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Idiopathic epilepsy syndromes DRAFT...

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1 Idiopathic epilepsy syndromes PANISRA SUDACHAN, M.D. Pediatric Neurologist Pediatric Neurology Department Prasat Neurological Institue Epilepsy course 26 August 2017 Unknown Immune Infectious Structural Etiology Metabolic Genetic Epilepsy types Focal Generalized Combined Generalized & Focal Unknown Focal Epilepsy Syndromes Seizure types Generalized onset Unknown onset Focal onset Classification 1964 1970 1981 1989 2001 2006 2010 2013 2017 Non epileptic events Semiology & EEG Finding
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Page 1: Idiopathic epilepsy syndromes DRAFT 2017thaiepilepsysociety.com/.../Idiopathic-epilepsy-syndromes_DRAFT1_2017.pdf · Low voltage SPK and Gen d/c minority of cases. Activation: Eye

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Idiopathicepilepsysyndromes

PANISRASUDACHAN,M.D.PediatricNeurologistPediatricNeurologyDepartmentPrasat NeurologicalInstitue

Epilepsycourse

26August2017

Unknown

Immune

Infectious

Structural

Etiology

Metabolic

Genetic

Epilepsytypes

Focal GeneralizedCombinedGeneralized&Focal

UnknownFocal

EpilepsySyndromes

SeizuretypesGeneralized

onsetUnknownonset

Focalonset

Classification

• 1964

• 1970

• 1981

• 1989

• 2001

• 2006

• 2010

• 2013

• 2017

Nonepilepticevents

Semiology&

EEGFinding

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Identifyofepilepsysyndromesbasedon:

• Typicalageonset

• Seizuretypes

• specificEEG characteristics

• Otherfeaturesorrelatedsymptoms

• Implicationsfortreatment,management,andprognosis

3

no approvedILAEepilepsysyndromes

https://www.epilepsydiagnosis.org

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EasyguideforEpilepsysyndrome“Patterndiagnosis”

SeizuresEpilepsy

Epilepsysyndrome

Development

Ageofonset

Seizuretypes

IEDs

Testing:GeneticMRI

Epilepsysyndrome

Age

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Idiopathicepilepsysyndromes

•Asyndromicthatisonlyepilepsy,withnounderlyingstructuralbrainlesionorotherneurologicalsignsorsymptoms.Thesearepresumedtobegeneticandareusuallyage-dependent.

IdiopathicEpilepsySyndromes

§ Idiopathic/Genetic GeneralizedEpilepsy

§ Idiopathic/Self-LimitedFocalEpilepsy

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‘Idiopathic/GeneticGeneralizedEpilepsies’

ChildhoodAbsenceEpilepsy

GeneralizedTonic-Clonic

SeizuresAlone

JuvenileAbsenceEpilepsy

JuvenileMyoclonicEpilepsy

ChildhoodabsenceepilepsyAgeofonset 2-12years(peak5-6years)

Seizuretype Absenceonly (multipledaily,brief,LOA)

EEGIEDS:3HzGeneralizedspikesandwaves;Normalbackground,OIRDA

Tests:Genetic SLC2A1,GABRG2andCACNA1A

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Childhoodabsenceepilepsy

O1

O2

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1SEC

Generalized3-Hz-spike-and-waves

JuvenileabsenceepilepsyAgeofonset 8-20years(peak9-13years)

SeizuretypeAbsence(notfrequent,notsevere,awareness)GTCatonset->AbsenceinadolescentGCs(80%ofcases,uponawakening)

EEGIEDS:3-6HzGeneralizedspikes/polyspikes andwaves,normalbackground,OIRDA(may)Ictal:Absence:Regular3-6HzGSWorPSW

GCs :EEGobscurebyartifact,generalizedfastrhythmicspikes-tonicphase,spikeandslowwavesandpostictalperiodslowing

Tests:Genetic GABRG2,CACNA1A andothers

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JAE-EEG

Juvenileabsenceepilepsy

JuvenilemyoclonicepilepsyAgeofonset 8-25years(peak9-13years) 5%ofcasesfromCAE

SeizuretypeMyoclonic (mandatory),especiallyonawakening(within30min-1hr)GTCs (>90%)precededbyseriesofmyoclonic,Absence (1/3ofcases,briefer<3seconds)

EEGIEDS:3.5-6HzGSW/PSW,normalbackground,fragmentshyperventilation mayprovokedabsence,<10%szinducedbyvisualstimuliIctal:singlegeneralizedPSWcorrelateswithmyoclonicseizures

Tests:GeneticComplexorMendelianCACNB4,GABRA1,CLCN2,GABRDandEFHC1,Microdeletions,suchasthe15q13.3microdeletion andothers

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EpilepsywithGTCsaloneAgeofonset 5-40years(peak11-23years)

SeizuretypeGCsespecially onawakening(within1-2hr ofwakening)infrequent,typicallyprovokedbysleepdeprivation,PHofchildhoodabsenceepilepsy

EEGIEDS:GSW/PSW(½ofcasesseenonlyduringsleep)fragmented,intermittentphotoparoxysmal response,normalBG(noslowing)Ictal:GCs:IctalEEGpatterns

Tests:Genetic complex inheritance,CLCN2andothers.

‘Self-LimitedFocalEpilepsies’

BenignEpilepsywithCentroTemporalSpikes(BECTS)

Self-limitedfrontal/temporal/parietallobeepilepsies

Self-LimitedOccipitalEpilepsyofChildhood:Panayiotopoulostype

(early-onset):PS

COE-GGastaut type(lateonset)

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BenignChildhoodEpilepsywithCentrotemporal Spikes

Ageofonset 3-14years(peak8-9years)

Seizuretype

Fronto-parietalopercular features–hemifacial(lip,mouth andtongue),clonic movements(withmaybeunilateral),laryngealsymptoms,articulardifficulty(aphasia),swallowing orchewingmovementsandhypersalivation,brief(<5minutes),Few,(may)secondarilygeneralize(typicallynocturnal events) (notGTCduringawake)Self-limited usuallyresolvedbyage13years(occasionallyoccuruptoage18years)

BenignChildhoodEpilepsywithCentrotemporal Spikes

EEG

IEDs:Highamp.Centrotemporal SpikesorSharp-and-slowwavecomplexes,max.negativityinCT(C3/C4andT3/T4)andmax.positivityF,increasedduringdrowsinessandsleep,unilat orbilat,(may)SPKoutsideCTregion(midline,parietal,frontalandoccipital), (may)photoresponsive (age.10yrs),10-20%-bysensorystimulioffingersortoesIctal:rare toobtainedictalrecording

Tests:Genetic Complexinheritance,GRIN2Agene

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BenignChildhoodEpilepsywithCentrotemporal Spikes

BenignChildhoodEpilepsywithCentrotemporalSpikes

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BenignChildhoodEpilepsywithCentrotemporal Spikes

BenignChildhoodEpilepsywithCentrotemporal Spikes

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PanayiotopoulossyndromeAgeofonset 1-14years(peak3-6years)

Self-limiting,resolvebyage11-13years

Seizuretype Autonomicfeaturesmainlyemetic (nausea,retching,vomiting),pupillary (mydriasis),circulatory (pallor,cyanosis),heartandrespiratorychange.Apnea andasystolecanoccur(severecase).Prolongduration,butwithoutresidualneurodeficit,someofcase- fronto-parietalopercular (25%mayautonomicSE),infrequent

Tests:Genetic unknowngene,complex(reportinsibling)

Panayiotopoulossyndrome

EEG

IEDs::MultifocalSPK/SW90%NormalsingleEEG10%Occipitalspikes 60%ofpatientsLowvoltageSPKandGend/cminorityofcases.

Activation:Eyeclosure(eliminationofcentralvisionandfixationoffsensitivity)mayactivateoccipitalspikes.EEGabnormalityisenhancedbysleepdeprivationandbysleepIctal:Unilateral,oftenposterioronset,withrhythmicslow(thetaordelta)activityintermixedwithsmallspikes

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Panayiotopoulossyndrome

PS:EEGOccipitalSpikes

Panayiotopoulossyndrome

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Lateonsetchildhoodoccipitalepilepsy(Gastaut type)Ageofonset 5months-19years(peak8-9years)

Seizuretype

Seizureswithvisualauraoccurfromawakestates,brief(typicalseconds,most<3minutes,rarelyupto20minutesVisualaura;multi-coloredcirclesinperipheralvisionincreasedinvolvedandmovinghorizontallytotheotherside,thesemaybefollowedbydeviationofeyesorheadturning(ipsilateral)May Otheroccipitalfeatures;ictalblindness,complexvisualhallucinations,visualillusions,orbitalpain,eyelidflutteringorrepetitiveeyeclosure,ictalheadacheorN/VMay spreadoutsidetheoccipitallobe resultinginhemiparesthesia,dyscognitivefeatures,hemiclonic

Lateonsetchildhoodoccipitalepilepsy(Gastaut type)

EEG

IEDs:Occipitalspikes orSW (may)onlyduringsleep,20%ofcasesmayco-existwithCT,frontalorGSW,BGnormalActivation:bysleepdeprivationandbysleep,20-90%ofcases–inducedbyfixation-offsensitivity(eliminationofcentralvision)Ictal: duringoculo-clonic seizureorictalblindness:BGactivityreductionandthenoccipitalfasterrhythmswithspikesoflowamplitude,thesemaybeslowerSW

Tests:Genetic Unknown

Prognosis Self-limitingEasilycontrolled(50-60%remissionin2-4yearsafteronset)90%dramaticresponsetocarbamazepine

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SummaryofIGE

IGE CAE JAE JME GTCSA

Age onset childhood Juvenile Juvenile Juvenile

Seizuretype Absence AbsenceGTCs

MyoclonicGTCs,Absence

GTCs

EEG 3HzGSW 3-6Hz GSW 3.5-6Hz GSW GSW/PSW

SummaryofSFE

SFE PS BECTS COE-G

Age onset Infantile1-14(3-6)yrs

Childhood Childhood

Seizuretype Autonomic(Emetic)

Perisylvian Occipital

EEG Multifocal 90%Occipital60%

Centrotemporal Occipital


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