Epilepsy in Children and Adolescents Epilepsy in Children and Adolescents Edited by James W. Wheless, MD DepartmentofPediatricNeurology UniversityofTennesseeHealthScienceCenter LeBonheurComprehensiveEpilepsy ProgramandNeuroscienceInstitute LeBonheurChildren’sHospital Memphis,TN,USA A John Wiley & Sons, Ltd., Publication Thiseditionfirstpublished2013(cid:1)C 2013byJohnWiley&Sons,Ltd Wiley-BlackwellisanimprintofJohnWiley&Sons,formedbythemergerofWiley’sglobalScientific, TechnicalandMedicalbusinesswithBlackwellPublishing. Registeredoffice: JohnWiley&Sons,Ltd,TheAtrium,SouthernGate,Chichester,WestSussex, PO198SQ,UK Editorialoffices: 9600GarsingtonRoad,Oxford,OX42DQ,UK TheAtrium,SouthernGate,Chichester,WestSussex,PO198SQ,UK 111RiverStreet,Hoboken,NJ07030-5774,USA Fordetailsofourglobaleditorialoffices,forcustomerservicesandforinformationabouthowtoapplyfor permissiontoreusethecopyrightmaterialinthisbookpleaseseeourwebsiteat www.wiley.com/wiley-blackwell. 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LibraryofCongressCataloging-in-PublicationData Epilepsyinchildrenandadolescents/[editedby]JamesW.Wheless. p. ; cm. Includesbibliographicalreferencesandindex. ISBN978-0-470-74123-8(cloth) I.Wheless,James. [DNLM: 1.Epilepsy. 2.Adolescent. 3.Anticonvulsants–therapeuticuse. 4.Child. 5.Epilepsy–therapy. WL385] 616.85(cid:2)300835–dc23 2012024368 AcataloguerecordforthisbookisavailablefromtheBritishLibrary. Wileyalsopublishesitsbooksinavarietyofelectronicformats.Somecontentthatappearsinprintmaynotbe availableinelectronicbooks. Coverimage:4yearoldwithLennox-GastautSyndrome,slowspikeandwavecomplexesinsleep,courtesyof DrJamesW.Wheless. Setin10/12ptTimesbyAptaraInc.,NewDelhi,India FirstImpression2013 Contents Listofcontributors xiii Preface xvii Section1 Epidemiologyandclassificationof childhoodepilepsies 1 Sectioneditor:PhillipL.Pearl 1 Epidemiologyandcommoncomorbiditiesofepilepsyinchildhood 3 JaySalpekar,MatthewByrne,andGeorgannFerrone 1.1 Epidemiology 3 1.2 Incidenceandprevalence 4 1.3 Genderandage 4 1.4 Classification 5 1.5 Febrileseizures 6 1.6 Etiology 6 1.7 Psychiatriccomorbidity 7 1.8 Psychologicalandpsychosocialstressrelatedtochronicdisease 7 1.9 Psychiatricsymptomsrelatedtomedicationsideeffects 8 1.10 Psychiatriccomorbidityrelatedtoepilepsypathophysiology 8 1.11 Attention-deficit/hyperactivitydisorder(ADHD) 9 1.12 Anxiety 10 1.13 Depression 11 1.14 Intellectualanddevelopmentaldisabilities(IDD) 12 1.15 Conclusion 12 References 13 vi CONTENTS 2 Classificationanddefinitionofseizuresandepilepsy syndromesinchildhood 17 SusanE.CombsandPhillipL.Pearl 2.1 Introduction 17 2.2 Purposeandgoalsofdefinitionsandclassification 17 2.3 Systemsofclassificationanddefinitions 18 2.4 Seizures 18 2.5 Generalizedseizures 19 2.6 Focalseizures 22 2.7 Syndromes 23 2.8 Specificage-relatedepilepsysyndromes 25 2.9 Futuredirections 34 Acknowledgements 34 References 34 3 Initiatingandwithdrawingmedicalmanagement 37 DavidT.HsiehandBhagwanIndurMoorjani 3.1 Initiatingmedicalmanagement 37 3.2 Thechancesofseizurerecurrenceafterthefirstunprovokedseizure 39 3.3 Seizurerecurrence 42 3.4 Thepossibleadverseeffectsofseizurerecurrence 42 3.5 Therisksofinitiatingantiepilepticdrugtherapy 44 3.6 Thebenefitsofinitiatingantiepilepticdrugtherapy 45 3.7 Howtoinitiatetreatmentwithantiepilepticdrugs 45 3.8 Specialcircumstances 48 3.9 Summary:initiatingmedicalmanagement 48 3.10 Withdrawingmedicalmanagement 49 3.11 Thelong-termprognosisofchildhood-onsetepilepsy 50 3.12 Whentoconsiderdiscontinuingantiepilepticdrugtherapy 51 3.13 Riskfactorsforseizurerecurrenceafterdiscontinuation 51 3.14 Therisksofdiscontinuingantiepilepticdrugtherapy 54 3.15 Thebenefitsofdiscontinuingantiepilepticdrugtherapy 55 3.16 Howtodiscontinueantiepilepticdrugs 55 3.17 Specialcircumstances 55 3.18 Summary:withdrawingmedicalmanagement 56 3.19 Disclaimer 57 References 57 4 Commongeneticandneurocutaneousdisordersin childhoodepilepsy 59 DewiFrancesT.Depositario-Cabacar,WilliamMcClintock, andTomReehal 4.1 Idiopathicepilepsies 60 4.2 Symptomaticepilepsies 63 4.3 Epilepsyincommonchromosomalabnormalities 63 CONTENTS vii 4.4 Epilepsyinmetabolicandmitochondrialdisorders 65 4.5 Epilepsyinmalformationsofcorticaldevelopment 66 4.6 Neurocutaneousdisorders 67 4.7 Summary 70 References 70 Section2 Diagnostic evaluationofchildhoodepilepsies 73 Sectioneditor:DavidF.Clarke 5 Evaluatingthechildwithseizures 75 KristenParkandSusanKoh 5.1 Emergentdiagnosisandmanagement 76 5.2 Subsequentevaluation 79 5.3 Additionalneurodiagnosticevaluation 84 References 87 6 TheuseofEEGinthediagnosisofchildhoodepilepsy 90 DavidF.Clarke 6.1 TechnicalaspectsoftheEEG 91 6.2 MethodsusedtoincreaseEEGyield 91 6.3 WhenshouldanEEGbeordered? 92 6.4 EEGfindingsinepilepsyandepilepsysyndromes 93 6.5 NeonatalEEGs 94 6.6 TheEEGinfocalepilepsy 96 6.7 TheEEGofgeneralizedepilepsy 99 6.8 Specificdisease-relatedepilepsysyndromes 104 6.9 Conclusion 105 References 105 7 Imagingofpediatricepilepsy 107 AsimF.Choudhri 7.1 Introduction 107 7.2 Imagingconsiderations 107 7.3 Congenitalmalformations 117 7.4 Neoplasms 124 7.5 Acquired/idiopathicabnormalities 126 References 127 8 Non-epilepticparoxysmaleventsofchildhood 129 SuchetaM.Joshi 8.1 Introduction 129 8.2 Breath-holdingspells 130 8.3 Parasomnias 131 viii CONTENTS 8.4 Benignparoxysmalpositionalvertigoofchildhood 133 8.5 Syncope 134 8.6 Paroxymalnon-epilepticevents(PNEs)withapsychiatricorbehavioral basis 134 8.7 Hyperekplexia 136 8.8 Alternatinghemiplegiaofchildhood 136 8.9 Movementdisorders 137 8.10 Sandifersyndrome 138 8.11 Conclusion 138 References 139 Section3 Principlesoftreatment 143 Sectioneditor:JamesW.Wheless 9 Pharmacologyofantiepilepticdrugs 145 JamesW.Wheless 9.1 Pharmacokinetics 146 9.2 Pharmacogenomics 155 References 157 10 Therapeuticefficacyofantiepilepticdrugs 159 JamesW.Wheless 10.1 Efficacy-basedtreatmentguidelines 160 10.2 Antiepilepticdrugselectionbasedonspecificpediatric epilepsysyndromes 164 10.3 Influenceofcomorbiditiesinchildrenwithepilepsy 171 10.4 Conclusions 172 References 172 11 Adverseeffectsofantiepilepticdrugs 175 JamesW.Wheless 11.1 Introduction 175 11.2 Specificdrugs 179 11.3 At-riskprofilesandmonitoring 189 References 191 12 Vagusnervestimulationtherapyandepilepsysurgery 193 KateVanPoppelandJamesW.Wheless 12.1 Vagusnervestimulation 195 12.2 Epilepsysurgery 203 12.3 Conclusions 215 References 215 CONTENTS ix 13 Dietarytherapiestotreatepilepsy 219 JamesW.Wheless 13.1 History 220 13.2 Efficacy 221 13.3 Mechanismofaction 228 13.4 Selectionofcandidatesforthediet 232 13.5 Initiationandmaintenance 234 13.6 Complications 236 13.7 Theketogenicdietinthetwenty-firstcentury 239 References 239 Resources 240 Websites 241 Section4 Generalizedseizuresandgeneralized epilepsysyndromes 243 Sectioneditor:AmyL.McGregor 14 Idiopathicgeneralizedepilepsies 245 AmyL.McGregor 14.1 Clinicalfeatures 246 14.2 Naturalhistory 248 14.3 Genetics 248 14.4 Treatment 248 14.5 Classification 249 14.6 Myoclonicepilepsyininfancy 249 14.7 Childhoodabsenceepilepsy(CAE) 250 14.8 Juvenileabsenceepilepsy(JAE) 252 14.9 Juvenilemyoclonicepilepsy(JME) 254 14.10 Epilepsywithgeneralizedtonic-clonicseizuresalone(IGE-GTCs) 256 14.11 Epilepsywithmyoclonicabsence 257 14.12 Epilepsywithmyoclonic-atonicseizures/Doosesyndrome 258 14.13 Febrileseizuresplus(FS+) 259 14.14 Eyelidmyocloniawithabsences(EMA)/Jeavonssyndrome 260 14.15 Summary 262 References 264 15 Cryptogenicandsymptomaticgeneralizedepilepsies: epilepsieswithencephalopathy 267 KarenKeough 15.1 Neonatal-onsetepilepsieswithencephalopathy 268 15.2 Infantile-onsetepilepsieswithencephalopathy 270 15.3 Epilepsieswithencephalopathywithonsetlaterininfancy 275 15.4 Epilepsieswithencephalopathywithonsetafterinfancy 277 x CONTENTS 15.5 Continuousspikewaveofsleep(CSWS)andLandau–Kleffner syndrome(LKS) 279 References 280 Section5 Partial-onsetseizuresandlocalization-related epilepsysyndromes 283 Sectioneditor:JamesW.Wheless 16 Idiopathicpartialepilepsies 285 FreedomF.PerkinsJr 16.1 Benigninfantileseizures 286 16.2 Benignchildhoodepilepsywithcentrotemporalspikes 287 16.3 Childhoodoccipitalepilepsy(Panayiotopoulostype) 289 16.4 Late-onsetchildhoodoccipitalepilepsy(Gastauttype) 292 References 294 17 Cryptogenicandsymptomaticpartialepilepsies 296 StephenFulton 17.1 Etiology 296 17.2 Seizurephenomena 297 17.3 Temporallobeepilepsy 297 17.4 Extratemporalepilepsy 303 17.5 Occipitallobeepilepsy 306 17.6 Parietallobeepilepsy 307 17.7 Hypothalamichamartoma 307 17.8 Otherlocalizingandlateralizingsigns 308 References 309 Section6 Epilepsiesrelativetoage, etiology, orduration 311 Sectioneditor:Yu-TzeNg 18 Neonatalseizures 313 EricV.Hastriter 18.1 Significanceofneonatalseizures 313 18.2 Pathophysiologyofneonatalseizures 314 18.3 Classificationandclinicalfeaturesofneonatalseizures 316 18.4 Electrographicseizures 317 18.5 Monitoringandrecording 317 18.6 Etiologyofneonatalseizures 321 18.7 Metaboliccausesforneonatalseizures 323 18.8 Inbornerrorsofmetabolism 323 CONTENTS xi 18.9 Treatment 327 18.10 Chronicpostnatalepilepsyandtheneedforlong-termtreatment 328 18.11 PotentialadverseeffectsofantiepilepticdrugsontheimmatureCNS 329 18.12 Conclusion 329 References 330 19 Febrileseizures 333 MarieFranciscaGrill 19.1 Introduction 333 19.2 Definition 333 19.3 Incidenceandprevalence 334 19.4 Pathophysiology 334 19.5 Prognosis 334 19.6 Initialevaluationandmanagement 335 19.7 Long-termmanagement 338 19.8 Managementinpractice 341 19.9 Genetics 342 19.10 Parentcounseling 343 19.11 Conclusion 344 References 344 20 Statusepilepticusinchildhood 346 Yu-TzeNgandRamaMaganti 20.1 Definition 346 20.2 Epidemiology 349 20.3 Pathophysiology 349 20.4 Etiology 350 20.5 Diagnosisandinvestigations 351 20.6 EEGpatternsinstatusepilepticus 352 20.7 Treatment 356 20.8 Prognosis 359 References 359 Index 365