Febrile infection-related epilepsy syndrome

Febrile infection-related epilepsy syndrome (FIRES) is a severe postinfectious neurological disorder that presents with status epilepticus in a previously normal child (or less commonly adult) after a febrile illness.

Terminology

FIRES has received several names in the literature:

  • acute encephalopathy of obscure origin in infants and children
  • acute encephalitis with refractory, repetitive partial seizures (AERRPS)
  • acute encephalopathy with inflammation-mediated status epilepticus
  • devastating epilepsy in school-aged children (DESC)
  • idiopathic catastrophic epileptic encephalopathy
  • fever-induced refractory epileptic encephalopathy in school-age children
  • severe refractory status epilepticus owing to presumed encephalitis

Treatment and prognosis

Two-thirds of the surviving children suffer from mild to severe cognitive impairment.

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Article Information

rID: 49208
Section: Pathology
Synonyms or Alternate Spellings:

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