| 摘要: |
| 目的:总结儿童抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎临床特点,以提高临床医师对该病的认识。方法:回顾性分析2015年12月至2017年3月于江西省儿童医院神经内科就诊的19例抗NMDAR脑炎患儿的临床表现、辅助检查、治疗、预后。结果:临床主要表现为精神行为异常、抽搐、运动障碍、睡眠障碍;10例头颅MRI异常;17例脑电图异常,主要表现为背景活动慢;5例脑脊液常规异常,淋巴细胞增高为主;2例血清抗NMDAR抗体IgG阴性,17例血清抗NMDAR抗体阳性及19例脑脊液抗体阳性;17例予一线免疫治疗,15例好转;随访16例,15例均好转,其中2例复发。结论:儿童抗NMDAR脑炎无明显性别差异,临床主要表现为精神异常、抽搐、运动障碍、睡眠障碍,出现中枢性通气障碍及合并肿瘤较少见,约半数头颅MRI表现异常,主要为颞叶受损;脑电图主要表现为背景活动慢,脑脊液无明显特异改变,少部分血清免疫抗体可为阴性;多数一线免疫治疗有效,大部分预后良好,复发率低于成人。 |
| 关键词: 抗N-甲基-D-天冬氨酸受体 脑炎 儿童 |
| DOI：doi:10.13407/j.cnki.jpp.1672-108X.2018.09.005 |
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| 基金项目: |
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| Clinical Analysis of 19 Children with Anti-N-Methyl-D-Aspartate Receptor Encephalitis |
| Huang Zhixin1, Zhong Jianmin1, Huang Zhi2, Chen Yong1 |
| (1. Jiangxi Children's Hospital, Jiangxi Nanchang 330006, China; 2. Children's Hospital of Chongqing Medical University, Chongqing 400014, China) |
| Abstract: |
| Objective: To summarize the clinical characteristics of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis in children, so as to increase the awareness of the disease. Methods: Retrospective analysis was conducted on 19 children with anti-NMDAR from Neurology Department of Jiangxi Children's Hospital from Dec. 2015 to Mar. 2017, in terms of clinical manifestations, auxiliary examination, treatment and prognosis. Results: The main clinical manifestations were abnormal mental behavior, convulsion, movement disorder and sleep disturbance; ten cases of cranial MRI were abnormal; seventeen cases of EEG were abnormal, the main performances were slow background; five cases of cerebrospinal fluid routine were abnormal, the lymphocyte growth was dominant; two serum anti-NMDAR antibodies were negative for IgG, and the remaining serum and cerebrospinal fluid antibodies were positive; seventeen patients were treated with first-line immunotherapy and 15 patients were improved; 16 patients were followed up, 15 patients were improved, among which 2 patients relapsed. Conclusion: There is no significant gender difference in anti-NMDAR encephalitis of children. The main clinical manifestations are abnormal mental behavior, convulsion, movement disorder and sleep disturbance, central ventilation disorders and tumors are rare. About half of the cranial MRI are abnormal, mainly performed as the damage of temporal lobe; the main performances of EEG are slow background; there are no specific changes in cerebrospinal fluid, and a small number of serum immune antibodies is negative; most first-line immunotherapy is effective, most of the prognosis is good, and the recurrence rate of children is lower than that of adults. |
| Key words: anti-N-methyl-D-aspartate receptor encephalitis children |
