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) |
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Pages (from-to) | 939-945 |
Number of pages | 7 |
Journal | Annals of Neurology |
Volume | 80 |
Issue number | 6 |
DOIs | |
State | Published - Dec 1 2016 |
Externally published | Yes |
Bibliographical note
Publisher Copyright:© 2016 American Neurological Association