title: Febrile infection-related epilepsy syndrome: a study of 12 patients.
abstract: OBJECTIVE
  To analyze the electroclinical features, neuroimaging findings, treatment, and outcome of 12 patients with febrile infection-related epilepsy syndrome (FIRES).
  
  
  METHODS
  This is a retrospective study of 12 children with FIRES with a mean time of follow-up of 6.5 years carried out at the Garrahan Hospital of Buenos Aires between 1997 and 2012.
  
  
  RESULTS
  Eight males and four females had focal status epilepticus preceded by febrile infection with a mean age at presentation of 8.5 years. In the acute period, the treatment included antiepileptic drugs (AEDs) in all cases, immunotherapy in 10 cases, and burst-suppression coma in eight. The ketogenic diet was tried in two, plasmapheresis in one, and rituximab in one. Two patients treated with IVIG and one patient given steroids had a good response, but in this phase only three patients had a prolonged good response to IVIG and a ketogenic diet. No patients died in this period. In the chronic epilepsy phase, all children had seizures arising from neocortical regions. All patients had refractory epilepsy, and most mental retardation, and behavioral disturbances. All received different AEDs and in this phase a third patient was put on a ketogenic diet. One patient was operated without good results. Only two cases had a good outcome after 2 and 10 years of follow-up.
  
  
  CONCLUSIONS
  FIRES is a well-defined severe epileptic syndrome, probably in the group of epileptic encephalopathies, characterized by focal or multifocal seizures arising from the neocortical regions with an unknown etiology. Immunoglobulin and the ketogenic diet may be considered a potentially efficacious treatment.
authors: Servicio de Neurología, Hospital de Pediatría Prof Dr Juan P Garrahan, Buenos Aires, Argentina. rhcaraballo@arnet.com.ar
authors: Roberto H
authors: RH
authors: Caraballo
authors: Gabriela
authors: G
authors: Reyes
authors: Maria Francisca Lopez
authors: MF
authors: Avaria
authors: Maria Celeste
authors: MC
authors: Buompadre
authors: Mariana
authors: M
authors: Gonzalez
authors: Sebastian
authors: S
authors: Fortini
authors: Ricardo
authors: R
authors: Cersosimo
mesh_terms: D000212:Acyclovir / Q000627:therapeutic use; D000293:Adolescent; D000998:Antiviral Agents / Q000627:therapeutic use; D001118:Argentina; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D018792:Encephalitis, Viral / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous; D007964:Leukocytosis; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000503:physiopathology*; D013226:Status Epilepticus / Q000209:etiology / Q000503:physiopathology; D013577:Syndrome; D016896:Treatment Outcome; D014474:Unconsciousness / Q000209:etiology / Q000503:physiopathology
chemical_list: D000998:Antiviral Agents; D016756:Immunoglobulins, Intravenous; D000212:Acyclovir
keywords: Encephalopathy; Epilepsy; Febrile; Focal status epilepticus; Neocortical; Refractory