关键词: 脱髓鞘自身免疫疾病； MOG抗体病； 复发； 儿童

Abstract: 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.

Key words: demyelinating autoimmune disease; MOG antibody disease; recurrence; child