Febrile infection-related epilepsy syndrome (FIRES)

Last revised by Rohit Sharma on 6 Feb 2024

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

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

The clinical presentation is with new-onset refractory status epilepticus (NORSE) following (2 weeks to 24 hours prior to development of seizures) a nonspecific febrile illness 2. CSF analysis reveals pleocytosis or elevated protein levels in nearly 75% of cases 3,4.

  • T2 and FLAIR: hyperintensity (acute phase), hypointensity (chronic phase)

  • DWI/ADC: no restricted diffusion

  • T1 C+ (Gd): no enhancement

Treatment is with aggressive initiation and uptitration of antiseizure medications (including anesthesia and ketogenic diet) and early consideration of immunomodulatory treatments (e.g. corticosteroids, intravenous immunoglobulin, rituximab, tocilizumab, anakinra, etc.) 2.

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

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