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J Child Neurol. 2017 Jan;32(1):35-40. doi: 10.1177/0883073816669450. Epub 2016 Sep 29.

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

Author information

1
1 Division of Child Neurology, Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
2
2 Division of Child Neurology, Texas Children's Hospital, Houston, TX, USA.
3
3 NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA.
4
4 Division of Child Neurology, University of Iowa School of Medicine, Iowa City, IA, USA.
5
5 Division of Child Neurology, Northwestern University Medical School, Robert H. Lurie Children's Hospital, Chicago, IL, USA.
6
6 Departments of Neurology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

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.

KEYWORDS:

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

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PMID:
27655472
DOI:
10.1177/0883073816669450
[Indexed for MEDLINE]

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