What is Febrile infection-related epilepsy syndrome?
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.
Other condition names
AERRPS
Acute encephalitis with refractory repetitive partial seizures
Acute non-herpetic encephalitis with severe refractory status epilepticus
DESC syndrome
Devastating epileptic encephalopathy in school-aged children
FIRES
Fever-induced refractory epileptic encephalopathy in school-aged children
Idiopathic catastrophic epileptic encephalopathy
Severe refractory status epilepticus owing to presumed encephalitis