Febrile infection–related epilepsy syndrome (FIRES): A nonencephalitic encephalopathy in childhood
- 1 July 2010
- Vol. 51 (7), 1323-1328
- https://doi.org/10.1111/j.1528-1167.2010.02535.x
Abstract
Encephalitis is generally presumed, even when seizures follow banal febrile infection, and pathogen detection in cerebrospinal fluid fails. This retrospective multicenter case series reports on 22 previously healthy children aged 3-15 years (median 6.5 years) with prolonged or recurrent seizures occurring 2-14 days (median 5 days) after fever onset (19 children with respiratory or nonspecific infections). Cerebrospinal fluid studies revealed 2-42 cells/microl (median 5 cells/microl) and no pathogens. Electroencephalography showed diffuse slowing or multifocal discharges. Neuroimaging demonstrated normal findings in 10 children. Brain biopsies were performed in seven children showing gliosis but no inflammation. Anesthetic barbiturates were used in 14 children with refractory status epilepticus, and immunotherapy in 9. Two children died, eight remained in a state of impaired consciousness, eight developed therapy-refractory epilepsies, two had behavioral disturbances, and two recovered. The lack of evidence for encephalitis suggests another infection-related pathogenesis of this disastrous epileptic encephalopathy. Therefore, we propose the term "febrile infection-related epilepsy syndrome" (FIRES).This publication has 8 references indexed in Scilit:
- Epileptic encephalopathy in children possibly related to immune-mediated pathogenesisBrain & Development, 2010
- Analysis of status epilepticus related presumed encephalitis in childrenEuropean Journal of Paediatric Neurology, 2008
- Clinical Characteristics of Acute Encephalopathy of Obscure Origin: A Biphasic Clinical Course Is a Common FeatureNeuropediatrics, 2006
- Devastating epileptic encephalopathy in school-aged children (DESC): A pseudo encephalitisEpilepsy Research, 2006
- Severe Refractory Status Epilepticus Owing to Presumed EncephalitisJournal of Child Neurology, 2005
- Idiopathic catastrophic epileptic encephalopathy presenting with acute onset intractable statusSeizure, 2003
- Outcome of Severe Refractory Status Epilepticus in ChildrenEpilepsia, 2001
- THE ACUTE ENCEPHALOPATHIES OF OBSCURE ORIGIN IN INFANTS AND CHILDRENBrain, 1961