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Clinical Medicine and the Nervous System Series Editors: John P. Conomy and Michael Swash Titles in the Series already published Headache Richard Peatfield Forthcoming titles in the Series The Heart and Stroke Edited by Anthony J. Furlan Electrophysiological Diagnosis E. M. Sedgewick Epilepsy: Electroclinical Syndromes Edited by Hans Liiders and Ronald P. Lesser With 87 Figures Springer-Verlag London Berlin Heidelberg New York Paris Tokyo Hans Liiders, MD, PhD Head, Section of Epilepsy and Clinical Neurophysiology, Department of Neurology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44\06, U.S.A. Ronald P. Lesser, MD Director of Adult Seizure Center, Department of Neurology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44\06, U.S.A. ,S'eries Editors John P. Conomy, MD Chairman, Department of Neurology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, Ohio 44\06, U.S.A. Michael Swash, MD, FRCP, MRCPath Consultant Neurologist, Neurology Department, The London Hospital, Whitechapel, London El I BB ISBN-l3: 978-1-4471-1403-1 e-ISBN-13: 978-1-4471-1401-7 DOl: 10.1007/978-1-4471-1401-7 Library of Congress Cataloging-in-Publication Data Epilepsy: electroclinical syndromes (Clinical medicine and the nervous system) Includes bibliographies and index. I. Epilepsy - Diagnosis. 2. Electroencephalography. I. Liiders, Hans. II. Lesser, Ronald P., 1946- III. Series. [DNLM: I. Electroencephalography. 2. Epilepsy. WL 385 E64127] RC373.E6 1987 616.8'5307'547 86-11917 The work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, re-use of illustrations, broadcasting, reproduction by photocopying, machine or similar means, and storage in data banks. © Springer-Verlag Berlin Heidelberg 1987 Softcover reprint ofthe hardcover 1st edition 1987 The use of names, trademarks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature. Filmset by Latimer Trend & Company Ltd, Plymouth Printed by Henry Ling Limited, The Dorset Press, Dorchester 2128/3916-543210 Series Editors' Foreword Epilepsy is among the most common scourges afflicting the health of humankind and perhaps the most terrifying. In one form or another, it is suffered by one in everyone hundred people on earth, with a disproportionate prevalence at the early and late extremes of life. There is nothing sacred or sanctifying about it in spite of Hippoc rates' terming epilepsy "The Sacred Disease" in a famous treatise. There is nothing ennobling about it despite its occasional designa tion as a "noble disorder" by virtue of i ts having affected the likes of Alexander of Macedon, Julius Caesar and other persons of royal lineage. From time to time, epilepsy is hailed as a condition which is artistically inspirational; Fyodor Dostoyevsky's dependence on his own personal experience with complex partial epilepsy as a source of imagery in the transfiguration scenes of The Brothers Karamazov and as a source of experience in The Idiot is often cited in this respect. In fact, for all its victims in human history, epilepsy has been a sad burden which has disrupted and shortened life, causing suffering and castigation for the duration of their terrestrial journey. Throughout the recorded history of epilepsy, now about 5000 years in the writing, men and women have experienced what Fernand Braudel and Emmanuel LeRoy Baudrie have termed L 'Histoire Immobile, or unchanging history. Only in the last century have the increasingly powerful tools of neuropharmacology and electro physiologic diagnosis been developed to allow epilepsy to be pre cisely diagnosed and effectively treated. Even more recently have the techniques been developed to allow surgical treatment of a small but important group of epileptic persons. This volume, the second in the Clinical Medicine and the Nervous System series, deals with epilepsy in a newer but very important nosology. Doctors Liiders and Lesser have organized the entire issue of epilepsy about the denominator of electrophysiology. Together with an outstanding group of contributors, they have defined the vi Series Editor's Foreword clinical presentation, natural history, genetics, risk factors and treatment of the epilepsies based on a diagnostic method which itself illuminates the fundamental pathology of the epilepsies, and which provides a rational basis for treatment. Illustrations are abundant in this text and are in every instance presented in the context of clinical problems. We asked the authors to produce a textbook of practical use to clinicians, novice or expert. We feel they have far exceeded that request and have produced a work of practical benefit to the scholar, scientist and clinician which will be of value for many years to come. Cleveland, Ohio John P. Conomy London Michael Swash Preface The development of electroencephalography marked the beginning of a new era in epileptology. Early on, relatively specific markers for epilepsy were described. Interestingly, and probably also unexpec tedly, these markers were not only present during ictal episodes but also inter-ictally and not infrequently could be easily detected by a short 20-min sample of scalp EEG activity. The EEG test became, therefore, a routine test in the evaluation of all epileptics. Of great importance, the EEG abnormality was different in the different epileptic syndromes so that it could be used to define the type of epilepsy a patient was suffering. The prominent position which electroencephalography plays in the International Classification of Seizures, as also in the recently proposed International Classifica tion of Epileptic Syndromes, clearly underlines this point. Finally, electroencephalography has been one of the most important tools in advancing our understanding of the pathophysiology of epileptic seizures. Both editors were orginally trained as "pure" electroencephalog raphers but eventually also became epileptologists. This transition gave us an opportunity to apply the facts we had learned as electroencephalographers to the clinical management of patients with epilepsy. It also made us realize the close relationship between, and the importance of integrating, the two specialties of electro encephalography and epileptology. This book follows the same philosophy. Each chapter is written either by authors specialized in both fields or by an epileptologist matched with an electroencephalographer of the same institution, who worked in close collaboration to produce a balanced chapter. The plan was to discuss the main epileptic syndrome from an electroclinical point of view. In addition, this book contains one chapter which discusses the problem of classification of epilepsies in an attempt to relate the viii Preface subsequent specific syndromes with the entities identified by the International Committee. We hope that this book will contribute to a better understanding of epileptic syndromes and, particularly, that it will make a contribution to closer interaction between electro encephalographers and epileptologists. Cleveland, H. Liiders Ohio R. P. Lesser Contents 1 The Classification of Epileptic Seizures and Epileptic Syndromes Roger J. Porter .................................................. . 2 Neonatal Seizures Peter Kellaway and Eli M. Mizrahi.............................. 13 3 Infantile Spasms (West Syndrome) Barbara F. Westmoreland and Manuel R. Gomez............... 49 4 Lennox-Gastaut Syndrome Warren T. Blume.................................................. 73 5 Febrile Seizures Gerald Erenberg and Harold H. Morris III...................... 93 6 Absence Seizures Manuel R. Gomez and Barbara F. Westmoreland............... 105 7 Juvenile Myoclonic Epilepsy Dudley S. Dinner, Hans Liiders, Harold H. Morris III, and Ronald P. Lesser................................... ................. 131 8 Generalized Tonic-CIonic Epilepsies Bruce J. Fisch and Timothy A. Pedley........................... 151 9 Progressive Myoclonic Epilepsy Hiroshi Shibasaki.................................................. 187 10 Generalized Status Epilepticus A. David Rothner and Harold H. Morris III.................... 207 x Contents 11 Simple Partial Seizures Ronald P. Lesser, Hans Liiders, Dudley S. Dinner, and Harold H. Morris III......................... ................ ..... 223 12 Complex Partial Seizures Frank W. Sharbrough ............................................. 279 13 Benign Focal Epilepsy of Childhood Hans Liiders, Ronald P. Lesser, Dudley S. Dinner, and Harold H. Morris III. ............... ......... .... ................. 303 14 Focal Status Epilepticus: Modern Concepts Antonio V. Delgado-Escueta and David M. Treiman ........... 347 Subject Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 393