中枢神经系统脱髓鞘疾病英文课件

中枢神经系统脱髓鞘疾病

DemyelinatingDiseasesoftheCentral

NervousSystem

哈医大二院神经科中枢神经系统脱髓鞘疾病

Demyelinating1

Chapter1

Intraduction1.Concept

Agroupofdiseasesofthebrainandspinalcordinwhichdemyelinationisaprominentfeature.Chapter1Intraducti22.PathologicFindings①

Destructionofthemyelinsheathsofnerves;②Infiltrationofinflammatorycellsinaperivasculardistribution;中枢神经系统脱髓鞘疾病英文课件3③Aparticulardistributionoflesion,oftenperivenousandprimarilyinwhitematter,eitherinmultiplesmalldisseminatedfociorinlargerfoci;④Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian,thesecondarydegenerationoffibertracts.③Aparticulardistributionof4中枢神经系统脱髓鞘疾病英文课件5中枢神经系统脱髓鞘疾病英文课件6多发性硬化

MultipleSclerosis

1.ConceptMultipleSclerosisisakindofautoimmunediseasescharacterizedbydemyelinationofCNS.Duetoitshighincidence,chronicityandtendencytoattackyoungadults,ithasbecomeoneofthemostimportantdiseasesofCNS.多发性硬化

MultipleSclerosis

1.Co72.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)环境因素(environment)(4)2.EtiologyAndPathogenesis1)83.EpidemiologyIncidenceofMSassociatedwithlatitude.ContactingwithsomekindofenvironmentmayplayanimportantroleintheincidenceofMS.3.EpidemiologyIncidenceofM9Hereditymaybeanimportantfactor.MSassociatedwiththeHLA-DRlocusonthesixthchromosome,HLA-DR2expressstronglyandthen-DR3,

B7andA3.Hereditymaybeanimporta104.

PathologicFindings

Characteristic:MultipledemyelinatedplaquesinwhitematterofCNS.

Position:Whitematteraroundthelateralventriclesandspinalcord,opticnerve,brainstemandcerebellar.

Acutestage:hyperemia,ondema,demyelination,

infiltrationofinflammatorycellsinperivasculardistribution.Recoverystage:Astrocyteproliferition,formingofastrocyticscab.

4.PathologicFindings

Charact11中枢神经系统脱髓鞘疾病英文课件12小脑及桥脑脱髓鞘病灶小脑及桥脑脱髓鞘病灶13桥脑脱髓鞘病灶桥脑脱髓鞘病灶145.ClinicalManifestations1)

Prodrome:Thesymptomsevolvedmoreslowly,overseveralweeksormonths.2)AcuteorsubacuteonsetRelapsing-remitting.3)Earlysymptomsandsigns:

①Weaknessornumbness,sometimesboth,inoneormorelimbsistheinitialsymptominabouthalfofthepatients;

5.ClinicalManifestations1)P15②

Thevisuallossinoneorbotheyes;③Nystagmus(fig1)andpalsyofeyemuscles(internuclearophthalmoplegia,

oneandahalfsyndrome)④Impairmentofotherbrainnerves;⑤Ataxia:Charcot’ssyndrom⑥Sensationdisorder:Romberg’ssign,Lhermitte’ssign;⑦

Attacksyndrom⑧Otherclinicalfeature②Thevisuallossinoneorbo16中枢神经系统脱髓鞘疾病英文课件176.LaboratoryandotherassistantTests1.CSFTest1)NumberofMNC<15106/L2)IgG-Index>0.7;oligoclonalbands(OB)positive;3)MBP,PLP,MAG,MOGAbsandAb-secretingcells4)CSF-Alb/serum-Alb>1.7(probabilityofMS)6.Laboratoryandotherassista182.Evokedpotentials:

①visualevokedpotentials(VEP);②brainstemauditoryevokedpotentials(BAEP);③somatosensoryevokedpotentials(SEP).3.MRI:

①preiventricularplaques;②regularplaquesinbrainstem,cerebellumandspinalcord;③atrophysymptom.(1,2,3,4,5,6)2.Evokedpotentials:19中枢神经系统脱髓鞘疾病英文课件20中枢神经系统脱髓鞘疾病英文课件21

Diagnosticcriteria1.ClinicaldefiniteMS(CDMS):

①twotimesofattackandtwolesions;②twoattacks,onelesionandonesubclinicalevidence;2.LaboratorysupporteddefiniteMS(LSDMS):

①Twoattacks,onesubclinicalevidenceandCSF/OB/IgG;②Oneattack,twolesionsandCSFOB/IgG;③Oneattack,onelesion,onesubclinicalevidenceandCSFOB/IgG;

Diagnosticcriteria1.223.ClinicalprobableMS(CPMS):

①twoattacks,onelesion;②oneattack,twolesions;③oneattack,onelesionandothersubclinicalevidence;4.LaboratorysupportedprobableMS(LSPMS)

Twoattacks;CSFOB/IgG;TwoattacksinvolvingdifferentpartofCNS,intermissionatlestonemonth;eachattackmustcontinuefor24hs.3.ClinicalprobableMS(CPMS23DifferentialDiagnosis1.

急性播散性脑脊髓炎

2.

脑动脉炎、脑干炎、脊髓血管畸形3.颈椎病脊髓型

4.热带痉挛性截瘫

5.大脑淋巴瘤DifferentialDiagnosis1.急性播散性24

Treatment目前尚无一种特效疗法，治疗的主要目的是：1.急性活动期抑制其炎症性脱髓鞘过程，遏止病情的进展。

2.尽量预防能促发的外因，减少复发次数，延长缓解间歇期。

3.预防并发症。4.对症及支持疗法。

Treatment目前尚无一种特效疗法，治疗的主要目251.Relapsing-RemittingMS：

①ACTHandSteoids：methylprenisolone,prednison,dexamethasone;②IFN-;③Azathioprine；④Immuneglublin（Ig).

中枢神经系统脱髓鞘疾病英文课件262.ProgressiveMS：

①Methotrexate,MTX;

②Cyclosphoamide;③CyclosporineA;④Plasmatransplantation.

3.

对症治疗及预防感染避免疲劳：如过度劳累、紧张、疫苗接种、妊娠、分娩等。

2.ProgressiveMS：

①Met27视神经脊髓炎

(Neuromyelitisoptica,NMO)1.Introduction：AcuteorsubacutedemyelinatingdiseaseinvolvedopticNandspinalcordatthesametime.2.EtiologyAndPathogenesis：与遗传素质和种族差异有关。西方-脑干；东方-视神经和脊髓；25%MS突发球后视神经炎；多方研究证实白种人对MS易感；非白种人对NMO易感。3.Pathology：Demyelination,plaqueofsclerosis,

infiltriationofinflammatorycellsinperivasculardistribution.

视神经、视交叉及胸颈段易受累。视神经脊髓炎

(Neuromyeli28中枢神经系统脱髓鞘疾病英文课件29

颈髓脱髓鞘

视神经炎

30中枢神经系统脱髓鞘疾病英文课件31ClinicalManifestations1、年轻居多，21-41岁。2、特征：急性横贯性脊髓炎和双侧同时或相继出现的ON。70%可在数日内有截瘫。3、急性起病可在数小时或数日内单或双眼失明，眼眶痛。4、脊髓症状可横贯、不对称、或呈播散性；特征为快速进展的双下肢瘫，感觉脱失平面、括约肌障碍等，1/3病人有

Lhermitte征、根痛。ClinicalManifestations1、年轻居多，32一、辅助检查1、CSF细胞数增加，73%单相、82%复发。2、复发病人脊髓MRI88%出现纵向融合超过数个节段，钆强化和肿胀常见。二、鉴别诊断1、单纯球后神经炎2、MS表现为NMO临床模式。3、亚急性视神经病三、治疗大剂量甲强冲击疗法一、辅助检查33急性播散性脑脊髓炎（ADEM）1、概述：是一种广泛累及脑和脊髓白质的急性炎症性疾病(感染出疹或疫苗接种)爆发型：急性出血性白质脑炎(AHL)2、病因及发病机制：病毒感染，脑组织+FAC可诱发EAE，认为ADEM是急性