脱髓鞘幻灯2009医大课件.ppt

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1、DemyelinatingdiseaseoftheCNS中枢神经系统脱髓鞘疾病TianjinmedicalUniversityGeneralhospitalZhouGuangxi天津医科大学总医院神经内科周广喜7702OverviewDemyelinativediseaseisagroupofdiseasesofbrainandspinalcordinwhichdestructionofmyelinisaprominentfeature.Myelinsheathsplayimportanceroleinprotectingandsupportingfornervousfiberandtran

2、sportingofmaterialtoneuronsandmaintainingofneurons’ionicenvironment.Theformationofmyelinsheathsinboththecentralandperipheralnervoussystemfollowsasimilarpattern.Centralmyelinisformedbyanextensionofmembraneoftheoligodendrocytewrappingtheaxons,whereasperipheralmyelinisformedbytheextensionoftheSchwannc

3、ellmembrane.ThecommonestacceptedthepathologiccriteriaofdemyelinativediseaseinCNS:1.Destructionofthemyelinsheathsofnervefibers;2.Relativesparingofotherelementsofnervoustissue,i.e.ofaxiscylinders,nervecell,andsupportingstructure,andarelativelackofWallerian,orsecondary,degenerationofffibertracts.3.Apa

4、rticulardistributionoflesionsoftenisperivenousandprimarilyinwhitematter.ClassificationofdemyelinationdiseaseinCNSDysmyelinativetype(leukodystrophy)Itresultsfromfailuretoformnormallyconstitutedmyelin,duetogeneticenzymaticdisorders,suchasadrenoleukodystrophy(ADL),Globodleucodystrophy(Krabbe),Metachro

5、maticleucodystrophy(MLD)DemyelinatingtypeItisbreakdownofnormallyconstitutedmyelin.ClassificationsofthedemyelinationdiseaseMultiplesclerosis1.relapsing-remitting,RR2.primaryprogressive,PP3.secondaryprogressive,SP4.progressiverelapsing,PRNeuromyelitisoptica(Devic)Diffusecerebralsclerosis(Schilder)&co

6、ncentricsclerosisofBaloAcutedisseminatedencephalomyelitisCentralpontinemyelinnolysisMultiplesclerosisMultiplesclerosisisthemostcommondemyelinatingdisorder.Itisadiseaseofthecentralwhitematterwith“lesionsseparatedintimeandspace”.Generally,MSischaracterizedclinicallybyremitting-relapsingcourse.Thedise

7、aseusuallyoccursinyoungadults.Thepeakageofonsetis20-40and95%patientsoccursbetween10-60years.Morefemalesthanmalesareaffected.Etiologyandpathogenesis1.Geographicalinfluence:Populationsresidingbetweentropicala