视神经脊髓炎谱系疾病患者临床特点分析

目的　分析并总结视神经脊髓炎谱系疾病患者的临床特点，为临床诊治提供参考依据。方法　回顾性分析中日友好医院神经眼科联合门诊2016年3月至2018年12月收治的75例视神经脊髓炎谱系疾病患者，对患者的一般情况、临床症状特点、实验室检查以及影像学检查结果进行分析，以及对比水通道蛋白4（aquaporin-4，AQP4）-IgG阳性和AQP4-IgG阴性患者的临床症状异同点。结果　75例患者中，男12例、女63例，男女比例接近为1∶5，发病年龄7~77岁，病程1个月~30 a，发病次数1~20次，16例（21.3%）患者起病前有明显诱因，其余59例（78.7%）患者无明显诱因发病。首次发病症状视神经炎发病28例（37.3%），脊髓炎发病18例（24.0%），视神经和脊髓同时受累19例（25.3%），颅内发病10例（13.3%），有19例患者伴随其他疾病，包括干燥综合征、系统性红斑狼疮等；45例（60.0%）患者检测了视神经脊髓炎特异性抗体（AQP4-IgG），其中31例（68.9%）患者呈现AQP4-IgG阳性表现，13例（28.9%）患者呈现AQP4-IgG阴性表现，尚有1例（2.2%）患者呈现髓鞘少突胶质细胞糖蛋白抗体阳性表现。结论　视神经脊髓炎谱系疾病多发于女性，大部分患者无明显诱因起病，复发率较高，首发病症累及视神经为主，其特异性抗体AQP4-IgG阳性患者多见，应予以早期诊断和治疗。

Clinical characteristics of patients with neuromyelitis optica spectrum disorders

Objective To analyze and summarize the clinical characteristics of patients with neuromyelitis optica(NMO)spectrum disorders,and to provide reference for clinical diagnosis and treatment.Methods A retrospective analysis was conducted in 75 patients with NMO spectrum disorders admitted to the Neuro-ophthalmology Clinic of China-Japan Friendship Hospital from March 2016 to December 2018.General conditions,clinical symptoms,results of laboratory examinations and imaging examinations were analyzed,and the similarities and differences of clinical symptoms between aquaporin-4(AQP4)-IgG positive and AQP4-IgG negative patients were compared.Results Among 75 patients,there were 12 males and 63 females.The ratio of males to females was close to 1:5.The onset age ranged from 7 to 77 years.The duration of the disease was from about one month to more than 30 years.The incidence of the disease was from one time to 20 times.Sixteen patients(21.3%)had obvious predisposition before onset,the remaining 59 patients(78.7%)had no obvious predisposition.The first onset symptoms were optic neuritis in 28 patients(37.3%),myelitis in 18 patients(24.0%),optic nerve and spinal cord involvement in 19 patients(25.3%),intracranial onset in 10 patients(13.3%),and 19 patients had other diseases,including Sjogren’s syndrome and systemic lupus erythematosus.Examination of NMO specific antibodies(AQP4-IgG)was conducted in 45 patients(60.0%),among of which 31 patients(68.9%)had AQP4-IgG positive manifestations,13 patients(28.9%)showed AQP4-IgG negative manifestations and 1 patient(2.2%)had myelin oligodendrocyte glycoprotein antibody positive manifestations.Conclusion NMO spectrum disorders occur mostly in women.Most of the patients have no obvious inducement.The recurrence rate is high.The initial symptom mainly involves optic nerves.The patients with positive specific antibody AQP4-IgG are more common.Early diagnosis and treatment should be taken.