Proposal to optimize evaluation and treatment of Febrile infection-related epilepsy syndrome (FIRES): A Report from FIRES workshop

被引：41

作者：

Koh, Sookyong ^{[1
]}

Wirrell, Elaine ^{[2
]}

Vezzani, Annamaria ^{[3
]}

Nabbout, Rima ^{[4
]}

Muscal, Eyal ^{[5
]}

Kaliakatsos, Marios ^{[6
]}

Wickstrom, Ronny ^{[7
]}

Riviello, James J. ^{[8
]}

Brunklaus, Andreas ^{[9
]}

Payne, Eric ^{[2
]}

Valentin, Antonio ^{[10
]}

Wells, Elizabeth ^{[11
]}

Carpenter, Jessica L. ^{[11
]}

Lee, Kihyeong ^{[12
]}

Lai, Yi-Chen ^{[13
]}

Eschbach, Krista ^{[14
]}

Press, Craig A. ^{[14
]}

Gorman, Mark ^{[15
]}

Stredny, Coral M. ^{[15
]}

Roche, William ^{[1
]}

Mangum, Tara ^{[16
]}

机构：

[1] Emory Univ, Sch Med, Dept Pediat, Atlanta, GA 30322 USA

[2] Mayo Clin, Child & Adolescent Neurol & Epilepsy, Rochester, MN USA

[3] Inst Ric Farmacol Mario Negri IRCCS, Dept Neurosci, Milan, Italy

[4] Paris Descartes Univ, Necker Enfants Malad Hosp, AP HP,Imagine Inst, Reference Ctr Rare Epilepsies,Dept Pediat Neurol, Paris, France

[5] Baylor Coll Med, Dept Pediat, Sect Pediat, Rheumatol, Houston, TX 77030 USA

[6] Great Ormond St Hosp Sick Children, Dept Neurol, London, England

[7] Karolinska Inst, Dept Womens & Childrens Hlth, Neuropediat Unit, Stockholm, Sweden

[8] Baylor Coll Med, Dept Pediat, Houston, TX 77030 USA

[9] Royal Hosp Children, Paediat Neurosci Res Grp, Glasgow, Lanark, Scotland

[10] Kings Coll Hosp NHS Trust, Dept Clin Neurophysiol, Dept Basic & Clin Neurosci Psychol & Neurosci, London, England

[11] Childrens Natl Hlth Syst, Ctr Neurosci & Behav Med, Washington, DC USA

[12] Advent Hlth Children, Comprehens Epilepsy Ctr, Orlando, FL USA

[13] Baylor Coll Med, Jan & Duncan Neurol Res Inst, Houston, TX 77030 USA

[14] Univ Colorado, Sch Med, Dept Pediat, Denver, CO USA

[15] Boston Childrens Hosp, Dept Neurol, Boston, MA USA

[16] Phoenix Childrens Hosp, Dept Pediat, Phoenix, AZ USA

来源：

EPILEPSIA OPEN | 2021年 / 6卷 / 01期

关键词：

anakinra; cytokines; epileptic encephalopathy; immune activation; neuroinflammation; new-onset refractory status epilepticus; INTERLEUKIN-1 RECEPTOR ANTAGONIST; STATUS EPILEPTICUS; RAT HIPPOCAMPUS; INFLAMMATORY CYTOKINES; WAVE DISCHARGES; KETOGENIC DIET; PHASE-II; EPILEPTOGENESIS; SEIZURES; IL-1-BETA;

D O I：

10.1002/epi4.12447

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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-1 beta) are recommended.

引用

页码：62 / 72

页数：11

共 68 条

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