Department of Pediatrics, Emory University School of Medicine, Atlanta, GA, USA.
Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, MN, USA.
Epilepsia Open. 2021 Jan 13;6(1):62-72. doi: 10.1002/epi4.12447. eCollection 2021 Mar.
Febrile infection-related epilepsy syndrome (FIRES) is a rare catastrophic epileptic encephalopathy that presents suddenly in otherwise normal children and young adults causing significant neurological disability, chronic epilepsy, and high rates of mortality. To suggest a therapy protocol to improve outcome of FIRES, workshops were held in conjunction with American Epilepsy Society annual meeting between 2017 and 2019. An international group of pediatric epileptologists, pediatric neurointensivists, rheumatologists and basic scientists with interest and expertise in FIRES convened to propose an algorithm for a standardized approach to the diagnosis and treatment of FIRES. The broad differential for refractory status epilepticus (RSE) should include FIRES, to allow empiric therapies to be started early in the clinical course. FIRES should be considered in all previously healthy patients older than two years of age who present with explosive onset of seizures rapidly progressing to RSE, following a febrile illness in the preceding two weeks. Once FIRES is suspected, early administrations of ketogenic diet and anakinra (the IL-1 receptor antagonist that blocks biologic activity of IL-1β) are recommended.
发热相关感染性癫痫综合征（FIRES）是一种罕见的灾难性癫痫性脑病，突然发生于原本正常的儿童和年轻成人，导致严重的神经功能障碍、慢性癫痫和高死亡率。为了提出改善 FIRES 预后的治疗方案，2017 年至 2019 年期间，在美国癫痫学会年会上举办了相关研讨会。一组国际儿科癫痫专家、儿科神经重症专家、风湿病专家和对 FIRES 有兴趣和专业知识的基础科学家聚集在一起，提出了 FIRES 诊断和治疗的标准化方法的算法。难治性癫痫持续状态（RSE）的广泛鉴别诊断应包括 FIRES，以便在临床病程早期开始经验性治疗。对于两周前有发热性疾病，随后出现癫痫发作迅速进展为 RSE 的所有年龄大于 2 岁的既往健康患者，都应考虑 FIRES。一旦怀疑 FIRES，建议早期给予生酮饮食和阿那白滞素（IL-1 受体拮抗剂，可阻断 IL-1β 的生物学活性）。
