儿童抗-NMDA受体脑炎临床特征和预后因素分析

Clinical characteristics and prognostic factors of anti-N-methyl-D-aspartate receptor encephalitis in children

目的探讨儿童抗-NMDA受体脑炎的临床特征和预后因素。方法收集和分析2015年1月—2017年12月确诊为抗-NMDA受体脑炎的51例患儿的临床资料,以及出院6个月的短期预后评估结果。结果 51例患儿中男21例、女30例,平均年龄(7.36±3.24)岁。最常见临床症状为运动障碍45例、人格改变43例、癫痫42例、认知障碍16例。2例自行转院。在49例接受治疗的患儿中,静脉注射免疫球蛋白45例,同时给予甲基泼尼松龙41例;血浆置换8例;接受利妥西单抗二线治疗8例,其中6例为静脉注射免疫球蛋白和甲基泼尼松龙治疗无效后、2例在接受血浆置换治疗无效后予利妥西单抗治疗。7例失访,42例出院后6个月随访评估短期预后良好23例。多元logistic回归模型分析发现,认知障碍(OR=23.97,95%CI:1. 12～513. 30,P=0. 042)与脑MRI异常(OR=14. 29,95%CI:1. 36～150. 10,P=0. 027)是短期预后差的独立危险因素。结论抗-NMDA受体脑炎常见运动障碍、人格改变、癫痫发作和认知障碍,MRI异常和认知障碍是短期预后不良的重要预测因子。

Objective To explore the clinical features and prognostic factors of anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis in children. MethodThe clinical data of anti-NMDA receptor encephalitis in 51 children from January 2015 to December 2017 were collected and analyzed, and the short-term prognostic results at 6 months after discharge were evaluated. ResultsIn 51 children (21 males and 30 females) with an average age of (7.36±3.24) years, the most common clinical symptoms were motor disorder (45 cases), personality change (43 cases), epilepsy (42 cases) and cognitive impairment (16 cases). Two cases were transferred to other hospital by themselves. In 49 children treated, 45 received intravenous immunoglobulin and 41 received methylprednisolone at the same time. Eight children received plasma exchange. The second-line treatment with rituximab were given to eight patients, among whom 6 patients had previously received ineffective intravenous immunoglobulin and methylprednisolone therapy and 2 patients had previously received ineffective plasma exchange therapy. Seven children were lost to follow-up. Forty-two children completed the assessment at 6-month follow-up after discharge, and 23 children had good short-term prognosis. Multiple logistic regression analysis showed that cognitive impairment (OR=23.97, 95%CI : 1.12~513.30, P=0.042) and abnormal brain MRI (OR=14.29, 95%CI: 1.36~150.10, P=0.027) were independent risk factors for poor short-term prognosis. ConclusionAnti-NMDA receptor encephalitis is commonly manifested as motor disorder, personality change, epilepsy and cognitive impairment. Abnormal MRI and cognitive impairment are important predictors of short-term poor prognosis.