目的:分析总结儿童急性坏死性脑病(ANEC)的临床特点、诊疗经过、头部影像学变迁及预后随访情况，以期提高临床医师对此疾病的认识。方法:回顾性收集2014年1月至2020年12月入住中国医科大学附属盛京医院儿科重症监护病房(PICU)临床诊断为ANEC的13例患儿资料，其中男7例，女6例；年龄中位数为30个月。分析其临床表现、实验室检查、诊疗经过及头部影像学资料，并对存活患儿进行电话随访。结果:13例患儿入院前均有发热和抽搐症状，发热距离出现意识障碍的时间中位数为48 h，头颅磁共振成像呈对称性多灶性脑损害。13例患儿均应用糖皮质激素治疗，10例输注人免疫球蛋白治疗，8例行血浆置换治疗。13例中死亡7例，随访5例，身高、体质量发育同健康儿童，免疫力正常，1例运动智力同健康同龄儿，儿童整体表现分类量表评分良好，余4例留有不同程度后遗症，均表现为语速慢和运动受限。结论:ANEC多发生于冬季和年幼儿，病情进展迅速，发热抽搐后很快出现意识障碍，死亡率高；早期头部影像学特征性改变是诊断的重要依据，存活患儿多遗留后遗症，多表现为语速慢和运动受限，经系统康复治疗后远期效果良好。“,”Objective:To analyze and summarize the clinical characteristics, diagnosis and treatment, head imaging changes and prognosis of acute necrotizing encephalopathy of childhood (ANEC), aiming to improve the understanding of this disease.Methods:Clinical data of 13 ANEC patients (there were 7 males and 6 females, the median age was 30 months) admitted to the Pediatric Intensive Care Unit (PICU) of Shengjing Hospital, China Medical University from January 2014 to December 2020 were retrospectively analyzed, including clinical manifestations, laboratory examinations, diagnosis and treatment procedures, and head imaging data.Survivors were followed up through telephone.Results:All patients had fever and convulsions before admission, and the median time between fever and consciousness disturbance was 48 hours.The magnetic resonance imaging scans showed symmetrical multifocal brain damages.All 13 patients used glucocorticoid treatment, 10 cases used human immunoglobulin treatment, 8 cases used plasma exchange treatment.A total of 7/13 patients died.Five children were regularly followed up, who presented normal height and weight development and normal immunity.One child had normal motor intelligence with the acceptable Pediatric Overall Performance Category Score, and the remaining 4 children had certain sequelae, mainly manifesting as slow speech speed and limited movement.Conclusions:ANEC mainly occurs in winter and young children with a high mortality.It progresses rapidly, and consciousness disturbance appears soon after fever and convulsion.Survivors usually have sequelae, mostly manifesting as slow speech speed and limited movement, which can be significantly improved after systematic rehabilitation treatment.