The expanding spectrum of febrile infection-related epilepsy syndrome (FIRES)

Abstract

In recent years, a number of phenomena included in the group of probable epileptic encephalopathies of autoimmune origin have been described. Their common denominator is abrupt onset, usually after a self-limited febrile illness, and a clinical picture of repetitive seizures or difficult-to-control status epilepticus. These symptoms occur mostly in children and have received a wide range of names, despite their highly similar presentations with minor distinctive aspects. Such denominations include acute encephalitis with refractory repetitive partial seizures (AERRPS), febrile infection-related epilepsy syndrome (FIRES) or new-onset refractory status epilepticus (NORSE). Recently, several authors have advocated a simplification of this heterogeneous range of diagnoses, with Ismail and Kossoff suggesting the definition of a single post-infectious syndrome with common characteristics. We report a case of acute encephalopathy after a febrile illness with complete remission of symptoms following antiepileptic and steroid treatment. We conducted an extensive workup including Video-EEG monitoring and speculate on the nature and classification of these syndromes and their growing clinical spectrum.