Febrile infection-related epilepsy syndrome
Febrile infection-related epilepsy syndrome
Definition
A rare, potentially fatal, epileptic encephalopathy characterized by explosive-onset of recurrent multifocal and bilateral tonic-clonic seizures following an unspecific febrile illness. The syndrome develops without a clear acute structural, toxic or metabolic cause, in a patient without previous epilepsy. FIRES is a subgroup of new-onset refractory status epilepticus (NORSE), and requires a preceding febrile infection as a mandatory feature.
Also known as acute encephalitis with refractory repetitive partial seizures, acute non-herpetic encephalitis with severe refractory status epilepticus, AERRPS, DESC syndrome, devastating epileptic encephalopathy in school-aged children, Febrile Infection-Related Epilepsy Syndrome (FIRES), fever-induced refractory epileptic encephalopathy in school-aged children, FIRES, idiopathic catastrophic epileptic encephalopathy, severe refractory status epilepticus owing to presumed encephalitis — per MONDO