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 Hundallah, Asif Doja, Eric Payne, Elaine Wirrell, Sookyong Koh, Jessica L Carpenter, James Riviello
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...
December 2020: Annals of Clinical and Translational Neurology