Febrile Infection-Related Epilepsy Syndrome (FIRES): A Literature Review and Case Study

Febrile infection-related epilepsy syndrome (FIRES) is a catastrophic epileptic syndrome that strikes previously healthy children aged 3-15 years and has an unknown pathogenesis and few treatments. These children experience a nonspecific febrile illness that is followed by prolonged refractory status epilepticus. Although the etiology is unknown, FIRES has a biphasic presentation, with the acute phase beginning as seizure activity lasting 1-12 weeks, then followed by the chronic phase, which is characterized by refractory seizures that cluster every 2-4 weeks, and may continue to be multifocal and independent. Treatment of FIRES is difficult, typically unresponsive to antiepileptic drugs. Some children resolve temporarily with drug-induced burst suppression comas. Other therapies such as a ketogenic diet have limited benefit. The outcome varies with the length of the acute phase and is usually poor, with up to 30% of cases ending in death and 66-100% of survivors having intellectual disability. The authors present a case of a 6-year-old child presenting with FIRES and refractory status epilepticus, which continued despite multidrug therapy. The patient underwent immunomodulatory therapy with the eventual resolution of status, but she developed a chronic, moderately severe encephalopathy, including intractable epilepsy. This case highlights the challenges of FIRES and the potential of immunomodulatory therapies for children with this disorder.