| 摘要: |
| ［摘要］ 目的 分析儿童自身免疫性脑炎（AE）的临床特征。方法 回顾性分析2020年5月至2024年9月潍坊市人民医院收治的36例AE患儿的临床资料，对其临床表现、辅助检查结果、治疗及预后进行总结分析。结果 36例患儿中男20例（55.56%），女16例（44.44%）。最小发病年龄为1岁，最大发病年龄为13岁6个月，平均发病年龄为（7.21±3.88）岁。急性起病（<1周）16例（44.44%），亚急性起病（1周～3个月）20例（55.56%）。21例（58.33%）发病前存在前驱感染症状。癫痫发作是最常见症状［19例（52.78%）］。36例脑脊液（CSF）的性状及透明度均基本正常。21例（58.33%）CSF有核细胞数增多（>5×106/L），3例（8.33%）CSF氯升高（>130 mmol/L），14例（38.89%）CSF蛋白异常，7例（19.44%）CSF葡萄糖异常。所有患儿的CSF标本均未检测到病原体。36例患儿中抗N-甲基-D-天冬氨酸受体（NMDAR）抗体阳性10例（27.78%），抗髓鞘少突胶质细胞糖蛋白（MOG）抗体阳性7例（19.44%），抗接触蛋白相关样蛋白2（CASPR2）抗体阳性6例（16.67%）。28例（77.78%）存在脑电图（EEG）异常。35例患儿接受颅脑磁共振成像（MRI）检查，其中14例（40.00%）表现异常。接受免疫治疗的患儿中68.75%（22/32）完全康复，无神经精神系统后遗症。结论 学龄期是儿童AE的发病高峰期，多以急性或亚急性起病，常伴前驱感染史，癫痫发作是最常见症状。抗NMDAR脑炎占儿童AE比例最高。AE患儿EEG异常率较高，但缺乏特异性。早期规范化免疫治疗对大多数AE患儿效果显著。 |
| 关键词: 自身免疫性脑炎 儿童 自身抗体 临床特征 |
| DOI：10.3969/j.issn.1674-3806.2025.06.18 |
| 分类号: |
| 基金项目: |
|
| Analysis on clinical characteristics of 36 cases of autoimmune encephalitis in children |
|
XU Li1, WANG Qianyun2, SHAO Xianli2, ZHANG Fan1, ZHAO Huafeng2
|
|
1.School of Clinical Medicine, Shandong Second Medical University, Weifang 261042, China; 2.Department of Pediatric Internal Medicine, Weifang People′s Hospital, Weifang 261041, China
|
| Abstract: |
| ［Abstract］ Objective To analyze the clinical characteristics of autoimmune encephalitis(AE) in children. Methods A retrospective analysis was conducted on the clinical data of 36 pediatric patients with AE who were admitted to Weifang People′s Hospital from May 2020 to September 2024. Their clinical manifestations, auxiliary examination results, treatments and prognosis were summarized and analyzed. Results Among the 36 patients, 20 patients were male(55.56%) and 16 patients were female(44.44%). The minimum age of the patient at disease onset was 1 year, the maximum age of the patient at disease onset was 13 years and 6 months, and the average age of the patient at disease onset was (7.21±3.88)years. There were 16 patients(44.44%) with acute onset(<1 week) and 20 patients(55.56%) with subacute onset(1 week to 3 months). Twenty-one patients(58.33%) had prodromal infection symptoms before disease onset. Epileptic seizures were the most common symptom［19 patients(52.78%)］. The cerebrospinal fluid(CSF) properties and transparency of 36 patients were basically normal. The number of nucleated cells in the CSF was increased(>5×106/L) in 21 patients(58.33%), and the CSF chlorine was increased(>130 mmol/L) in 3 patients(8.33%), and abnormal CSF protein occurred in 14 patients(38.89%), and abnormal CSF glucose occurred in 7 patients(19.44%). No pathogens were found in the CSF specimens of all the patients. Among the 36 patients, anti-N-methyl-D-aspartate receptor(NMDAR) antibody was positive in 10 patients(27.78%), and anti-myelin oligodendrocyte glycoprotein(MOG) antibody was positive in 7 patients(19.44%), and anti-contactin-associated protein-like 2(CASPR2) antibody was positive in 6 cases(16.67%). Twenty-eight patients(77.78%) had electroencephalogram(EEG) abnormalities. Thirty-five patients underwent cerebral magnetic resonance imaging(MRI) examinations, among whom 14 patients(40.00%) showed abnormal manifestations. Among the patients who received immunotherapy, 68.75%(22/32) of them had a complete recovery without any neuropsychiatric sequelae. Conclusion School age period is the peak period for the onset of AE in children. Its clinical manifestations mostly present with acute or subacute onset, often accompanied by a history of prodromal infection, and epileptic seizures are the most common symptom. The children with anti-NMDAR encephalitis account for the highest proportion of the children with AE. The abnormal rate of EEG in AE children is relatively high, but the abnormities lack specificity. Early standardized immunotherapy has a significant effect on most AE children. |
| Key words: Autoimmune encephalitis(AE) Children Autoantibodies Clinical characteristics |
