MOG抗体相关脑炎临床特点与预后分析

Clinical manifestations and prognosis of MOG-IgG associated encephalitis based on single-centered cohort data

目的基于单中心队列临床数据总结MOG抗体相关脑炎的临床与预后特点。方法收集2017年1月至2019年6月北京协和医院MOG抗体相关疾病患者数据库资料,回顾分析合并脑炎(大脑、小脑、脑干等受累)事件患者与无脑炎事件患者的临床特点和预后差异。结果本研究共纳入MOG抗体相关疾病患者86例,其中脑炎患者56例(合并抗NMDAR脑炎4例)。与非脑炎组患者年龄〔中位数(上、下四分位数)29.9(12.2,38.4)岁〕及发病年龄〔中位数(上、下四分位数)24.9(11.8,33.0)岁〕比较,脑炎组〔分别为17.2(12.0,29.0)岁、13.9(7.4,24.6)岁〕更低(均P<0.05);脑炎组与非脑炎组患者性别比例、非脑炎相关临床表现无明显差异;两组患者绝大多数缓解期均应用小剂量糖皮质激素和免疫抑制剂治疗,脑炎组年化复发率〔中位数(上、下四分位数)〕、末次EDSS评分〔中位数(范围)〕分别为0.464(0.271,0.839)、0(0~2)分,非脑炎组分别为0.192(0,1.009)、0(0~8.5)分,两组差异无统计学意义(P>0.05)。脑炎组患者脑炎相关常见表现包括癫痫发作〔19例(34%)〕、意识障碍〔7例(12%)〕、肢体无力〔4例(7%)〕等,极少数患者仅出现发热、头痛等。头颅MRI病灶分布较为广泛,幕上深部白质病灶、皮层或皮层下白质、脑干、基底节、丘脑受累常见,少数有小脑、胼胝体受累。结论脑炎是MOG抗体相关疾病非常重要的临床分型之一,发病年龄越小的患者更易经历脑炎发作。

Objective To analyze clinical manifestations and prognosis features of patients with myelin oligodendrocyte glycoprotein(MOG)-immunoglobulin G(IgG)-associated encephalitis based on single-centered data.Methods Patients with MOG-IgG associated disorders(MOGAD)were included at Peking Union Medical College Hospital between January,2017 and June,2019.Differences of the clinical manifestations and prognosis features between patients with encephalitis(in which cerebellum,brainstem,or diencephalon were involved)and those without encephalitis were reviewed retrospectively.Results 86 patients with MOGAD were included and 56 had encephalitis events(4 accompanied by anti-NMDAR encephalitis).Compared with patients without encephalitis,those with encephalitis had a younger median(IQR)age[17.2(12.0,29.0)vs.29.9(12.2,38.4),P<0.05]and age of disease onset[13.9(7.4,24.6)vs.24.9(11.8,33.0),P<0.05].No significant gender predominance difference was revealed,neither the symptoms other than encephalitis were different between the two groups.Majority of both groups take low dose glucocorticoids and immunosuppressants for prevention therapy.The median(IQR)annualized relapse rate(ARR)and median(range)expanded disability status scale(EDSS)score at last follow-up of patients with encephalitis were 0.464(0.271,0.839)and 0(0-2),while ARR and last EDSS score of those without encephalitis were 0.192(0,1.009)and 0(0-8.5),neither revealed significant differences between groups(P>0.05).Patients with encephalitis commonly presented with symptoms of seizure(n=19,34%),disturbance of consciousness(n=7,12%),limb weakness(n=4,7%),and a few patients solely presented as fever and headache.Brain lesions of MRI distributed widely in supratentorial deep white matter,cortical grey matter or juxtacortical white matter,brainstem,thalamus,basal ganglia,cerebellum and corpus callosum.Conclusions Encephalitis is a significant clinical phenotype of MOGAD and younger patients may be inclined to experience encephalitis attacks.