Rasmussen's syndrome treated with anakinra

J Neurol. 2024 Feb;271(2):723-726. doi: 10.1007/s00415-023-12072-8. Epub 2023 Nov 3.

Abstract

Background and objective: Rasmussen's encephalitis (RE) is a rare chronic neurological disorder, characterized by unilateral inflammation of the cerebral cortex, refractory focal epilepsy or epilepsia partialis continua, hemiparesis, and progressive cognitive decline. Interleukin-1 (IL-1) plays an important role in neuroinflammation as a key element in the activation of the inflammatory IL-1β-IL-1 receptor type 1 (IL-1R1) axis. Anakinra, an IL-1 inhibitor, is successfully used in patients with new onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

Methods and results: We present 38-year-old male with RE having right-sided hemiparesis and continuous spasms being unresponsive to immune modulatory therapies like pulse steroid, intravenous immunoglobulin and anti-seizure drugs. After treatment with anakinra for three weeks, the continuous spasms almost completely subsided, and his muscle strength returned to normal.

Discussion: Anakinra may be considered as a treatment option in patients with RE and refractory seizures.

Keywords: Anakinra; Epilepsia Partialis Continua; Immunotherapy; Interleukin-1; Rasmussen’s encephalitis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Drug Resistant Epilepsy*
  • Encephalitis*
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use
  • Interleukin-1
  • Magnetic Resonance Imaging
  • Male
  • Paresis
  • Spasm

Substances

  • Interleukin 1 Receptor Antagonist Protein
  • Interleukin-1