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Fundamentals of Neurologic Disease This page intentionally left blank i-xii.Davis.fm 3/2/05 3:18 PM Page i Fundamentals of Neurologic Disease Larry E. Davis, M.D. With Molly K. King, M.D. Jessica L. Schultz, M.D. Neurology Service New Mexico VA Health Care System Albuquerque, New Mexico and Departments of Neurology and Neuroscience School of Medicine University of New Mexico Albuquerque, New Mexico i-xii.Davis.fm 3/2/05 3:18 PM Page ii Demos Medical Publishing, Inc., 386 Park Avenue South, New York, New York 10016 © 2005 by Demos Medical Publishing, Inc. 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. Illustrations by Yvonne Wylie Walston, CMI © Creative Imagery, Inc. Library of Congress Cataloging-in-Publication Data Davis, Larry E. (Larry Ernest) Fundamentals of neurologic disease : an introductory text / Larry E. Davis, with Molly K. King, Jessica L. Schultz ; illustrations by Yvonne Wylie Walston. p. cm. Includes bibliographical references and index. ISBN 1-888799-84-6 (pbk. : alk. paper) 1. Neurology. 2. Nervous system—Diseases. [DNLM: 1. Nervous System Diseases. WL 300 D262f 2005] I. King, Molly K., 1962- II. Schultz, Jessica L., 1974- III. Title. RC346.D385 2005 616.8—dc22 2004030159 i-xii.Davis.fm 3/2/05 3:18 PM Page iii Contents 1 Approach to the patient with a neurologic problem: Key steps in neurological diagnosis and treatment 1 2 Neurologic examination 9 3 Common neurologic tests 23 4 Disorders of muscle 39 Duchenne muscular dystrophy (Muscular dystrophies) 40 Dermatomyositis (Inflammatory myopathy) 44 Primary hyperkalemic periodic paralysis (channelopathies) 45 5 Disorders of the neuromuscular junction 49 Myasthenia gravis 49 Botulism 53 6 Disorders of peripheral nerves 57 Diabetic distal symmetrical polyneuropathy 58 Carpal tunnel syndrome 61 Bell’s palsy 63 7 Disorders of the spinal cord and vertebral bodies 67 Amyotrophic lateral sclerosis 68 Transverse myelitis and myelopathy 73 Low back pain with radiculopathy 74 iii i-xii.Davis.fm 3/2/05 3:18 PM Page iv iv CONTENTS 8 Disorders of the brainstem and cerebellum 79 Lateral medullary infarction (Wallenberg syndrome) 81 Spinocerebellar ataxia (SCA 1) 84 9 Disorders of the cerebrovascular system 87 Ischemic strokes (embolic and lacunar) 87 Transient ischemic attacks 93 Hemorrhagic strokes 94 Spontaneous intracranial hemorrhage 94 Saccular aneurysms 96 10 Disorders of myelin 101 Myelin 101 Multiple sclerosis 102 Guillain-Barré syndrome 105 11 Disorders of higher cortical function 109 Prefrontal lobe 110 Limbic system 110 Parietal lobe 111 Aphasias 113 Intelligence 115 Neurologic changes of normal aging 115 Dementia 116 Alzheimer’s disease 117 Mental retardation 120 12 Disorders of the extrapyramidal system 123 Essential tremor 124 Parkinson’s disease 126 Huntington’s disease 130 13 Central nervous system infections 133 Bacterial meningitis 134 Brain abscess 137 Herpes simplex virus encephalitis 139 Prion diseases 141 i-xii.Davis.fm 3/2/05 3:18 PM Page v CONTENTS v 14 Brain tumors 145 Brain herniation syndromes 145 Cerebral edema 146 Glioblastoma multiforme — malignant astrocytoma 147 Meningioma 149 Pituitary adenoma 150 Cerebral metastases 152 15 Seizures and status epilepticus 155 Primarily generalized tonic-clonic seizures and secondarily generalized partial seizures (Grand mal seizure) 156 Absence seizure (Petit mal seizure) 158 Infantile spasms (West’s syndrome) 159 Complex partial seizure (Localization-related, temporal lobe, or psychomotor seizure) 160 Status epilepticus 161 16 Coma and cerebral death 165 Coma 165 Cerebral death 171 17 Disorders of the developing nervous system 173 Anencephaly 174 Chiari type I and II malformations 176 Phenylketonuria (Phenylalanine hydroxylase deficiency) 178 Tay-Sach’s disease (Hexosaminidase A deficiency) 179 Down syndrome 181 18 Traumatic brain injury and subdural hematoma 185 Traumatic brain injury 185 Chronic subdural hematoma 190 19 Neurologic complications of alcoholism 193 Drunkenness and alcoholic coma 194 Alcoholic tremulousness and hallucinosis 194 Alcohol-withdrawal seizures 195 Delirium tremens 195 Wernicke’s encephalopathy and Korsakoff’s psychosis syndrome 195 i-xii.Davis.fm 3/2/05 3:18 PM Page vi vi CONTENTS Alcoholic cerebellar degeneration 196 Alcoholic polyneuropathy 198 Fetal alcohol syndrome 198 20 Disorders of pain and headache 201 Pain 201 Headache pain 202 Tension-type headache 203 Migraine headache 204 21 Disorders of the vestibular system 209 Principles of vertigo management 212 Benign paroxysmal positional vertigo 212 Meniere’s disease 215 Glossary of common neurologic terms 219 Index 227 i-xii.Davis.fm 3/2/05 3:18 PM Page vii Preface This textbook is intended for students who wish to The later chapters are divided into chapters that learn the basic principles of neurology and to review common diseases present at different neu- understand common neurologic diseases. We roanatomic sites along the neuroaxis from muscle selected 58 neurologic diseases based on their fre- to the cerebral cortex and chapters on diseases that quency, ability to represent that category of neuro- have a similar pathophysiology. Each chapter logic disease, value in teaching neuroscience begins with an overview to understand the com- concepts, and diagnostic importance. mon features of this group of diseases. Selected We recognized that most introductory courses diseases are then discussed with an emphasis on on neurologic diseases are short and lack suffi- the pathophysiology, major clinical features, major cient time for a student to extensively read a com- laboratory findings, and the principles of disease prehensive neurology textbook. In addition, while management. Our book covers both adult and abbreviated versions of neurology textbooks cover pediatric neurologic diseases. a myriad of neurologic diseases in telegraphic This book does not cover detailed aspects of dis- style, they are difficult to comprehend unless one ease variants, all possible laboratory tests, drug already knows about the disease. Thus, many stu- dosages, or many related neurologic diseases as it is dents finish the course with a spotty understand- not designed for the specific treatment of neuro- ing of neurology. We designed our book to be read logic patients. Our goal is to provide broad and from cover to cover, giving the reader a more integrated coverage of the fundamentals of com- thorough understanding of the fundamentals of mon neurologic diseases in the context of a neurology. competent examination strategy, the disease patho- The first chapters cover the basic approach a physiology, and the principles of disease manage- neurologist takes when encountering a patient with ment. We hope that students will find the book a neurologic problem, the key elements of the neu- structure and context useful in their studies and that rologic exam, and an overview of common neuro- it will contribute to the instructors’ efforts to sup- logic tests. We discuss how to use the history and port students in their learning. neurologic exam to localize the patient’s problem to specific neuroanatomic site(s) and to use the neu- Larry E. Davis, M.D. roanatomic information along with results of Molly K. King, M.D. appropriate laboratory tests to establish a diagnosis. Jessica L. Schultz, M.D. vii This page intentionally left blank i-xii.Davis.fm 3/2/05 3:18 PM Page ix Acknowledgments Dr. Davis is indebted to his late father, Lloyd, for Finally, we thank everyone who helped in the his years of encouragement in his pursuit of schol- preparation of this book. In particular, we are arly endeavors; to his wife, Ruth Luckasson, for her grateful to Yvonne Walston, CMI, of Creative support, ideas, patience, and encouragement dur- Imagery, Inc. whose artwork improved the clarity ing the many hours of writing this book; and to his of the chapters, to Dr. Blaine Hart, who con- children Meredith, Colin, and Charles. tributed neuroimaging illustrations, to Drs. Mark Dr. King thanks Dr. Joe Bicknell for his enthu- Becher and Mario Kornfeld who contributed neu- siastic mentoring and Dr. Kurt Fiedler for his ropathology illustrations, and to Diana Schneider, inspiring, never-ending pursuit of knowledge. Dr. PhD, President of Demos Medical Publishing, who Schultz would like to thank her husband, Brandon, supported the creation of this book. for his support and unfailing patience during the creation of this book. ix This page intentionally left blank i-xii.Davis.fm 3/2/05 3:18 PM Page xi Foreword A detailed patient history and physical examina- anatomical and pathological correlation and pro- tion remain the underpinnings of neurologic diag- vides many tables and line drawings that help in nosis. Imaging and laboratory testing are understanding the anatomy and physiological important, but the ability to piece together clues in basis of disease and the differential diagnosis of the patient’s story and to localize lesions by the neurologic illnesses. Too often introductory vol- findings on the neurologic examination still sepa- umes on neurology are overwhelming in bulk and rate the good neurologist from the main body of complexity. The authors of this volume have suc- physicians. Medical students who choose neurol- ceeded in presenting a gentler and concise intro- ogy for a career often cite two factors that have duction to this fascinating subject. influenced them—first, the spectacular basic
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