摘要： 目的 总结晚发型（LO-NMOSDs）和极晚发型视神经脊髓炎谱系疾病（VLO-NMOSDs）临床特征、治疗方案及预后。方法 纳入2015年1月至2021年12月首都医科大学附属北京同仁医院收治的68例首次发作的视神经脊髓炎谱系疾病病例，包括55例LO-NMOSDs和13例VLO-NMOSDs，对比分析二者性别、发病特点（首发症状）、残疾程度[扩展残疾状态量表（EDSS）评分]、合并症（自身免疫性疾病或肿瘤）、血清抗水通道蛋白4（AQP4）抗体和自身免疫抗体表达变化、视神经和脊髓影像学特点、治疗及预后。结果 与LO-NMOSDs患者相比，VLO-NMOSDs患者发病达峰时EDSS评分更高（Z=-2.119，P=0.034），而性别、首发症状、合并其他自身免疫性疾病或肿瘤、血清抗AQP4抗体和自身免疫抗体阳性率、视神经和脊髓受累部位、治疗方案、急性期治疗后14 d内EDSS评分等项指标组间差异无统计学意义（均P>0.05）。结论 VLO-NMOSDs与LO-NMOSDs患者具有相似的人口学特征、临床特征、治疗方案和短期预后，但前者发病达峰时残疾程度更严重，未来将进一步探究其长期预后。

关键词: 视神经脊髓炎, 水孔蛋白质类, 自身抗体, 老年人

Abstract: Objective To summarize the clinical features, treatment options and prognosis of late-onset and very late-onset neuromyelitis optica spectrum disorders (LO-NMOSDs and VLO-NMOSDs). Methods Total 68 patients with NMOSDs admitted to Beijing Tongren Hospital, Capital Medical University from January 2015 to December 2021 were included, while 55 with LO-NMOSDs and 13 with VLO-NMOSDs. The sex, first-onset symptoms, Extended Disability Status Scale (EDSS), complications, anti-aquaporin 4 (AQP4) antibody and autoimmune antibody, imaging examinations (optic nerve and spinal cord), therapeutic schedule and prognosis were compared between 2 groups. Results Compared with LO-NMOSDs group, VLO-NMOSDs group had higher EDSS scores at nadir (Z=-2.119, P=0.034), but there were no significant differences in sex, first-onset symptoms, combination with other autoimmune diseases or tumors, anti-AQP4 antibody and autoimmune antibody, optic nerve and spinal cord involvement sites, therapeutic schedule and EDSS scores within 14 d after acute treatment (P>0.05, for all). Conclusions Patients with VLO-NMOSDs and LO-NMOSDs have similar demographic characteristics, clinical characteristics, therapeutic schedule and short-term prognosis, but the VLO-NMOSDs has more severe disability at nadir, and its long-term prognosis remains to be further explored.

Key words: Neuromyelitis optica, Aquaporins, Autoantibodies, Aged