title: Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases.
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.
authors.affiliation: 1 Division of Child Neurology, Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
authors.forename: Jacqueline S
authors.initials: JS
authors.lastname: Gofshteyn
authors.affiliation: 2 Division of Child Neurology, Texas Children's Hospital, Houston, TX, USA.
authors.forename: Angus
authors.initials: A
authors.lastname: Wilfong
authors.affiliation: 3 NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA.
authors.forename: Orrin
authors.initials: O
authors.lastname: Devinsky
authors.affiliation: 3 NYU Comprehensive Epilepsy Center, Department of Neurology, NYU School of Medicine, New York, NY, USA.
authors.forename: Judith
authors.initials: J
authors.lastname: Bluvstein
authors.affiliation: 4 Division of Child Neurology, University of Iowa School of Medicine, Iowa City, IA, USA.
authors.forename: Joshi
authors.initials: J
authors.lastname: Charuta
authors.affiliation: 4 Division of Child Neurology, University of Iowa School of Medicine, Iowa City, IA, USA.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Ciliberto
authors.affiliation: 5 Division of Child Neurology, Northwestern University Medical School, Robert H. Lurie Children's Hospital, Chicago, IL, USA.
authors.forename: Linda
authors.initials: L
authors.lastname: Laux
authors.affiliation: 1 Division of Child Neurology, Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA.|6 Departments of Neurology and Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.
authors.forename: Eric D
authors.initials: ED
authors.lastname: Marsh
mesh_terms: D000208:Acute Disease; D000927:Anticonvulsants / Q000627:therapeutic use*; D002185:Cannabidiol / Q000627:therapeutic use*; D002648:Child; D002908:Chronic Disease; D000069279:Drug Resistant Epilepsy / Q000188:drug therapy* / Q000209:etiology; D000073376:Epileptic Syndromes / Q000188:drug therapy* / Q000209:etiology; D005334:Fever / Q000150:complications*; D006801:Humans; D007239:Infections / Q000150:complications; D008297:Male; D013226:Status Epilepticus / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants; D002185:Cannabidiol
keywords: cannabidiol; febrile infection-related epilepsy syndrome (FIRES); pediatric epilepsy; refractory status epilepticus