目的：研究视神经脊髓炎谱系疾病（NMOSD）的临床特点，探讨治疗进展。方法：回顾性分析13例 NMOSD患者的临床表现、辅助检查结果、治疗及预后，并进行分析。结果：13例NMOSD患者中男女比例 1∶3.3，平均（45±14）岁。急性脊髓炎11例，视神经炎9例，延髓最后区综合征2例，伴发皮肤瘙痒2例，肢体痉 挛性疼痛3例。9例患者血和/或脑脊液AQP4-IgG阳性，3例抗体阴性患者中1例MOG-IgG阳性。11例MRI 表现为脊髓长节段脱髓鞘信号伴强化，1例延髓背侧异常信号，1例视神经异常信号伴强化。大部分患者急 性期给予大剂量激素冲击或免疫球蛋白或血浆置换治疗，部分患者给予硫唑嘌呤预防复发。11 例患者 （84.6%）好转，1例患者（7.7%）无好转，1例患者（7.7%）自行缓解。8例（61.5%）呈复发病程，5例（38.5%）为 单相病程。结论：NMOSD临床表现多种多样，除了典型的核心症状以外，还有很多非典型症状。大剂量激 素冲击、血浆置换和免疫球蛋白治疗仍是目前急性期首选治疗方案，硫唑嘌呤、吗替麦考酚酯和利妥昔单抗 是预防发作的一线用药。

To investigate the clinical characteristics and treatment progression of neuromyelitis optica spectrum disorders(NMOSD). Methods: The clinical manifestations, auxiliary examination results, treatment, and prognosis of 13 NMOSD patients were retrospectively analyzed. Results: The proportion of men to women in the 13 NMOSD cases was 1∶3.3, and the average age was (45±14) years. There were 11 cases of acute myelitis, 9 cases of optic neuritis, 2 cases of area postrema syndrome, 2 cases of pruritus, and 3 cases of spastic pain in limbs. There were 9 cases positive for AQP4-IgG in blood and/or cerebrospinal fluid, and of the 3 cases negative for antibody, 1 was MOG-IgG positive. MRI showed demyelinating signal with intensification in the long segment of the spinal cord in 11 cases, abnormal signals on the dorsum of the medulla in 1 case, and optic nerve abnormal signals with strengthening in 1 case. Most patients in acute stage were given high-dose corticosteroid, immunoglobulin, or plasma exchange treatment, and some patients received azathioprine in prevention of recurrence. Eleven patients (84.6% ) showed improvement, 1 patient (7.7% ) showed no improvement, and 1 patient (7.7% ) showed spontaneous improvement. Eight patients (61.5% ) had a course of recurrence, and 5 patients (38.5% ) had a monophasic course. Conclusion: The clinical manifestations of NMOSD are varied. Beside the typical core symptoms, there are also many atypical symptoms. Current treatment during the acute phase includes high-dose glucocorticoid, plasma exchange, and immunoglobulin therapy. Azathioprine, mycophenolate mofetil, and rituximab are first-line drugs for prevention.