目的 分析儿童MOG抗体病(MOG-AD)复发的危险因素,为临床预判疾病复发提供科学依据。方法 收集 2016年1月-2020年6月71例MOG抗体阳性且临床首次发生中枢神经系统急性脱髓鞘综合征患儿的临床资料;随访6个 月~4年,比较复发与无复发两组患儿的临床资料、实验室检查和影像学特点、治疗及随访结果。结果 71例患儿中男29例、 女42例,中位年龄6.3(4.4~9.0)岁。首次发作时的临床特点以发热(35例)、偏瘫(28例)、脊髓炎(28例)、脑病(27例)、 头痛(22例)、癫痫(20例)、视神经炎(ON,18例)为主。头颅MRI均可见颅内多发病灶,累及大脑皮层下、白质、基底节区、 视神经等多部位;脊髓MRI异常35例,表现为脊髓单段、多节段或全脊髓病变。中位随访时间36(18 ~ 48)月。复发26例 (36.62%),男8例、女18例,中位年龄6.0(4.0~9.0)岁。临床最终诊断为急性播散性脑脊髓炎最为常见(34例),其次为 ON(18例)。二分类logistic回归分析发现,首次发作临床特点表现为ON、前驱感染为呼吸道感染、首次治疗仅予静脉注 射甲基泼尼松龙(IVMP)是复发的独立危险因素(P
Objective To analyze the risk factors for recurrence of MOG antibody disease (MOG-AD) in children, and to provide scientific basis for clinical prediction of disease recurrence. Methods The clinical data of 71 children with positive MOG antibody and central nervous system (CNS) acute demyelinating syndromes (ADS) developed for the first time from January 2016 to June 2020 were collected. The follow-up period ranged from 6 months to 4 years. The clinical data, laboratory examination and imaging characteristics, treatment and follow-up results of recurrent and nonrecurrent groups were compared. Results Among the 71 children, 29 were boys and 42 were girls, with a median age of 6.3 (4.4~9.0) years. The clinical characteristics of first episode were fever (35 cases), hemiplegia (28 cases), myelitis (28 cases), encephalopathy ( 27 cases), headache ( 22 cases), epilepsy ( 20 cases) and optic neuritis (ON, 18 cases). Brain MRI showed multiple intracranial lesions involving subcortical, white matter, basal ganglia, optic nerve and other parts of the brain. MRI of spinal cord showed abnormalities in 35 cases, presenting as single-segment and multi-segment spinal cord lesion or total myelopathy. The median follow-up was 36 ( 18 ~ 48 ) months. There were 26 recurrent children ( 36 . 62 %), including 8 boys and 18 girls, with a median age of 6 . 0 ( 4 . 0 ~ 9 . 0 ) years. The most common clinical diagnosis was acute diffuse encephalomyelitis ( 34 cases), followed by ON ( 18 cases). Binary logistic regression analysis found that first episode characterized by ON, the presence of prodromal respiratory tract infection, and intravenous methylprednisolone (IVMP) alone in the first treatment were independent risk factors for relapse (P< 0.05). Conclusions The clinical phenotypes of MOG-AD in children are diverse. The first episode characterized by ON, the presence of prodromal respiratory tract infection, and IVMP alone in the first treatment were important predictors of recurrence.