Febrile Infection-Related Epilepsy Syndrome Treated with Anakinra

被引:213
作者
Kenney-Jung, Daniel L. [1 ,2 ]
Vezzani, Annamaria [3 ]
Kahoud, Robert J. [1 ,2 ]
LaFrance-Corey, Reghann G. [1 ]
Ho, Mai-Lan [4 ]
Muskardin, Theresa Wampler [2 ]
Wirrell, Elaine C. [1 ,2 ]
Howe, Charles L. [1 ,5 ,6 ]
Payne, Eric T. [1 ,2 ]
机构
[1] Mayo Clin, Dept Neurol, 200 First St SW, Rochester, MN 55905 USA
[2] Mayo Clin, Dept Pediat, 200 First St SW, Rochester, MN 55905 USA
[3] Mario Negri Inst Pharmacol Res, Inst Hospitalizat & Sci Care, Milan, Italy
[4] Mayo Clin, Dept Radiol, 200 First St SW, Rochester, MN 55905 USA
[5] Mayo Clin, Dept Neurosci, 200 First St SW, Rochester, MN 55905 USA
[6] Mayo Clin, Dept Immunol, 200 First St SW, Rochester, MN 55905 USA
关键词
JUVENILE IDIOPATHIC ARTHRITIS; REFRACTORY STATUS EPILEPTICUS; AGE-CHILDREN FIRES; KETOGENIC DIET; SEIZURES; ENCEPHALOPATHY; IL-1; EPILEPTOGENESIS; INTERLEUKIN-1; PATHOGENESIS;
D O I
10.1002/ana.24806
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
收藏
页码:939 / 945
页数:7
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