Sai Yang, Zhang Xiao, Feng Mei, Tang Jingwen, Liao Hongmei, Tan Lihong
Department of Neurology, The Children's Hospital of Hunan, Changsha, Hunan 410007, P.R. China.
Exp Ther Med. 2018 Aug;16(2):1442-1448. doi: 10.3892/etm.2018.6329. Epub 2018 Jun 20.
The aim of the present retrospective study was to investigate the diagnosis, treatment and prognosis of pediatric anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis. A total of 23 pediatric patients with anti-NMDAR encephalitis were included in the present study. The clinical data, laboratory test results, imaging examination, treatment outcomes, and follow-up records were reviewed and analyzed. A total of 8 patients exhibited prodromal symptoms, including fever, cough, and vomiting. Clinical symptoms included epilepsy, convulsions, ataxia, coma, dyskinesia, personal behavior change and hallucinations. A total of 20 cases had the initial neurologic symptoms of dyskinesia or seizure, whereas 3 cases exhibited psychiatric symptoms of personal behavior change and hallucinations. Furthermore, pediatric patients >6 years old had more psychiatric symptoms than those ≤6 years. A total of 20 cases exhibited abnormal electroencephalography records, with 1 case of extreme δ brush. A total of 10 cases exhibited abnormal brain magnetic resonance imaging detection. Furthermore, the CSF protein contents for pediatric patients ≤6 years old was significantly higher than those >6 years. For treatment, 18 pediatric patients received the first-line treatment of methylprednisone and intravenous injection of immunoglobulin, and 6 cases were subjected to the second-line treatment of rituximab. A total of 2 patients underwent plasma exchange and/or cyclophosphamide treatment. In follow-up, 12 cases reported no convulsion, whereas 11 cases had moderate or severe neurological and psychiatric sequelae. The recovery rate for pediatric patients ≤6 years old was significantly higher than those >6 years. Anti-NMDAR encephalitis is commonly seen in pediatric patients, mainly with initial neurological symptoms. These patients could respond to immunotherapy, and younger pediatric patients typically have a better prognosis.
本回顾性研究的目的是调查儿童抗N-甲基-D-天冬氨酸受体(抗NMDAR)脑炎的诊断、治疗及预后。本研究共纳入23例儿童抗NMDAR脑炎患者。对其临床资料、实验室检查结果、影像学检查、治疗结果及随访记录进行回顾分析。共有8例患者出现前驱症状,包括发热、咳嗽和呕吐。临床症状包括癫痫、惊厥、共济失调、昏迷、运动障碍、个人行为改变和幻觉。共有20例患者最初的神经系统症状为运动障碍或癫痫发作,而3例患者出现个人行为改变和幻觉等精神症状。此外,6岁以上儿童患者的精神症状比6岁及以下儿童更多。共有20例患者脑电图记录异常,其中1例出现极端δ刷。共有10例患者脑磁共振成像检测异常。此外,6岁及以下儿童患者的脑脊液蛋白含量显著高于6岁以上患者。治疗方面,18例儿童患者接受了甲泼尼龙和静脉注射免疫球蛋白的一线治疗,6例患者接受了利妥昔单抗的二线治疗。共有2例患者接受了血浆置换和/或环磷酰胺治疗。随访中,12例患者报告无惊厥,而11例患者有中度或重度神经和精神后遗症。6岁及以下儿童患者的恢复率显著高于6岁以上患者。抗NMDAR脑炎在儿童患者中较为常见,主要表现为最初的神经系统症状。这些患者对免疫治疗有反应,年龄较小的儿童患者通常预后较好。