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 Hundallah; Asif Doja; Eric Payne; Elaine Wirrell; Sookyong Koh; Jessica L. Carpenter; James Riviello

doi:10.1002/acn3.51229

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

Neurology

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5 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 language	English (US)
Pages (from-to)	2467-2474
Number of pages	8
Journal	Annals of Clinical and Translational Neurology
Volume	7
Issue number	12
DOIs	https://doi.org/10.1002/acn3.51229
State	Published - Dec 2020

ASJC Scopus subject areas

General Neuroscience
Clinical Neurology

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Lai, Y. C., Muscal, E., Wells, E., Shukla, N., Eschbach, K., Hyeong Lee, K., Kaliakatsos, M., Desai, N., Wickström, R., Viri, M., Freri, E., Granata, T., Nangia, S., Dilena, R., Brunklaus, A., Wainwright, M. S., Gorman, M. P., Stredny, C. M., Asiri, A., ... Riviello, J. (2020). Anakinra usage in febrile infection related epilepsy syndrome: an international cohort. Annals of Clinical and Translational Neurology, 7(12), 2467-2474. https://doi.org/10.1002/acn3.51229

Lai, YC, Muscal, E, Wells, E, Shukla, N, Eschbach, K, Hyeong Lee, K, Kaliakatsos, M, Desai, N, Wickström, R, Viri, M, Freri, E, Granata, T, Nangia, S, Dilena, R, Brunklaus, A, Wainwright, MS, Gorman, MP, Stredny, CM, Asiri, A, Hundallah, K, Doja, A, Payne, E, Wirrell, E, Koh, S, Carpenter, JL & Riviello, J 2020, 'Anakinra usage in febrile infection related epilepsy syndrome: an international cohort', Annals of Clinical and Translational Neurology, vol. 7, no. 12, pp. 2467-2474. https://doi.org/10.1002/acn3.51229

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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.",

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

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AU - Lai, Yi Chen

AU - Muscal, Eyal

AU - Wells, Elizabeth

AU - Shukla, Nikita

AU - Eschbach, Krista

AU - Hyeong Lee, Ki

AU - Kaliakatsos, Marios

AU - Desai, Nevedita

AU - Wickström, Ronny

AU - Viri, Maurizio

AU - Freri, Elena

AU - Granata, Tiziana

AU - Nangia, Srishti

AU - Dilena, Robertino

AU - Brunklaus, Andreas

AU - Wainwright, Mark S.

AU - Gorman, Mark P.

AU - Stredny, Coral M.

AU - Asiri, Abdurhman

AU - Hundallah, Khalid

AU - Doja, Asif

AU - Payne, Eric

AU - Wirrell, Elaine

AU - Koh, Sookyong

AU - Carpenter, Jessica L.

AU - Riviello, James

PY - 2020/12

Y1 - 2020/12

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AB - 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.

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

Abstract

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