Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases
Cannabidiol as a Potential Treatment for Febrile Infection-Related Epilepsy Syndrome (FIRES) in the Acute and Chronic Phases
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DOI：
10.1177/0883073816669450
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发表时间：
2017-01-01
影响因子：
1.9
通讯作者：
Marsh, Eric D.
中科院分区：
文献类型：
--
作者：
Gofshteyn, Jacqueline S.;Wilfong, Angus;Marsh, Eric D.
发热性感染相关癫痫综合征（FIRES）是一种影响正常儿童的发热性疾病后的毁灭性癫痫。FIRES表现为超难治性癫痫持续状态的急性期，所有患者均进展为持续难治性癫痫的慢性期。典型的结果是严重的脑病或死亡。作者介绍了来自5个中心的7名患有FIRES的儿童，他们对抗癫痫药物或其他治疗没有反应，他们在疾病的急性或慢性阶段接受了紧急或扩大研究方案的大麻二酚（Epidiolex，GW Pharma）。开始使用大麻二酚后，7名患者中有6名的癫痫发作频率和持续时间有所改善。1例患者因继发于异氟烷的多器官衰竭死亡。平均停用4种抗癫痫药物。目前，5例受试者可以走动，1例在辅助下行走，4例可以言语。虽然这是一个开放标签的病例系列，但作者增加了大麻二酚作为FIRES的可能治疗方法。
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.