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.
MONDO:0800500
childhood-onset epilepsy syndrome with developmental and/or epileptic encephalopathy
is_a
AERRPS
exact
DESC syndrome
exact
FIRES
exact
Febrile Infection-Related Epilepsy Syndrome (FIRES)
exact
acute encephalitis with refractory repetitive partial seizures
exact
acute non-herpetic encephalitis with severe refractory status epilepticus
exact
devastating epileptic encephalopathy in school-aged children
exact
fever-induced refractory epileptic encephalopathy in school-aged children
exact
idiopathic catastrophic epileptic encephalopathy
exact
severe refractory status epilepticus owing to presumed encephalitis
exact
status epilepticus owing to presumed encephalitis
related