Epilepsy: Electroclinical Syndromes

404 Pages·1987·9.66 MB·English

by  Roger J. Porter (auth.)

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
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© Springer-Verlag Berlin Heidelberg 1987
Softcover reprint ofthe hardcover 1st edition 1987
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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