以癫痫发作为首发症状的MOG抗体相关疾病患者临床分析

Clinical analysis of MOG antibody-related diseases with seizures as the initial symptom

目的分析以癫痫发作为首发症状的髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(myelin oligodendrocyte glycoprotein-IgG associated disorders,MOG-AD)的临床特点。方法回顾性分析2018年11月至2022年1月河北省儿童医院神经内科收治的15例以癫痫发作为首发症状的MOG-AD患儿的一般信息、临床表现、实验室检查、治疗及随访等资料。结果15例患儿中,男4例,女11例;起病年龄4~11岁,中位年龄为9岁。除癫痫发作症状外,还可表现为发热6例(40.00%)、头痛5例(33.33%)。共29次癫痫发作中,全面性发作9次,局灶性发作20次,2次癫痫持续状态。脑脊液白细胞数存在不同程度升高(6×106/L~341×106/L);视频脑电图以局灶性或弥漫性慢波为主的非特异性改变9例,局灶性癫痫放电4例,正常4例;头颅核磁显示单侧皮层病变5例,白质病变5例,脑膜强化4例,正常3例。15例均予以静脉注射丙种球蛋白联合激素治疗,4例予以抗癫痫发作药物治疗,患儿多数预后良好,2例复发。结论以癫痫发作为首发症状的MOG-AD多见于学龄期儿童,常伴发热、头痛,脑脊液、脑电图及磁共振影像学检查结果多符合脑炎特点,部分患儿表现为孤立性癫痫发作,需尽早完善MOG抗体检测明确诊断,早期启动免疫治疗。

Objective To analyze the clinical characteristics of myelin oligodendrocyte glycoprotein-IgG associated disorders(MOG-AD)with seizures as the initial symptom.Methods The general information and clinical manifestations of 15 children with MOG-AD with seizure as the initial symptom admitted to the department of Neurology of Hebei Children s Hospital were retrospectively analyzed.Results Among the 15 cases,4 were males and 11 were females;the onset age ranged from 4 years old to 11 years old,and the median age was 9 years old.In addition to seizure as the initial symptom,the patients also presented with fever(6 cases,40.00%)and headache(5 cases,33.33%).Of the 29 seizures,there were 9 generalized seizures,20 focal seizures,and 2 status epilepticus.Cerebrospinal fluid(CSF)leukocytes were increased to varying degrees(6×106/L-341×106/L);the video electroencephalo-graph(EEG)of 9 cases showed focal or diffuse slow wave-based non-specific changes,4 cases showed focal epileptic discharge,and 4 cases were normal.Brain magnetic resonance imaging(MRI)showed unilateral cortical lesions in 5 cases,white matter lesions in 5 cases,meningeal enhancement in 4 cases,and normal in 3 cases.Fifteen patients received intravenous injection of gamma globulin combined with hormone therapy,and 4 patients received anti-seizure medication treatment.The prognoses were good in most cases,with 2 cases of recurrence.Conclusions MOG-AD with seizure as the initial symptom is more common in school-age children,often accompanied by fever and headache.The results of cerebrospinal fluid tests,EEG,and MRI are in line with the characteristics of encephalitis,and some cases show isolated epilepsy.MOG antibody detection is necessary as soon as possible to confirm the diagnosis and start immunotherapy early.