Febrile infection-related epilepsy syndrome (FIRES): Pathogenesis, treatment, and outcome: A multicenter study on 77 children

Uri Kramer, Ching Shiang Chi, Kuang Lin Lin, Nicola Specchio, Mustafa Sahin, Heather Olson, Rima Nabbout, Gerhard Kluger, Jainn Jim Lin, Andreas Van Baalen

Research output: Contribution to journalArticle

Abstract

Purpose: 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. Key Findings: 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). Significance: 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.

Original languageEnglish
Pages (from-to)1956-1965
Number of pages10
JournalEpilepsia
Volume52
Issue number11
DOIs
Publication statusPublished - Nov 2011

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Multicenter Studies
Epilepsy
Fever
Infection
Intravenous Immunoglobulins
Ketogenic Diet
Coma
Status Epilepticus
Learning Disorders
Brain Diseases
Therapeutics
Anticonvulsants
Anesthesia
Retrospective Studies
Steroids

Keywords

  • Burst-suppression
  • Children
  • Epilepsy
  • Refractory
  • Status epilepticus

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Febrile infection-related epilepsy syndrome (FIRES) : Pathogenesis, treatment, and outcome: A multicenter study on 77 children. / Kramer, Uri; Chi, Ching Shiang; Lin, Kuang Lin; Specchio, Nicola; Sahin, Mustafa; Olson, Heather; Nabbout, Rima; Kluger, Gerhard; Lin, Jainn Jim; Van Baalen, Andreas.

In: Epilepsia, Vol. 52, No. 11, 11.2011, p. 1956-1965.

Research output: Contribution to journalArticle

Kramer, U, Chi, CS, Lin, KL, Specchio, N, Sahin, M, Olson, H, Nabbout, R, Kluger, G, Lin, JJ & Van Baalen, A 2011, 'Febrile infection-related epilepsy syndrome (FIRES): Pathogenesis, treatment, and outcome: A multicenter study on 77 children', Epilepsia, vol. 52, no. 11, pp. 1956-1965. https://doi.org/10.1111/j.1528-1167.2011.03250.x
Kramer, Uri ; Chi, Ching Shiang ; Lin, Kuang Lin ; Specchio, Nicola ; Sahin, Mustafa ; Olson, Heather ; Nabbout, Rima ; Kluger, Gerhard ; Lin, Jainn Jim ; Van Baalen, Andreas. / Febrile infection-related epilepsy syndrome (FIRES) : Pathogenesis, treatment, and outcome: A multicenter study on 77 children. In: Epilepsia. 2011 ; Vol. 52, No. 11. pp. 1956-1965.
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AU - Sahin, Mustafa

AU - Olson, Heather

AU - Nabbout, Rima

AU - Kluger, Gerhard

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AU - Van Baalen, Andreas

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