Febrile infection-related epilepsy syndrome

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

Inheritance type

Not applicable

Age of Onset

  • All ages
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