Orphanet: Febrile infection related epilepsy syndrome

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Febrile infection-related epilepsy syndrome

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • 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
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: G40.5
  • ICD-11: 8A63.Y
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 11005
  • MedDRA: -
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