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Febrile infection-related epilepsy syndrome

Disease definition

Febrile infection-related epilepsy syndrome (FIRES) describes an explosive-onset, potentially fatal acute epileptic encephalopathy that develops in previously healthy children and adolescents following the onset of a non-specific febrile illness.

ORPHA:163703

  • 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: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: G40.5
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 11005
  • MedDRA: -

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