Wang Juan, Xie Lingling, Jiang Li
Department of Neurology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, No. 136, Zhongshan 2nd Road, Yuzhong District, Chongqing, 400014, China.
Chongqing Key Laboratory of Neurodevelopment and Cognitive Disorders, Chongqing, 400014, China.
Acta Epileptol. 2025 Jan 10;7(1):3. doi: 10.1186/s42494-024-00187-y.
Febrile infection-related epilepsy syndrome (FIRES) is a rare epilepsy syndrome with unclear pathogenesis, characterized by fever-induced, super-refractory status epilepticus and high mortality. Studies have shown that ketogenic diet (KD) is effective in controlling convulsions in FIRES, but its mechanisms are unclear. This paper intends to summarize the mechanisms by which KD may exert effects against FIRES. Clinical studies have shown that patients with FIRES have elevated levels of various inflammatory factors such as interleukin (IL)-6, IL-8, IL-10, and so on. KD may exert anti-FIRES effects through several potential inflammatory pathways, including nuclear factor -κB (NF-κB) and NLR family pyrin domain containing 3 (NLRP3). Furthermore, the Kyoto Encyclopedia of Genes and Genomes (KEGG) network suggested that KD may play an anti-inflammatory role through several pathways such as cellular senescence and neutrophil extracellular trap formation. These mechanisms need to be further investigated.
发热感染相关癫痫综合征(FIRES)是一种发病机制不明的罕见癫痫综合征,其特征为发热诱发的超难治性癫痫持续状态和高死亡率。研究表明,生酮饮食(KD)对控制FIRES中的惊厥有效,但其机制尚不清楚。本文旨在总结KD可能对FIRES发挥作用的机制。临床研究表明,FIRES患者体内多种炎症因子水平升高,如白细胞介素(IL)-6、IL-8、IL-10等。KD可能通过多种潜在的炎症途径发挥抗FIRES作用,包括核因子-κB(NF-κB)和含NLR家族pyrin结构域蛋白3(NLRP3)。此外,京都基因与基因组百科全书(KEGG)网络表明,KD可能通过细胞衰老和中性粒细胞胞外陷阱形成等多种途径发挥抗炎作用。这些机制有待进一步研究。