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视神经脊髓炎

Neuromyelitis Optica.

作者信息

Patterson Sarah L, Goglin Sarah E

机构信息

Division of Rheumatology, Department of Medicine, University of California, San Francisco, 400 Parnassus Avenue, San Francisco, CA 94143, USA.

Division of Rheumatology, Department of Medicine, University of California, San Francisco, 400 Parnassus Avenue, San Francisco, CA 94143, USA.

出版信息

Rheum Dis Clin North Am. 2017 Nov;43(4):579-591. doi: 10.1016/j.rdc.2017.06.007. Epub 2017 Aug 31.

Abstract

Neuromyelitis optica (NMO), formerly known as Devic disease, is an autoimmune astrocytopathic disease characterized by transverse myelitis and optic neuritis. Most patients demonstrate a relapsing course with incomplete recovery between attacks, resulting in progressive disability. The pathogenesis involves production of aquaporin-4 antibodies (AQP4-IgG) by plasmablasts in peripheral circulation, disruption of the blood-brain barrier, complement-mediated astrocyte injury, and secondary demyelination. The diagnosis relies on characteristic clinical manifestations in the presence of serum AQP4-IgG positivity or specific neuroimaging findings, and exclusion of alternative etiologies. Current treatment involves aggressive immunosuppression with pulse-dose steroids during acute attacks and long-term immunosuppression for attack prevention.

摘要

视神经脊髓炎(NMO),以前称为德维克病,是一种自身免疫性星形细胞病,其特征为横贯性脊髓炎和视神经炎。大多数患者表现为复发型病程,发作期间恢复不完全,导致进行性残疾。发病机制涉及外周循环中浆母细胞产生水通道蛋白4抗体(AQP4-IgG)、血脑屏障破坏、补体介导的星形细胞损伤以及继发性脱髓鞘。诊断依赖于血清AQP4-IgG阳性或特定神经影像学表现时的特征性临床表现,并排除其他病因。目前的治疗包括急性发作期用大剂量脉冲类固醇进行积极免疫抑制以及用于预防发作的长期免疫抑制。

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