视神经脊髓炎谱系疾病临床特点分析及治疗进展

Clinical Characteristics Analysis and Treatment Progression of Neuromyelitis Optica Spectrum Disorders

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-Ig G阳性,3例抗体阴性患者中1例MOG-Ig G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。

Objective: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.