Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis
Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis
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DOI：
10.3389/fneur.2019.00596
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发表时间：
2019-06-06
影响因子：
3.4
通讯作者：
Zhang, Linmei
中科院分区：
文献类型：
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作者：
Zhang, Min;Li, Wenhui;Zhang, Linmei
目的：抗N-甲基-D-天冬氨酸受体(抗-NMDAR)脑炎是儿童自身免疫性脑炎最常见的类型。本研究旨在探讨我国儿童抗NMDAR脑炎的临床特点和远期疗效。方法：对2015年7月至2018年11月在复旦大学儿童医院就诊的0~18岁儿童抗NMDAR脑炎患者进行回顾性研究。结果：纳入34例抗NMDAR脑炎患者，年龄5个月~14岁，中位年龄7岁，女性18例。中位随访期为20个月(6~39个月)。18例(52.9%)患者以癫痫为首发症状，10例(29.4%)患者有异常行为或认知功能障碍。30例(88.2%)患者在病程中出现两种以上症状。未发现肿瘤。12例(35.2%)患者有异常脑脊液(CSF)表现，包括白细胞增多和蛋白浓度升高。18例(52.9%)脑MRI表现正常。免疫治疗前脑电活动异常27例(79.4%)，癫痫样放电16例(47.0%)。所有患者均接受一线免疫治疗，其中30例(88.2%)和4例(11.8%)分别获得良好(改良Rankin评分0~2)和较差(MRS评分3~6)。结果好和差的两组患者的初始MRS评分有显著差异。18例癫痫患者中14例(77.7%)接受了抗癫痫药物(AED)治疗，12例(85.7%)在最后一次随访时发作缓解。12例患者中10例(83.3%)在1年内退出AED治疗。结论：大多数患者达到惊厥缓解，因此抗NMDAR脑炎的儿童患者可能不需要长期使用AEDs。在我们的患者中，83.3%的患者对一线免疫治疗敏感，取得了良好的效果。免疫治疗前较高的MRS评分预示着不良结果，这突显了对抗NMDAR脑炎患者进行全面评估的必要性。
Objective: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is the most common form of autoimmune encephalitis in pediatric patients. In this study, we aimed to investigate the clinical features and long-term outcomes of pediatric patients with anti-NMDAR encephalitis in China.Methods: We conducted a retrospective study of children (age range: 0-18 years) with anti-NMDAR encephalitis treated at Children's Hospital of Fudan University between July 2015 and November 2018. Demographic characteristics, clinical features, treatment, and outcomes were reviewed.Results: Thirty-four patients with anti-NMDAR encephalitis were enrolled (age range: 5 months to 14 years; median age: 7 years; female: 18). The median follow- up duration was 20 months (range: 6-39 months). Eighteen (52.9%) patients initially presented with seizures and 10 (29.4%) with abnormal (psychiatric) behaviors or cognitive dysfunction. Thirty (88.2%) patients exhibited more than two symptoms during the disease course. No neoplasms were detected. Twelve (35.2%) patients had abnormal cerebrospinal fluid (CSF) findings, including leukocytosis, and increased protein concentration. Eighteen (52.9%) patients exhibited normal brain MRI findings. Electroencephalography revealed abnormal background activity in 27 (79.4%) patients, and epileptiform discharges in 16 (47.0%) patients prior to immunotherapy. All patients received first-line immunotherapy, with 30 (88.2%) and four (11.8%) patients achieving good (Modified Rankin Scale [mRS] score of 0-2) and poor outcomes (mRS score of 3-6), respectively. Initial mRS scores differed significantly between the good and poor outcome groups. Fourteen out of 18 patients (77.7%) with seizures accepted anti-epileptic drug (AED) administration, and seizure freedom was achieved in 12 out of 14 (85.7%) patients at the last follow-up. Ten of these 12 (83.3%) patients withdrew from AED treatment within 1 year.Conclusions: Most patients achieved seizure freedom, so long-term use of AEDs may not be necessary for pediatric patients with anti-NMDAR encephalitis. Among our patients, 83.3% were sensitive to first-line immunotherapy and achieved good outcomes. Higher mRS scores before immunotherapy predicted poor outcomes, highlighting the need for a comprehensive assessment of patients with anti-NMDAR encephalitis.