INTRACTABLE SEIZURES Diagnosis, Treatment, and Prevention ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY Editorial Board: NATHAN BACK, State UniversitvofNew York atBuffalo IRUN R. COHEN, The Weizmann Institute ofScience DAVID KRITCHEVSKY, WistarInstitute ABELLAITHA, N. S. Kline Institutefor PsychiatricResearch RODOLFO PAOLETTI, University ofMilan Recent Volumes in this Series Volume 491 THE MOLECULAR IMMUNOLOGY OFCOMPLEX CARBOHYDRATES-2 Edited by Albert M. Wu Volume 492 NUTRITION AND CANCER PREVENTION: New Insights into the Role of Phytochemicals Edited under the auspices ofthe American Institute for CancerResearch Volume 493 NEUROIMMUNE CIRCUITS, DRUGS OF ABUSE, AND INFECTIOUS DISEASES Edited by Herman Friedman, Thomas W. Klein, and John J. Madden Volume 494 THE NIDOVIRUSES (CORONAVIRUSES AND ARTERIVIRUSES) Edited by Ehud Lavi, Susan R. Weiss, and Susan T. Hingley Volume 495 PROGRESS IN BASIC AND CLINICAL IMMUNOLOGY Edited by Andrzej Mackiewicz, Maciej Kurpisz, and Jan Zeromski Volume 496 NONINVASIVE ASSESSMENT OFTRABECULAR BONE ARCHITECTURE AND THE COMPETENCE OF BONE Edited by Sharmila Majumdar, Ph.D., and Brian K. Bay, Ph.D. Volume 497 INTRACTABLE SEIZURES: Diagnosis, Treatment, and Prevention Edited by W. McIntyre Burnham, Peter L. Carlen, and Paul A. Hwang Volume 498 DIABETES AND CARDIOVASCULAR DISEASE: Etiology, Treatment, and Outcomes Edited by Aubie Angel, Naranjan DhaHa, Grant Pierce, and Pawan Singal Volume 499 FRONTIERS IN MODELING AND CONTROL OF BREATHING Edited by Chi-Sang Poon and Homayoun Kazemi Volume 500 BIOLOGICAL REACTIVE INTERMEDIATES VI: Chemical and Biological Mechanisms ofSusceptibility to and Prevention ofEnvironmental Diseases Edited by Patrick M. Dansette, Robert Snyder, Marcel Delaforge, G. Gordon Gibson, Helmut Greim, David J. JoHow, Terrence J. Monks, and I. Glenn Sipes AContinuationOrderPlanisavailableforthisseries.Acontinuationorderwillbringdeliveryofeachnewvolume immediatelyuponpublication.Volumesarebilledonlyuponactualshipment.Forfurtherinformationpleasecontact thepublisher. INTRACTABLE SEIZURES Diagnosis, Treatment, and Prevention Edited by w. McIntyre Bumham University of Toronto Toronto, Ontario, Canada Peter L. Carlen Toronto Western Research Institute Toronto, Ontario, Canada and Paul A. Hwang University of Toronto Toronto, Ontario, Canada SPRINGER SCIENCE+BUSINESS MEDIA, LLC Library of Congress Cataloging-in-Publieation Data lntractable seizures: diagnosis, treatment, and prevention/edited by W. McIntyre Bumham, Peter L. Carlen, and Paul A. Hwang. p. ; em. - (Advances in experimental medicine and biology; v. 497) lncludes bibliographical references and index. ISBN 978-1-4613-5503-8 ISBN 978-1-4615-1335-3 (eBook) DOI 10.1007/978-1-4615-1335-3 1. Epilepsy. 1. Bumham, W. McIntyre, 1939- II. Carlen, Peter L. III. Hwang, Paul A. 1949- IV. Series. [DNLM: 1. Seizures-diagnosis. 2. Seizures-prevention & control. 3. Seizures-therapy. WL 3401614 2001] RC372 .1495 2001 616.8'53-dc21 2001042722 ISHN 978-1-4613-5503-8 ©2002 Springer Scienec+Business Media New York Originally publisbcd by Kluwer Academic I Plenum Publisher,. New York in 2002 Softcover reprinl of the hardcover 1s I edilion 2002 10 9 8 7 6 5 4 3 2 I A C.I.P. record for this book is available from the Library of Congress AII rights reserved No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, reeording, or otherwise, without written permission from the Publisher To absent friends, Gerhard Fromm, Eric Lothman, and Frank Morrell, and to our teacher, Pierre Gloor PREFACE About 20%ofthepeoplewho haveepilepsyhave seizuresthatare resistant to anti convulsant medications. Another 20% have seizures which are only partially responsive todrugs. Thesedrug-resistantseizuresarecalled"intractable",andthepatientswho have them-about Iin250inthegeneralpopulation-presentamajorchallengetophysicians, neurologists and epilepsy associations. The present volume describes the symptomatology of the major "intractable" syndromes,themostappropriatedrugsforeach, andthepossibilitiesfor surgicalcontrol. Research related to the causes and effects of intractable seizures is presented, and new directions in therapy are discussed. vii ACKNOWLEDGMENTS The present volume originated in a conference sponsored by the Bloorview Chil dren's Hospital Foundation. The editors would like to thank the Foundation for their generoussupport. Wewouldalsoliketo thankMs. AllaVilneroftheBloorviewEpilepsy Research Programand Mr. David Banhfor theirdedicated workin preparingthis book. ix ABBREVIATIONS ACPD amino-cyclopentane-l,3-dicarboxylate ACTH Corticotrophin AED Antiepileptic Drugs AEM Antiepileptic Medications AHP Afterhyperpolarization AMPA amino-3-hydroxy-5-methyl-4-isoxazoleproprionic acid AVMs Arteriovenous Malformations BZP Benzodiazepine CAl Field CAl of the Hippocampus CA2 Field CA2 of the Hippocampus CA3 Field CA3 of the Hippocampus Ca2+ Calcium Ions Cl- Chloride Ions CNS Central Nervous System CPS Complex Partial Seizures CRH Corticotropin-Releasing Hormone CSF Cerebrospinal Fluid CT Computerized Tomography DAG Diacyl Glycerol EAA Excitatory Amino Acids EEG Elecroencephalography EcoG Electrocorticography ESES Electrical Status Epilepticus of Sleep FCD Focal Cortical Dysplasia G G-protein GEPR Genetically Epilepsy Prone Rats GSH glutathione HHE Hemiconvulsions Hemiplegia Epilepsy Syndrome IP3 inositol-I.4.5-triphosphate IS Infantile Spasms KA Kainic Acid LGS Lennox-Gastaut Syndrome LTP Long-Term Potentiation MCT Medium-Chain Triglyceride mGluRs Metabotropic Glutamate Receptors MK801 (+)-5-methyl-l0,II-dihydro-5H-dibenzo[a,d]cycloheptan-5,10-imine maleate xi xii Abbreviations MR Magnetic Resonance MTS Mesial Temporal Sclerosis Na+ Sodium Ions NGF Nerve Growth Factor NMDA N-methyl, D-aspartate NO Nitric Oxide nREM Non Rapid Eye Movement Sleep (SlowWave Sleep) PCP phencyclidine PET Positron Emission Tomography PDS Paroxysmal Depolarization Shift PIP phosphatidyl inositol-4,5-diphosphate 2 PKC Phosphokinase C PLC Phospholipase C PNS Peripheral Nervous System PTE Posttraumatic Epilepsy REM Rapid Eye Movement Sleep (Dream Sleep) SE Status Epilepticus SPECT Single Photon Emission Computed Tomography SRS Spontaneous Recurrent Seizures VGB Vigabatrin VPA Valproate WS West's Syndrome TABLE OF CONTENTS 1. Intractable Epilepsy in Adults 110 E. Leppik Complex Partial Seizures 2. Clinical Description and Diagnosis 9 Warren T. Blume 3. Drug Therapy for Complex Partial Seizures 19 Joseph Bruni 4. Surgery for Complex Partial Seizures 27 John P. Girvin West's Syndrome (Infantile Spasms) 5. Clinical Description and Diagnosis 33 Richard A. Hrachovy 6. Drug Therapy for West's Syndrome 51 Catherine Chiron and Oliver Dulac 7. Surgery for West's Syndrome 57 Harold 1. Hoffman Lennox-Gastaut Syndrome 8. Clinical Description and Diagnosis 61 Ernst Niedermeyer 9. Drug Therapy in Lennox-Gastaut Syndrome 77 Kevin Farrell 10. Surgery in Lennox-Gastaut Syndrome: Corpus Callosum Division for Children 87 John R. Gates xiii