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

doi:10.1002/ana.24806

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

Research output: Contribution to journal › Article › peer-review

118 Scopus citations

Abstract

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 language	English (US)
Pages (from-to)	939-945
Number of pages	7
Journal	Annals of neurology
Volume	80
Issue number	6
DOIs	https://doi.org/10.1002/ana.24806
State	Published - Dec 1 2016

ASJC Scopus subject areas

Neurology
Clinical Neurology

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title = "Febrile infection-related epilepsy syndrome treated with anakinra",

abstract = "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.",

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

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AU - Kenney-Jung, Daniel L.

AU - Vezzani, Annamaria

AU - Kahoud, Robert J.

AU - LaFrance-Corey, Reghann G.

AU - Ho, Mai Lan

AU - Muskardin, Theresa Wampler

AU - Wirrell, Elaine C.

AU - Howe, Charles L.

AU - Payne, Eric T.

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AB - 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.

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Febrile infection-related epilepsy syndrome treated with anakinra

Abstract

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