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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.Myelinsheathsplayimportanceroleinprotectingandsuppor
2、tingfornervousfiberandtransportingofmaterialtoneuronsandmaintainingofneurons’ionicenvironment.Theformationofmyelinsheathsinboththecentralandperipheralnervoussystemfollowsasimilarpattern.Centralmyelinisformedbyanextensionofmembraneoftheoligodendrocytewrappingtheaxons,wherea
3、speripheralmyelinisformedbytheextensionoftheSchwanncellmembrane.ThecommonestacceptedthepathologiccriteriaofdemyelinativediseaseinCNS:1.Destructionofthemyelinsheathsofnervefibers;2.Relativesparingofotherelementsofnervoustissue,i.e.ofaxiscylinders,nervecell,andsupportingstru
4、cture,andarelativelackofWallerian,orsecondary,degenerationofffibertracts.3.Aparticulardistributionoflesionsoftenisperivenousandprimarilyinwhitematter.ClassificationofdemyelinationdiseaseinCNSDysmyelinativetype(leukodystrophy)Itresultsfromfailuretoformnormallyconstitutedmye
5、lin,duetogeneticenzymaticdisorders,suchasadrenoleukodystrophy(ADL),Globodleucodystrophy(Krabbe),Metachromaticleucodystrophy(MLD)DemyelinatingtypeItisbreakdownofnormallyconstitutedmyelin.ClassificationsofthedemyelinationdiseaseMultiplesclerosis1.relapsing-remitting,RR2.prim
6、aryprogressive,PP3.secondaryprogressive,SP4.progressiverelapsing,PRNeuromyelitisoptica(Devic)Diffusecerebralsclerosis(Schilder)&concentricsclerosisofBaloAcutedisseminatedencephalomyelitisCentralpontinemyelinnolysisMultiplesclerosisMultiplesclerosisisthemostcommondemyelinat
7、ingdisorder.Itisadiseaseofthecentralwhitematterwith“lesionsseparatedintimeandspace”.Generally,MSischaracterizedclinicallybyremitting-relapsingcourse.Thediseaseusuallyoccursinyoungadults.Thepeakageofonsetis20-40and95%patientsoccursbetween10-60years.Morefemalesthanmalesareaf
8、fected.Etiologyandpathogenesis1.Geographicalinfluence:Populationsresidingbetweentropicala