Myelin oligodendrocyte glycoprotein antibody-associated optic neuritis: an update

ABSTRACT Myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-associated optic neuritis has been established as a new entity of immune-mediated optic neuropathy. Patients usually present with recurrent optic neuritis, often bilaterally with initially severe vision loss and optic disc edema. However, in contrast to aquaporin 4-IgG-seropositive neuromyelitis optica spectrum disorder, visual recovery tends to be more favorable, with good response to steroid treatment. Another important differential diagnosis of myelin oligodendrocyte glycoprotein-IgG--associated optic neuritis is multiple sclerosis. Close monitoring for signs of relapse and long-term immunosuppression may be considered to maintain optimal visual function. The diagnosis can be made on the basis of the presence of a specific, usually serological, antibody against myelin oligodendrocyte glycoprotein (IgG; cell-based assay), and a demyelinating event (optic neuritis, myelitis, brainstem syndrome, or cortical lesions with seizures). The clinical spectrum of this newly recognized inflammatory demyelinating disease is expanding rapidly. We briefly review the epidemiological characteristics, clinical manifestations, diagnostic considerations, and treatment options of myelin oligodendrocyte glycoprotein-IgG-associated optic neuritis.

摘要：髓鞘少突胶质细胞糖蛋白免疫球蛋白G（myelin oligodendrocyte glycoprotein-immunoglobulin G，IgG）相关性视神经炎已被确立为一种新型免疫介导性视神经病变。患者通常表现为复发性视神经炎，多为双侧受累，初始即出现严重视力丧失与视盘水肿。然而，与水通道蛋白4-IgG（aquaporin 4-IgG）血清阳性的视神经脊髓炎谱系疾病不同，此类患者的视力恢复往往预后更佳，且对类固醇治疗反应良好。髓鞘少突胶质细胞糖蛋白-IgG相关性视神经炎的另一重要鉴别诊断为多发性硬化（multiple sclerosis）。为维持最佳视觉功能，可考虑密切监测复发征象并实施长期免疫抑制治疗。该疾病的诊断可基于两项依据：一是存在针对髓鞘少突胶质细胞糖蛋白的特异性抗体（通常为血清学抗体，采用基于细胞的检测法（cell-based assay）检测，IgG型），二是存在脱髓鞘事件，包括视神经炎、脊髓炎、脑干综合征或伴癫痫发作的皮层病变。这种新近被认知的炎症性脱髓鞘疾病的临床谱系正快速拓展。本文简要综述了髓鞘少突胶质细胞糖蛋白-IgG相关性视神经炎的流行病学特征、临床表现、诊断要点及治疗方案。