摘要：

视神经脊髓炎是主要累及视神经和脊髓的自身免疫性中枢神经系统疾病，水通道蛋白4是主要靶抗原，其特异性抗体NMO-IgG 阳性患者以女性多见，临床症状较重，同时出现双侧视神经炎或视神经炎和脊髓炎，受累脊髓节段较长。而在NMO-IgG 阴性患者血清中可以检出抗水通道蛋白1（AQP1）抗体和抗髓鞘少突胶质细胞糖蛋白（MOG）抗体，抗AQP1 抗体阳性患者女性少见，长节段脊髓病变多见，视神经炎少见；抗MOG 抗体阳性患者男性多见，视神经炎多见，尤其是双侧视神经同时受累，胸腰髓受累多见。

关键词: 视神经脊髓炎, 水通道蛋白质4, 水通道蛋白质1, 髓鞘, 少突神经胶质, 糖蛋白类, 综述

Abstract:

Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system (CNS) which primarily involves the optic nerve and spinal cord. Aquaporin 4 (AQP4) is the main objective antigen, and its specific antibody was NMO-IgG. It was found in clinic that most of NMO-IgG-positive patients were female, whose clinical symptoms were more severe, bilateral optic neuritis (BON) or optic neuritis (ON) and myelitis were more likely to appear at the same time, and involved spinal segments were longer. Recent studies discovered that anti-aquaporin 1 (AQP1) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies existed in the serum of patients with NMO-IgG-negative. It was discovered that low proportions of women, more cases of long-segment spinal cord lesion, and rare cases of ON appeared in anti-AQP1 antibody-positive patients. Most of anti-MOG antibody-positive patients were male. ON was common, especially bilateral optic nerves involved at the same time, and thoracolumbar involvement was common.

Key words: Neuromyelitis optica, Aquaporin 4, Aquaporin 1, Myelin sheath, Oligodendroglia, Glycoproteins, Review