title: Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children.
abstract: OBJECTIVE
  To explore the correlations between treatment modalities and selected disease parameters with outcome in febrile infection-related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy with a yet undefined etiology.
  
  
  METHODS
  We conducted a retrospective multicenter study on children who had been included in eight studies published between November 2001 and July 2010. Additional data were retrieved from six of the eight participating centers.
  
  
  RESULTS
  The 77 enrolled patients presented with prolonged refractory status epilepticus. A preceding febrile infection had been reported in 96% of them. Treatment modalities included antiepileptic drugs (a median of six), intravenous immunoglobulin (IVIG, 30 patients), steroids (29 patients), burst-suppression coma (BSC, 46 patients), and other less conventional agents. There was no evidence of efficacy for those treatment modalities except for IVIG (two patients), a ketogenic diet (one patient), and a prolonged cycle of barbiturate anesthesia coma (one patient). Nine patients (11.7%) died during the acute phase of FIRES. Only 12 of the 68 surviving patients (18%) retained normal cognitive level, but most of them had learning disabilities. Sixty-three patients (93%) had refractory epilepsy at follow-up. Cognitive levels at follow-up were significantly associated with duration of BSC (p = 0.005) and younger age at FIRES onset (p = 0.02).
  
  
  CONCLUSIONS
  The outcome of FIRES is poor. No therapeutic agent was efficacious in shortening the acute phase, with the possible exception of a ketogenic diet. Treatment by inducing a prolonged BSC was associated with a worse cognitive outcome.
authors: Pediatric Epilepsy Unit, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel. umkramer@netvision.net.il
authors: Uri
authors: U
authors: Kramer
authors: Ching-Shiang
authors: CS
authors: Chi
authors: Kuang-Lin
authors: KL
authors: Lin
authors: Nicola
authors: N
authors: Specchio
authors: Mustafa
authors: M
authors: Sahin
authors: Heather
authors: H
authors: Olson
authors: Rima
authors: R
authors: Nabbout
authors: Gerhard
authors: G
authors: Kluger
authors: Jainn-Jim
authors: JJ
authors: Lin
authors: Andreas
authors: A
authors: van Baalen
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D001323:Autoantibodies / Q000276:immunology; D001921:Brain / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology / Q000473:pathology; D013226:Status Epilepticus / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D001323:Autoantibodies