Clinical features of central nerve system inflammatory demyelinating diseases associated with anti-myelin oligodendrocyte glycoprotein antibodies in children
10.3760/cma.j.issn.2095-428X.2019.24.005
- VernacularTitle: 儿童抗髓鞘少突胶质细胞糖蛋白抗体相关中枢神经系统炎性脱髓鞘病临床特征
- Author:
Weihua ZHANG
1
;
Xiaotun REN
1
;
Tongli HAN
1
;
Hua CHENG
2
;
Jiuwei LI
1
;
Shuai GONG
1
;
Weixing FENG
1
;
Xiaoyun ZHU
1
;
Changhong REN
1
;
Ji ZHOU
1
;
Changhong DING
1
;
Haitao REN
3
;
Hongzhi GUAN
3
;
Fang FANG
1
Author Information
1. Department of Neurology, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing 100045, China
2. Department of Radiology, Beijing Children′s Hospital, Capital Medical University, National Center for Children′s Health, Beijing 100045, China
3. Department of Neurology, Peking Union Medical College Hospital, Beijing 100730, China
- Publication Type:Journal Article
- Keywords:
Child;
Myelin oligodendrocyte glycoprotein;
Central nerve system inflammatory demyelination di-seases;
Optic neuritis;
Monophasic;
Recurrent
- From:
Chinese Journal of Applied Clinical Pediatrics
2019;34(24):1858-1861
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To describe the clinical manifestations of central nerve system inflammatory demyelinating disease associated with anti-myelin oligodendrocyte glycoprotein antibody (MOG-IDD) in children, and to explore the clinical characteristics of the children.
Methods:The clinical and laboratory characteristics of the patients diagnosed in Beijing Children′s Hospital, Capital Medical University, from October 2016 to August 2018 were described, and the clinical data of the patients with unipolar and recurrent diseases were compared.
Results:A total of 50 patients were included, among whom the ratio of male to female was 24:26, and the average age of onset was (6.7±3.1) years old (0.4-12.6 years old). There was no significant difference in the age of onset between boys and girls(t=0.712, P=0.480). The main symptoms included fever (31/50 cases), encephalopathy (26/50 cases) and optic neuritis (22/50 cases), etc.In the last follow-up, 26 patients (52.0%) had a monophasic course and 24 patients (48.0%) had a recurrent course.There were age differences in encephalopathy and ataxia in the first episode of [(5.7±2.8) years old vs.(8.1±3.0) years old, (5.0±2.5) years old vs. (7.7±3.0) years old](t=2.746, P=0.009; t=2.837, P=0.007). The average number of recurrence was (2.1±1.4) times (1-7 times), in which 17 cases (70.8%) of recurrence presented within 12 months and 20 cases (83.3%) of recurrence presented within 24 months after onset.Convulsion incidences of recurrent cases were 10 cases and 13 cases respectively in the first episode and recurrent courses, which were significantly higher than those of monophasic cases (4 cases, 4 cases)(χ2=7.912, P=0.005; χ2=8.365, P=0.004). All patients were sensitive to first-line immunotherapy.Seven patients with recu-rrence were treated with mycophenolatemofetil, and 17 patients with repeated first-line therapy.In the last follow-up, all patients were in remission and 2 patients had mild neurological dysfunction.
Conclusions:MOG-IDD can occur in childhood.Encephalopathy and optic neuritis are the most common symptoms.Encephalopathy and ataxia are more common in young children.Convulsions may indicate the course of recurrence.
