儿童MOG抗体阳性中枢神经系统脱髓鞘病临床特征研究

Clinical features analysis of central nervous system demyelinating disease in children with positive MOG antibody

目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的中枢神经系统脱髓鞘病(central nervous system demyelinating disease)临床、影像学等特征。方法回顾性分析2017年12月至2019年8月无锡市儿童医院诊断的7例MOG抗体阳性中枢神经系统脱髓鞘病患儿的病例资料、随访情况等资料。结果7例患儿中,男2例,女5例,最终诊断2例为急性播散性脑脊髓炎(ADEM),2例为视神经脊髓炎谱系疾病(NMOSD),1例为视神经炎(ON),1例为急性横贯性脊髓炎(ATM),1例尚不能归类。脑脊液检查6例白细胞升高,2例蛋白升高。采用细胞转染色免疫荧光法(CBA)检测MOG抗体,7例血清均阳性,4例脑脊液阳性。影像学检查多部位受累,存在颅内病灶6例,脊髓2例。7例患儿静脉糖皮质激素和丙种球蛋白治疗效果好,随访7个月-2年不等,有2例复发。结论MOG抗体与多种儿童中枢神经系统脱髓鞘病有关,与感染和疫苗接种可能有关,对激素和丙种球蛋白治疗敏感,有易复发的特点。

Objective To investigate the clinical and radiological features of children with central nervous system demyelinating disease and seropositivity to myelin-oligodendrocyte glycoprotein(MOG).Methods The datum of cases and follow-up situation were retrospectively analyzed in children with central nervous system demyelinating disease and seropositivity to MOG who were hospitalized in Wuxi Children’s Hospital from December 2017to August 2019.Results Seven patients including 2males and 5females were included in this study.Two patients were finally dignosed with acute disseminating encephalomyelitis(ADEM),two with neuromyelitis optica spectrum disorders(NMOSD),one with optic neuritis(ON),one with acute transverse myelitis(ATM)and one can’t be categorized.In cerebrospinal fluid examination,white blood cells were elevated in 6cases and protein was increased in 2cases.Cell transfection immunofluorescence(CBA)was used to detect anti-MOG antibodies.All 7cases were positive for sera and 4cases were positive for cerebrospinal fluid.Multi-site involvement was found in imaging studies.Six cases had intracranial lesions and 2case of spinal cord.In 7patients,intravenous glucocorticoids and gamma globulin were effective.The follow-up ranged from 7 months to 2years,and 2patients had recurrence.Conclusion MOG antibodies are associated with a variety of central nervous system demyelinating diseases in children.It may be related to infection and vaccination.It is sensitive to hormones and gamma globulin and has the characteristics of easy recurrence.