CY Mo, CL Yuen, TH Fung

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a very rare disease of unknown pathogenesis. With no established diagnostic criteria in place, diagnosis is made purely on clinical grounds. These children typically experience a prolonged course of refractory status epilepticus with poor response to antiepileptic drugs, hence disease management remains a formidable challenge. Outcomes for these patients are usually poor, with significant neurodevelopmental morbidity and even mortality. Here we report a 6-year-old Chinese girl who was diagnosed to have FIRES, treated aggressively and was able to recover with a relatively good neurological outcome.