Yang Sai, Yang Liming, Liao Hongmei, Chen Mei, Feng Mei, Liu Shulei, Tan Lihong
Department of Neurology, Hunan Children's Hospital, Changsha, China.
Front Pediatr. 2021 Apr 22;9:605042. doi: 10.3389/fped.2021.605042. eCollection 2021.
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.
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。