Ochiai Kenta, Omata Taku, Sano Kentaro, Sakuma Hiroshi, Takanashi Jun-Ichi
Department of Pediatrics, Tokyo Women's Medical University Yachiyo Medical Center, Yachiyo, JPN.
Department of Brain and Neurosciences, Tokyo Metropolitan Institute of Medical Science, Tokyo, JPN.
Cureus. 2025 Jan 16;17(1):e77568. doi: 10.7759/cureus.77568. eCollection 2025 Jan.
Febrile infection-related epilepsy syndrome (FIRES) is a type of new-onset refractory status epilepticus (NORSE) that occurs in previously healthy children. Conventional antiepileptic drugs (AEDs) often fail to control seizures, necessitating the use of high-dose anesthetics, which can lead to severe complications and poor outcomes. Perampanel has shown promise in the treatment of refractory epilepsy; however, its role in FIRES remains underexplored. We report the case of a 13-year-old boy with FIRES, which was characterized by refractory status epilepticus following a febrile illness. Although some AEDs were administered, high-dose thiopental and ventilator support were required. Perampanel at a starting dose of 2 mg/day and titrated to 8 mg/day enabled successful weaning from thiopental and extubation. The patient eventually became seizure-free on clobazam and levetiracetam. On follow-up, the patient exhibited memory and behavioral issues, along with bilateral hippocampal atrophy on MRI. This case demonstrated the potential role of perampanel in managing FIRES by reducing the need for prolonged barbiturate use and ventilator dependence. Although there was persistent cognitive impairment, which was likely secondary to hippocampal damage, perampanel showed a favorable safety profile. This case suggested that perampanel is a valuable addition to FIRES treatment. Further studies are required to confirm its efficacy.
发热感染相关性癫痫综合征(FIRES)是一种新发难治性癫痫持续状态(NORSE),发生于既往健康的儿童。传统抗癫痫药物(AEDs)往往无法控制癫痫发作,因此需要使用高剂量麻醉剂,这可能导致严重并发症和不良预后。吡仑帕奈在难治性癫痫治疗中已显示出前景;然而,其在FIRES中的作用仍未得到充分探索。我们报告了一例13岁患有FIRES的男孩病例,其特征为发热性疾病后出现难治性癫痫持续状态。尽管使用了一些AEDs,但仍需要高剂量硫喷妥钠和呼吸机支持。起始剂量为2mg/天的吡仑帕奈,并滴定至8mg/天,使患者成功停用硫喷妥钠并拔管。患者最终在服用氯巴占和左乙拉西坦后无癫痫发作。随访时,患者出现记忆和行为问题,MRI显示双侧海马萎缩。该病例证明了吡仑帕奈在管理FIRES方面的潜在作用,即减少长期使用巴比妥类药物的需求和对呼吸机的依赖。尽管存在持续性认知障碍,这可能继发于海马损伤,但吡仑帕奈显示出良好的安全性。该病例表明吡仑帕奈是FIRES治疗中有价值的补充药物。需要进一步研究来证实其疗效。