Febrile infection-related epilepsy syndrome

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Disease Overview

FIRES (Febrile Infection-Related Epilepsy Syndrome) is a sub-type of cryptogenic new-onset refractory status epilepticus (NORSE). NORSE describes a condition in which a healthy person who has not had seizures before, begins having seizures. Over a few days, the seizures increase in frequency and length and evolve into status epilepticus (SE). SE is a prolonged seizure or cluster of seizures during which a person does not regain consciousness between seizures.[7818][14869] The seizure activity is considered refractory because it cannot be controlled with standard anti-seizure medications. Cryptogenic means all possible known causes have been ruled out. People with the sub-type FIRES have a fever a day to two weeks before the beginning of the seizures. The fever may or may not be present when SE begins, but no infection can be found to be causing SE. SE can last for days, weeks, or even months.[7818][14869]

Of note, FIRES previously was considered a separate condition that occurred only in children, while NORSE was described only in adults. However, there is now consensus that FIRES and NORSE without a prior fever can both be described in people of any age.[7818][14922]

You can learn more about FIRES on GARD’s NORSE Information Page.


Synonyms

  • Status epilepticus owing to presumed encephalitis
  • Febrile infection-related epilepsy syndrome
  • FIRES
  • Acute encephalitis with refractory repetitive partial seizures
  • AERRPS
  • Acute non-herpetic encephalitis with severe refractory status epilepticus
  • Devastating epileptic encephalopathy in school-aged children
  • DESC syndrome
  • Fever-induced refractory epileptic encephalopathy in school-aged children
  • Idiopathic catastrophic epileptic encephalopathy
  • Severe refractory status epilepticus owing to presumed encephalitis

For more information, visit GARD.

National Organization for Rare Disorders