Gofshteyn Jacqueline S, Wilfong Angus, Devinsky Orrin, Bluvstein Judith, Charuta Joshi, Ciliberto Michael A, Laux Linda, Marsh Eric D
1 Division of Child Neurology, Pediatric Regional Epilepsy Program, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
2 Division of Child Neurology, Texas Children's Hospital, Houston, TX, USA.
J Child Neurol. 2017 Jan;32(1):35-40. doi: 10.1177/0883073816669450. Epub 2016 Sep 29.
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.
发热感染相关癫痫综合征（FIRES）是一种在发热性疾病后影响正常儿童的严重癫痫。FIRES表现为急性期的超难治性癫痫持续状态，所有患者都会进展到慢性期的持续性难治性癫痫。典型的结局是严重脑病或死亡。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均无反应，在疾病的急性期或慢性期，根据紧急或扩大的研究方案接受了大麻二醇（Epidiolex，GW制药公司）治疗。开始使用大麻二醇后，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名患者因异氟烷导致的多器官衰竭死亡。平均停用了4种抗癫痫药物。目前，5名受试者可独立行走，1名需辅助行走，4名能进行言语交流。虽然这是一个开放标签的病例系列，但作者将大麻二醇作为FIRES的一种可能治疗方法。
