MOG抗体相关皮质脑炎患者的临床特征分析  

Clinical characteristics of patients with MOG antibody-associated cerebral cortical encephalitis

作　　者：陈云[1] 杨再兰 周浩[1] 邰赵婧 刘开宇 CHEN Yun;YANG Zaian;ZHOU Hao(Department of Pediatric Neurology,Guizhou Branch of Shanghai Children's Medical Center,Shanghai Jiaotong University School of Medicine,Guizhou Provincial People's Hospital,Guiyang(550002),Guizhou,China)

机构地区：[1]上海交通大学附属上海儿童医学中心贵州医院神经康复科,贵州省人民医院,贵州贵阳550002

出　　处：《癫痫与神经电生理学杂志》2024年第5期284-292,F0002,共10页Journal of Epileptology and Electroneurophysiology

摘　　要：目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关皮质脑炎的临床特征,提高对该病诊断的准确性,减少临床误诊。方法回顾性分析2021年9月至2024年1月上海交通大学附属上海儿童医学中心贵州医院确诊的10例MOG抗体相关疾病(MOGAD)患儿的临床资料。结果10例患儿中男性6例、女性4例,发病中位年龄为11.5(5,13)岁,起病至确诊MOGAD的中位时间为17.5(8,42)d。癫痫发作为最常见的首发症状,其次为视力下降。脱髓鞘事件包括急性播散性脑脊髓炎、大脑皮质脑炎(CCE)、视神经炎、脊髓炎及多灶性神经功能缺损等。头颅MRI显示7例患儿有颅内多发病灶,3例为单一病灶,5例为脊髓受累,5例为视神经受累。6例患儿血清及脑脊液(CSF)MOG抗体均为阳性,单独血清或CSF抗体阳性各2例,合并NMDAR抗体2例,合并NMDAR及抗GFAP抗体1例。5例患儿病程早期表现为CCE,头颅MRI显示为皮层T2-FLAIR高信号,伴或不伴脑膜强化,其中4例以癫痫发作为首发症状,初始均被误诊为病毒性脑膜脑炎,误诊率高达40%。与其他病种比较,CCE病例更常合并癫痫发作、脑电图异常、CSF压力增高及有核细胞数增多。8例患儿接受了一线治疗,2例接受了二线治疗;8例症状完全恢复,2例遗留轻微症状。结论MOGAD临床表现多样,CCE易被误诊为病毒性脑膜脑炎,早期识别并及时启动免疫治疗,患儿通常预后良好。Objective To investigate the clinical characteristics of myelin oligodendrocyte glycoprotein(MOG)antibody-associated cerebral cortical encephalitis in children to improve the accuracy of diagnosis of the disease and reduce clinical misdiagnosis.Methods Retrospective analysis was conducted on clinical data of 10 children diagnosed with MOG antibody-associated disease(MOGAD)at Guizhou Hospital of Shanghai Children's Medical Center affiliated with Shanghai Jiao Tong University from September 2021 to January 2024.Results Among 10 patients,there were 6 males and 4 females,with a median age of onset of 11.5(5,13)years.The median time from onset to diagnosis of MOGAD was 17.5(8,42)days.Epilepsy was the most common initial symptom,followed by visual acuity loss.Demyelinating events included acute disseminated encephalomyelitis,cerebral cortical encephalitis(CCE),optic neuritis,myelitis and multifocal neurological deficits,etc..Brain MRI showed that multiple intracranial lesions in 7 cases,single lesion in 3 cases,while spinal cord and optic nerve involved in 5 cases,respectively.There were 6 cases with MOG antibody positive in both sera and cerebrospinal fluid(CSF),2 cases with MOG positive in sera and 2 cases with MOG positive in CSF.Two cases were positive for NMDAR antibodies in combination,and 1 case was positive for anti-NMDAR and anti-GFAP antibodies in combination.Five cases exhibited CCE at the early disease stage,and cranial MRI showed high T2-FLAIR signal in the cortex with or without meningeal enhancement,among them,4 cases presented with epileptic seizures as the initial symptom and they were initially misdiagnosed as viral meningoencephalitis,with a misdiagnosis rate of up to 40%.When compared with other diseases,CCE cases were more commonly associated with seizures,abnormal EEG,increased CSF pressure and elevated number of nucleated cells.Eight cases received the first-line treatment and 2 cases received the second-line treatment.Eight cases showed complete recovery of symptoms,while 2 cases remained with m

关 键 词：髓鞘少突胶质细胞糖蛋白抗体病 大脑皮质脑炎 癫痫发作 误诊 免疫治疗 

分 类 号：R744.5[医药卫生—神经病学与精神病学] R512.3[医药卫生—临床医学]