多发性硬化.ppt

Casediscussion Departmentofneurology ward2 病例分析 1 定位诊断 右侧顶枕叶 99年8月出现双眼左侧视物不清 查体双眼左侧同向偏盲 视乳头水肿 提示视觉皮层受损 记忆力与计算力差 提示大脑皮层受损 结合头MRI右顶枕异常长T1 长T2信号 GD DTPA环形增强 颈髓 C3 C4 本次以左侧颈肩疼痛起病 提示左侧颈髓脊膜受累 左侧肢体活动无力 查体左肢近端肌力4 5 远端肌力5 5 左侧病理征阳性 考虑左侧锥体束病变 结合颈段MRI示C3 4颈髓左侧异常信号 病例分析 2 定性诊断 多发性硬化临床确诊 multiplesclerosis clinicaldefinite 女性患者 反复发作缓解病程 2次发作 先后出现局灶神经功能障碍2个病灶 右顶枕及颈髓 99年手术活检病理证实为活动性脱髓鞘改变 目前CSF蛋白增高 24小时IgG合成率升高 MBP升高 Consensusdefinitionsofsubgroupsofmultiplesclerosis Relapsing remittingmultiplesclerosisClearlydefineddiseaserelapseswithfullrecoveryorwithsequelaeandresidualdeficituponrecovery periodsbetweendiseaserelapsescharacterizedbyalackofdiseaseprogression SecondaryprogressivemultiplesclerosisInitialrelapsing remittingdiseasecoursefollowedbyprogressionwithorwithoutoccasionalrelapses minorremissionsorplateaus PrimaryprogressivemultiplesclerosisDiseaseprogressionfromonsetwithoccasionalplateausandtemporaryminorimprovementsallowed Progressive relapsingmultiplesclerosisProgressivediseasefromonset withclearacuterelapses withorwithoutfullrecovery TypeofMSPercentageofPatientsBenignMS10 Relapsing remittingMS40 Secondarychronic40 ofpatientswithoriginalprogressiverelapsing remittingMSPrimaryprogressiveMS10 Classification pathogenesis GeneticSusceptibility EpidemiologicstudieshaveidentifiedageneticsusceptibilitytoMS Thegeneticinfluenceappearstovaryamongdifferentpopulations AutoimmuneCauses InMSimmunedysfunctioncanbedetectedlocallyinCNS CSFaswellassystemicallyinperipheralcirculation ViralCauses Molecularmimicry DistributionofDemyelinatingPlaquesinCNS PlaquesweredemonstratedtooccuranywherewithinthewhitematteroftheCNS butthemostfrequentlyaffectedsitesaretheopticnerves thebrainstem thecerebellumandthespinalcord Lesionsintheselocationsoftencorrelatewithclinicalsymptoms Inthecerebralhemispheresperiventriculardistributionofplaquesisoftenseen Whenplaquesareadjacenttothecortex subcorticalmyelinatedfibersareoftenspared Plaqueslocatednearbythegraymattermayrarelyspreadintothegraymatter includingdeepnucleiandthecortex Thereoftenisaxonsparingwithintheplaque Clinicaldiagnosis Primarilyyoungadults withpeakageofonsetatabout30years itdoesoccurinchildrenandadolescents 3 to5 andpersonsovertheageof50years 9 ThecriteriadesighedbyPoserin1983establishtwomajorgroups definiteMSandprobableMS eachwithtwosubgroups clinicalandlaboratorysupported Paraclinicalevidenceincludesevokedpotentials CSFanalysis CT orMRIdata MRIisbyfarthebestparaclinicaltest depictingabnormalitiesin95 ofpatientswithclinicallydefiniteMS DiagnosticMRIcriteriacommonlyusedformultiplesclerosis BiopsydiagnosisofMS Histologicfeatures activedemyelinatingprocessperivenularcuffsoflymphocytesandplasmacells sheetsoffoamymacrophagescontainingintrocytoplasmicneutrallipids myelindebris Reactivemononucleatedandmultinucleatedastrocytes Largeprotoplasmicglialcellswithfragmentedchromatin Creutzfeldtcells Myelinabsent axonrelativelypreserved Neuroimagingandpathology Diffuselyorringenhanceinglesionsarecharacterizedbyhypercellularity thepresenceofmacrophages extensivelossofmyelin occasionallywithedema withorwithoutcystformation Nonenhancingdemyelinatinglesionsarecharacterizedbyfibrillaryastrocytosis withfewmacrophagesandfewornoinflammatorycellsofothertypes Activedemyelinatingprocessvsmalignantglioma Difficultpointstodifferentiateactivedemyelinatingprocessesfromgliomas hypercellularitypleomorphicastrocytes includinglargeCreutzfeldtcells Mitoticfigures Necrosiscysticchanges Activedemyelinatingprocessvsmalignantglioma Histologicfeturesfavoringdiagnosisofareactiveprocess