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Pediatric Pediatric Epilepsy Epilepsy Surgery A. Arzimanoglou, JH. Cross, WD. Gaillard, A . P A H. Holthausen, P. Jayakar, P. Kahane, G. Mathern r Surgery z e im a n d o g lo i u , a JH . t C In 1997, Jean AICARDI, one of the most brilliant child neurologists of our era, commented on r r o “How the view has changed” in the field of pediatric epilepsy surgery: “Surgery for epilepsy has ss i now become a realistic therapeutic option for selected children and the field is likely to increase in , W c the near future. It is now realised that procrastination, in the hope that new antiepileptic drugs will D become efficacious, is not justified for some forms of epilepsy that can often be recognised from . G E a onset or after a relatively short course. It is also becoming clear that surgery is not reserved for a illa p highly selected population of patients with normal intelligence and focal seizures, but may also help r d a proportion of more severe epilepsies if a realistic view of the problem and perspectives is taken by , H i the partners”. . H le A. Arzimanoglou o Twenty years later, the most obvious and probably still the strongest reasons against epilepsy ltha p u JH. Cross surgery in children are cultural and psychological. This book, written by members of the ad hoc se s n ILAE Task Force for pediatric epilepsy surgery and experts in the field, is the result of continued , y P collaborative working between pediatric epilepsy surgery centres over the years. Authors . WD. Gaillard Ja critically review all available data and set out the key elements of presurgical evaluation, the y a S k specific electro-clinical presentations per etiology, the range of outcomes to be monitored, and a H. Holthausen r the surgical techniques used today. , u P . K r P. Jayakar a What becomes obvious when reviewing all available data is that the key to h a g optimized outcome in children with epilepsy is early appropriate recognition of ne possible surgical candidates and timely referral to centres with expertise. , G e P. Kahane . M r a G. Mathern th y e r n ISBN : 978-2-7420-1424-8 Pediatric Pediatric Epilepsy Epilepsy Surgery A. Arzimanoglou, JH. Cross, WD. Gaillard, A . P A H. Holthausen, P. Jayakar, P. Kahane, G. Mathern r Surgery z e im a n d o g lo i u , a JH . t C In 1997, Jean AICARDI, one of the most brilliant child neurologists of our era, commented on r r o “How the view has changed” in the field of pediatric epilepsy surgery: “Surgery for epilepsy has ss i now become a realistic therapeutic option for selected children and the field is likely to increase in , W c the near future. It is now realised that procrastination, in the hope that new antiepileptic drugs will D become efficacious, is not justified for some forms of epilepsy that can often be recognised from . G E a onset or after a relatively short course. It is also becoming clear that surgery is not reserved for a illa p highly selected population of patients with normal intelligence and focal seizures, but may also help r d a proportion of more severe epilepsies if a realistic view of the problem and perspectives is taken by , H i the partners”. . H le A. Arzimanoglou o Twenty years later, the most obvious and probably still the strongest reasons against epilepsy ltha p u JH. Cross surgery in children are cultural and psychological. This book, written by members of the ad hoc se s n ILAE Task Force for pediatric epilepsy surgery and experts in the field, is the result of continued , y P collaborative working between pediatric epilepsy surgery centres over the years. Authors . WD. Gaillard Ja critically review all available data and set out the key elements of presurgical evaluation, the y a S k specific electro-clinical presentations per etiology, the range of outcomes to be monitored, and a H. Holthausen r the surgical techniques used today. , u P . K r P. Jayakar a What becomes obvious when reviewing all available data is that the key to h a g optimized outcome in children with epilepsy is early appropriate recognition of ne possible surgical candidates and timely referral to centres with expertise. , G e P. Kahane . M r a G. Mathern th y e r n Pediatric Epilepsy Surgery ISBN:978-2-7420-1424-8 Publishedby ÉditionsJohnLibbeyEurotext 127,avenuedelaRépublique, 92120Montrouge,France. Tél.:0033(1)46730660 Fax:0033(1)40840999 e-mail:[email protected] Internetwebsite:http://www.jle.com JohnLibbeyEurotext 34AnyardRoad,Cobham SurreyKT112LA UnitedKingdom ©2016,JohnLibbeyEurotext.Allrightsreserved. Unauthorizedduplicationcontravenesapplicablelaws. ItisprohibitedtoreproducethisworkoranypartofitwithoutauthorisationofthepublisheroroftheCentre Françaisd’ExploitationduDroitdeCopie(CFC),20,ruedesGrands-Augustins,75006Paris. Pediatric Epilepsy Surgery AlexisArzimanoglou J.HelenCross WilliamD.Gaillard HansHolthausen PrasannaJayakar PhilippeKahane GaryMathern ■PEDIATRICEPILEPSYSURGERY CONTENTS Epilepsysurgeryinchildren:Timeiscritical J.HelenCross,AlexisArzimanoglou,PhilippeKahane,HansHolthausen, GaryMathern,WilliamD.Gaillard,PrasannaJayakar |XIX SectionI.PRESURGICALEVALUATIONINCHILDREN Editors:PrasannaJayakar,WilliamD.Gaillard Chapter1:Theroleandlimitsofseizuresemiology J.HelenCross |3 Whatcanbedeterminedfromsemiologyinchildren? |3 Agedependenceofsemiology |4 Chapter2:TheroleandlimitsofsurfaceEEGandsourceimaging PrasannaJayakar,DouglasNordli,O.CarterSnead |7 SurfaceEEG |8 Generalprinciples |8 Developmentalsubstrates–Maturationalissues |9 Theepilepticgenerator |10 Technicalissues |11 Practical“dosanddon’ts”–Tipstoavoidpitfalls |11 Sourceimaging |15 Background |15 Basicprinciplesofsourceimaging |15 Localizationoftheepileptogeniczoneinconjunctionwithothernon-invasive neurophysiologicalandimagingmodalities |17 Contributiontothedecisionmakingrelevanttotheindicationforinvasivemonitoring |19 ESI/MSI:prosandcons |20 EEG-triggeredfMRI |21 Chapter3:Theroleandlimitsofstructuralandfunctionalneuroimaging WilliamD.Gaillard,ChimaOluigbo |25 Structuralimaginginpresurgicalevaluation |26 Functionalimagingtoidentifytheepileptogeniczone |29 Mappingeloquentcortex |32 Chapter4:Theroleandlimitationsofcognitiveevaluation MaryLouSmith,MadisonBerl |39 Purposesofthepresurgicalneuropsychologicalevaluation |40 Domainsincludedintheneuropsychologicalevaluation andguidelinesfordeterminingassessmenttoolswithinthosedomains |40 School-agechildren |41 Considerationsforyoung(preschool)andchildrenwithintellectualdisability |45 Intracarotidanaestheticproceduresinchildren |45 Challengesforandlimitationsofthepresurgicalneuropsychologicalevaluationinchildren |48 Futuredirections |48 Chapter5:Theroleandlimitsofbehavioralandpsychiatricevaluation JaySalpekar,DavidDunn |51 Overlapofepilepsyandpsychiatricconditions |51 Neurologicpathwaystopsychiatricillness |52 Commonpsychiatriccomorbidities |53 Attentiondeficithyperactivitydisorder(ADHD) |53 Anxietyanddepression |53 Comorbidityindrug-resistantepilepsy |54 Surgeryinneurologyandpsychiatry |54 Roleofneurobehavioralevaluation |55 Elementsofneurobehavioralevaluation |55 Riskandbenefitanalysis |56 V Limitsofneurobehavioralevaluation |57 Researchdirectionsforthefuture |57 ■PEDIATRICEPILEPSYSURGERY Chapter6:IntracranialEEGrecordingsandelectricalstimulation LauraTassi,PrasannaJayakar,TomPieper,PhilippeKahane |61 Generalindications |62 iEEGmethods:subdural/depth,SEEG,ECoG |63 Subduralelectrodesandcombinationofsubdural/depthelectrodes |63 Stereo-electro-encephalography(SEEG) |65 Intra-operativeelectro-corticography(ECoG) |68 Definingtheepileptogeniczone(EZ):spontaneousandprovoked |69 Ictalonsetzone |70 Irritative,continuousepileptiformdischarges,andfunctionaldeficitzones |72 Definingcriticalcortex:intra-operativeandextra-operative |72 Extra-operativeelectricalstimulation |72 Intra-operativeelectricalstimulation |75 Chapter7:Whyistheepilepsycaseconferenceimportant? ElaineWyllie,ImadNajm |79 Chapter8:Socio-economicaspectsandepilepsysurgeryinchildren GrahamFieggen,JoWilmshurst |83 Whatisrequired? |84 Developing(“resourcepoor”)countries |85 Africa |85 SouthAsia |87 China |88 MiddleEast |88 LatinAmerica |88 Developed(“resourceequipped”)countries |88 Specificpaediatricissues |89 SectionII.SEMIOLOGYINCHILDREN Editors:PhilippeKahane,J.HelenCross Chapter9:Thetemporallobe:Semiology AndrasFogarasi |95 Ictalsignsoftemporallobeseizures |95 Behavioralchange |95 Ictalemotionalsigns |96 Ictalmotorsigns |96 Autonomicsymptoms |97 Agedependencyofdifferentperi-ictalsigns |97 Etiologyandseizuresemiology |99 Lateralizingsignsinchildhoodtemporallobeseizures |99 Interobserveragreementonchildhoodseizuresemiology |100 FurtherpitfallsofTLEseizuresemiology |101 Chapter10:Thefrontallobe:Semiology AndrasFogarasi,IngridTuxhorn,PhilippeKahane |103 Frontallobefunctionalanatomyrelevanttoseizuresemiology |103 Clinicalpatternsoffrontallobeictalsemiology |104 Tonicseizures |104 Versiveseizures |104 Hypermotorseizures |106 Motorstereotypies |107 Autonomicseizure |107 Differentialdiagnosisandfrontallobeepilepsysyndromes |107 Dopediatricfrontallobeseizuresandepilepsydifferfromadults? |108 Chapter11:Theposteriorcortex:Semiology StefanoFrancione,RobertoMai |111 Semiologyofseizuresoriginatingfromtheparietallobe |113 Semiologyofseizuresoriginatingfromtheoccipitallobe |113 Particularfindingsinyoungchildren |114 Auras |115 Oculo-motormanifestations |116 Simplemotorandcomplexmotorbehaviours |117 VI EEGfeatures |118 Contents■ Chapter12:Theinsula:Semiology PetiaDimova |121 Insularlobesemiologyinchildren:isthereanyspecificity? |124 Auras |126 Vegetativesigns |126 Motormanifestations |126 SectionIII.ETIOLOGYANDEPILEPSYSURGERY Editors:HansHolthausen,AlexisArzimanoglou,PrasannaJayakar Chapter13:Focal(isolated)corticaldysplasiaTypeI HansHolthausen,PavelKrsˇek,IngmarBlümcke |133 HistologicalclassificationofFCDTypeI–workinprogress |133 ImaginginisolatedFCDTypeI |137 MRIinisolatedFCDTypeI |137 PETandSPECTinisolatedFCDTypeI |139 SeizuresinFCDTypeI |140 EEGinFCDTypeI |140 Presurgicalwork-upandepilepsysurgeryinFCDTypeI |140 Chapter14:FocalcorticaldysplasiaTypeII LauraTassi,IngmarBlümcke,DeepakGill |145 HistopathologicalandMRaspectsofFCDtypeII |146 Histopathologicalaspects |146 Neuroradiologicalmethodologyanddiagnosis |148 Clinicalandneurophysiologicalaspects |149 Clinicaldata |149 EEGandvideo-EEGfeatures |149 Ancillarytechniques |150 Invasivetechniquesandsurgicaloutcome |150 Invasivemonitoring:whenandwhy |150 Surgeryandoutcome |154 Chapter15:Othermalformationsofcorticaldevelopment CarmenBarba,FrankRitter,RenzoGuerrini,AngusA.Wilfong |157 Heterotopias |158 Subependymal(periventricular)nodularheterotopias |158 Subcorticalnodularheterotopias |159 Subcorticalbandheterotopias |161 Polymicrogyria |162 Chapter16:Tuberoussclerosis MichaelDuchowny,A.SimonHarvey,HowardWeiner |167 Clinicalpresentation |167 Surgicalreferralandpre-operativeassessment |168 IntracranialEEGrecordings |170 Surgicalapproaches |171 Outcome |173 Chapter17:Hemimegalencepahlyanddiffusehemisphericmalformations ofcorticaldevelopment ChristineBulteau-Peyrie,TaisukeOtsuki,OlivierDelalande |175 Specificepilepsysurgeryindications |175 Recommendedpresurgicalevaluation |177 Clinicalconsiderations |177 BrainMRI |177 InterictalandictalvideoEEG |178 Availableresults |178 Seizures |178 Contra-lateralhemiplegiaandhomonymouslateralhemianopia |180 Suggestedfollow-up |181 Chapter18:Benigntumors(WHOgradesIandII) HansHolthausen,BertilRydenhag,NandanYardi,IngmarBlümcke |185 VII Neuro-pathologicalclassificationofbraintumorsWHOgradeIandII frequentlyassociatedwithepilepsy(so-calledLEATs) |186 ■PEDIATRICEPILEPSYSURGERY MRIinpatientswithepilepsyinassociationwithbraintumors |187 Incidences,prevalences,frequenciesinpediatricbraintumors |190 Braintumorsandepileptogenicity |191 Medicaltherapy/AEDsinchildrenwithepilepsyinassociationwithbraintumors |191 Surgeryinchildrenwithseizuresassociatedwithbenigntumors |192 Tumor-surgeryvs.epilepsysurgeryandlesionectomyvs.tailoredresection |192 Radiotherapyandchemotherapyinpatientswithepilepsyandbenignsupratentorialbraintumors (WHOgradeIandII) |199 SurgeryinpatientswithepilepsyinassociationwithbraintumorsWHOgradeIIandhigher |200 Chapter19:Vascularcausesandperinatalhypoxic-ischemicevents Eeva-LiisaMetsähonkala,HansHolthausen,Yu-TzeNg,EijaGaily |207 Prenatalandperinatallesions |208 Arterialischemicstrokesandporencephaliccystsinchildren |208 Venousstrokeandcerebralsinovenousthrombosis |209 Intracranialhemorrhageandhemorrhagicstrokeinterminfants |209 Watershedlesionsandulegyriaduetohypoxicischemicevents |210 Vascularlesionsinpretermchildren |210 Seizuretypesandepilepsysyndromesinpre/perinatalvascularlesions |210 Presurgicalevaluationinpre/perinatalvascularlesions |211 Surgeryinpre-andperinatalvascularlesionsandlesionscausedbyhypoxicischemicevents |215 Postoperativeoutcome |217 Epilepsysurgeryinvascularlesionsoccurringinchildhood |220 Cavernomas |221 Hematologicaltestingforneurosurgery |223 Chapter20:Sturge-Webersyndrome AlexisArzimanoglou,EricKossoff |229 Clinicalpresentation |229 Diagnosticworkup |230 Medicalandsurgicaltreatment |231 Chapter21:Hypothalamichamartoma JohnF.Kerrigan,PhilippeKahane,MartineFohlen,AlexisArzimanoglou |239 History |239 Epidemiology |240 Genetics |240 Anatomicalaspects |241 Pathologyandpathophysiology |241 Clinical-EEGfeaturesandnaturalhistory |243 Gelasticanddacrysticseizures |244 Otherseizuretypes |244 Cognitionandbehaviour |245 Psychiatricsymptoms |246 HHandepilepsy:whichpartofthebrainisseizing? |247 Intrinsicepileptogenesis |247 Secondaryepileptogenesis |248 Therunning-downphenomenon |250 Presurgicalevaluation |251 Magneticresonance(MR)imaging |251 Electroencephalographyandvideo-EEGseizuremonitoring |251 Neuropsychologicalorneurodevelopmentaltesting |251 ElectrocorticographyandinvasiveEEGmonitoring |251 Treatment |252 Chapter22:Cerebello-pontinehamartoma A.SimonHarvey,AlexisArzimanoglou |261 Clinicalpresentation |261 Pathologicalandfunctionalconsiderations |262 Surgicaltreatmentandoutcomes |264 Chapter23:Epilepsiesfollowingcerebralinfections HansHolthausen,GeorgiaRamantani |267 Epilepsypost-viralencephalitis |270 Epilepsypost-herpessimplexvirusencephalitis |270 Epilepsiespost-bacterialmeningitis(EPBM) |275 Epilepsyinneurocysticercosis(NCC) |278 VIII Epilepsysurgeryinrarepost-infectionepilepsy |280 Contents■ Chapter24:Rasmussenencephalitis AdamL.Hartman,CynthiaF.Salorio |285 Historicalperspective |285 Diagnosis |285 Pathology |288 Treatment |288 Medicaltreatment |288 Surgery |289 Outcomes |290 Timingofsurgery |294 Post-surgicalmanagement |295 Directionsforfutureresearch |295 Chapter25:Post-traumaticepilepsy BrianJ.Dlouhy,MatthewD.Smyth,DavidD.Limbrick,Jr. |299 Epidemiology |300 Pathophysiology |301 Treatment |301 Chapter26:Mesialtemporallobeepilepsyinchildren PavelKrsˇek,MichaelDuchowny,ArthurCukiert |305 Pathologicalsubstratesofpediatrictemporallobeepilepsy |305 Riskfactorsforpediatrictemporallobeepilepsy |305 Roleofdualpathologyinpediatrictemporallobeepilepsy |307 Clinicalmanifestationsoftemporallobeepilepsyinchildren |309 EEGfeaturesofpediatrictemporallobeepilepsy |310 Surgicaloutcomeinchildrenwithhipocampalsclerosisanddualpathology |310 Timingofsurgeriesinrelationtoneuropsychologicaloutcome |310 Surgicaltechnique |311 Outcomeregardingseizures |311 Chapter27:EpilepsysurgeryinMRI-negativepatients ThomasBast,PhilippeKahane,PrasannaJayakar |315 MRI-negativefocalepilepsy |316 DifferencebetweenMRI-negativeandnon-lesionalepilepsy |316 Selectionofsurgicalcandidates |318 Presurgicaldiagnosticwork-up |319 OutcomeafterMRI-negativeepilepsysurgery |324 Chapter28:Landau-Kleffnersyndrome MariaClark,RebeccaGreenaway,BrianNeville |329 Background,clinicalpresentationanddifferentialdiagnosis |329 Multiplesubpialtransectionsofthedorsalsurfaceofthetemporalgyrusintothesylvianfissure |330 Mesialsubpialtransectionindications |330 Recommendedpresurgicalevaluation |331 Availableresults |331 Specificfollow-up |332 Newideasaboutaetiology |333 SectionIV:SURGICALTECHNIQUESFORTHENEUROLOGIST Editors:GaryMathern,WilliamD.Gaillard,AlexisArzimanoglou Chapter29:Lesionectomy ThomasBlauwblomme,WilliamHarkness,ChristianSainte-Rose |337 Patientselection |338 Generalprinciples |338 Anesthesiaprotocol |338 Imageguidance |339 Skin/bone/dura |339 Delineationofthelesion |339 Hemostasis/closure |340 Safety |340 Deepseatedlesion |340 Eloquentareas |340 Completeresectionboundaries |341 IX Lesion-specificconsiderations |341 Anatomicorelectrophysiologicallimits? 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