TY - JOUR
T1 - Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases
AU - Gofshteyn, Jacqueline S.
AU - Wilfong, Angus
AU - Devinsky, Orrin
AU - Bluvstein, Judith
AU - Charuta, Joshi
AU - Ciliberto, Michael A.
AU - Laux, Linda
AU - Marsh, Eric D.
N1 - Publisher Copyright:
© SAGE Publications.
PY - 2017/1/1
Y1 - 2017/1/1
N2 - 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.
AB - 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.
KW - cannabidiol
KW - febrile infection-related epilepsy syndrome (FIRES)
KW - pediatric epilepsy
KW - refractory status epilepticus
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U2 - 10.1177/0883073816669450
DO - 10.1177/0883073816669450
M3 - Article
C2 - 27655472
AN - SCOPUS:85006387041
SN - 0883-0738
VL - 32
SP - 35
EP - 40
JO - Journal of child neurology
JF - Journal of child neurology
IS - 1
ER -