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北京大学第一医院儿童医学中心,北京 102627
[ "吴 晔,北京大学第一医院儿童医学中心儿童神经内科主任,二级教授,主任医师,医学博士,博士生导师。在国内外期刊以第一作者或通信作者发表论文180余篇;先后承担国家自然科学基金项目4项、“十三五”重大新药创制专项子课题及“十四五”重点研发计划课题。获宋庆龄儿科医学奖(2023年,第一完成人)。任职中华医学会儿科学分会临床药理学组副组长、中国抗癫痫协会神经调控专业委员会副主任委员、中国抗癫痫协会药物委员会副主任委员、中国抗癫痫协会教育委员会副主任委员;《中国循证儿科杂志》执行副主编,《中华儿科杂志》编委,Epilepsia Open杂志副主编等。研究方向为遗传性及获得性脑白质病的发生机制、癫痫相关皮质发育畸形的分子机制、抗癫痫发作药物临床药理学以及癫痫的神经调控。Email:dryewu@263.net。" ]
收稿:2024-12-15,
修回:2025-01-04,
录用:2025-01-13,
纸质出版:2025-03-28
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吴晔,桑田,管巧.热性感染相关性癫痫综合征[J].重庆医科大学学报,2025,50(0):275-279.
Wu Ye,Sang Tian,Guan Qian.Febrile infection-related epilepsy syndrome[J].Journal of Chongqing Medical University,2025,50(0):275-279.
吴晔,桑田,管巧.热性感染相关性癫痫综合征[J].重庆医科大学学报,2025,50(0):275-279. DOI: 10.13406/j.cnki.cyxb.003752.
Wu Ye,Sang Tian,Guan Qian.Febrile infection-related epilepsy syndrome[J].Journal of Chongqing Medical University,2025,50(0):275-279. DOI: 10.13406/j.cnki.cyxb.003752.
热性感染相关性癫痫综合征(febrile infection-related epilepsy syndrome,FIRES)是一种发生在既往健康的儿童和青少年的炎症相关急性脑病,以热性感染后出现的超级难治性癫痫持续状态为临床特征,多数预后不良。近年来随着对该病认识的加深,治疗理念也有所转变,更提倡在规范抗癫痫发作治疗的基础上,早期及时免疫治疗和综合支持。本文从FIRES的定义、临床特征、辅助检查、发病机制、诊断及评估、治疗、预后和未来研究方向进行了全面阐述,以期提高认识,改善预后。
Febrile infection-related epilepsy syndrome is an inflammation-related acute encephalopathy that occurs in previously healthy children and adolescents. It is clinically characterized by super-refractory status epilepticus that occurs after a febrile infection and is often associated with poor prognosis. In recent years,with a deeper understanding of this disease,the treatment approaches have shifted to early and timely immunotherapy and comprehensive support on the basis of standardized anti-epileptic seizure treatment. This review comprehensively elaborates the definition,clinical characteristics,auxiliary examinations,pathogenesis,diagnosis and evaluation,treatment,prognosis,and future research directions of febrile infection-related epilepsy syndrome,aiming to enhance understanding and improve prognosis.
Hirsch LJ , Gaspard N , van Baalen A , et al . Proposed consensus definitions for new-onset refractory status epilepticus(NORSE),febrile infection-related epilepsy syndrome(FIRES),and related conditions [J ] . Epilepsia , 2018 , 59 ( 4 ): 739 - 744 .
Specchio N , Wirrell EC , Scheffer IE , et al . International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood:position paper by the ILAE task force on nosology and definitions [J ] . Epilepsia , 2022 , 63 ( 6 ): 1398 - 1442 .
Wickstrom R , Taraschenko O , Dilena R , et al . International consensus recommendations for management of new onset refractory status epilepticus(NORSE) including febrile infection-related epilepsy syndrome(FIRES):summary and clinical tools [J ] . Epilepsia , 2022 , 63 ( 11 ): 2827 - 2839 .
Wickstrom R , Taraschenko O , Dilena R , et al . International consensus recommendations for management of new onset refractory status epilepticus(NORSE) including febrile infection-related epilepsy syndrome(FIRES):summary and clinical tools [J ] . Epilepsia , 2022 , 63 ( 11 ): 2827 - 2839 .
Sakuma H , Thomas T , Debinski C , et al . International consensus definitions for infection-triggered encephalopathy syndromes [J ] . Dev Med Child Neurol , 2025 , 67 ( 2 ): 195 - 207 .
Specchio N , Pietrafusa N . New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome [J ] . Dev Med Child Neurol , 2020 , 62 ( 8 ): 897 - 905 .
Farias-Moeller R , Bartolini L , Staso K , et al . Early ictal and interictal patterns in FIRES:the Sparks before the blaze [J ] . Epilepsia , 2017 , 58 ( 8 ): 1340 - 1348 .
Culleton S , Talenti G , Kaliakatsos M , et al . The spectrum of neuroimaging findings in febrile infection-related epilepsy syndrome (FIRES):a literature review [J ] . Epilepsia , 2019 , 60 ( 4 ): 585 - 592 .
Meletti S , Giovannini G , D’Orsi G , et al . New-onset refractory status epilepticus with claustrum damage:definition of the clinical and neuroimaging features [J ] . Front Neurol , 2017 , 8 : 111 .
Hanin A , Cespedes J , Dorgham K , et al . Cytokines in new-onset refractory status epilepticus predict outcomes [J ] . Ann Neurol , 2023 , 94 ( 1 ): 75 - 90 .
Tan TH , Perucca P , O’Brien TJ , et al . Inflammation,ictogenesis,and epileptogenesis:an exploration through human disease [J ] . Epilepsia , 2021 , 62 ( 2 ): 303 - 324 .
Clarkson BDS , LaFrance-Corey RG , Kahoud RJ , et al . Functional deficiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome [J ] . Ann Neurol , 2019 , 85 ( 4 ): 526 - 537 .
Lin WS , Hsu TR . Hypothesis:Febrile infection-related epilepsy syndrome is a microglial NLRP3 inflammasome/IL-1 axis-driven autoinflammatory syndrome [J ] . Clin Transl Immunology , 2021 , 10 ( 6 ): e1299 .
Koh S , Wirrell E , Vezzani A , et al . Proposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES):a Report from FIRES workshop [J ] . Epilepsia Open , 2021 , 6 ( 1 ): 62 - 72 .
Lai YC , Muscal E , Wells E , et al . Anakinra usage in febrile infection related epilepsy syndrome:an international cohort [J ] . Ann Clin Transl Neurol , 2020 , 7 ( 12 ): 2467 - 2474 .
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