目的 总结儿童急性坏死性脑病（ANEC）的临床特征、治疗过程和预后。方法 回顾性分析2016年6月至2022年12月确诊ANEC患儿的临床资料。对存活患儿进行电话随访，应用儿童整体表现分类量表（POPC）评估患儿生存质量。结果 共纳入26例ANEC患儿，男女比例为1.6∶1，中位年龄36.0（23.0~69.0）个月。发病季节以夏季常见（42.3%），其次为冬季（34.6%）；前驱感染以流感病毒感染多见（34.6%），其次为人类疱疹病毒6B型及新型冠状病毒。所有患儿均有发热伴不同程度意识障碍，24例出现惊厥。神经影像学以对称性丘脑受累（100%）为典型改变，多伴有基底节区（50.0%）、脑干（61.5%）、小脑（26.9%）等部位受累。23例患儿接受甲基泼尼松龙激素冲击治疗，住院病死率为 23.1%（6/26），出院6个月后累积病死率为30.7%（8/26）。6个月后随访到14例存活患儿，11例接受康复治疗，其中POPC 评估2分1例，3分4例，4分6例，5分1例，6分2例。3例新型冠状病毒感染后ANEC患儿3个月随访POPC评估结果为2分2例，6分1例。结论 华南地区ANEC于夏季和冬季高发，新型冠状病毒感染可诱发ANEC；临床表现为发热、抽搐、意识障碍三联征，典型影像学改变为双侧丘脑受累。早期免疫治疗及综合康复训练有助于减少神经系统后遗症的严重程度。

Objective To summarize the clinical characteristics, diagnosis and treatment and prognosis of acute necrotizing encephalopathy of childhood (ANEC). Methods The clinical data of ANEC patients from June 2016 to December 2022 were retrospectively analyzed. Survivors were followed up through telephone. Survivors' life quality was measured using the pediatric overall performance category scale (POPC). Results A total of 26 children with ANEC were included, with a male to female ratio of 1.6:1 and a median age of 36.0 (23.0-69.0) months. The most common season was summer (42.3%), followed by winter (34.6%). Influenza virus was the most common preceding infection (34.6%), followed by human herpesvirus 6B and SARS-CoV-2. All patients had fever and different degrees of consciousness disorder, and 24 had convulsions. Symmetrical thalamic involvement (100%) was the typical change in imaging, with basal ganglia (50.0%), brainstem (61.5%), cerebellum (26.9%) and other sites involved. Twenty-three patients received methylprednisolone shock therapy, and the in-hospital fatality rate was 23.1% (6/26). The cumulative case fatality rate 6 months after discharge was 30.7% (8/26). The 14 surviving children were followed up regularly for 6 months, and 11 received rehabilitation treatment. One child received two points for the POPC, four received three, six received four, one received five, and two received six. The POPC results of 3 patients with ANEC after SARS-CoV-2 infection at 3 months follow-up were 2 in 2 children and 6 in one child. Conclusions ANEC occurs frequently in summer and winter in South China, and can be induced by SARS-CoV-2 infection. The typical signs of ANEC are fever, convulsion, and disturbance of consciousness, and the typical imaging changes were bilateral thalamic involvement. Early immunotherapy and comprehensive rehabilitation training can reduce the severity of neurological sequelae.