Febrile infection-related epilepsy syndrome: Refractory status epilepticus and management strategies

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a rare, catastrophic epileptic syndrome that strikes previously healthy children. Its pathogenesis is unknown, it has few treatments, and it is typically refractory. In FIRES, refractory status epilepticus or a cluster of seizures starts a few days after the onset of an acute febrile illness, and it may continue as drug-resistant epilepsy, with neuropsychological impairments occurring without latency. Clinical knowledge and guidelines on FIRES are limited because it is sporadic and extremely rare. To date, the absence of specific bio-markers poses a significant diagnostic challenge; nevertheless, early diagnosis is very important for optimal management. Despite treatment with multiple immunotherapies and anti-seizure medications, the majority of patients with FIRES are left with significant cognitive disabilities and refractory epilepsy. This review aims to highlight the most recent insights into the clinical fea-tures, terminology, epidemiology, pathogenesis, diagnostic challenges, and therapeutic options associated with FIRES.