Febrile infection-related epilepsy syndrome treated with anakinra

Ann Neurol. 2016 Dec;80(6):939-945. doi: 10.1002/ana.24806. Epub 2016 Nov 14.

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.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural

MeSH terms

  • Child, Preschool
  • Female
  • Humans
  • Infectious Encephalitis / cerebrospinal fluid
  • Infectious Encephalitis / complications*
  • Infectious Encephalitis / drug therapy
  • Inflammation Mediators / cerebrospinal fluid
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use*
  • Recombinant Proteins / therapeutic use
  • Seizures, Febrile / cerebrospinal fluid
  • Seizures, Febrile / complications*
  • Seizures, Febrile / drug therapy
  • Status Epilepticus / cerebrospinal fluid
  • Status Epilepticus / complications*
  • Status Epilepticus / drug therapy*
  • Syndrome

Substances

  • Inflammation Mediators
  • Interleukin 1 Receptor Antagonist Protein
  • Recombinant Proteins