Febrile infection‐related epilepsy syndrome treated with anakinra

作者：Daniel Kenney‐Jung, Annamaria Vezzani, Robert J. Kahoud, Reghann G. LaFrance‐Corey, Mai‐Lan Ho, Theresa Wampler Muskardin, Elaine Wirrell, Charles L. Howe, Eric T. Payne · 发表于：Annals of Neurology · 年份：2016 · DOI：10.1002/ana.24806 · 被引用次数：263 · 研究领域：Infectious Encephalopathies and Encephalitis、Infectious Diseases and Tuberculosis、Bacterial Infections and Vaccines

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.