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A Behavioral Screen for Heat-Induced Seizures in Mouse Models of Epilepsy
06:58

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Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77

Uri Kramer1, Ching-Shiang Chi, Kuang-Lin Lin

  • 1Pediatric Epilepsy Unit, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel. umkramer@netvision.net.il

Epilepsia
|September 3, 2011
PubMed

Insights

Febrile infection-related epilepsy syndrome (FIRES) has a poor prognosis, with no consistently effective treatments identified. Prolonged burst-suppression coma may worsen cognitive outcomes in pediatric patients.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Background:

  • Febrile infection-related epilepsy syndrome (FIRES) is a severe form of epileptic encephalopathy with unknown causes.
  • FIRES is characterized by prolonged refractory status epilepticus following a febrile infection.

Purpose of the Study:

  • To investigate the relationship between various treatment strategies and disease parameters with patient outcomes in FIRES.
  • To identify potential therapeutic agents and prognostic factors for FIRES.

Main Methods:

  • A retrospective multicenter study analyzed data from 77 pediatric patients diagnosed with FIRES.
  • Data were collected from eight published studies and six participating centers between November 2001 and July 2010.

Main Results:

  • Most patients (96%) had a preceding febrile infection and received multiple treatments, including antiepileptic drugs, IVIG, steroids, and burst-suppression coma (BSC).
  • No treatment demonstrated consistent efficacy, with only a ketogenic diet showing potential benefit in one case. Mortality was 11.7%.
  • Long-term outcomes were poor, with 93% experiencing refractory epilepsy and only 18% of survivors having normal cognitive function. Cognitive outcomes correlated with BSC duration and younger age at onset.

Conclusions:

  • FIRES has a dismal prognosis with limited treatment options.
  • No therapeutic agent effectively shortened the acute phase, though a ketogenic diet showed promise.
  • Prolonged BSC was associated with worse cognitive outcomes, highlighting the need for careful treatment selection.
Abstract

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