Tian Maoqiang, Li Juan, Lei Wenting, Shu Xiaomei
Department of Pediatrics, Zunyi Medical University, First Affiliated Hospital, Zunyi, Guizhou Province, China.
Neuropediatrics. 2019 Apr;50(2):111-115. doi: 10.1055/s-0038-1675607. Epub 2019 Jan 8.
Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARe) was originally described as a paraneoplastic disease with more than 50% cases involving a tumor. However, tumor incidence in anti-NMDARe in children is much lower. Herpes simplex virus-induced anti-NMDARe has been well-described; however, findings on Japanese encephalitis virus (JEV)-induced anti-NMDARe are scarce. Here, we describe a 7-year-old boy who presented with fever and headache that progressed to seizures and disturbance of consciousness. Brain magnetic resonance imaging (MRI) revealed abnormalities in the bilateral globus pallidus. The diagnosis of JE was made based on a positive JE antibody test results in serum and cerebrospinal fluid. Antiviral and symptomatic therapies led to rapid recovery. Four weeks after the onset of JE, the patient presented with emotional and behavioral disturbances, sleep difficulty, and extrapyramidal symptoms. MRI showed symmetrical lesions in the bilateral thalami and basal ganglia which were expanded than those on the original scan. Antibodies against NMDAR were detected and immunotherapy led to significant recovery. This case and our literature review suggest that JEV may be a clinically important cause of anti-NMDARe in children. Patients with JE-induced anti-NMDARe present with symptoms similar to those of patients with primary anti-NMDARe. Most patients with JE-induced anti-NMDARe showed a good response to first-line immunotherapies.
抗 N-甲基-D-天冬氨酸受体脑炎(抗 NMDAR 脑炎)最初被描述为一种副肿瘤性疾病,超过 50%的病例与肿瘤有关。然而,儿童抗 NMDAR 脑炎的肿瘤发生率要低得多。单纯疱疹病毒引起的抗 NMDAR 脑炎已有充分描述;然而,关于日本脑炎病毒(JEV)引起的抗 NMDAR 脑炎的研究结果却很少。在此,我们报告一名 7 岁男孩,他最初出现发热和头痛,随后进展为癫痫发作和意识障碍。脑磁共振成像(MRI)显示双侧苍白球异常。根据血清和脑脊液中 JEV 抗体检测结果阳性确诊为乙型脑炎。抗病毒和对症治疗使病情迅速恢复。乙型脑炎发病四周后,患者出现情绪和行为障碍、睡眠困难及锥体外系症状。MRI 显示双侧丘脑和基底节有对称性病变,较初次扫描时扩大。检测到抗 NMDAR 抗体,免疫治疗使病情显著恢复。该病例及我们的文献综述表明,JEV 可能是儿童抗 NMDAR 脑炎的一个重要临床病因。JEV 引起的抗 NMDAR 脑炎患者的症状与原发性抗 NMDAR 脑炎患者相似。大多数 JEV 引起的抗 NMDAR 脑炎患者对一线免疫治疗反应良好。