febrile infection-related epilepsy syndrome
MONDO:0015584
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.
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Febrile infection-related epilepsy syndrome
Febrile infection-related epilepsy syndrome (FIRES) is onset of severe seizures (status epilepticus) following a febrile illness in someone who was previously healthy. The seizures may initially be focal; however, often become tonic-clonic. Complications often include intellectual disability, behavioral problems, and ongoing seizures. The underlying cause is unclear. Often there is an upper respiratory tract or gastroenteritis one day to two weeks before onset. Diagnosis involves extensive testing to rule out other possible causes. It is a type of new-onset refractory status epilepticus (NORSE). The seizures are often resistant to treatment. High doses of benzodiazepines or barbiturates are often used, with care taking place in the intensive care unit. A ketogenic diet may help in some cases. The medications anakinra or tocilizumab have been tried. The risk of death, despite treatment is about 12%. The condition newly affects about one in a million children per year. Onset is generally in children between the ages of 2 and 17. Males appear to be more commonly affected than females. Cases consistent with the condition were first described in 1961, with the current name coming into use in 2010.
Background only. Canonical identity remains MONDO; clinical-trial relationships come from ClinicalTrials.gov.Drugs being studied
Disease identity and terminology: MONDO. Trial relationships: ClinicalTrials.gov.