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视神经脊髓炎谱系疾病(NMOSD)主要包括急性脊髓炎、视神经炎和最后区综合征，其中水通道蛋白4(AQP4)-IgG阳性NMOSD患者较阴性患者症状更严重，复发更频繁，恢复更不完全，早期即可致残，但其确切发病机制尚不明确。本文现围绕AQP4-IgG阳性NMOSD发病机制的最新研究进展综述如下。“,”Neuromyelitis optica spectrum disorder (NMOSD) is a central nervous system inflammatory disease characterized by aquaporin-4 immunoglobulin G (AQP4-IgG). The core clinical manifestations include acute myelitis, optic neuritis and area postrema syndrome. AQP4-IgG-positive NMOSD patients have severe symptoms, frequent relapses, less complete recovery and early disability. In recent years, AQP4-IgG-positive NMOSD has gradually been recognized, but its exact mechanism is still unclear. This paper reviews the recent advance in pathogenesis of AQP4-IgG positive NMOSD.