120例视神经脊髓炎谱系疾病临床特点分析  

The clinical characteristics of 120 neuromyelitis optica spectrum disorder patients

作　　者：尤小凡[1] 胡洪涛[1] 叶静[2] YOU Xiaofan;HU Hongtao;YE Jing(Department of Neurology, Beijng Jishuitan Hospital, Beijing 100096, China)

机构地区：[1]北京积水潭医院神经内科,北京100096 [2]首都医科大学宣武医院神经内科,北京100096

出　　处：《中风与神经疾病杂志》2018年第4期333-337,共5页Journal of Apoplexy and Nervous Diseases

摘　　要：目的基于2015年诊断标准分析视神经脊髓炎谱系疾病(NMOSD)患者的临床特点和影像学特征。方法回顾性分析120例NMOSD患者的临床特点、影像学特征、以及对比水通道蛋白4(AQP4)抗体阳性和阴性患者的临床异同点。结果女性∶男性=2.75∶1,平均发病年龄37.1岁,平均病程21个月,80%患者为复发病程。起病症状视神经炎和脊髓炎分别占41.7%和40%。15.8%患者伴有自身免疫病;5.8%患者伴有内分泌疾病。78.1%的患者AQP4抗体阳性,抗体阳性组疾病严重程度评分显著高于抗体阴性组(P=0.026)。长节段横贯性脊髓炎占83.7%。头部MRI异常见于36.7%(44/120)患者。28.3%(34/120)患者脑脊液白细胞升高(>5×10~6/L),脑脊液蛋白49.5 mg/dl(正常值:15~45 mg/dl);30.8%(37/120)患者寡克隆区带阳性;52.2%(24/46)患者髓鞘碱性蛋白阳性;40%(48/120)患者脑脊液免疫球蛋白G升高。结论 2015年诊断标准有助于NMOSD诊断,NMOSD长节段脊髓炎多见,短节段脊髓炎不能排除NMOSD,AQP4抗体阳性患者病情更重,应重视早期诊断。Objective To describe the clinical characteristics of neuromyelitis optica spectrum disorder in a series of Chinese patients. Methods This was a retrospective study of 120 patients,according to the 2015 criteria. Data on the clinical features,magnetic resonance imaging,and aquaporin-4（ AQP4） antibody status were analyzed. Results The female to male ratio was 2. 75∶ 1,and the mean onset age was 37. 1 years. The median disease duration was 21 months. Eighty percent of the patients were relapsing course. Optic neuritis（ 41. 7%） and myelitis（ 40%） were common onset attacks. Some of the patients（ 15. 8%） were associated with autoimmune diseases and 5. 8% of the patients were associated with endocrinopathies. AQP4-antibody was detected in 78. 1% of the patients. The patients with positive AQP4-antibody had significantly higher Expanded Disability Status Scale scores（ P = 0. 026）. Longitudinally extensive transverse myelitis was found in83. 7% of the patients. Brain magnetic resonance imaging was abnormal in 36. 7%（ 44/120） of the patients. Elevated cerebrospinal fluid cells（ 〉5 × 10^6/L） were seen in 28. 3%（ 34/120） of the patients. The median of cerebrospinal fluid protein was 49. 5 mg/dl（ normal range： 15 - 45 mg/dl）. Thirty-seven patients（ 37/120,30. 8%） had positive cerebrospinal fluid oligoclonal bands,24 patients（ 24/46,52. 2%） had abnormal myelin basic protein,and 48 patients（ 48/120,40%）had an elevated immunoglobulin G index. Conclusion The new 2015 criteria helps to facilitate the diagnoses of neuromyelitis optica spectrum disorders. Longitudinally extensive transverse myelitis was common in NMOSD patients,but short myelitis can not excluse NMOSD. AQP4 antibody positive patients were more severely affected.

关 键 词：视神经脊髓炎谱系疾病 临床特点 水通道蛋白4抗体 核磁影像 

分 类 号：R744.52[医药卫生—神经病学与精神病学]