Medicine and Health Sciences Postgraduation Program, School of Medicine, Pontificial Catholic University of Rio Grande Do Sul (PUCRS), Porto Alegre, Brazil. Electronic address: https://twitter.com/DaissyMora.
Medicine and Health Sciences Postgraduation Program, School of Medicine, Pontificial Catholic University of Rio Grande Do Sul (PUCRS), Porto Alegre, Brazil; Neuroinflammation and Neuroimmunology Lab, Brain Institute of Rio Grande Do Sul, Porto Alegre, Brazil.
Curr Opin Neurobiol. 2022 Oct;76:102618. doi: 10.1016/j.conb.2022.102618. Epub 2022 Aug 13.
Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune inflammatory disease of the central nervous system. Most of the cases are positive for autoantibodies targeting the water channel aquaporin-4 (AQP4-IgG). Activated B and T cells, innate immunity cells, pro-inflammatory cytokines, and activated complement contribute to the formation of the NMOSD lesions. Optic neuritis, longitudinally extensive myelitis, and area postrema syndrome are core clinical manifestations. NMOSD diagnosis is based on clinical manifestations, magnetic resonance imaging findings, and AQP4-IgG positivity. Cell-based assays are the preferred method for the detection of AQP4-IgG. Acute relapses are treated with IV methylprednisolone or plasma exchange. Recent advances on the NMOSD immunobiology led to approved treatments such as eculizumab, satralizumab, and inebilizumab.
视神经脊髓炎谱系疾病（NMOSD）是一种罕见的中枢神经系统自身免疫性炎症性疾病。大多数病例的自身抗体针对水通道蛋白 4（AQP4-IgG）。激活的 B 和 T 细胞、先天免疫细胞、促炎细胞因子和激活的补体有助于 NMOSD 病变的形成。视神经炎、长节段横贯性脊髓炎和延髓后正中综合征是核心临床表现。NMOSD 的诊断基于临床表现、磁共振成像发现和 AQP4-IgG 阳性。基于细胞的检测是检测 AQP4-IgG 的首选方法。急性复发采用 IV 甲基强的松龙或血浆置换治疗。NMOSD 免疫生物学的最新进展带来了依库珠单抗、satralizumab 和 inebilizumab 等已批准的治疗方法。
