title: Febrile infection-related epilepsy syndrome treated with anakinra.
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.
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.|Departments of Pediatrics, Mayo Clinic, Rochester, MN.
authors: Daniel L
authors: DL
authors: Kenney-Jung
authors: Institute of Hospitalization and Scientific Care-Mario Negri Institute of Pharmacological Research, Milan, Italy.
authors: Annamaria
authors: A
authors: Vezzani
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.|Departments of Pediatrics, Mayo Clinic, Rochester, MN.
authors: Robert J
authors: RJ
authors: Kahoud
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.
authors: Reghann G
authors: RG
authors: LaFrance-Corey
authors: Departments of Radiology, Mayo Clinic, Rochester, MN.
authors: Mai-Lan
authors: ML
authors: Ho
authors: Departments of Pediatrics, Mayo Clinic, Rochester, MN.
authors: Theresa Wampler
authors: TW
authors: Muskardin
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.|Departments of Pediatrics, Mayo Clinic, Rochester, MN.
authors: Elaine C
authors: EC
authors: Wirrell
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.|Departments of Neuroscience, Mayo Clinic, Rochester, MN.|Departments of Immunology, Mayo Clinic, Rochester, MN.
authors: Charles L
authors: CL
authors: Howe
authors: Departments of Neurology, Mayo Clinic, Rochester, MN.|Departments of Pediatrics, Mayo Clinic, Rochester, MN.
authors: Eric T
authors: ET
authors: Payne
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D000069544:Infectious Encephalitis / Q000134:cerebrospinal fluid / Q000150:complications* / Q000188:drug therapy; D018836:Inflammation Mediators / Q000134:cerebrospinal fluid; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D011994:Recombinant Proteins / Q000627:therapeutic use; D003294:Seizures, Febrile / Q000134:cerebrospinal fluid / Q000150:complications* / Q000188:drug therapy; D013226:Status Epilepticus / Q000134:cerebrospinal fluid / Q000150:complications* / Q000188:drug therapy*; D013577:Syndrome
chemical_list: D018836:Inflammation Mediators; D053590:Interleukin 1 Receptor Antagonist Protein; D011994:Recombinant Proteins