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DemyelinatingDiseasesoftheCentralNervousSystem 哈医大二院神经科 Chapter1Intraduction 1 ConceptAgroupofdiseasesofthebrainandspinalcordinwhichdemyelinationisaprominentfeature 2 PathologicFindings Destructionofthemyelinsheathsofnerves Infiltrationofinflammatorycellsinaperivasculardistribution Aparticulardistributionoflesion oftenperivenousandprimarilyinwhitematter eitherinmultiplesmalldisseminatedfociorinlargerfoci Arelativeintegrityoftheaxiscylindersinthelesionsandalackofwallerian thesecondarydegenerationoffibertracts 多发性硬化MultipleSclerosis 1 ConceptMultipleSclerosisisakindofautoimmunediseasescharacterizedbydemyelinationofCNS Duetoitshighincidence chronicityandtendencytoattackyoungadults ithasbecomeoneofthemostimportantdiseasesofCNS 2 EtiologyAndPathogenesis 1 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 3 Epidemiology IncidenceofMSassociatedwithlatitude ContactingwithsomekindofenvironmentmayplayanimportantroleintheincidenceofMS Hereditymaybeanimportantfactor MSassociatedwiththeHLA DRlocusonthesixthchromosome HLA DR2expressstronglyandthen DR3 B7andA3 4 PathologicFindings Characteristic MultipledemyelinatedplaquesinwhitematterofCNS Position Whitematteraroundthelateralventriclesandspinalcord opticnerve brainstemandcerebellar Acutestage hyperemia ondema demyelination infiltrationofinflammatorycellsinperivasculardistribution Recoverystage Astrocyteproliferition formingofastrocyticscab 小脑及桥脑脱髓鞘病灶 桥脑脱髓鞘病灶 5 ClinicalManifestations 1 Prodrome Thesymptomsevolvedmoreslowly overseveralweeksormonths 2 AcuteorsubacuteonsetRelapsing remitting 3 Earlysymptomsandsigns Weaknessornumbness sometimesboth inoneormorelimbsistheinitialsymptominabouthalfofthepatients Thevisuallossinoneorbotheyes Nystagmus fig1 andpalsyofeyemuscles internuclearophthalmoplegia oneandahalfsyndrome Impairmentofotherbrainnerves Ataxia Charcot ssyndrom Sensationdisorder Romberg ssign Lhermitte ssign Attacksyndrom Otherclinicalfeature 6 LaboratoryandotherassistantTests 1 CSFTest1 NumberofMNC0 7 oligoclonalbands OB positive 3 MBP PLP MAG MOGAbsandAb secretingcells4 CSF Alb serum Alb 1 7 probabilityofMS 2 Evokedpotentials visualevokedpotentials VEP brainstemauditoryevokedpotentials BAEP somatosensoryevokedpotentials SEP 3 MRI preiventricularplaques regularplaquesinbrainstem cerebellumandspinalcord atrophysymptom 1 2 3 4 5 6 Diagnosticcriteria 1 ClinicaldefiniteMS CDMS twotimesofattackandtwolesions twoattacks onelesionandonesubclinicalevidence 2 LaboratorysupporteddefiniteMS LSDMS Twoattacks onesubclinicalevidenceandCSF OB IgG Oneattack twolesionsandCSFOB IgG Oneattack onelesion onesubclinicalevidenceandCSFOB IgG 3 ClinicalprobableMS CPMS twoattacks onelesion oneattack twolesions oneattack onelesionandothersubclinicalevidence 4 LaboratorysupportedprobableMS LSPMS Twoattacks CSFOB IgG TwoattacksinvolvingdifferentpartofCNS intermissionatlestonemonth eachattackmustcontinuefor24hs DifferentialDiagnosis 1 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 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 1 Relapsing RemittingMS ACTHandSteoids methylprenisolone prednison dexamethasone IFN Azathioprine Immuneglublin Ig 2 ProgressiveMS Methotrexate MTX Cyclosphoamide CyclosporineA Plasmatransplantation 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 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 颈髓脱髓鞘视神经炎 ClinicalManifestations 1 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 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 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MS 或其变异型 3 病理 脑和脊髓多数脱髓鞘病灶 小静脉周围炎性反应 形成血管袖套 ClinicalManifestations 1 Prodrome2 Typeofencephalitis3 Typeofmeningitis4 Typeofmyelitis 1 LabTeste1 WBC pressureofCSFornormal Pr IgGandOBpositive 2 AbnormalofEEG 3 CTscanshowsthelesionsofmultiplediffusionsubcortexlowdensity MRIsh