Pediatric Neurology: A Case-Based Review Tena Rosser, MD Children’s Hospital Los Angeles University of Southern California Keck School of Medicine Los Angeles, California Acquisitions Editor: Frances DeStefano Managing Editor: Scott Scheidt Developmental Editor: Lisa Consoli Project Manager: Fran Gunning Marketing Manager: Kimberly Schonberger Design Coordinator: Stephen Druding Manufacturing Coordinator: Kathleen Brown Compositor: International Typesetting and Composition Printer: Edwards Brothers Copyright © 2007 Lippincott Williams & Wilkins 530 Walnut Street Philadelphia, PA 19106 All rights reserved. This book is protected by copyright. No part of this book may be reproduced in any form or by any means, including photocopying, or utilized by any information storage and retrieval system with- out written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews. Materials appearing in this book prepared by individuals as part of their official duties as U.S. government employees are not covered by the above-mentioned copyright. Printed in the USA Library of Congress Cataloging-in-Publication Data Pediatric neurology : a case-based review / [edited by] Tena Rosser ; contributing authors, Arthur Partikian . [et al.]. p. ; cm. Includes bibliographical references and index. ISBN-13: 978-0-7817-7888-6 ISBN-10: 0-7817-7888-3 1. Pediatric neurology. 2. Pediatric neurology––Case studies. I. Rosser, Tena. II. Partikian, Arthur. [DNLM: 1. Nervous System Diseases––Case Reports. 2. Nervous System Diseases––Examination Questions. 3. Child. 4. Infant. WS 18.2 P36997 2007] RJ486.P433 2007 618.92'8––dc22 2006011452 Care has been taken to confirm the accuracy of the information presented and to describe generally ac- cepted practices. 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Lippincott Williams & Wilkins customer service representatives are available from 8:30 am to 6 pm, EST. 06 07 08 09 10 3 4 5 6 7 8 9 10 CONTRIBUTING AUTHORS Children’s Hospital Los Angeles University of Southern California Keck School of Medicine Los Angeles, California Arthur Partikian, MD Tena Rosser, MD Kiarash Sadrieh, MD Children’s National Medical School George Washington University School of Medicine Washington, District of Columbia Taeun Chang, MD John Crawford, MD Tammy Tsuchida, MD, PhD Adeline Vanderver, MD iii To my mentors and colleagues in the neurology department at Children’s National Medical Center, Washington, DC. To my family. v CONTENTS Contributing Authors iii Dedication v Preface ix Abbreviations xi Introduction xiii Case 1 . 1 Case 31 . 159 Case 2 . 5 Case 32 . 167 Case 3 . 11 Case 33 . 173 Case 4 . 17 Case 34 . 177 Case 5 . 23 Case 35 . 183 Case 6 . 31 Case 36 . 189 Case 7 . 35 Case 37 . 193 Case 8 . 41 Case 38 . 197 Case 9 . 45 Case 39 . 201 Case 10 . 49 Case 40 . 205 Case 11 . 53 Case 41 . 211 Case 12 . 59 Case 42 . 217 Case 13 . 65 Case 43 . 223 Case 14 . 69 Case 44 . 229 Case 15 . 75 Case 45 . 233 Case 16 . 81 Case 46 . 239 Case 17 . 87 Case 47 . 247 Case 18 . 93 Case 48 . 253 Case 19 . 97 Case 49 . 257 Case 20 . 103 Case 50 . 263 Case 21 . 111 Case 51 . 269 Case 22 . 117 Case 52 . 273 Case 23 . 123 Case 53 . 277 Case 24 . 127 Case 54 . 283 Case 25 . 133 Case 55 . 287 Case 26 . 137 Case 56 . 293 Case 27 . 141 Case 57 . 299 Case 28 . 145 Case 58 . 305 Case 29 . 149 Case 59 . 311 Case 30 . 153 Case 60 . 317 Index by Category of Disease 323 Index 325 vii PREFACE The idea for the development of this book arose while preparing for the oral portion of the American Board of Psychiatry and Neurology board examination. I quickly came to realize that there was no current published collection of cases in pediatric neurology that might serve as a study aid. Although this book will likely appeal most to adult and pediatric neurology residents preparing for the oral board examination, it is also intended to be of interest to a broader audience of practic- ing adult and child neurologists, general pediatricians, and medical students. Although it is difficult to recreate the oral board examination experience on paper, this book is written as a case-based review with discussions structured according to the format used in the neurology oral boards. Both common and rare neurologic disorders are covered in the text, but the majority of cases are based on actual patient presentations. The cases demonstrate the diverse and fascinating dis- ease processes found in the field of child neurology. The vignettes in this book are presented randomly and not by disease category to simulate the experience of the oral boards. However, for individuals who wish to perform more focused study on a particular area of child neurology, an index by disease category is provided in the back of the book. Overall, this book is intended to help readers gain a foundation of knowledge in pediatric neurology and develop an organized approach to clinical decision making. ix ABBREVIATIONS ACTH Adrenocorticotropic hormone ANA Antinuclear antibody BAER Brainstem auditory evoked response BUN Blood urea nitrogen CBC Complete blood count CIDP Chronic inflammatory demyelinating polyneuropathy CK Creatine kinase cm Centimeter CMAP Compound muscle action potential CMV Cytomegalovirus CNS Central nervous system CSF Cerebrospinal fluid CT Computed tomography DNA Deoxyribonucleic acid DWI Diffusion-weighted imaging EBV Epstein-Barr virus ECG Electrocardiogram ECHO Echocardiogram EEG Electroencephalogram EMG Electromyography ER Emergency room ESR Erythrocyte sedimentation rate FDA Food and Drug Administration kg Kilogram HIV Human immunodeficiency virus HMA Homovanillic acid HSV Herpes simplex virus HTLV-1 Human T-cell lymphotropic virus type 1 ICP Intracranial pressure ICU Intensive care unit IQ Intelligence quotient IV Intravenous xi xii ABBREVIATIONS PEDIATRIC NEUROLOGY: A CASE-BASED REVIEW IVIG Intravenous immunoglobulin LDL Low-density lipoprotein MELAS Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes mg Milligram mm Millimeter MRA Magnetic resonance angiography MRI Magnetic resonance imaging MRV Magnetic resonance venography NCS Nerve conduction study NCV Nerve conduction velocity NICU Neonatal intensive care unit NMDA N-methyl-D-aspartate NPO Nothing per os (mouth) PCR Polymerase chain reaction PT Prothrombin time PTT Partial thromboplastin time RPR Rapid plasmin reagin SPECT Single-photon emission computed tomography SSEP Somatosensory-evoked potential SSPE Subacute sclerosing panencephalitis TORCH Toxoplasmosis, other, rubella, CMV, HIV, HSV infections TSH Thyroid-stimulating hormone VDRL Venereal Disease Research Laboratory VER Visual-evoked response VLDL Very-low-density lipoprotein VMA Vanillylmandelic acid INTRODUCTION Preparing for the oral neurology boards is a challenging task. It is important to understand from the outset that the purpose of this test is to evaluate the candi- date’s thinking process and to make sure that the candidate is a safe physician with good clinical judgment. Of course, this requires a basic fund of knowledge and a certain level of experience. Adequate preparation, which involves under- standing what will be asked of you during the test and being familiar with a broad range of neurologic diseases in both adults and children, can significantly ease the stress of the process. Preparation for such an important examination does not happen overnight. It will likely be insufficient to read a child neurology textbook several weeks before the test. You will need time to familiarize yourself with all aspects of this field and recall the cases in pediatric neurology that you encountered during your training. Thus, begin studying for the exam as early as possible. Practice is another important element of preparation. During the exam, whether with a live patient or with vignettes, you are asked to review and syn- thesize information in an organized fashion in a limited amount of time. Although residency may prepare you for this to some degree, the oral boards demand a certain amount of succinctness that may not come naturally to everyone. Developing the clinical skills and thought processes to perform a physical exam or discuss a case in a timely fashion requires practice. Try to take a history and perform a physical examination in 30 minutes on patients whom you currently see. Also, reading through vignettes alone or with colleagues as often as possible before the exam definitely will make the task easier when you are asked to do it in front of examiners. THE EXAMINATION The oral neurology boards consist of three 1-hour-long parts.