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.
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.
- 1. Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children.
- 2. Febrile infection-related epilepsy syndrome | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. (2016, January 11). Retrieved November 15, 2016, from https://rarediseases.info.nih.gov/diseases/11005/febrile-infection-related-epilepsy-syndrome
- 3. Sakuma H, Tanuma N, Kuki I, Takahashi Y, Shiomi M, Hayashi M. Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry. 2015 Jul;86(7):820-2.