Neurodevelopmental Disorders: Diagnosis and Treatment Randi Jenssen Hagerman Oxford University Press Neurodevelopmental Disorders DEVELOPMENTAL PERSPECTIVES IN PSYCHIATRY Series Editor Jarnes C. Harris, M.D., Johns Hopkins University Tuberous Sclerosis Complex, Third Edition Manual Gomez, M.D., Julian Sampson, M.D., and Vicky Whittemore, PH.D. Neurodevelopmental Disorders: Diagnosis and Treatment Randi Jenssen Hagerman, M.D. Neurodevelopmental Disorders Diagnosis and Treatment Randi Jenssen Hagerman New York Oxford • Oxford University Press 1999 Oxford University Press Oxford New York Athens Auckland Bangkok Bogota Buenos Aires Calcutta Cape Town Chennai Dar es Salaam Delhi Florence Hong Kong Istanbul Karachi Kuala Lumpur Madrid Melbourne Mexico City Mumbai Nairobi Paris Sao Paulo Singapore Taipei Tokyo Toronto Warsaw and associated companies in Berlin Ibadan Copyright © 1999 by Oxford University Press, Inc. Published by Oxford University Press, Inc. 198 Madison Avenue, New York, New York 10016 Oxford is a registered trademark of Oxford University Press All rights reserved. No part of this publication 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 permission of Oxford University Press. Library of Congress Cataloging-in-Publication Data Hagerman, Randi Jenssen, 1949- Neurodevelopmental disorders : diagnosis and treatment / Randi Jenssen Hagerman. p. cm.—(Developmental perspectives in psychiatry) Includes bibliographical references and index. ISBN 0-19-512314-X 1. Developmental disabilities. 2. Pediatric neuropsychology. 3. Developmental neurobiology 4. Cognitive neuroscience. I. Title. II. Series. [DNLM: 1. Mental Retardation. 2. Developmental Disabilities. WM 300 H144n 1999] RJ506.D47H34 1999 618.92'8-dc21 98-54687 DNLM/DLC for Library of Congress 9 8 7 6 5 43 Printed in the United States of America on acid-free paper This book is dedicated to my husband, Paul, my daughters, Karin and Hillary, and my mother, Helen Kalantar. Their tolerance, support, love, and sense of adventure have made all things possible. This page intentionally left blank PREFACE This book includes nine chapters that review selected syndromes that I have seen in my clinical and research experience over the last 20 years. Some of these syndromes are common, such as fetal alcohol syndrome and Tourette syndrome, and others are rare, such as Smith-Magenis syndrome. Each of these disorders has an interesting cognitive and behavioral phenotype that necessitates a unique treatment approach, and yet common themes are seen in almost all of the chapters, such as the treatment for hyperactivity or attention deficits. The commonalities and differences in each of these disorders have been fascinating to me and, I hope, to the reader of this book. We can learn treatment approaches to one disorder that may be beneficial to several others. I have had the good fortune to work with an outstanding clinical team of professionals who have taught me about a global approach to treatment in which interventions can work synergistically to push for the most optimal outcome possible. We have also worked closely with the schools to utilize medical interven- tion to enhance educational endeavors. The separation of medical and educational professionals is institutional in our society, but we can learn so much from each other. This book highlights a global approach to treatment that includes medications to improve cognitive and behavior problems, language and motor therapy, educational interventions, vocational approaches, and computer technol- ogy to enhance the functioning of children and adults with these syndromes. A wide variety of professionals in the medical and educational field should find this book useful, and parents who are interested in more detailed information about their child's syndrome will also benefit from reading this book. I find the fast-moving field of genetics fascinating, and I have tried to document the latest molecular findings in each of these disorders. Although the information is somewhat technical at times, the benefits of understanding the underlying molecular mechanisms for specific behavioral or cognitive phenotypes are worth- viii PREFACE while from an intellectual viewpoint and from a treatment perspective now and in the future of molecular interventions. Our clinical team is best known for our research and writings regarding fragile X syndrome, but what we have learned from this disorder we have applied to the other syndromes discussed here. Much of the treatment information for these disorders is anecdotal, with the exception of Tourette syndrome, for which con- trolled studies of psychopharmacological interventions exist. Hopefully our efforts in compiling treatment information will stimulate further research and controlled trials of a variety of interventions. This book was written because so much good information passes my way on a daily basis that I could not keep track of it, or even remember it, unless I wrote it down in a cognitive framework for myself and for my students and team members. I began this book during my sabbatical in Australia, and Danuta Loesch of LaTrobe University in Melbourne and Bogden Mdzewski provided the aca- demic environment, intellectual stimulation, and gracious hospitality of their home which made this writing possible. I am deeply grateful to them. Our clinical and research team provides a daily learning environment for me, and the innovative ideas of team members can be found throughout this book. I am very thankful to Tracy Kovach, Kristen Gray, Cathy Bodine, and Lisa Noble for all they have taught me regarding computers and assistive technology; to Marcia Braden, Rebecca O'Connor, Jennifer Hills, Nancy Mann, Craig Knip- penberg, and Bill Sobesky, for their expertise in educational and psychological interventions; to Tracy Stackhouse, Clare Summers, and Lucy Miller for their expertise in sensory integration occupational therapy; to Sarah Scharfenaker for her expertise in speech and language interventions; and to Louise Cane for her expertise in genetic counseling. The physicians who work with me, including Edward Goldson, Sheila Tunnell, and Pamela McKenzie, have been a constant source of support and feedback for new ideas and issues. Most important, our students, residents, and fellows, some of whom are supported by the MCH Training Grant no. MCJ089413, have provided great support, intellectual stimulation, and review of the information in this book. These individuals include Karen Riley, Dick Taylor, Sheila Tunnell, Jason Coe, JeffSidders, Matt Holden, Susan Endres, Brenda Sanger, Wade Heinrichs, Rhoda Amaria, Aaron Jodeh, Kathryn Farrow and Kurt Weaver. Kurt did an exceptional job of compiling the appendices; he was funded by the William Rosen Fund of the National Fragile X Foundation. The support staff of the Child Development Unit at The Children's Hospital, including Barbara Wheeler, Mela Barrios, and Jacque Woods, have been invalu- able to me and provided the time and opportunity to complete this book. I am also deeply grateful to the patients and families who have taught me about their children. Many parents have reviewed these chapters and provided valuable insight and they are acknowledged at the end of each chapter. Permission was obtained from each patient and parent for use of the pictures included in this book. Each chapter was reviewed by experts in each field, and I am thankful for the time of these gracious professionals, including Mary Linden, Edward Riley, Sarah Mattson, Heather Carmichael Olson, William McMahon, Bruce Bender, PREFACE ix Arthur Robinson, Jim Salbenblatt, Michele Mazzocco, Ron Rosenfeld, Merlin Butler, Suzanne Cassidy, Joseph Wagstaff, Janet Thomas, David Manchester, Paul Wang, Ursula Bellugi, Ann C. M. Smith, and A. J. Allen. Their critiques improved this book immensely. Most important, this book was possible because of the dedicated and intense effort of two women, Susan Wood and Megan Lampe, who each spent 6 months of almost full-time effort to complete this book. Their creativity in Internet and library searches, their word processing expertise, and their knowledge of English pulled this book together. If I didn't have a family, I would dedicate this book to Susan and Megan. Their friendship and support have been wonderful. Finally, financial support for both clinical work and research has been essen- tial. Louise Gane and Sandy Morrison have done a tremendous amount to organize both foundation support and private family support, and I am greatly indebted to them.I thank the Kenneth Kendal King Foundation, the Denver Foundation, the Comprecare Foundation, the Wallace Genetic Foundation, and the Janssen Research Foundation for their support of our clinical work and research. I am very thankful for the efforts of Felix de la Cruz, National Institute of Child Health and Development, the National Fragile X Foundation, and the FRAXA Foundation for promoting fragile X research both at our center and elsewhere. NICHD grant no. HD36071 supports some of the fragile X research reported here. I am greatly indebted to the support of families who have donated money and carried out fund raisers to support both clinical work and research regarding fragile X syndrome at our center. Jim and Vicky Mulvey, Margaret Israel, Eric and Elissa Boory, Shannon Geis, Pam and James Vershbow, and Lori and Chris Beesley stand out remarkably in this regard, and I am deeply grateful. I continue to learn on a daily basis from the families and patients that I evaluate and treat. I hope this book gives these well-deserving families something in return. Denver, Colorado R. J. H. February 1999