febrile infection-related epilepsy syndrome

Rare Disease

MONDO:0015584

Also Known As

AERRPSDESC syndromeFIRESFebrile Infection-Related Epilepsy Syndrome (FIRES)acute encephalitis with refractory repetitive partial seizuresacute non-herpetic encephalitis with severe refractory status epilepticusdevastating epileptic encephalopathy in school-aged childrenfever-induced refractory epileptic encephalopathy in school-aged childrenidiopathic catastrophic epileptic encephalopathysevere refractory status epilepticus owing to presumed encephalitisstatus epilepticus owing to presumed encephalitis

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.