Epilepsy in Children

528 Pages·2002·10 MB·English

by  Alexis Arzimanoglou

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Aicardi’s Epilepsy in Children
Third Edition
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Aicardi’s Epilepsy in Children
Third Edition
Alexis Arzimanoglou, M.D.
Head, Pediatric Epilepsy Unit
Department of Child Neurology and Metabolic Disorders
and
Institut National de la Santé et de la Recherche Médicale
University Hospital Robert Debré
Paris, France
Renzo Guerrini, M.D.
Professor of Child Neurology and Psychiatry
Head of the Epilepsy, Neurophysiology and Neurogenetics Unit
Department of Child Neurology and Psychiatry
University of Pisa and Research Institute Stella Maris Foundation
Pisa, Italy
Jean Aicardi, M.D., F.R.C.P.
Honorary Professor of Child Neurology
Institute of Child Health, University College London;
Research Director
Institut National de la Santé et de la Recherche Médicale;
Honorary Consultant
Department of Child Neurology and Metabolic Disorders
University Hospital Robert Debré
Paris, France
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Library of Congress Cataloging-in-Publication Data
Arzimanoglou, A.
Aicardi’s epilepsy in children / Alexis Arzimanoglou, Renzo Guerrini, Jean Aicardi.—
3rd ed.
p. ; cm.
Rev. ed. of: Epilepsy in children / Jean Aicardi. 2nd ed. c1994.
Includes bibliographical references and index.
ISBN 0-7817-2698-0
1. Epilepsy in children. I. Title: Epilepsy in children. II. Guerrini, Renzo.
III. Aicardi, Jean. IV. Aicardi, Jean. Epilepsy in children. V. Title.
[DNLM: 1. Epilepsy—classification—Child. 2. Epilepsy—classification—infant. 3.
Epilepsy—diagnosis—Child. 4. Epilepsy—diagnosis—Infant. 5. Epilepsy—therapy—
Child. 6. Epilepsy—therapy—Infant. WL 385 A797a2004]
RJ496.E6A43 2004
618.92′853—dc22
2003054693
Care has been taken to confirm the accuracy of the information presented and to de-
scribe generally accepted practices. However, the authors and publisher are not responsi-
ble for errors or omissions or for any consequences from application of the information in
this book and make no warranty, expressed or implied, with respect to the currency, com-
pleteness, or accuracy of the contents of the publication. Application of this information in
a particular situation remains the professional responsibility of the practitioner.
The authors and publisher have exerted every effort to ensure that drug selection and
dosage set forth in this text are in accordance with current recommendations and practice
at the time of publication. However, in view of ongoing research, changes in government
regulations, and the constant flow of information relating to drug therapy and drug reac-
tions, the reader is urged to check the package insert for each drug for any change in indi-
cations and dosage and for added warnings and precautions. This is particularly important
when the recommended agent is a new or infrequently employed drug.
Some drugs and medical devices presented in this publication have Food and Drug Ad-
ministration (FDA) clearance for limited use in restricted research settings. It is the re-
sponsibility of the health care provider to ascertain the FDA status of each drug or device
planned for use in their clinical practice.
10 9 8 7 6 5 4 3 2 1
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Contents
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
Section I.General Aspects
1. Epilepsy: Overview and Definitions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
2. Classification of Epileptic Seizures and Epilepsies . . . . . . . . . . . . . . . . . . . . . . . . 7
Section II. Major Types of Epileptic Seizures in Childhood and
Corresponding Epileptic Syndromes
3. Infantile Spasms and Related Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14
4. Lennox-Gastaut Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 38
5. Dravet Syndrome: Severe Myoclonic Epilepsy or Severe Polymorphic Epilepsy of
Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
6. Epilepsies with Predominantly Myoclonic Seizures . . . . . . . . . . . . . . . . . . . . . . . 58
7. Myoclonic Epilepsies Associated with Progressive Degenerative Disorders:
Progressive Myoclonic Epilepsies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81
8. Epilepsies with Typical Absence Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88
9. Epilepsies with Tonic-Clonic Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 105
10. Epilepsies Characterized by Partial Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114
11. Landau-Kleffner Syndrome and Syndrome of Continuous Spike-Waves
of Slow Sleep . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 176
Section III.Epileptic Manifestations Relative to Age,Cause,Duration,
and Precipitation of Seizures
12. Neonatal Seizures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
13. Epilepsy in Infants . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 210
14. Febrile Convulsions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220
15. Occasional Seizures Other Than Febrile Convulsions . . . . . . . . . . . . . . . . . . . . . . 235
16. Status Epilepticus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 241
17. Stimulus-Sensitive Epilepsies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 262
18. Posttraumatic Seizures and Posttraumatic Epilepsy . . . . . . . . . . . . . . . . . . . . . . . . 279
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vi CONTENTS
19. Epilepsy as the Presenting Manifestation of Structural Brain Lesions . . . . . . . . . . 284
20. Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312
Section IV.Selected Aspects of Diagnosis,Prognosis,and Treatment
of Convulsive Disorders in Children
21. Diagnosis and Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 325
22. General Aspects of Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
23. General Principles of Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354
24. Medical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
25. Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
Subject Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 503
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Preface
Since the second edition of this book, an enormous amount of new knowledge on epilepsy has accumulated at
an increasing pace on both its basic and clinical aspects. Novel techniques of investigation and new forms of
therapy, both medical and surgical, have been developed. Advances in genetics have clarified the etiology of
some forms of monogenic epilepsy, and they may shortly provide some understanding of the more common
syndromes with a multifactorial inheritance. Genetics have been shown to play a major role in some cases of
ionic channel dysfunction. New concepts about the mechanisms of seizures and their propagation have led to
the development of the concept of the epileptic network. New antiepileptic agents, some of which have been
rationally rather than empirically devised, have been introduced as a result of the better knowledge of the mech-
anisms of excitatory or inhibitory neurotransmission. Surgical treatment, which has benefitted immensely
from the spectacular progress that has occurred in the techniques of structural and functional imaging, has be-
come an established form of therapy, and new surgical methods such as stimulation are being tested.
Such remarkable technical developments have evolved in parallel with the refinements in diagnostic capa-
bilities, leading to the more precise delineation of epilepsy syndromes and to a better knowledge of their nat-
ural history and sensitivity to drugs. The result has been the identification of more rational indications and con-
traindications for the use of antiepileptic agents on a syndromic basis, as well as the better overall management
of the patients. The realizations that epilepsy may consist of more than simply having seizures and that cogni-
tive or behavioral abnormalities or both may result from infraclinical paroxysmal neuronal activity, such as oc-
curs in the Landau-Kleffner syndrome or the syndrome of continuous spike-waves of sleep, emphasize some
of the peculiar aspects of epilepsy in childhood. They underline the probable role of paroxysmal epileptic brain
dysfunction on the development of the brain, possibly as a result of the plastic changes induced by the epilep-
tic process.
The incorporation of at least some of these recent findings while still retaining the clinical orientation of
the book necessitated the development of a new edition. Collecting this mass of data and literature and re-
viewing them in an accessible manner proved to be a formidable challenge beyond the abilities of a single au-
thor, so a collective endeavor was required. The three authors of this edition have long shared a similar ap-
proach to the study of epilepsy in children, as well as having considerable experience in its clinical diagnosis
and management. We hope this common experience maintains the homogeneity of the text and avoids dis-
crepancies. We have reviewed, discussed, and amended all chapters together in an effort to achieve a degree of
coherence analogous to that of a single-authored book.
The general structure of the book is the same as that of the previous two editions. The volume is divided
into four parts. The first part discusses general notions about seizure disorders, including definitions of the ba-
sic terms that are necessary to the understanding of the subsequent chapters, and the problems of classifica-
tion of epileptic seizures and epilepsies. The second part describes the major types of epileptic seizures and the
epilepsy syndromes in which the particular seizures are the major manifestation. For each type of seizure, an
attempt has been made to delineate the associated syndromes and to assess the validity and usefulness of these
syndromes for prognosis and treatment. This conforms with the clinical approach that uses the symptom as a
starting point for reaching the most precise diagnosis possible. The third part deals with those epileptic mani-
festations for which the age of occurrence or the precipitating factors are more informative than the clinical
characteristics. General etiologies, both genetic and lesional, are also discussed in this section. The fourth part
is concerned with the general aspects of diagnosis, prognosis, and treatment, while taking into account that the
specific aspects of these problems are considered in the discussion of the individual syndromes.
Jean Aicardi
Alexis Arzimanoglou
Renzo Guerrini
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Acknowledgments
The authors thank the persons who directly or indirectly contributed to this book. Many of the opinions and
ideas expressed here were discussed over the years with our faithful collaborators, Jean-Jacques Chevrie,
Françoise Goutières, Edouard Hirsch, Philippe Kahane, and Lucio Parmeggiani. Their contribution to our un-
derstanding of the many problems of epilepsy in children and their approach to dealing with them have been
invaluable.
This book is also a tribute to the memory of Professor Henri Gastaut and to Dr. Joseph Roger, pioneers in
the field of epilepsy, whose examples have been a constant stimulation to us. Thanks are also due to Drs. Pierre
Thomas, Françoise Salefranque, Jeanne Misès, and Perrine Plouin for their contributions to the illustrations.
We also thank Brigitte Tricot, who, for the third time, did the considerable secretarial work for this text. In
addition, we express our appreciation to the editorial team of Lippincott Williams & Wilkins, especially Anne
Sydor, Jenny Kim, and Christiana Sahl for their support, help, and understanding.
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