目的 回顾性分析儿童免疫相关性脑干脑炎临床特点及治疗预后。方法 收集 2017年3月至2022年10月在神经内科确诊为免疫相关性脑干脑炎患儿的临床资料、治疗及预后随访结果。结果 共收集13 例患儿相关资料，男8例，女5例，首次起病中位数年龄5.0（1.9~7.1）岁，12例起病前 1 月内存在前驱事件。临床表现包括常见的首发的神经系统症状为肢体活动障碍69.2 %（9/13）、面瘫46.2 %（6/13）、意识水平下降46.2 %（6/13）。病程中常见的神经系统体征为颅神经受累76.9 %（10/13），肌力下降69.2 %（9/13）及意识水平下降46.2 %（6/13）。急性期30.8 %（4/13）的患儿出现脑脊液蛋白-细胞分离现象，30.8 %（4/13）的患儿血清抗GQ1b抗体阳性，53.8 %（7/13）的患儿头颅MRI存在脑干异常信号。急性期免疫治疗包括静脉输注甲基泼尼松龙冲击联合静脉输注免疫球蛋白治疗，仅1例患儿效果差，予免疫吸附治疗后好转。急性期出院后末次随访时间为12（6~24）个月，随访期间无复发，仅2例患儿遗留后遗症。结论 儿童免疫相关的脑干脑炎多在5岁左右起病，起病前多有前驱事件，常见的神经系统症状及体征为肢体瘫痪、颅神经麻痹伴意识水平下降。多数患儿一线免疫治疗效果好，较少遗留神经系统后遗症。

Objective To retrospectively analyze the clinical features and prognosis of immune-related brainstem encephalitis in children. Methods The clinical data, treatment and prognostic follow-up results of children diagnosed with immune-related brainstem encephalitis in the Department of Neurology, from March 2017 to October 2022 were collected. Results A total of 13 children (8 boys and 5 girls) were included. The median age of first onset was 5.0 (1.9-7.1) years old, and 12 had precursor events within 1 month before onset. The common initial neurological symptoms in clinical manifestations were limb movement disorder (69.2 %, 9/13), facial paralysis (46.2 %, 6/13), and decreased consciousness level (46.2 %, 6/13). The common neurological signs during the course of the disease were cranial nerve involvement (76.9 %, 10/13), decreased muscle strength (69.2 %, 9/13) and decreased consciousness level (46.2 %, 6/13). In the acute stage, 30.8 % (4/13) of the children showed protein-cell separation in cerebrospinal fluid, 30.8 % (4/13) of the children had positive serum anti-GQ1b antibody, and 53.8 % (7/13) of the children had abnormal brain stem signals on head MRI. The immunotherapy during acute phase was intravenous methylprednisolone combined with immunoglobulin. Only 1 patient had poor effect, but improved after immunoadsorption therapy. The median follow-up time after discharge was 12 (6-24) months, during which there was no recurrence and only 2 cases of residual sequelae. Conclusions Immune-related brainstem encephalitis in children usually occurs at about 5 years of age with prodromal events. The common neurological symptoms and signs include limb paralysis, cranial nerve paralysis and decreased consciousness level. Most children with first-line immunotherapy have good outcomes and few neurological sequelae.