视神经脊髓炎谱系疾病  

Neuromyelitis optica spectrum disorders

作　　者：王维治[1] 王化冰[2] Wang Weizhi;Wang Huabing(Department of Neurology,the Second Affiliated Hospital,Harbin Medical University,Harbin 150086,China;Department of Neurology,Neuroinfection and Neuroimmunology Center,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China)

机构地区：[1]哈尔滨医科大学附属第二医院神经科,哈尔滨150086 [2]首都医科大学附属北京天坛医院神经内科神经病学中心神经感染免疫科,北京100070

出　　处：《中华神经科杂志》2022年第5期511-519,共9页Chinese Journal of Neurology

摘　　要：视神经脊髓炎谱系疾病(NMOSD)是免疫介导的中枢神经系统炎性脱髓鞘疾病,以视神经炎和脊髓炎为主要临床征象,病因主要与水通道蛋白4(AQP4)抗体相关,AQP4是免疫攻击的靶抗原。NMOSD以视神经炎、纵向延伸的长节段横贯性脊髓炎、延髓最后区综合征、脑干综合征、间脑综合征和大脑综合征等为临床特征。近年来在NMOSD的病因机制、临床诊断和新型作用靶点的单克隆抗体药物治疗等研究领域均获得长足进步,推动了临床神经病学的发展。Neuromyelitis optica spectrum disorders(NMOSD)is an immune mediated inflammatory demyelinating disease of the central nervous system.Optic neuritis and longitudinally extensive transverse myelitis are the main clinical signs,and the etiology is mainly related to aquaporin 4(AQP4)antibody.AQP4 is the target antigen of immune attack.NMOSD is characterized by optic neuritis,longitudinally extended transverse myelitis,medulla area postrema syndrome,brainstem syndrome,diencephalic syndrome and cerebral syndrome.In recent years,the etiological mechanism,clinical diagnosis and monoclonal antibodies targeting new mechanisms of NMOSD have made great progress,which promoted the development of clinical neurology.

关 键 词：视神经脊髓炎谱系疾病 中枢神经系统 脱髓鞘疾病 水通道蛋白4 视神经炎 纵向延伸的长节段横贯性脊髓炎 

分 类 号：R744.52[医药卫生—神经病学与精神病学]