Anakinra usage in febrile infection related epilepsy syndrome: an international cohort. Issue 12 (4th December 2020)

• Record Type: Journal Article
• Authors: Lai, Yi‐Chen; Muscal, Eyal; Wells, Elizabeth; Shukla, Nikita; Eschbach, Krista; Hyeong Lee, Ki; Kaliakatsos, Marios; Desai, Nevedita; Wickström, Ronny; Viri, Maurizio; Freri, Elena; Granata, Tiziana; Nangia, Srishti; Dilena, Robertino; Brunklaus, Andreas; Wainwright, Mark S.; Gorman, Mark P.; Stredny, Coral M.; Asiri, Abdurhman; Hundallah, Khalid; Doja, Asif; Payne, Eric; Wirrell, Elaine; Koh, Sookyong; Carpenter, Jessica L.; Riviello, James
• Is Part Of: Annals of clinical and translational neurology. Volume 7:Issue 12(2020)
• Page Start: 2467
• Page End: 2474
• Publication Date: 2020-12-04
• Subjects: Nervous system -- Diseases -- Periodicals; Neurology -- Periodicals; 616.8005
• DOI: 10.1002/acn3.51229
• Languages: English
• ISSNs: 2328-9503

Abstract

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.