摘要： 视神经脊髓炎谱系疾病包括经典视神经脊髓炎、视神经脊髓型多发性硬化、视神经脊髓炎限定型，以及伴系统性自身免疫性疾病或视神经脊髓炎特征性影像学表现的孤立性视神经炎或脊髓炎。其特征性神经眼科表现包括双侧同时或相继发生的视神经炎、视盘水肿和盘周渗出，视功能恢复差，对糖皮质激素有较好的反应性和依赖性。这些临床特征与水通道蛋白4 抗体检测和头部MRI 检查相结合，可为视神经脊髓炎谱系疾病与多发性硬化的早期鉴别诊断提供帮助。视神经脊髓炎谱系疾病可能出现某些特征性眼球运动异常，尚待进一步研究。

关键词: 视神经脊髓炎, 自身免疫疾病, 眼科学, 综述

Abstract: Neuromyelitis optica spectrum disorders (NMOSDs) include classic neuromyelitis optica (NMO), opticospinal multiple sclerosis (OSMS), limited form of NMO and isolated optic neuritis or myelitis accompanied by either systemic autoimmune diseases or typical MRI findings of NMO. The common neuro-ophthalmic features of NMOSDs include simultaneous or consecutive bilateral optic neuritis, more commonly seen optic disk edema and surrounding exudate, poor visual recovery, steroid responsiveness and dependency. Combined with serum aquaporin 4 (AQP4) antibody and brain MRI examination, these clinical features can be helpful to the early differential diagnosis between NMOSDs and MS. Some types of eye movement abnormalities have been reported in patients with NMOSDs, but further investigation needs to be done before the specificity of these features are confirmed.

Key words: Neuromyelitis optica, Autoimmune diseases, Ophthalmology, Review