MESIAL TEMPORAL SCLEROSIS - nan.com.np · • Mesial temporal sclerosis in epilepsy: Vincent R....
Transcript of MESIAL TEMPORAL SCLEROSIS - nan.com.np · • Mesial temporal sclerosis in epilepsy: Vincent R....
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MESIALTEMPORALSCLEROSIS
DrVijayKAggarwalConsultantRadiologist-MRISpecialistNorvic Hospital
ClinicalFellowshipNeuroradiology,USAClinicalFellowshipBodyMRI,USA
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Learningobjectives
1.ToreviewtheMRanatomyofthetemporallobe,especiallymedialregion.
2.TodiscussoptimalMRItemporallobeprotocol.
3.TodescribeprimaryaswellassecondarymagneticresonancefeaturesofMTS.
4.TodiscusstheroleofMRspectroscopyandfunctionalimagingmethodsinthepre-surgicalworkupoftemporallobeepilepsy(TLE).
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Temporallobeepilepsy
Causes:• Mesialtemporalsclerosis (MTS):~70%• Temporallobetumours:~10%MCGanglioglioma.• Corticaldysplasia:5-10%• Vascularmalformations:~5%• Trauma• Infection• Congenital• Temporalpoleencephalocele - Rare
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CASE
• 25-year-male
• Hadexperiencedcomplexpartialseizuressincechildhood.
• Seizures,whichfeaturedautomatismsanddystonicposturing,hadstartedfollowingafebrileillnesswithaprolongedseizurewhenhewasfouryearsofage.
• Unsuccessfulcontrolofseizuresusingmultipleantiepilepticdrugsresultedinweeklyseizures,renderinghimunabletodrive.
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Anatomy
• HippocampusisaprominentC-shapedstructurebulginginthefloorofthetemporalhornofthelateralventricle.
• Consistsofcornuammonis(CA1-CA4).
• Otherregionsthattogethercomprisethehippocampalformation consistofthedentategyrus,thesubicularcomplex,andtheentorhinalcortex.
• Basedonitsextrinsicconnectivity,thehippocampalformationreceivesavastamountofhighlyprocessedmultimodalsensoryinformationthatisfunneledintothehippocampalformationmainlybytheentorhinalcortex.
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Anatomy
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MRImaginganatomyoftemporallobe
CoronalT2-weighted(A)andsagittalT13-dimensionalinversionrecovery(B)images,showingmesialtemporallobestructures:sylvianfissure(1);superior(2),medial(3),inferior(4)temporalgyri;parahippocampalgyrus(5);collateralwhitematter(6);uncus(U);amygdala(A);andhead(H),body(B),andtail(T)ofthehippocampus.
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MTS
• Characterizedbypatternofneuronallosswithinthehippocampusaffectingprincipallythepyramidalcelllayersofthecornuammonis(CA)andthegranulelayerofthedentategyrus.
• Changesinclude:Ø SelectivelossofinhibitoryinterneuronsØ AbnormalspoutingofaxonsØ ReorganizationofneuraltransmitterreceptorsØ AlterationsinsecondmessengersystemsØ Hyperexcitabilityofthegranulecells.
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FigA:Normalhippocampushistopath.FigB:SurgicalspecimenshowalmostcompletelossofneuronsfromtheCA1sectorofthehippocampalcortexaswellasfromCA3andCA4.ThereisrelativepreservationofneuronalnumbersinCA2.Thereisalsodepletionofneuronsfromthedentate.Theneuronaldepletionisaccompaniedbymoderateastrocyticgliosis.Thefeaturesareofhippocampalsclerosis.
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• Hippocampalformationisnotuniformlyaffected,withthedentategyrus,andtheCA1,CA4andtoalesserdegreeCA3sectionsofthehippocampusbeingprimarilyinvolved.
• Histologicallythereisneuronalcellloss,gliosisandsclerosis.
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Pathophysiology
• Notcompletelyunderstood.
• Itispostulatedthataninsulttothedevelopingbrainduringchildhood, suchasacomplicatedfebrileseizureorencephalitis,damagesthedentateinterneuronsystem.
• Thedamageddentategyrusbecomesreorganized,leadingtoanaberranthyperexcitablesynapticsystem.
• Thisisclinicallymanifestedasrecurrentseizures,orepilepsy
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Lateraldiagramofthecircuitryassociatedwiththehippocampus.
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Clinical Investigations
• MRIisthemodalityofchoicetoevaluatethehippocampus,howeverdedicatedTLEprotocolneedstobeperformedifgoodsensitivityandspecificityistobeachieved.
• Thinsectionobliquecoronalsequencesatrightanglestothelongitudinalaxisofthehippocampusarerequired,tominimizevolumeaveraging.
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EpilepsyProtocolMRI
RoutineMRimagingincludes:
• T1W,T2W,FLAIR,DiffusionweightedandSWIsequences inaxialplanewith5mmslicethickness.
• T1Inversion recovery(IR),FLAIRandT2Woblique coronal imagescoveringwholebrainareacquired.
• Obliquecoronalplane isperpendicular tothelongaxisofhippocampus ortheparahippocampal convolution.
• Forhippocampal volumetry, aObliquecoronal threedimensionalgradientechosequenceslicethickness0.85mm, interslicegap1.3mm)isobtained perpendicular tolongaxisofhippocampus ortheparahippocampalconvolution(onlyinselected cases).
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MR features of hippocampal sclerosis
Primarysigns:1.Smallatrophicunilateralhippocampus.2.HyperintensityonT2W/FLAIRimages.3.Lossofthehippocampalinternalarchitectureandthatof
normaldigitationsofthehead.
Ø Oftenmentioned,butprobablyoneoftheleastspecificfindings,isenlargementofthetemporalhornofthelateralventricle.Ifanything,caremustbetakennottoallowanasymmetricenlargedhorntotrickyouintothinkingthehippocampusisreducedinsize.
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DiagramofacoronalT1-weightedMRimageshowingclassicfindingsassociatedwithmesial temporalsclerosis.
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MR features of hippocampal sclerosis. Primary signs: 1.Small left atrophic unilateral hippocampus. 2. Hyperintensity on T2W images. 3. Loss of the hippocampal internal architecture and that of normal digitations of the head.
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RightmesialtemporalsclerosisoncoronalobliqueT2W
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BilateralmesialtemporalsclerosiscoronalobliqueT2W
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Whensevereandlongstanding,additionalassociatedfindingsinclude:
Ø Atrophyoftheipsilateralfornixandmamillarybody.
Ø Increasedsignalandoratrophyoftheanteriorthalamicnucleus
Ø Atrophyofthecingulategyrus
Ø Increasedsignaland/orreductioninthevolumeoftheamygdala.
Ø Dilatationoftemporalhornandtemporallobeatrophy
SecondarySigns
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SecondarySignsØ Collateralwhitematterandentorhinalcortexatrophy
Ø Thalamicandcaudateatrophy
Ø Ipsilateralcerebralhypertrophy
Ø Contralateralcerebellarhemiatrophy
Ø Reducedwhitemattervolumeintheparahippocampalgyrus.
Ø Reductioninthevolumeofthesubiculum
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Secondarysigns:1. Atrophyofcaudate2. Atrophyofthalamus3. Atrophyoffornix4. UnilateralatrophyofRtemporallobe5. AtrophyofRmamillary body6. Dilatedtemporalhorn
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CoronalobliqueT2-Wshowingsecondarysignsofmesialtemporalsclerosis
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T1IR-atrophywithhypointensityoftheleftHippocampus(arrow).
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AxialT2-weightedFLAIRimageshowshyperintensityoftherighthippocampuswithvolumeloss(whitearrows),characteristicofrightmesialtemporalsclerosis.
Mesialtemporalsclerosisinepilepsy:VincentR.Spano,BMSc(H)andDavidJ.Mikulis,MD
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CoronalobliqueFLAIRofptwithpersistentrefractoryseizures,after rightselectivetranscorticalamygdalohippocampectomy(*).Noterighthippocampus(arrow)isatrophicandshowshyperintensesignalcompatiblewithsclerosis,atrophyoftherightfornix.
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3yearsmalewithleftMTSandleftMBatrophy
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13yearsfemalewithrightMTSatrophyofrightMB
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18yearsmalewithleftMTS
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51yearsfemalewithleftMTSandleftMBatrophy
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MRSpectroscopy
• N-acetylaspartate(NAA)occursinneuronsbutnotinmatureglialcells.
• Thus,itisconsideredamarkerofneuronalabundanceorfunction.
• Incomparison,creatineactivityandcholineactivityareassociatedmorewithglialcellsthanwithneurons.
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MR Spectroscopy
• Findingstypicallyrepresentneuronaldysfunction:
Ø DecreasedNAAØ DecreasedNAA/ChoandNAA/CrratiosØ Increasedlipidandlactatesoonafterasseizure
Ø ThecorrespondencebetweenthedecreasedNAA:creatineratioandthesideofMTSorEEG-detectedseizureonset(EEGseizureonset)isashighas90%intemporallobeepilepsy.
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MRPerfusion
• DemonstratessimilarchangestoSPECTwithbloodperfusiondependingonwhenthescanisobtained.
Ø Ictalscan:Hyperperfusion.Ø Interictalscan:Hypoperfusion.
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SPECTscaninapatientpresentingwithepilepsyshowinghypoperfusion inrighttemporalregioninbothinterictalandictalphases.
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BloodoxygenationleveldependentBOLDfunctionalMRI
• ClinicalfMRIisbasedonbloodoxygenationlevel-dependent(BOLD)contrast.
• BOLDsignalresponsearisesfromlocalizedhemodynamicchangesinducedbyregionallyincreasedneuronalactivityassociatedwithprocessingastimulusorperformingacognitivetaskdefinedbytheparadigm.
• BOLDfMRIisahighspatialresolutiontechniquewithoutionizingradiationthatmapsphysiologicandmetabolicconsequencesofalteredelectricalactivityinthebrain.
• fMRIhasthepotentialtopredictthepossibledeficitsinlanguage,andinvisual,motor,andsensoryfunctionsthatwouldarisefromthesurgicalintervention.
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A16-year-oldwithcorticaldysplasia(thickarrow)involvingprecentralgyrus ontheleftside.
Real-timefMRIobtainedafterrightfingertappingvsrestshowsactivationofprimaryhandmotorarea(thinarrow)placedclosetothelesion.
Ifresectionextendstoprimaryhandmotorarea,thepatientislikelytodeveloppostprocedureneurologicdeficit.
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Conclusion1.MRI formsthemainstayforstructuralandfunctional
neuroimaginginpatientswithepilepsy.
2.TheMRIdemonstrationofalesionverymuchhelpsinfurtherpresurgicalevaluation.InpatientswithnegativeMRI,PETandSPECTmaybeusedascomplimentarytoolsinthepresurgicalworkup.
3.Neuroimaginghashelpedtounderstandthepathophysiologyofepilepsybetterandalsotoprognosticatetheoutcomeofmedicalandsurgicaltreatments.
4.Advancementsinneuroimaginghavealsoprovidednoninvasivetoolstodetecttheepileptogenicfocus.
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References
• Mesialtemporalsclerosisinepilepsy:VincentR.Spano,BMSc(H)andDavidJ.Mikulis,MD
• MagneticResonanceImagingofMesialTemporalSclerosis(MTS):Whatradiologistsoughttoknow? P.Singh1,G.Mittal2,R.Kaur2,K.Saggar1;
• Anatomyofthehippocampalformation.SchultzC1,EngelhardtM
• MRofMesialTemporalSclerosis:HowMuchIsEnough?RichardBronen,YaleUniversitySchoolofMedicine,NewHaven,Conn
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THANKYOU