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

doi:10.1177/0883073816669450

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

Journal article

Peer reviewed

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 and Eric D Marsh

Journal of child neurology, Vol.32(1), pp.35-40

01/2017

DOI: 10.1177/0883073816669450

PMID: 27655472

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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.

Acute Disease

Fever - complications

Humans

Status Epilepticus - etiology

Anticonvulsants - therapeutic use

Status Epilepticus - drug therapy

Male

Treatment Outcome

Epileptic Syndromes - drug therapy

Drug Resistant Epilepsy - drug therapy

Drug Resistant Epilepsy - etiology

Epileptic Syndromes - etiology

Cannabidiol - therapeutic use

Child

Chronic Disease

Infection - complications

Details

Title: Subtitle: Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases
Creators: Jacqueline S Gofshteyn - 1 Division of Child Neurology, Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA
Angus Wilfong - 2 Division of Child Neurology, Texas Children's Hospital, Houston, TX, USA
Orrin Devinsky - 3 NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA
Judith Bluvstein - 3 NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA
Joshi Charuta - 4 Division of Child Neurology, University of Iowa School of Medicine, Iowa City, IA, USA
Michael A Ciliberto - 4 Division of Child Neurology, University of Iowa School of Medicine, Iowa City, IA, USA
Linda Laux - 5 Division of Child Neurology, Northwestern University Medical School, Robert H. Lurie Children's Hospital, Chicago, IL, USA
Eric D Marsh - 6 Departments of Neurology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
Resource Type: Journal article
Publication Details: Journal of child neurology, Vol.32(1), pp.35-40
Publisher: United States
DOI: 10.1177/0883073816669450
PMID: 27655472
ISSN: 1708-8283
eISSN: 1708-8283
Language: English
Date published: 01/2017
Academic Unit: Neurology; Stead Family Department of Pediatrics; Neurology (Pediatrics)
Record Identifier: 9983905521602771

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