Abstract
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.