Febrile infection-related epilepsy syndrome treated with anakinra

Daniel L. Kenney-Jung, Annamaria Vezzani, Robert J. Kahoud, Reghann G. LaFrance-Corey, Mai Lan Ho, Theresa Wampler Muskardin, Elaine C. Wirrell, Charles L. Howe, Eric T. Payne

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81 Scopus citations


Febrile infection-related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and an unclear etiology. Anakinra is a recombinant version of the human interleukin-1 receptor antagonist used to treat autoinflammatory disorders. This is the first report of anakinra for treatment of a child with super-refractory status epilepticus secondary to FIRES. Anakinra was well tolerated and effective. Cerebral spinal fluid analysis revealed elevated levels of proinflammatory cytokines before treatment that normalized on anakinra, suggesting a potential pathogenic role for neuroinflammation in FIRES. Further studies are required to assess anakinra efficacy and dosing, and to further delineate disease etiology. Ann Neurol 2016;80:939–945.

Original languageEnglish (US)
Pages (from-to)939-945
Number of pages7
JournalAnnals of neurology
Issue number6
StatePublished - Dec 1 2016

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Kenney-Jung, D. L., Vezzani, A., Kahoud, R. J., LaFrance-Corey, R. G., Ho, M. L., Muskardin, T. W., Wirrell, E. C., Howe, C. L., & Payne, E. T. (2016). Febrile infection-related epilepsy syndrome treated with anakinra. Annals of neurology, 80(6), 939-945. https://doi.org/10.1002/ana.24806