FIRES—Pathophysiology, therapeutical approach, and outcome

Abstract

Background: The acronym FIRES stands for febrile infection-related epileptic syndrome, which is a rare epileptic syndrome in the pediatric population. The initial presentation of FIRES is similar to febrile seizures (FS). Both start after a febrile episode; however, in FIRES the epileptic seizure evolves into a super refractory status epilepticus within days despite appropriate treatment. FIRES needs to be diagnosed early and treated by a multidisciplinary team to control the status epilepticus (SE) as fast as possible. Limiting the duration of the SE is paramount for the prevention of catastrophic sequelae such as severe neurologic disabilities or even death. Objective/Conclusion: We describe possible pathophysiological mechanisms and summarize important clinical features of FIRES. The aim of this review is to raise awareness, foster early recognition and improve neurologic long-term outcomes. Moreover, we propose a diagnostic approach and list therapeutic options providing an algorithm.