Hu Wandong, Li Lili, Zhao Fen, Zhang Huan, Zhang Hongwei
Department of Neurology, Children's Hospital Affiliated to Shandong University, Jinan, Shandong, China.
Front Pediatr. 2025 Aug 4;13:1567095. doi: 10.3389/fped.2025.1567095. eCollection 2025.
Developmental epileptic encephalopathy (DEE) is a severe neurological disorder caused by underlying genetic abnormalities and frequent epileptic activity. It is characterized by early-onset, drug-resistant epilepsy, abnormal electroencephalogram (EEG) findings, and developmental delay or regression. DEE is associated with high rates of disability and mortality. The ketogenic diet (KD) is a well-established non-pharmacological treatment for refractory epilepsy and has demonstrated therapeutic efficacy in several DEE subtypes. In certain cases, it may reduce or even eliminate the need for pharmacological interventions. This review discusses the current clinical application of KD in children with DEE and summarizes key factors influencing its therapeutic effectiveness.
发育性癫痫性脑病（DEE）是一种由潜在遗传异常和频繁癫痫发作引起的严重神经系统疾病。其特征为早发性、耐药性癫痫、异常脑电图（EEG）表现以及发育迟缓或倒退。DEE与高致残率和死亡率相关。生酮饮食（KD）是一种已确立的难治性癫痫非药物治疗方法，并且在几种DEE亚型中已显示出治疗效果。在某些情况下，它可能减少甚至消除药物干预的需求。本综述讨论了KD在DEE儿童中的当前临床应用，并总结了影响其治疗效果的关键因素。
