儿童流感相关急性坏死性脑病的病理机制 与分层治疗
Pathogenic Mechanisms and Stratified Treatment of Influenza-Associated Acute Necrotizing Encephalopathy in Children
DOI: 10.12677/acm.2026.1651938, PDF,   
作者: 林雯雯, 蒋 莉*：重庆医科大学附属儿童医院神经内科，儿童少年健康与疾病国家临床医学研究中心，儿童发育疾病研究教育部重点实验室，儿童神经发育与认知障碍重庆市重点实验室，重庆
关键词: 流感相关急性坏死性脑病；发病机制；免疫治疗；分层治疗；Influenza-Associated Acute Necrotizing Encephalopathy； Pathogenesis； Immunotherapy； Stratified Treatment
摘要: 流感相关急性坏死性脑病(influenza-associated acute necrotizing encephalopathy, IANE)以双侧丘脑对称性病变及快速进展的神经系统功能障碍为特征。其核心病理机制为感染诱发的细胞因子风暴，IL-6作为其关键效应分子，而遗传易感基因则可能提高疾病发生与复发的风险。目前IANE尚无统一治疗方案，临床上主要围绕抗病毒治疗、免疫调节和重症监护支持展开，常用的免疫治疗包括糖皮质激素、静脉注射免疫球蛋白(IVIG)、IL-6受体拮抗药物及血浆置换等。基于ANE-SS评分、脑干受累情况、IL-6水平及遗传背景构建的多维度风险分层是实现IANE个体化治疗的核心前提。低中危患儿可采用高剂量糖皮质激素单药或联合IVIG，高危患儿则建议早期启动糖皮质激素联合托珠单抗的强化方案，并根据病情演变动态调整。以风险分层为指导实施阶梯化与精准化治疗，有助于降低病死率并改善远期神经功能预后。
Abstract: Influenza-associated acute necrotizing encephalopathy (IANE) is characterized by rapidly progressive neurological dysfunction accompanied by symmetrical bilateral thalamic lesions. The central pathogenic mechanism involves infection-induced cytokine storm, in which interleukin-6 (IL-6) acts as a key effector molecule, while genetic susceptibility loci may increase the risk of disease onset and recurrence. Currently, there is no standardized treatment protocol for IANE. Management mainly focuses on antiviral therapy, immunomodulation, and intensive care support, with commonly used immunotherapeutic interventions including corticosteroids, intravenous immunoglobulin (IVIG), IL-6 receptor antagonists, and plasma exchange. A multidimensional risk stratification model incorporating ANE severity score (ANE-SS), brainstem involvement, IL-6 levels, and genetic background constitutes the basis for individualized treatment in IANE. High-dose corticosteroid monotherapy or its combination with IVIG may be appropriate for patients at low to intermediate risk, whereas early initiation of an intensified regimen combining corticosteroids with tocilizumab is recommended for high-risk patients, with dynamic adjustment according to clinical evolution. Risk-stratified, stepwise, and precision-based therapeutic strategies may help reduce mortality and improve long-term neurological outcomes.
文章引用：林雯雯, 蒋莉. 儿童流感相关急性坏死性脑病的病理机制 与分层治疗[J]. 临床医学进展, 2026, 16(5): 1368-1376. https://doi.org/10.12677/acm.2026.1651938

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