Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases

Jacqueline S. Gofshteyn, Angus Wilfong, Orrin Devinsky, Judith Bluvstein, Joshi Charuta, Michael A. Ciliberto, Linda Laux, Eric D. Marsh*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

66 Scopus citations

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a devastating epilepsy affecting normal children after a febrile illness. FIRES presents with an acute phase with super-refractory status epilepticus and all patients progress to a chronic phase with persistent refractory epilepsy. The typical outcome is severe encephalopathy or death. The authors present 7 children from 5 centers with FIRES who had not responded to antiepileptic drugs or other therapies who were given cannabadiol (Epidiolex, GW Pharma) on emergency or expanded investigational protocols in either the acute or chronic phase of illness. After starting cannabidiol, 6 of 7 patients' seizures improved in frequency and duration. One patient died due to multiorgan failure secondary to isoflourane. An average of 4 antiepileptic drugs were weaned. Currently 5 subjects are ambulatory, 1 walks with assistance, and 4 are verbal. While this is an open-label case series, the authors add cannabidiol as a possible treatment for FIRES.

Original languageEnglish (US)
Pages (from-to)35-40
Number of pages6
JournalJournal of child neurology
Volume32
Issue number1
DOIs
StatePublished - Jan 1 2017

Keywords

  • cannabidiol
  • febrile infection-related epilepsy syndrome (FIRES)
  • pediatric epilepsy
  • refractory status epilepticus

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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