血清 M OG 抗体阳性炎性脱髓鞘疾病患者临床特征分析

摘要

ABSTRACT : Objective To investigate the clinical features of patients with M OG antibody positive inflammatory demyelinating disease Methods T he clinical data of 36 patients with serum M OG antibody positive inflammatory demyelinating disease (M OG antibody positive group ) and 87 patients with serum AQP4 antibody positive NM OSD (AQP4 antibody positive group ) were retrospectively analyzed. T he clinical characteristics of the two groups were analyzed and compared. Results When compared with the AQP4 antibody positive NM OSD patients ,the M OG antibody positive group have statistically significant differences in the proportion of men and women (1∶1.77 vs.1∶6.25 , P＝0.005 ) and age at onset [ (23.17 ± 14.37 ) years old vs. (35.91 ± 12.91 ) years old , P＝ 0.000 ].Females were more common than male in those two groups and the M OG antibody positive group was mainly children and youth. T he proportion of ADEM patients [7 cases (19.4%) vs. 0 (0%) , P＝0.000] in the first demyelinating event in the M OG antibody positive group was higher than that in the AQP 4 antibody positive group. T he proportions of patients with myelitis [11 cases (30.6%) vs. 47 cases (50.4%) ,P＝0.018] and brainstem syndrome [5 cases (13.9%) vs. 25 cases (28.7%) , P＝0.049] in the M OG antibody positive group were lower than those of the AQP4 antibody positive group. T he proportion of patients with seizures [5 cases (13.9%) vs. 0 (0%) ,P＝0.002] in the M OG antibody positive group was higher than that of the AQP4 antibody positive group. In MRI studies , total braia lesions [27 cases (75.0%) vs. 33 cases (37.9%) ] and NM O atypical lesions [23 cases (85.2%) vs. 4 cases (12.1%) ] in the M OG antibody positive group were higher than those of the AQP4 antibody positive group ,and the typical NM O lesions in the brain [4 cases (14.8%) vs. 29 cases (87.9%) ] in the M OG antibody positive group were lower than the AQP 4 antibody positive group (both P ＝ 0.000 ) . Only 14.8% of the patients with M OG antibody positive inflammatory demyelinating disease have typical NM O brain lesions. T he EDSS score [(2.36 ± 1.66 ) points vs. (3.83 ± 1.66 ) points , P＝0.000] was lower in the M OG antibody positive group than in the AQP4 antibody positive group at the last time of the visit. Conclusions MOG antibody positive inflammatory demyelinating disease patients have a small difference in male and female propotions. When compared with AQP4 antibody positive NMOSD patients ,the age of onset of MOG antibody positive inflammatory demyelinating disease is younger ,the first episode is mainly ADEM , optic neuritis and myelitis ; seizures is more Commonly observed ,MOG antibody positive patients rarely have NMO typical brain lesions , are prone to involve the lower spinal cord and have a good prognosis.%目的 探讨MOG抗体阳性炎性脱髓鞘疾病患者的临床特征.方法 回顾性分析36例血清MOG抗体阳性炎性脱髓鞘疾病患者(M OG抗体阳性组)与87例血清AQP4抗体阳性NM OSD患者(AQP4抗体阳性组)的临床信息,分析比较两组患者的临床特征.结果 MOG抗体阳性组与AQP4抗体阳性组患者相比,患病男女比例(1︰1.77 vs.1︰6.25 ,P＝0.005 )及发病年龄〔(23.17 ± 14.37 )岁vs. (35.91 ± 12.91 )岁,P＝0.000〕差异存在统计学意义,两组患者均以女性为多,M OG抗体阳性组患者以儿童和青年为主. M OG抗体阳性组首发脱髓鞘事件中ADEM 患者比例高于AQP4抗体阳性组〔7例(19.4%) vs.0例(0%) ,P＝0.000〕,脊髓炎〔11例(30.6%) vs.47例(50.4%) ,P＝0.018)〕和脑干综合征〔5例(13.9%) vs.25例(28.7%) ,P＝0.049)〕患者比例低于AQP4抗体阳性组. MOG抗体阳性组患者出现肢体抽搐症状患者比例高于 AQP4抗体阳性组〔5例(13.9%) vs.0(0%) ,P＝ 0.002〕.影像学表现上,MOG 抗体阳性组头部总病灶〔27 例(75.0%)vs.33 例(37.9%)〕及NMO非典型病灶〔23例(85.2%) vs.4例(12.1%)〕患者比例高于AQP4抗体阳性组,头部NMO典型病灶〔4例(14.8%) vs.29例(87.9%)〕患者比例低于AQP4抗体阳性组(均 P＝0.000) ,仅14.8% MOG抗体阳性炎性脱髓鞘疾病患者存在典型NM O头部病灶. M OG抗体阳性组患者截访时EDSS评分低于AQP4抗体阳性组〔(2.36 ± 1.66)分 vs.(3.83 ± 1.66)分,P＝0.000〕.结论 MOG抗体阳性炎性脱髓鞘疾病患者男女比例差异较小,与AQP4抗体阳性NMOSD患者相比发病年龄小,首次发作以ADEM 、视神经炎及脊髓炎为主,易出现肢体抽搐症状,少见NMO典型头部病灶,倾向于累及下段脊髓,预后较好.