population—only to the number correct compared to useful test. Reliability coefficients are calculated for a the total number of items. For example, function at test across three conditions. One is test-retest, meaning the 50th percentile is average performance, whereas a the capacity of the test to yield a similar score if given grade of 50% on a test would be considered failing. a second time to a child. Another is alternate-form reliability, where the child is tested with an alternate Even more straightforward appeal exists for age- form of the test, measuring the same trait and in the and grade-equivalent scores. These scores are obtained same way as the initial testing. A third kind refers to by discerning the average raw score performance on internal stability in a test, where in the ideal test, item a test for children of a given age or grade level. The responses are compared to another item on the test individual patient’s score on that test is compared to demonstrate the equivalence of items in measuring to that value. Grade equivalencies are expressed as the construct in a replicable manner. Active judgments tenths of a grade (for example, a grade equivalency of must be made in the choice of tests, with reliability 4.1 represents the beginning of fourth grade). Despite coefficients reviewed in the process of test selection. their appeal, there are limitations with these forms of derived scores. First, a grade-equivalency value does Validity not mean that a child is performing at that particu- lar level within his or her own school, as the curricu- This is another vital consideration in the construction lar expectations of the school might be different from and use of standardized tests. Validity is the extent to the mean score established by the normative sample. which a test actually measures what it intends to mea- Some actual age- or grade-equivalency values might sure and affects the appropriateness with which infer- not have been earned by any specific member of a nor- ences can be made based on the test results. Validity mative sample, but instead are extrapolated or inter- of a given test is expressed as the degree of correlation, polated from other points of data. Furthermore, age with external criteria generally accepted as an indica- or grade equivalencies may not be comparable across tion of the trait or characteristic. different tests. The meaning of a first grader who obtains a raw score similar to a third grader is not that Validity is discussed primarily in terms of the child is functioning as a third grader in that sub- content—whether test items represent the domain ject. He or she shares that score, but the assumption being measured as claimed—or criterion—the rela- that the child in first grade has all the skills of a third tionship between test scores and a particular criterion grader is inappropriate. Similarly, a 12-year-old patient or outcome. The criterion may be concurrent, such as who achieves an age equivalency score of 8 years, comparison of performance on neuropsychological 4 months seldom actually functioned on the test the

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 25 test measures with neurophysiologic measures (eg, a physically healthy population. Motor and sensory computer tomography, electroencephalography). handicaps and neurologic impairment are not within the normative samples. Issues of validity predominate Alternatively, the criterion may be predictive—the here, though with transitory factors as noted previ- extent to which test measures relate in a predictive ously, reliability can be affected as well. Standardized fashion to a future criterion (eg, school achievement). procedures may have to be modified to ensure that a In the rehabilitation context, various events and con- patient is engaged in the testing in a meaningful way. tingencies may affect predictive validity. An appropri- ate determinant of predictive validity is the likelihood USES OF ASSESSMENT that the individual’s test performance reasonably reflects performance for a considerable period of time Psychological assessment has a wide variety of purposes after the test administration. Acute disruption in phys- in pediatric rehabilitation. These purposes encompass ical or emotional functioning could certainly interfere issues directly related to the medical setting, but often with intellectual efficiency, leading to nonrepresenta- have equal utility in educational planning. Unique to tive test results. In contrast, chronic conditions would the field of pediatric rehabilitation is this necessity for be less likely to invalidate the child’s performance from interaction between what are arguably the two biggest a predictive standpoint because significant change in public systems for children: medicine and education. performance as a function of illness or impairment Both have their productive and counterproductive would not be expected over time. With therapeutic forces and hold a vital place in the individual child’s interventions, a patient’s performance could improve, or teen’s life. Furthermore, both can act to hinder or so test results from prior to that would not be valid. potentiate the salutary effect of the other. The needs The more time that passes between test administra- and parameters of engagement with both is at the crux tions, the more likely extraneous factors can intervene of the navigation of development for our patients, and and dilute prior predictive validity. Anxiety, motiva- psychological assessment contributes significantly to tion, rapport, physical and sensory handicaps, bilin- this process. gualism, and educational deficiencies can all effect validity (15). For an inpatient population, the effects of Psychological testing is often associated solely acute medical conditions (eg, pain, the stress of hospi- with IQ testing. The intelligence quotient (IQ) con- talization, medical interventions themselves, fatigue) cept of intellectual development is too narrow for can also affect validity. Wendlend and colleagues (16) many of the applications in a pediatric rehabilitation noted that in a study of cognitive status post-polymy- setting. Instead, the evaluation of the broader aspect elitis, the deficit seen could well have been due to the of cognition is the more important activity. Cognitive effect of hospitalization as opposed to the disease. assessment covers testing the wide array of known components of the brain’s thinking skills. Assessing Construct validity refers to the extent to which the these intake, processing, and output modalities of test relates to relevant factors. Another important com- thinking, their individual elements or the combination ponent of validity is ecological validity, which refers of these skills are vital factors in school or in medi- to the extent to which test scores predict actual func- cal rehabilitation. School is children’s work, and the tionality in real-world settings. Test scores are typi- interface with this system is critical, as it is the arena cally obtained under highly structured clinical testing where many key adjustment and developmental issues situations, which include quiet conditions, few dis- are played out. Psychological adjustment—indeed, tractions, one-on-one guidance, explicit instructions, overall functioning—is intimately tied to cognitive praise, redirection, and so on. These conditions do status. Coping with frustration, functioning within a not represent typical everyday tasks or settings (17). group, and inhibiting for long-term goals, are exam- This disconnect between the test setting and real life ples of processes vital to school that have cognitive is especially relevant in children with brain-related ill- capacity at their center. ness or injury. These children, who have high rates of disordered executive functioning (eg, distraction Within the schools, the psychological assessment control, organization, planning, self-monitoring, etc.) performed has typically included only intellectual and benefit disproportionately from the highly directive achievement testing as prime components. Though that nature of clinical testing, and test scores may overesti- is changing in some settings, it is not yet common that mate true functional capacity for everyday tasks (18). cognitive processes are assessed. For the populations common to a rehabilitation medicine practice, many A test’s reliability affects validity in that a test conditions have brain involvement (eg, traumatic brain must yield reproducible results to be valid. However, injury). Their needs are clearly beyond the limitations as detailed previously, validity requires additional of typical school testings. Eligibility for services within elements. the special education system under the qualifying In the rehabilitation population, all of these issues have particular import. Most tests are developed on

26 Pediatric Rehabilitation conditions of traumatic brain injury (mandated by the learning environment. Results of psychological/ the federal government in 1998) cannot be done with- neuropsychological evaluations can be useful in dem- out consideration beyond IQ and achievement testing. onstrating such need related to cognitive issues. For Indeed, traumatic brain injury (TBI) as its own inclu- example, deficits in information processing speed can sion category was done to reflect the serious misunder- have a global effect on functioning within the group standing of the disorder when only evaluated by IQ and instructional environment of school. Accommodations achievement testing alone. The intellectual assessment such as reduction in homework, extended time for tests, of children with spina bifida needs explication beyond or lecture notes, among others, can all be sought with IQ testing as well. Often, the component parts of the the documentation provided by evaluation results. The Full-Scale IQ score are so divergent in children with issue of how long the accommodations are required spina bifida and other brain conditions that it does can be answered by repeated testing. An example is in not represent a true summary score. To understand a the case of a brain injury where recovery occurs and child’s condition fully, further assessment of cognitive accommodations may no longer be needed. processes needs to be done. Pertinent abilities are atten- tion, concentration, memory, and executive functions. It is important for the clinician to recognize the role In the wide array of conditions known to affect brain he or she can play in securing vital, but not typical, functioning there are primary and secondary effects. medical treatment for a patient. This includes speech Primary effects are seen from brain tumors, seizure and language or occupational therapy, cognitive reme- disorders, or cancer processes. Secondary effects on diation, or adjustment-focused cognitive behavioral cognitive processes are seen in the process of infectious work. The documentation of that need, based on the disease or cancer treatment. It is necessary to evaluate medical diagnosis or history, can be obtained much a broader array of abilities rather than relying solely on quicker and with the proper focus through the medical IQ to understand the full spectrum of required cogni- system in terms of both insurance coverage and proper tive skills for competent development. treatment frequency and formulation. Obtaining assess- ment from a public school system can be a lengthy pro- In order to promote the fuller understanding cess. For rehabilitation patients, this can waste valuable of medical conditions and their effects on cognitive time and, therefore, cannot meet the time frame needed functioning, the rehabilitation practitioner will often for an acute recovery. A typical school psychological be consulted for more specialized assessment to cap- assessment could miss acute issues and be even less ture the full nature of functioning within his or her likely to detect weaknesses that could hamper devel- patients. Input into the Individualized Educational opment or skill acquisition distant from the injury or Plan (IEP), which is the centerpiece of planning in the illness. Such evaluation needs the medical framework special education system, is essential in brain-based of rehabilitation psychology to be timely and pertinent. disorders to ensure full consideration of the medical Furthermore, with a rehabilitation psychology per- condition, its own process, and its unique effect on spective and knowledge, appropriate documentation brain functioning. The dynamic nature of recovery is emerges to secure services covered by medical insur- notably absent from most students receiving special ance or from legal settlement funds, if such exists. education services, but is often a primary part of the Keeping the intervention within the medical perspec- course in traumatic brain injury, brain infectious pro- tive can make it more integrated with disease or injury cesses, cancer, or strokes. The need for frequent reas- sequelae and, therefore, more targeted and appropriate sessment, specific remediation-focused services, or in terms of goals and treatment techniques. specialized support in re-entry to school are several of the unique concepts that are vital to sound educational It can be seen that the assessment of a child’s or planning in our population but are largely unknown to teen’s learning process is essential to both the school the traditional process of special education. This is the and medical setting. Memory processes, language most critical juncture of school and medical factors in abilities, planning, or capacity to inhibit are essential a pediatric rehabilitation process. functional elements in either system. The preference of one modality over another, or the explication of mem- As per Section 504 of the Rehabilitation Act, ory functioning, can be of great use in school issues accommodations are often sought on either a long-term and in rehabilitation. The need to master specialized or transitory basis in rehabilitation medicine patient tasks, such as wheelchair skills or self-catheterization, groups. These are efforts to “level the playing field” can be enhanced when general learning styles of an within the school setting in acknowledgement of dis- individual patient can be discerned. ability that skews a student’s ability to benefit from the standard educational setting. These students do not This understanding of a patient’s cognition can require the breadth or type of actual intervention or inform educating the patient about his or her medical service gained through special education classification, disorder, or the rationale about a medical procedure. but instead need modifications in the system in order The feelings of victimization that can evolve around a to demonstrate their capacities or adequately access painful surgery and the subsequent effect on adjust- ment or even personality formation are secondary

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 27 sources of potential morbidity in a child’s develop- on a developing brain (22) are all factors that argue for ment. The child’s or teen’s sense that he or she was the role of neuropsychological assessment in the care regarded enough in the consideration of procedures to of such patients. In the next section, the nature of this be included in the decision and planning process. The type of baseline testing will be explored. experience of this and the skill to be a meaningful participant are vital long-term skills and are promul- Understanding the individual experience of a gated by knowing the proper way to present material child or teen in relation to his or her body experience in a way to ensure understanding. Decisions about a is another use of assessment. Understanding the expe- child’s ability to benefit from a specific treatment such rience, whether through a questionnaire about pain, as biofeedback, relaxation training, or the varieties of assessment of specific mood states like depression or behavioral programming available are part of diagnos- anxiety, or a general personality assessment of that tics that guide treatment. patient, can be quite useful. Differential diagnosis can be important, as in the case of post-traumatic stress Change as the result of intervention can be quan- disorder, where cognitive symptoms of that disorder tified by assessment. However, change without overt can be mistaken for the effects of a mild brain injury intervention, but to chronicle the long-term outplay of or concussion. In that circumstance, the deficits are a medical condition, is arguably the most common use due to the effects of the stress and not to the mechan- of assessment in rehabilitation. The risk for long-term ical disruption of trauma. sequelae in traumatic brain injury or from cancer pro- cesses and treatment is well known (13,19). The serial TYPES OF ASSESSMENTS assessment of a patient, particularly through known critical developmental periods or illness interventions, The purpose of psychological assessment is to discern is at the core of sound pediatric rehabilitation practice. the status of an individual in relation to an appropri- A developmental lag becomes the object of treatment, ate peer group. Jerome Sattler discerns four pillars of whether to spur development or to teach compensa- child assessment as norm-referenced tests, interviews, tory strategies. As the physical process of a disease observations, and informal assessment (15). This is is monitored through traditional outpatient clinic vis- a broader list than many referral sources would rec- its, so the status cognitive/behavioral of functioning ognize, as typically “tests” are all that might be con- in relation to the demand of one’s medical condition sidered as psychological assessment or evaluation. or to changing developmental expectations is equally However, a central tenet in psychology is that test important to monitor. scores or results cannot be interpreted in isolation. Information from naturalistic settings must be sought Baseline assessment is the initiation of such a pro- through the methods of interview, observations, and cess. It most often has been understood as measuring informal assessments, as enumerated by Sattler. function at the outset of illness or injury against which to calibrate future change. Now this concept has been In a discussion of cognitive testing, the issues of expanded to include the characterization of a healthy single tests versus batteries is an important consider- child or teen prior to exposure to risk. Specifically, this ation. Single tests are designed to tap a specific dimen- paradigm defines the process of baseline assessment in sion of cognition, like verbal learning or visual–motor sports as regards the risk of concussion. Participation abilities. As useful as they are for more in-depth exami- in all sports has exploded in recent years in children nation of a single construct, this strength is a source of of all ages (20). With the use of baseline cognitive test- limitation as well. Seldom is the question at hand to be ing, the determination of a child’s or teen’s unique answered by examining a single ability. Abilities are not cognitive profile prior to a concussion are quanti- the unitary concepts that evolve from theoretic models. fied (21). In a much shorter time frame than the one The influence of other overarching cognitive abilities, implied previously for more serious illness or injury such as attention or processing speed, is not addressed processes, the degree of concussion and recovery are directly and is discernible only through observation. discerned by repeated testing post-concussion within Normative samples for single tests can be restricted and weeks or months. Cognitive assessment is generally not large enough or representative enough to draw firm regarded as essential in the diagnosis and monitor- conclusions as to standing within one’s peer group. ing of concussion, as delineated by the International Conference on Concussion in Sport held in Zurich in Therefore, the use of a test battery is preferred. The 2008. The increased vulnerability of the adolescent best-known example of a test battery is the Wechsler athlete relative to adults is well recognized as to dura- batteries for intelligence assessment, comprised of a tion of symptoms and differential recovery pattern. number of subtests. These collections cover an array of Furthermore, the effect of repeat concussion, treat- abilities. In neuropsychologic assessment, the concept ment options, school demands, restriction of exposure of a fixed battery versus a flexible battery exists. A fixed to risk (continued sports participation)—both during battery is a group of subtests developed to tap a spec- recovery and subsequently—and the potential effect trum of either a specific function—for instance, memory

28 Pediatric Rehabilitation or attention—or a comprehensive view of cognition. system, the same factors apply in understanding other The Wechsler memory scales and the NEPSY described medical disorders as well. She explains it as in the next section are examples of a battery for a spe- cific function, in the case of the memory scales, and a . . . biological risk associated with the medical condi- comprehensive assessment, in the case of the NEPSY. tion, moderated by the child’s development; by the time since onset of the condition; and by the reserve avail- Fixed batteries provide for the strongest basis for able within the child, family, school, and community. comparison of a patient’s performance across the sub- tests, as the norms are based on this arrangement of The interpretation of standardized tests must take tests, given in the established order to the normative these factors into account: issues about the course of group. Because all subjects receive the same subtests, a disease or injury recovery, the unique interface that there can be an expression of both strengths (what a the course of an illness or recovery has on the time- patient can do) and deficits. This is particularly use- table of childhood development, and the actual length ful in the construction of rehabilitation plans. A flex- of the struggle with the medical condition. Her inclu- ible battery is composed of a number of single tests, sion of the word “reserve” with which to respond and assembled with the patient’s referral question or cope dovetails with the requirement of assessment known medical condition in mind, with an eye to tap- that examines these factors as well. ping tests most likely to explicate suspected deficits. Some of these elements are captured in a good Lezak and colleagues (23) noted a survey of neu- history taking and/or record review. Reserve factors ropsychologists where 70% responded that they use a concerning coping and response are also gathered in flexible battery approach. They note the position that history but can additionally be tapped by standard- fixed batteries involve more testing than some patients ized questionnaires, whose responses are sought from need and can’t accommodate the practice of adding a variety of sources. These encompass figures from the tests either newly developed or needed to explicate a major settings in a child’s life (ie, parents and teach- deficit seen but in need of further examination. ers). The value of such instruments is that they can reference responses to those of a normative popula- Automated or computer use in testing has tion such that the degree of divergence from standard increased substantially since the 1980s. Prior to that, development can be expressed. Some include consis- automated and later computerized administration and tency scales that add information about the nature of scoring of tests was quite limited. Initially, comput- the responses given. erized testing of attention was developed (Gordon Diagnostic System, Connors Continuous Performance Culture-Sensitive Assessment Test). More recently, computerized tests have been developed for concussion diagnosis and monitoring Psychological assessments with culturally diverse chil- (as noted in the prior section), but also for research dren are challenging under any circumstances. Most purposes. Such techniques offer repeatability, sensi- measures have a culture bias in terms of content and tivity to subtle cognitive changes, and ease of admin- validity, and normative data are seldom adequately istration. Reliability, validity, and other considerations representative of diverse groups. Not all examiners are pertinent to general issues in more traditional so- sufficiently sensitive to the impact of cultural issues called pen-and-paper tests are pertinent to this type on test performance, and when interpreted without of assessment as well. caution, results can be misleading. The assessment of English language learners, children who have reduced Evaluation, then, is a robust and multifactorial mastery of the English language because their parents’ process, not to be confined to a set of test scores or primary language is not English, is particularly chal- descriptions of test performance, but also to include lenging. Use of interpreters or test translations carries natural setting data. The norm-referenced placement limitations, such as lack of equivalent concepts in the of a patient has a role, but the assessment setting in two languages, minimal provision for dialectical vari- and of itself imposes a high degree of structure. While ations, and possible changes in the level of difficulty this one-to-one administration is not replicated in real or meaning of translated words (15). life, it is necessary for the standardization of admin- istration and the reference to a normative sample, as Several “culture-fair” tests have been developed described previously. Therefore, the addition of per- to reduce culture bias by limiting the amount of ver- spectives from natural settings of the home, school, bal exchange, using more abstract content that is less and community are necessary, as is the consideration grounded in culture and language, and using more of the aspects of the medical condition. diverse groups during the norming process. This represents an important step in culturally sensitive Maureen Dennis (24) captured the interaction assessments, and some of these tests are discussed in of these factors in the following, which she calls an “outcome algorithm.” Though Dennis is specifically referring to disorders that affect the central nervous

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 29 following sections. However, there is no way to truly The Halstead Reitan Battery has been refined and eliminate cultural bias from tests, and demographic redefined over the years since Ward Halstead’s original data on normative groups must be carefully examined conceptualization in the 1940s to a larger series of tests before assuming that it is any more representative of to diagnose so-called brain damage for ages 14 and the specific patient than traditional tests. For example, above (25), and subsequently the downward extension many of the “culture-fair” tests are normed only on for ages 9–14, called the Halstead Neuropsychological children in the United States. Their use for students Test Battery (HRNB) for Older Children. It takes with different backgrounds, such as children from ref- approximately four to six hours to administer and uses ugee camps in Africa with little to no formal school- subtests from the adult Halstead Reitan Battery, with ing, is clearly limited. some modifications. The battery for children ages 5–8 is called the Reitan Neuropsychological Test Battery Culture-sensitive assessments in pediatric popu- and requires a similar time interval for administration. lations are made even more complicated by the fre- These batteries, in wide usage earlier, are criticized for quency of mild to severe motor impairment. Examiners a number of pivotal problems. The first is on concep- assessing individuals with motoric impairment rely tual grounds, in that the battery was not developed for heavily on tests of verbal cognitive skills and try children, but for adults, and is perhaps reflected in the to reduce the number of tasks that require speeded minimal assessment of memory, academics, and lan- or complex motor responses. Examiners assessing guage, with no direct measure of attention. The psycho- individuals from linguistically or culturally diverse metric properties are widely acknowledged to be quite backgrounds rely heavily on tests of nonverbal cog- poor, such that reliance on those alone for interpreta- nitive skills and try to reduce the verbal component. tion is inappropriate. Considerable clinical acumen is Examiners assessing individuals from linguistically required to interpret findings. Dean concludes a review diverse backgrounds with motor impairments are of the batteries saying, “The HRNB cannot be recom- limited indeed in terms of valid options. Even in mended for general clinical use without considerable pediatric groups that do not have motor impairment, training and familiarity with research on the battery the higher frequency of discrepancies in functioning (26).” Considering norms published in the interim, (significant strengths and weaknesses in a single Lezak et al. (23) is more favorable to the HRNB in say- individual, such as may be caused by damage to right ing that what statistics it yields are misappropriated by versus left hemisphere or cortical versus subcorti- “naïve clinicians,” implying the same point as Dean. cal areas) makes the traditional practice of assessing nonverbal skills and considering the results repre- The only neuropsychological battery ever developed sentative of general functioning highly questionable. specifically for children is the NEPSY-Developmental School and community-based clinicians may not be Neuropsychological Assessment (27), with the new- aware of the complexity of issues involved and may est version, the NEPSY-II (28), published in 2007. Both provide scores without adequate caution regarding batteries are based on the diagnostic principles of the limitations. Russian neuropsychologist Alexandr Luria. The origi- nal NEPSY had two forms and covered ages 3–4 and SPECIFIC INSTRUMENTS 5–12, with a core battery of 11 to 14 subtests repre- sented to tap five functional domains: attention and Neuropsychological Evaluation executive functions, sensorimotor functions, language, visuospatial processing, and memory and learning Originally, the neuropsychological assessment was functions. This original version was criticized for its directed at diagnosing the presence, nature, and site content and psychometric properties (29). It is well of brain dysfunction. The focus has shifted from diag- standardized, and though some instability is noted in nosis to assessment of a child’s function to identify some subtests, this may indeed reflect the reality of and implement effective management, rehabilitation, the developmental status of the brain. or remediation services. The most recent version has not been tested Neuropsychological Batteries enough to generate a literature on its strong points or weaknesses. It does expand the age range to 16 years, As mentioned earlier, neuropsychological batteries extending one ostensible benefit of a battery that cov- have been developed to provide a comprehensive eval- ers the childhood range, allowing for the ideal serial uation of cognitive abilities. The two most common assessment. The content has also changed, with tar- in practice today are the downward extensions of the geted groupings of subtests for various diagnoses, Halstead Reitan Neuropsychological Battery and the nonverbal elements, and new measures of executive NEPSY-II, developed specifically for children. functioning, memory and learning, which reportedly solves some of its statistical problems. A functional domain in social perception has been added as well.

30 Pediatric Rehabilitation Attention, Concentration, and The effects of anxiety about an illness process, its treat- Information Processing ment, and demands for coping can all affect attention, and in a competent diagnosis are differentiated from The processes of attention, concentration, and infor- primary brain disruption. mation processing are often central concerns for any patient with a medical condition involving the Because of the issue of time in competent atten- brain (30). In many ways, they form the basis on tion processes, computerized testing has real utility to which the other component processes occur. Overall control for calibration of presentation and response. cognitive productivity suffers from losses or failures to Absent a fully computerized administration, the use of develop these skills. taped auditory stimulus in attention testing allows for standardized presentation increments. Typically, the Attention has been conceptualized in a number computerized tasks involve visual stimulus and the of ways, generally relating to an organism’s recep- taped presentations involve auditory ones. This differ- tivity to incoming stimuli. Most do regard the issues entiation between verbal and nonverbal, or auditory of automatic attention processes versus deliberate/ versus visual, is necessary to capture these two cen- voluntary as central dimensions. Other characteristics tral aspects of stimulus processing. include sustained, purposeful focus—often referred to as concentration—and the ability to shift atten- Recent development of a battery of attention tasks tion as required by a stimulus. Being able to ward off for children, the Test of Everyday Attention-Children distractions is usually seen as part of concentration will be described next. It attempts to cover a number (31). Vigilance is conceptualized as maintaining atten- of aspects of attention processes and for the compar- tion on an activity for a period of time. There are the ison of subtest scores to allow for relative differentia- needs to respond to more than one aspect of a stim- tion of components. ulus or competing stimulus—the capacity to divide attention—alternating with shifts in focus. Inattention, slowness, and poor concentration have a wide-ranging effect on competent cognitive and The multitude of processes subsumed in the concept adaptive functioning. Other processes may be quite of attention are necessary because of the overall effect. competent, but attention and its aspects can be a pri- Most notable is the developmental nature of attention mary “rate limiting” factor. These should be addressed in childhood and adolescence. Increasing demands in in even a screening of functioning, whether at bed- school participation are seen in the shifting requirements side or in the clinic, both as an overall indicator of throughout the academic process. In the early grades, a current cognitive activity, but also as a harbinger for child is more directly engaged by the teacher, but as the developmental problems to come, signaling the need years progress, the capacity for independent (ie, volun- for more stringent monitoring. Commonly used tests tary/deliberate) processes grows. Attentional processes are described in Table 3.1. are a central aspect of the changing capacity of normal development. Attention’s vulnerability to normal varia- Problem-Solving and Executive Functioning Tests tion, as with fatigue or anxiety, is a part of typical func- tioning. Attentional processes require a certain “tone” to As with attention processes, deficits in these realms the brain’s functioning, attention and its concomitants can have a devastating effect on overall functioning. are often affected in brain disorders. Furthermore, with Cognitive process can be intact, but with executive acquired deficits in the disordered brain, the demands functioning impairments, the output can be substan- are higher, as an individual struggles with recognizing tially derailed. The basic tasks of life can suffer, along the need to attend along with implementing a specific with the ever-present demand in childhood to acquire compensatory task. new skills. These deficits can be more obscured in children than in adults, as there is a natural sup- Lezak and colleagues (23) note that underlying port of activity by parents or other family members. many attention problems is slowed processing. This Return to school can be the point at which executive can be misinterpreted as a memory disorder (32), as functioning problems can clearly be seen for the first competing stimuli in normal activity interrupt the time since an acquired illness or injury. Traumatic processing of the immediately preceding stimuli and brain injury presents a particular vulnerability to something is “forgotten,” in common parlance. The deficit in these skills. Executive functions are associ- discernment of this specific problem is important, as ated with the frontal and prefrontal areas of the brain, strategies alleviating the effects of slowed processing where, due to the mechanisms of closed head injury would be different from those for memory per se. and the shape of the brain and skull convexities, dam- age can be focused across the full range of severity. All of these aspects warrant examination, notably Rehabilitation efforts suffer, both in commitment to in those with a brain disorder, due to the overall effect the process and in learning strategies to compensate on functioning and the demand for acquisition of aca- for deficits (39). demic and adaptive behaviors throughout childhood.

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 31 3.1 Tests of Attention and Speed of Processing INSTRUMENT (REF.) DESCRIPTION COMMENTS Test of Everyday Attention Batteries of 8 or 9 tasks for ages 17 and Taps visual/auditory attention including dual Test of Everyday Attention-Children above; TEA-CH ages 6–16 tasks; selective, sustained and executive (TEA-Ch) (33) control Normed for ages 6–16 years; includes Gordon Diagnostic System (GDS) (34) 3 tasks: delay, vigilance, distractibility; has Freestanding and portable electronic preschool version for ages 4–5 yrs devices used for administration and calculation of summary data; parallel Paced Auditory Serial Addition Test (PASAT) Adding pairs of digits presented at 4 rates version for vigilance task; relies solely on Children’s Paced Auditory Serial Addition of speed, controlled by the audiotape visual task Test (CHIPASAT) (35) presentations; adult and child forms; ages 8 Continuous Performance Tests (36) and above Highly sensitive to deficits in processing speed; sensitive to mild disruption, but can Symbol Digit Modalities (SDMT) (37) Covers a category of tests; visual or auditory be stressful test to take, as many items can stimulus where must respond to a target be missed at normal ranges Trail-Making Test (TMT) (38) stimulus in the presence of distractors; various versions for ages 4 and up Many versions exist; sustained, vigilance and inhibition tapped; Connors Continuous Oral or written; requires visual scanning and Performance Test II and Test of Attention tracking to match preset symbol and number are well known. pairs Taps information processing; Spanish Subject draws lines to connect consecutively version with norms; seen as selectively numbered (Part A) and alternating numbers useful. and letters in order (Part B). Ages 9 and up Part of Halstead-Reitan battery; test of speed, visual search, attention, mental flexibility, and fine motor; needs interpretation with other tests; Part B is most sensitive The competent measurement of these skills Lezak (23) differentiates these brain skills as requires a multidimensional approach and is quite complex (40). Testing of these functions imposes “Questions about executive functions [are generally a degree of structure required by standardization phrased \\as how or whether a person goes about doing such that vital elements can be obscured. Attempts something (eg, Will you do it and, if so, how and when?); at quantification in real-life situations becomes par- questions about cognitive functions are generally ticularly important. Questionnaires for parents and phrased in terms of what or how much (eg, How much teachers elicit descriptions of behavior that can be do you know? What can you do?).” compared to normative expectations. Particularly for parents, this can be useful in understanding the There are many models, as in attention, as to what need for treatment. Teachers have a normal sam- comprises these skills and how to measure the com- ple of age-appropriate peers in the classroom and ponents, since it is far from a unitary concept. Again, can be more aware of such problems. In that cir- as in attention, the developmental progress of these cumstance, the questionnaire process can illumi- skills is a central aspect of childhood and adolescence. nate the component elements to be addressed, as a In the teenage patient, assessment of these skills is deficit in classroom performance can be composed vital, as adult-like capabilities for work, driving, and of many factors, with differing contributions to the independence can be severely affected and, in the par- overall presentation. The Behavior Rating Inventory ticular case of driving, have disastrous results. The of Executive Functions (BRIEF), described in the sec- enactment of graduated driver license requirements tion on “Psychosocial Evaluation,” is an instrument for teen driving in some states implies the central- focused on these behaviors. It covers the preschool ity of these skills and their necessity for that activity. period through adolescence, as well as a self-report Stepwise exposure and supervision of driving for teens questionnaire for older children, with basic forms for allows for a graduated experience before full driving teachers and parents to complete. privileges are granted. Specialized assessment through

32 Pediatric Rehabilitation rehabilitation-based driving evaluations using com- in the questionnaire approach discussed previously. puter simulation should be considered by a rehabilita- The effect of impairment in these skills can be wide- tion team in any teen with a history of brain disorder. spread and debilitating, especially as expectations for empathy and self-awareness increase in adolescence. Definitions of executive skills include the capac- ity for planning and flexible use of strategies, and the One of the questionnaires does differentiate these ability to generate, maintain, and shift cognitive sets; two factors. In the BRIEF (131), questions about such to use organized search strategies; and to use self- skills yield feedback for the behavioral regulation monitoring and self-correction, as well as the capacity composite, as differentiated from another composite to utilize working memory. It is distinct from general reflecting the cognitive aspect, metacognition. A list intelligence, though it does correlate at lower levels of tests that cover this wide-reaching domain is listed of intelligence. Again, as in attention skills, these in Table 3.2. skills are vulnerable and easily disrupted in many circumstances, as they are largely acquired through- Nonverbal/Visual–Perceptual out childhood as an essential central process of com- Function Tests petent development. Therefore, deficits acquired can be “silent” until they are called on for future devel- This type of cognitive task is seen as one of the two opment. The range of tasks is wide, from inhibiting major classes of cognitive input/output. At its base is behavior in the absence of visible authority to plan- the perceptual capacity of vision. From earliest infancy, ning how to accomplish several assignments due at humans already have sufficient visual perception to the same time. mimic another’s facial expression (29). Related to the developmental aspects of childhood, by age 9, visual Though the cognitive aspects are difficult to quan- processes are integrated with tactile and proprioceptive tify, the literature on these is substantial. However, the functions. Tests of visuoperceptual, visuospatial, and emotional and behavioral aspects of executive skills is visuomotor function are all within this domain and less studied (41). Executive skills act to regulate behav- include the ability to discriminate between objects, ior (42), inhibit and manage emotions, tolerate frustra- distinguish between left and right, judge spatial ori- tion, and provide persistence. Notable is the result of entation and the relationship among objects in space, limited empathy (ie, taking the position of the other). copy a model, understand symbolic representations They are observed collaterally in any sound testing of maps and routes, and solve nonverbal problems. process, but are captured better, to the extent possible, 3.2 Tests of Problem Solving and Executive Function INSTRUMENT (REF.) DESCRIPTION COMMENTS Halstead Category (HCT) (43) Versions exist for ages 5–9 and 9–14, as Machine and booklet forms; measures well as through adulthood; part of Halstead- conceptualization and abstraction abilities Wisconsin Sorting Test (WCST) (44) Reitan battery Tower of Hanoi (TOH) (45) Requires inference of correct sorting Tower of London (TOL) (46) Revised manual offers norms for ages 6.5 strategies and flexible use Stroop Color-Word Test (47) and above. Taps working memory, planning, rule use Matching Familiar Figures (MFFT) (48) and behavioral inhibition Fluency Tasks Verbal and Design Computer and standard administration; Taps inhibition, working memory, Delis-Kaplan Executive Function System ages 4 and up anticipatory planning (D-KEFS) (49) Test of inhibition, selective attention, and Arrange balls on pegs to match picture; switching sets norms for 7 and up Measures impulsivity Well-known test, quick administration in Taps self-monitoring, initiating, and shifting; paper form; several versions included in many batteries Battery aids comparison of subtest scores Must find identical match for stimulus picture; ages 6 and up Speeded tasks of response generating to verbal and nonverbal stimuli Battery of 6 subtests; age 8 and above

Chapter 3 Psychological Assessment in Pediatric Rehabilitation 33 Direct functional outputs include being able to navi- as distinct from language per se. One element can be gate the environment and depth perception. Testing of intact, while the other has significant impairment, and these functions can illuminate visual field cuts, visual evaluation distinguishes between the two. neglect, and apraxia. An acute injury most notably, but even an illness, However, these tasks often involve other aspects of may produce a frank aphasia, but this often resolves cognition, such as attention, memory, speed of think- from its most dramatic state. Deficits remain that are ing, and motor impairment. At the base is the requisite more subtle, but important to address. Common defi- of normal visual acuity, screened for in the pediatric cits after injury differ between an adult and a child. In clinic setting or in school admission testing. However, childhood and adolescence, deficits in word finding, after a brain injury or illness, the intactness of the dyscalculia, and problems with formulating written basic perceptual components of nonverbal function- language are common (29). Functional problems, like ing should not be assumed. Issues like cortical visual difficulty with instructions or following commands, defects or loss of binocular vision require examination require analysis to determine the degree of linguis- by vision specialists; in more severe TBIs, such injuries tic difficulty versus other factors, such as attention or are not uncommon. Consideration of the myriad factors memory. As is always the case, the determination of involved in this domain’s assessment requires examin- concurrent difficulty in other tasks provides the diag- ing other test performances to discern patterns, as well nostic information to hone in on the core problem(s). as factors that may affect visual function output but not be a deficit in these processes per se. The importance Components like phonologic processing, nam- of these functions as a basic component of input and ing, language comprehension (oral and written), and output of cognitive function is seen in their presence in understanding the syntactic structure of language, as all intelligence and neuropsychological batteries. Table well as the productive aspects of language, should be 3.3 provides a representative listing of these tests. covered. The profound impact of language deficits on academics makes it a particularly important aspect for Language Functioning Tests assessment. Problems with reading comprehension are often seen, and because reading is one of the pri- To examine this function is to assess what is felt to mary tools for learning once a child enters elementary be a central aspect of brain functioning and the foun- school, the impact can be widespread on competent dation of human thought. Language development is school functioning. well delineated, and proceeds from spoken to writ- ten competency in normal childhood. Receptive and There are many well-accepted tests to measure expressive abilities require separate assessment, as all these aspects. For receptive language, the Peabody do the modalities of written language. Speech as the Picture Vocabulary Test-III (54), Token Test (55), or the mechanical aspect of oral communication is assessed Bracken Basic Concept Scale-Revised (56) are widely used. These vary from single-word comprehension to grammatical/syntactical structure to linguistic 3.3 Tests of Nonverbal/Visual–Perceptual Function INSTRUMENT (REF.) DESCRIPTION COMMENTS Rey-Osterreith Complex Figure Test Copying of complex figure and a delayed Taps planning, visual organization, and (ROCF) (50) recall condition; alternate forms exist for memory for complex visual information repeat administration; ages 6 and up Beery Developmental Test of Visual-Motor Long-standing test with new additions; Integration. 4th ed. (VMI) (51) Design-copying test of 24 forms of visuoperceptual, visuomotor integration is progressive difficulty; supplemental tests of assessed Facial Recognition Tests (52) visual perception and motor coordination; Ages 3+; procedures for younger children Present in standalone tests, but also Wide Range Assessment Battery of in batteries, including memory aspect; Visual Motor Ability (WRAVMA) (53) Requires direct matching and side profiles of implications of right hemisphere function photos of human faces; norms for ages 6+ Sound psychometrics yield Battery of 3 tests: drawing, matching, and standard scores and percentile for each pegboard. Can be administered individually subtest or as a battery yielding a composite; ages 3–16.