Citations 2
Anakinra usage in febrile infection related epilepsy syndrome: an international cohort
Lai, Yi‐Chen , Muscal, Eyal , Wells, Elizabeth, Shukla, Nikita , Eschbach, Krista , Kaliakatsos, Marios , Desai, Nevedita, Wickström, Ronny , Viri, Maurizio , Freri, Elena , Granata, Tiziana , Nangia, Srishti, Brunklaus, Andreas , Wainwright, Mark S. , Gorman, Mark , Stredny, Coral M. , Asiri, Abdurhman, Hundallah, Khalid , Payne, Eric T. , Koh, Sookyong , Carpenter, Jessica L. , Riviello, James J. , Wainwright, Mark S , Brunklaus, Andreas , Lai, Yi-Chen , Eschbach, Krista , Shukla, Nikita Malani , Lee, Ki Hyeong , Dilena, Robertino , Doja, Asif , Wirrell, Elaine
Annals of Clinical and Translational Neurology | December 4, 2020 | Volume: 7 | Issue: 12 | Pages: 2467-2474
AbstractFebrile‐infection related epilepsy syndrome (FIRES) is a devastating neurological condition characterized by a febrile illness preceding new onset refractory status epilepticus (NORSE). Increasing evidence suggests innate immune dysfunction as a potential pathological mechanism. We report an international retrospective cohort of 25 children treated with anakinra, a recombinant interleukin‐1 receptor antagonist, as an immunomodulator for FIRES. Anakinra was potentially safe with only one child discontinuing therapy due to infection. Earlier anakinra initiation was associated with shorter duration of mechanical ventilation, ICU and hospital length of stay. Our retrospective data lay the groundwork for prospective consensus‐driven cohort studies of anakinra in FIRES.
Proposal to optimize evaluation and treatment of Febrile infection‐related epilepsy syndrome (FIRES): A Report from FIRES workshop
Koh, Sookyong , Wirrell, Elaine , Nabbout, Rima , Muscal, Eyal , Kaliakatsos, Marios , Wickström, Ronny , Riviello, James J. , Brunklaus, Andreas , Payne, Eric T. , Valentı́n, Antonio , Wells, Elizabeth, Carpenter, Jessica L. , Lee, Kihyeong, Eschbach, Krista , Press, Craig A. , Gorman, Mark , Stredny, Coral M. , Roche, William , Mangum, Tara, Brunklaus, Andreas , Payne, Eric, Lai, Yi-Chen , Eschbach, Krista , Wirrell, Elaine C , Vezzani, Annamaria , Lai, Yi‐Chen , Roche, William
Epilepsia Open | November 20, 2020 | Volume: 6 | Issue: 1 | Pages: 62-72
AbstractFebrile 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.