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1、中枢神经系统脱髓鞘疾病DemyelinatingDiseasesoftheCentral NervousSystemChapter1Intraduction1.ConceptAgroupofdiseasesofthebrainandspinalcordinwhichdemyelinationisaprominentfeature.2.PathologicFindings①Destructionofthemyelinsheathsofnerves;②Infiltrationofinflammatorycellsinaperivasculardistributi
2、on;③Aparticulardistributionoflesion,oftenperivenousandprimarilyinwhitematter,eitherinmultiplesmalldisseminatedfociorinlargerfoci;④Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian,thesecondarydegenerationoffibertracts.多发性硬化MultipleSclerosis1.ConceptMultipleScleros
3、isisakindofautoimmunediseasescharacterizedbydemyelinationofCNS.Duetoitshighincidence,chronicityandtendencytoattackyoungadults,ithasbecomeoneofthemostimportantdiseasesofCNS.2.EtiologyAndPathogenesis1)病毒感染及自身免疫反应：麻疹病毒、人类噬T淋巴细胞病毒(HTLV-I)分子模拟、细胞免疫、体液免疫(1,2,3,4)2)遗传因素(inheritedfactor)3)
4、环境因素(environment)(4)3.EpidemiologyIncidenceofMSassociatedwithlatitude.ContactingwithsomekindofenvironmentmayplayanimportantroleintheincidenceofMS.Hereditymaybeanimportantfactor.MSassociatedwiththeHLA-DRlocusonthesixthchromosome,HLA-DR2expressstronglyandthen-DR3,B7andA3.4.Pathologic
5、FindingsCharacteristic:MultipledemyelinatedplaquesinwhitematterofCNS.Position:Whitematteraroundthelateralventriclesandspinalcord,opticnerve,brainstemandcerebellar. Acutestage:hyperemia,ondema,demyelination,infiltrationofinflammatorycellsinperivasculardistribution.Recoverystage:Astr
6、ocyteproliferition,formingofastrocyticscab.小脑及桥脑脱髓鞘病灶桥脑脱髓鞘病灶5.ClinicalManifestations1)Prodrome:Thesymptomsevolvedmoreslowly,overseveralweeksormonths.2)AcuteorsubacuteonsetRelapsing-remitting.3)Earlysymptomsandsigns:①Weaknessornumbness,sometimesboth,inoneormorelimbsistheinitialsympt
7、ominabouthalfofthepatients;②Thevisuallossinoneorbotheyes;③Nystagmus(fig1)andpalsyofeyemuscles(internuclearophthalmoplegia,oneandahalfsyndrome)④Impairmentofotherbrainnerves;⑤Ataxia:Charcot’ssyndrom⑥Sensationdisorder:Romberg’ssign,Lhermitte’ssign;⑦Attacksyndrom⑧Otherclinicalfeature6.
8、Laboratoryandotherassistan
