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

被引:125
作者
Gofshteyn, Jacqueline S. [1 ]
Wilfong, Angus [2 ]
Devinsky, Orrin [3 ]
Bluvstein, Judith [3 ]
Charuta, Joshi [4 ]
Ciliberto, Michael A. [4 ]
Laux, Linda [5 ]
Marsh, Eric D. [1 ,6 ,7 ]
机构
[1] Childrens Hosp Philadelphia, Div Child Neurol, Pediat Reg Epilepsy Program, Philadelphia, PA 19104 USA
[2] Texas Childrens Hosp, Div Child Neurol, Houston, TX 77030 USA
[3] NYU Sch Med, Dept Neurol, NYU Comprehens Epilepsy Ctr, New York, NY USA
[4] Univ Iowa, Sch Med, Div Child Neurol, Iowa City, IA 52242 USA
[5] Northwestern Univ, Sch Med, Div Child Neurol, Robert H Lurie Childrens Hosp, Chicago, IL USA
[6] Univ Penn, Dept Neurol, Perelman Sch Med, Philadelphia, PA 19104 USA
[7] Univ Penn, Dept Pediat, Perelman Sch Med, Philadelphia, PA 19104 USA
关键词
pediatric epilepsy; cannabidiol; refractory status epilepticus; febrile infection-related epilepsy syndrome (FIRES); CHILDHOOD EPILEPSY; STATUS EPILEPTICUS; ENCEPHALOPATHY; EFFICACY; CHILDREN; ENCEPHALITIS; PATHOGENESIS; ANESTHESIA;
D O I
10.1177/0883073816669450
中图分类号
R74 [神经病学与精神病学];
学科分类号
100204 [神经病学];
摘要
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.
引用
收藏
页码:35 / 40
页数:6
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