Febrile infection-related epilepsy syndrome treated with anakinra.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 27770579.
- Also identified by DOI 10.1002/ana.24806 and PMC identifier 5225882.
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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.
Medical subject headings
- Infectious Encephalitis
- Interleukin 1 Receptor Antagonist Protein
- Seizures, Febrile
- Status Epilepticus