Anakinra usage in febrile infection related epilepsy syndrome: an international cohort

Yi Chen Lai, Eyal Muscal, Elizabeth Wells, Nikita Shukla, Krista Eschbach, Ki Hyeong Lee, Marios Kaliakatsos, Nevedita Desai, Ronny Wickström, Maurizio Viri, Elena Freri, Tiziana Granata, Srishti Nangia, Robertino Dilena, Andreas Brunklaus, Mark S. Wainwright, Mark P. Gorman, Coral M. Stredny, Abdurhman Asiri, Khalid HundallahAsif Doja, Eric Payne, Elaine Wirrell, Sookyong Koh, Jessica L. Carpenter, James Riviello

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

Febrile-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.

Original languageEnglish (US)
Pages (from-to)2467-2474
Number of pages8
JournalAnnals of Clinical and Translational Neurology
Volume7
Issue number12
DOIs
StatePublished - Dec 2020

ASJC Scopus subject areas

  • Neuroscience(all)
  • Clinical Neurology

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