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抗N-甲基-D-天冬氨酸受体脑炎患儿的临床特征及预后因素

Clinical Characteristics and Prognostic Factors of Children With Anti-N-Methyl-D-Aspartate Receptor Encephalitis.

作者信息

Yang Sai, Yang Liming, Liao Hongmei, Chen Mei, Feng Mei, Liu Shulei, Tan Lihong

机构信息

Department of Neurology, Hunan Children's Hospital, Changsha, China.

出版信息

Front Pediatr. 2021 Apr 22;9:605042. doi: 10.3389/fped.2021.605042. eCollection 2021.

Abstract

Anti-N-methyl-D-aspartate (anti-NMDA) receptor encephalitis is the most common autoimmune encephalitis in pediatric patients. The study aimed to investigate the clinical characteristics and prognostic factors of anti-NMDA receptor encephalitis in children in South China. This was a retrospective study of children diagnosed with anti-NMDA receptor encephalitis between 01/2014 and 12/2017 at Hunan Children's Hospital. Laboratory, brain magnetic resonance imaging (MRI), and electroencephalography data were collected. The short-term (6-month) outcomes were assessed using the Liverpool score by the same pediatric neurologist. The children were divided into good (scores 4-5) and poor (score <3) clinical outcomes. Among the 51 patients, 21 (41.2%) were male. The most common clinical symptoms were dyskinesia (88.2%), personality change (84.3%), seizure (82.4%), and cognitive disorder (31.4%). Two were transferred to another hospital, 45 (91.8%) received intravenous immunoglobulins, 41 (83.7%) received methylprednisolone, and 8 (16.3%) received plasma exchange. Eight (16.3%) received rituximab for second-line treatment, six after intravenous immunoglobulin and methylprednisolone treatment, and two after plasma exchange therapy failed. Seven were lost to follow-up. The short-term outcome was good in 23 patients. Cognitive disorder [odds ratio (OR): 23.97, 95% confidence interval (CI): 1.12-513.30, = 0.042) and abnormal brain MRI (OR: 14.29, 95% CI: 1.36-150.10, = 0.027] were independently associated with a poor short-term outcome after adjustment for age, GCS, and rituximab use. MRI abnormalities and cognitive disorders are independently associated with poor short-term outcomes in children with anti-NMDA receptor encephalitis. The use of rituximab is not associated with the 6-month outcomes.

摘要

抗N-甲基-D-天冬氨酸(抗NMDA)受体脑炎是儿科患者中最常见的自身免疫性脑炎。本研究旨在探讨中国南方儿童抗NMDA受体脑炎的临床特征及预后因素。这是一项对2014年1月至2017年12月期间在湖南省儿童医院诊断为抗NMDA受体脑炎的儿童进行的回顾性研究。收集了实验室检查、脑磁共振成像(MRI)和脑电图数据。由同一位儿科神经科医生使用利物浦评分评估短期(6个月)结局。将儿童分为临床结局良好(评分4 - 5分)和不良(评分<3分)两组。51例患者中,21例(41.2%)为男性。最常见的临床症状为运动障碍(88.2%)、性格改变(84.3%)、癫痫发作(82.4%)和认知障碍(31.4%)。2例转至其他医院,45例(91.8%)接受静脉注射免疫球蛋白治疗,41例(83.7%)接受甲泼尼龙治疗,8例(16.3%)接受血浆置换治疗。8例(16.3%)接受利妥昔单抗进行二线治疗,6例在静脉注射免疫球蛋白和甲泼尼龙治疗后使用,2例在血浆置换治疗失败后使用。7例失访。23例患者短期结局良好。在调整年龄、格拉斯哥昏迷量表(GCS)和利妥昔单抗使用情况后,认知障碍[比值比(OR):23.97,95%置信区间(CI):1.12 - 513.30,P = 0.042]和脑MRI异常(OR:14.29,95%CI:1.36 - 150.10,P = 0.027)与短期不良结局独立相关。MRI异常和认知障碍与抗NMDA受体脑炎患儿的短期不良结局独立相关。利妥昔单抗的使用与6个月结局无关。

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