Wu Pei-Yu, Chi Ching-Shiang, Tsai Chi-Ren, Yang Yao-Lun, Lee Hsiu-Fen
Division of Pediatric Neurology, Children's Medical Center, Taichung Veterans General Hospital, 1650, Taiwan Boulevard Sec. 4, Taichung 407, Taiwan.
Department of Post-Baccalaureate Medicine, College of Medicine, National Chung Hsing University, 250, Kuo Kuang Rd., Taichung 402, Taiwan.
Children (Basel). 2023 Jan 18;10(2):182. doi: 10.3390/children10020182.
Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common autoimmune encephalitis in children. There is a high probability of recovery if treated promptly. We aimed to analyze the clinical features and long-term outcomes of pediatric patients with anti-NMDA receptor encephalitis.
We conducted a retrospective study with definite diagnoses of anti-NMDA receptor encephalitis in 11 children treated in a tertiary referral center between March 2012 and March 2022. Clinical features, ancillary tests, treatment, and outcomes were reviewed.
The median age at disease onset was 7.9 years. There were eight females (72.7%) and three males (27.3%). Three (27.3%) patients initially presented with focal and/or generalized seizures and eight (72.7%) with behavioral change. Seven patients (63.6%) revealed normal brain MRI scans. Seven (63.6%) had abnormal EEG results. Ten patients (90.1%) received intravenous immunoglobulin, corticosteroid, and/or plasmapheresis. After a median follow-up duration of 3.5 years, one patient was lost to follow-up at the acute stage, nine (90%) had an mRS ≤ 2, and only one had an mRS of 3.
With the early recognition of anti-NMDA receptor encephalitis based on its clinical features and ancillary tests, we were able to treat patients promptly with first-line treatment and achieve favorable neurological outcomes.
抗N-甲基-D-天冬氨酸(NMDA)受体脑炎是儿童最常见的自身免疫性脑炎。若及时治疗,恢复的可能性很大。我们旨在分析儿童抗NMDA受体脑炎患者的临床特征和长期预后。
我们对2012年3月至2022年3月在一家三级转诊中心接受治疗的11例确诊为抗NMDA受体脑炎的儿童进行了回顾性研究。对临床特征、辅助检查、治疗及预后进行了回顾。
发病时的中位年龄为7.9岁。有8名女性(72.7%)和3名男性(27.3%)。3例(27.3%)患者最初表现为局灶性和/或全身性癫痫发作,8例(72.7%)表现为行为改变。7例(63.6%)患者脑部MRI扫描结果正常。7例(63.6%)脑电图结果异常。10例(90.1%)患者接受了静脉注射免疫球蛋白、皮质类固醇和/或血浆置换。中位随访时间为3.5年后,1例患者在急性期失访,9例(90%)改良Rankin量表(mRS)评分≤2,只有1例mRS评分为3。
基于抗NMDA受体脑炎的临床特征和辅助检查进行早期识别,我们能够及时用一线治疗方法治疗患者并取得良好的神经学预后。