报告一例视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorders,NMOSD)合并人体免疫缺陷病毒(human immunodeficiency virus,HIV)感染/获得性免疫缺陷综合征(acquired immune deficiency syndrome,AIDS)，并通过文献复习，总结其发病机制、临床特征、治疗及预后。检索文献包括7篇英文文献(8个病例)，1篇中文文献，共报道9例NMOSD合并HIV感染/AIDS病例，结合本文报道的1例共10例，其中5例为女性，5例为男性，3例HIV感染/AIDS为新发，其他病例的HIV感染/AIDS发病均早于NMOSD。临床表现上，7例均为视神经炎和脊髓炎同时或相继发生，2例表现为单相病程或复发性脊髓炎，1例仅表现为双眼相继发生的视神经炎，10例患者头或脊髓MRI均有典型的视神经或脊髓异常信号，伴或不伴强化。2例患者未进行水通道蛋白4(aquaporin protein-4,AQP4)抗体IgG检测，其余8例中5例AQP4抗体阳性、3例阴性。针对AIDS的治疗，10例患者中，8例接受了高效抗逆转录病毒治疗(highly active antiretroviral therapy,HAART)。针对NMOSD的治疗，10例患者中，急性期有8例患者接受糖皮质激素冲击治疗、3例患者接受血浆置换、2例接受丙种球蛋白治疗，序贯治疗期有6例患者接受免疫抑制剂治疗，其中1例因高胆红素血症停药。发生视神经炎的7例中，2例患者经治疗仍失明、5例视力部分恢复，发生脊髓炎的8例中，5例患者遗留截瘫或轻瘫、3例肌力部分恢复。1例因严重并发症去世。NMOSD合并HIV感染/AIDS临床较罕见，预后差，往往遗留严重的视力障碍及瘫痪等，临床治疗较为棘手，糖皮质激素和免疫抑制剂并非使用禁忌证，但制定治疗决策前需要充分考虑风险与获益的平衡。

A case of neuromyelitis optica spectrum disorders(NMOSD) complicated with human immunodeficiency virus(HIV) infection/acquired immunodeficiency syndrome(AIDS) was reported, and the pathogenesis, clinical characteristics, treatment and prognosis were summarized through the literature review. The retrieved literatures included seven English literatures (eight cases) and one Chinese literature, in which a total of nine cases of NMOSD co-infected with HIV infection/AIDS were reported. Combined with the case reported in this paper, the total number of cases was ten, among which five cases were female and five cases were male, three cases of HIV infection/AIDS were newly developed, and the other cases had earlier onset of HIV infection/AIDS than NMOSD. In terms of clinical manifestations, seven cases all had simultaneous or sequential optic nerve and myelitis, two patients presented with a uniphasic course or recurrent myelitis, and one case presented only with bilateral optic neuritis occurring sequentially in both eyes. All ten patients had typical abnormal signals of the optic nerve or spinal cord with or without enhancement on cranial or spinal MRI. Two patients did not undergo AQP4 antibody IgG testing , and of the remaining seven cases, five were positive for AQP4 antibodies and three were negative. For AIDS treatment, eight of the ten patients received highly active antiretroviral therapy(HAART). For NMOSD treatment, among the ten patients, eight patients received intravenous methylprednisolone,three patients received plasmapheresis, and two patients received intravenous immunoglobulin in the acute phase. Six patients received immunosuppressive therapy during the sequential treatment period, and one of them was discontinued due to hyperbilirubinemia. Of the seven cases with optic neuritis, two patients remained blind after treatment and five had partial recovery of vision. Of the eight cases with myelitis, five patients were left with paraplegia or mild paralysis, and three had partial recovery of muscle strength. One case died due to serious complications.NMOSD combined with HIV infection/AIDS is rare in clinic and has a poor prognosis. Patients are often left with severe visual impairment and paralysis. Clinical treatment is quite difficult, hormones and immunosuppressive agents are not considered as contraindications. Treatment decisions need to be made with fully considered about the balance of risks and benefits.