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Febrile infection‐related epilepsy syndrome treated with anakinra
Author(s) -
KenneyJung Daniel L.,
Vezzani Annamaria,
Kahoud Robert J.,
LaFranceCorey Reghann G.,
Ho MaiLan,
Muskardin Theresa Wampler,
Wirrell Elaine C.,
Howe Charles L.,
Payne Eric T.
Publication year - 2016
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.24806
Subject(s) - anakinra , medicine , status epilepticus , etiology , neuroinflammation , epilepsy , proinflammatory cytokine , dosing , disease , intensive care medicine , immunology , inflammation , psychiatry
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