Yoneno Shota, Baba Shimpei, Sumitomo Noriko, Miyamoto Kayoko, Takahashi Kan, Shimizu-Motohashi Yuko, Saito Takashi, Komaki Hirofumi
Department of Child Neurology, National Center of Neurology and Psychiatry, Tokyo, JPN.
Department of Nutrition, National Center of Neurology and Psychiatry, Tokyo, JPN.
Cureus. 2024 Jul 9;16(7):e64149. doi: 10.7759/cureus.64149. eCollection 2024 Jul.
Although the treatment strategy for febrile infection-related epilepsy syndrome (FIRES) is improving, current research focuses on acute management. Evidence for the management of the chronic phase is limited. We present the case of a 19-year-old woman with FIRES who showed excellent response to a ketogenic diet (KD) administered in the chronic phase. At the age of four years, she presented with new-onset super-refractory status epilepticus after a febrile episode. She was diagnosed with FIRES and had profound motor and cognitive deterioration and drug-resistant epilepsy. From the age of 17, she experienced numerous seizures that often led to status epilepticus with respiratory failure, necessitating laryngotracheal separation and nocturnal mechanical ventilation. To improve seizure control, we planned a KD for the first time 15 years after the onset of FIRES. We introduced a classic KD (ketogenic ratio, 3:1) using blended meals through gastrotomy. Two months after starting the KD, she experienced a decrease in seizure frequency and duration. Moreover, as unexpected stabilization of respiration was achieved, mechanical ventilation was stopped. Our case implies that KD may be a promising treatment option for patients with FIRES in the chronic phase, as is believed to be the case in the acute phase.
尽管发热感染相关性癫痫综合征（FIRES）的治疗策略正在改善，但目前的研究集中在急性期管理。关于慢性期管理的证据有限。我们报告一例19岁FIRES女性患者，其在慢性期接受生酮饮食（KD）治疗后反应良好。4岁时，她在一次发热发作后出现新发超难治性癫痫持续状态。她被诊断为FIRES，伴有严重的运动和认知功能衰退以及药物难治性癫痫。17岁起，她经历了无数次癫痫发作，常导致癫痫持续状态并伴有呼吸衰竭，需要进行气管切开和夜间机械通气。为改善癫痫控制，在FIRES发病15年后，我们首次计划采用生酮饮食。我们通过胃造口术采用混合餐引入经典生酮饮食（生酮比例为3:1）。开始生酮饮食两个月后，她的癫痫发作频率和持续时间有所下降。此外，由于呼吸意外地稳定下来，机械通气得以停止。我们的病例表明，生酮饮食可能是FIRES慢性期患者一种有前景的治疗选择，就像在急性期被认为的那样。
