Epilepsy in children This page intentionally left blank Epilepsy in children Second Edition Edited by The late SHEILA J WALLACE Paediatric Neurologist, Department of Child Health University Hospital of Wales, Cardiff, UK and KEVIN FARRELL Professor, Division of Pediatric Neurology University of British Columbia, and Director, Epilepsy Service British Columbia’s Children’s Hospital, Vancouver, British Columbia, Canada A member of the Hodder Headline Group LONDON First published in Great Britain in 2004 by Arnold, a member of the Hodder Headline Group, 338 Euston Road, London NW1 3BH http://www.arnoldpublishers.com Distributed in the United States of America by Oxford University Press Inc., 198 Madison Avenue, New York, NY 10016 Oxford is a registered trademark of Oxford University Press ©2004 Arnold All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronically or mechanically, including photocopying, recording or any information storage or retrieval system, without either prior permission in writing from the publisher or a licence permitting restricted copying. In the United Kingdom such licences are issued by the Copyright Licensing Agency: 90 Tottenham Court Road, London W1T 4LP. Whilst the advice and information in this book are believed to be true and accurate at the date of going to press, neither the author[s] nor the publisher can accept any legal responsibility or liability for any errors or omissions that may be made. In particular (but without limiting the generality of the preceding disclaimer) every effort has been made to check drug dosages; however it is still possible that errors have been missed. Furthermore, dosage schedules are constantly being revised and new side-effects recognized. For these reasons the reader is strongly urged to consult the drug companies’ printed instructions before administering any of the drugs recommended in this book. British Library Cataloguing in Publication Data A catalogue record for this book is available from the British Library Library of Congress Cataloging-in-Publication Data A catalog record for this book is available from the Library of Congress ISBN 0 340 80814 4 1 2 3 4 5 6 7 8 9 10 Commissioning Editor: Joanna Koster Development Editor: Sarah Burrows Project Editor: Zelah Pengilley Production Controller: Deborah Smith Cover Design: Lee-May Lim Index: Indexing Specialists (UK) Ltd Typeset in 10/12 Minion by Charon Tec Pvt. Ltd, Chennai, India Printed and bound in the UK by Butler & Tanner Ltd What do you think about this book? Or any other Arnold title? Please send your comments to [email protected] To the memory of Sheila Wallace and to my wife, Kate, and my children, Seonaid, Alastair and Nicholas, for their love and inspiration. This page intentionally left blank Contents Contributors xi Foreword by John M. Freeman xv Preface xvii Sheila Wallace – an appreciation by John BP Stephenson xix Abbreviations xxi 1 Definitions and classification of epileptic seizures and epilepsies 1 The late Olaf Henriksen and the late Sheila J Wallace 2 Paroxysmal nonepileptic disorders: differential diagnosis of epilepsy 4 John Stephenson, William P Whitehouse and Sameer Zuberi 3 Incidence and prevalence 21 Lars Forsgren 4 Aspects of etiology 27 4A Genetics 29 Robert Robinson and R Mark Gardiner 4B Chromosomal syndromes associated with epilepsy 43 Daniela T Pilz and David Ravine 4C Abnormalities of brain development 47 Renzo Guerrini and Lucio Parmeggiani 4D Epilepsy following acute brain injury 62 Kevin Farrell 4E Disorders of metabolism and neurodegenerative disorders associated with epilepsy 65 Bwee Tien Poll-The 4F Infection and postinfective causes of epilepsy 76 Anna Lecticia Ribeiro Pinto and Paulo Rogério M de Bittencourt 5 Pathology of childhood epilepsies 81 Hajime Miyata and Harry V Vinters 6 Pathophysiology of childhood epilepsies 95 Rüdiger Köhling and John GR Jefferys 7 Seizures in the neonate 111 Eli M Mizrahi 8 Febrile seizures 123 Sheila J Wallace viii Contents 9 Epilepsies with onset in the first year of life 131 9A Early epileptic encephalopathies 133 Shunsuke Ohtahara, Yasuko Yamatogi and Yoko Ohtsuka 9B West syndrome 142 Raili Riikonen 9C Partial epilepsies in infancy 148 Olivier Dulac and Jean-Paul Rathgeb 9D Benign myoclonic epilepsy in infancy 153 Natalio Fejerman 9E Severe myoclonic epilepsy in infancy (Dravet syndrome) 157 Natalio Fejerman 10 Epileptic syndromes with onset in early childhood 161 10A Myoclonic-astatic epilepsy 163 Jean Aicardi 10B Lennox-Gastaut syndrome 169 Jean Aicardi 11 Epileptic syndromes with onset in middle childhood 179 11A Childhood absence epilepsy 181 Roberto Michelucci and Carlo Alberto Tassinari 11B Epilepsy with myoclonic absences 189 Carlo Alberto Tassinari, Guido Rubboli, Elena Gardella and Roberto Michelucci 11C Eyelid myoclonia with absences 195 Colin D Ferrie 11D Benign partial epilepsies 199 Kazuyoshi Watanabe 11E Rasmussen syndrome 221 Mary B Connolly and Kevin Farrell 12 Epileptic syndromes with onset in late childhood or adolescence 225 12A Idiopathic generalized epilepsy with generalized tonic-clonic seizures only 227 Colin D Ferrie and CP Panayiotopoulos 12B Juvenile myoclonic epilepsy 231 Richard A Grünewald and CP Panayiotopoulos 12C Juvenile absence epilepsy 237 CP Panayiotopoulos 12D Benign partial seizures of adolescence 241 CP Panayiotopoulos 12E Reflex seizures and reflex epilepsies 243 Michael Koutroumanidis and CP Panayiotopoulos 13 Cognitive and behavioral manifestations of epilepsy in children 250 Thierry Deonna 14 Progressive myoclonus epilepsies 257 Charlotte Dravet and Alain Malafosse 15 Epilepsies symptomatic of structural lesions 268 Paolo Curatolo 16 Epilepsy in people with intellectual disability 281 Matti Sillanpää 17 Status epilepticus 290 John H Livingston 18 Neurophysiological investigations 304 Mary B Connolly and Peter KH Wong Contents ix 19 Imaging 329 19A Anatomical imaging 331 Ruben I Kuzniecky 19B Functional imaging 342 Csaba Juhász and Harry T Chugani 20 Investigation of the child with epilepsy 352 Katherine Wambera and Kevin Farrell 21 Pharmacology of antiepileptic drugs 358 Andrew Fisher and Philip N Patsalos 22 Treatment of epilepsy 385 22A Antiepileptic drugs (AEDs) 387 Blaise FD Bourgeois 22B The ketogenic diet 405 Eileen PG Vining and Eric H Kossoff 22C Vagal nerve stimulation 409 Jerome V Murphy and Sara Hudson 22D Surgery 413 Charles E Polkey 22E Behavioral therapy 430 JoAnne Dahl 23 Cognitive aspects 433 Dorothée GA Kasteleijn-Nolst Trenité and Anne de Saint-Martin 24 Psychiatric aspects 447 Sharon Davies and Isobel Heyman 25 Social aspects 463 David W Dunn and Joan K Austin 26 Special centers for childhood epilepsy 474 Frank MC Besag 27 The adolescent with epilepsy 478 Kevin Farrell Index 485