视神经脊髓炎谱系疾病的研究进展

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1、授予单位代码10089学号或申请号10010010002中国图书分类号R744.5+2HebeiMedicalUniversity硕士学位论文专业学位视神经脊髓炎谱系疾病的研究进展研究生：李艳导师：任士卿教授专业：神经病学二级学院：第三医院2018年3月目录中文摘要············································································································1英文摘要···················

2、·························································································2英文缩写············································································································3综述视神经脊髓炎谱系疾病的研究进展·············································

3、·······4致谢··················································································································22个人简历··········································································································23中文摘要视神经脊髓炎谱系疾病的研究进展摘要视神经脊髓炎

4、谱系疾病（neuromyelitisopticaspectrumdisorder，NMOSD）是一类自身免疫介导的以视神经和脊髓受累为主的中枢神经系统炎性脱髓鞘疾病，具有高复发率和高致残率的特点，多数患者遗留有严重的视力障碍和肢体功能障碍。因此减少复发及残疾累积是NMOSD治疗的重点和难点。水通道蛋白4抗体（aquaporin-4immunoglobulinGantibodies，AQP4-IgG）是NMOSD特异性的生物学标志物，2015年NMOSD诊断标准国际共识及2016年中国NMOSD诊断和治疗指南根据AQP4-Ig

5、G进行分层诊断，即AQP4-IgG阳性组和AQP4-IgG阴性组，同时针对急性期及缓解期提出了相应的治疗策略。目前NMOSD尚无确实有效的治疗药物，结合临床实践，应早期识别复发高危人群，予以个体化的疾病修饰治疗（diseasemodifyingtherapy）。本文将对NMOSD的发病机制、临床表现、复发及疾病修饰治疗等方面进行综述。关键词：视神经脊髓炎谱系疾病，水通道蛋白4抗体，发病机制，复发，疾病修饰治疗1英文摘要ResearchProgressofNeuromyelitisOpticaSpectrumDisorderA

6、BSTRACTNeuromyelitisopticaspectrumdisorder(NMOSD)isanimmunemediatedinflammatorydemyelinatingsyndromeofthecentralnervoussystemwithapredilectionfortheopticalnervesandspinalcord.Itischaracterizedbyhighrateofrelapseanddisability.Mostpatientsareleftwithseverevisualimpai

7、rmentsandmotordysfunction.Therefore,reducingrelapseanddisabilityaccumulationisthepriorityofthetreatments.Intermsofthespecificbiomarker--theaquaporin-4immunoglobulinGantibodies(AQP4-IgG),bothinternationalconsensusdiagnosticcriteriain2015andChineseguidelinesin2016cla

8、ssifiedNMOSDintosero-positivegroupandsero-negativegroupandmeanwhilecameupwithtreatmentstrategiesforacutephaseandremission.However,therearenopoten