The clinical and radiological features of children with first attack of inflammatory demyelinating central nervous system disorders and seropositivity to myelin-oligodendrocyte glycoprotein
10.3760/cma.j.issn.1006-7876.2018.02.004
- VernacularTitle:髓鞘少突胶质细胞糖蛋白抗体阳性的儿童中枢神经系统脱髓鞘病变首次发作的临床和影像学特点
- Author:
Min ZHANG
1
;
Wenhui LI
;
Haowei YANG
;
Linmei ZHANG
Author Information
1. 复旦大学附属儿科医院神经内科
- Keywords:
Demyelinating autoimmune diseases,CNS;
Glycoproteins;
Autoantibodies;
Child
- From:
Chinese Journal of Neurology
2018;51(2):97-104
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical and radiological features of children with first attack of inflammatory central nervous system disorders and seropositivity to myelin-oligodendrocyte glycoprotein (MOG).Methods The clinical course,cerebrospinal fluid (CSF),MRI studies,MOG status and outcomes were retrospectively analyzed in children with first attack of inflammatory central nervous system disorders and seropositivity to MOG who were hospitalized in Children's Hospital of Fudan University from January 2016 to April 2017.Results Thirteen patients including 8 males and 5 females were included in this study,the ratio of male/female was 1.6∶ 1,and the median age was six years.Ten patients were diagnosed with acute disseminating encephalomyelitis,and three with clinically isolated syndromes.Seven patients had elevated CSF lymphocyte cells,and five patients had elevated CSF protein.All the patients' sera were tested for the anti-MOG IgG,which ranged from 1∶10 to 1∶100 with cell-based assay.MRI results showed that multiple anatomical areas were involved.Twelve patients had brain lesions,in which 10 patients had multiple lobes involved and four had tumefactive demyelinating lesions.The affected anatomical areas included white matters in 11 cases,thalamus/basal ganglias in nine,corpus callosums in three,brainstems in 10,spinal cord in five.The MRI features were characterized by hazy,bilateral lesions without clear boundaries.Clinical symptoms were fully restored in all the patients after treated with intravenous globulin and methyl prednisone.The average follow-up time was 8.9 months,and none of the patients had clinical recurrence.Conclusions MOG was associated with many kinds of inflammatory demyelinating diseases of central nervous system in children.Most of them were diagnosed with acute disseminating encephalomyelitis which has an acute or sub acute clinical course.The clinical manifestations of patients showed diversity.Multiple anatomical areas were involved,and treatment with intravenous globulin and methyl prednisone was effective in the acute phase.All of the patients had a favorable outcome.
