视神经脊髓炎
视神经炎
髓鞘少突胶质细胞糖蛋白
医学
自身抗体
脊髓炎
脱髓鞘病
中枢神经系统
免疫学
抗体
横贯性脊髓炎
多发性硬化
病理
内科学
实验性自身免疫性脑脊髓炎
脊髓
精神科
作者
Sven Jarius,Friedemann Paul,Brian G. Weinshenker,Michaël Lévy,Ho Jin Kim,Brigitte Wildemann
标识
DOI:10.1038/s41572-020-0214-9
摘要
Neuromyelitis optica (NMO; also known as Devic syndrome) is a clinical syndrome characterized by attacks of acute optic neuritis and transverse myelitis. In most patients, NMO is caused by pathogenetic serum IgG autoantibodies to aquaporin 4 (AQP4), the most abundant water-channel protein in the central nervous system. In a subset of patients negative for AQP4-IgG, pathogenetic serum IgG antibodies to myelin oligodendrocyte glycoprotein, an antigen in the outer myelin sheath of central nervous system neurons, are present. Other causes of NMO (such as paraneoplastic disorders and neurosarcoidosis) are rare. NMO was previously associated with a poor prognosis; however, treatment with steroids and plasma exchange for acute attacks and with immunosuppressants (in particular, B cell-depleting agents) for attack prevention has greatly improved the long-term outcomes. Recently, a number of randomized controlled trials have been completed and the first drugs, all therapeutic monoclonal antibodies, have been approved for the treatment of AQP4-IgG-positive NMO and its formes frustes.
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