Misu Tatsuro, Fujihara Kazuo
Department of Multiple Sclerosis Therapeutics, Tohoku University Graduate School of Medicine.
Nihon Rinsho. 2013 May;71(5):823-8.
Neuromyelitis optica (NMO) is a disease characterized by severe optic neuritis and transverse myelitis with autoantibody against aquaporin 4 (AQP4), mainly localized at astrocyte foot processes. Loss of AQP4 and glial fibrillary acidic protein with relatively preserved myelin is the pathological hallmarks of active NMO lesions. Several experimental studies suggested the crucial role of AQP4 antibody with diverse mechanisms including antibody- and complement-induced cytotoxicity against astrocytes. In vivo studies demonstrated that T cell-mediated CNS inflammation is necessary for the access of AQP4 antibody into CNS. NMO patients often develop medullary lesions including area postrema, which lacks the blood-brain-barrier and is sensitive to emetic agents. NMO is now considered to be an autoimmune astrocytopathy, and is distinct from multiple sclerosis.
视神经脊髓炎（NMO）是一种以严重视神经炎和横贯性脊髓炎为特征的疾病，伴有针对水通道蛋白4（AQP4）的自身抗体，主要定位于星形胶质细胞足突。AQP4和胶质纤维酸性蛋白缺失而髓鞘相对保留是活动性NMO病变的病理特征。多项实验研究提示AQP4抗体通过多种机制发挥关键作用，包括抗体和补体诱导的对星形胶质细胞的细胞毒性作用。体内研究表明，T细胞介导的中枢神经系统炎症是AQP4抗体进入中枢神经系统所必需的。NMO患者常出现延髓病变，包括最后区，该区缺乏血脑屏障且对催吐剂敏感。NMO现被认为是一种自身免疫性星形胶质细胞病，与多发性硬化不同。
