Erschienen in:
03.11.2023 | Short Commentary
Rasmussen’s syndrome treated with anakinra
verfasst von:
Abdullah Arcan, Esra Koçhan Kızılkılıç, Ayşegül Gündüz, Rümeysa Unkun, Annamaria Vezzani, Çiğdem Özkara
Erschienen in:
Journal of Neurology
|
Ausgabe 2/2024
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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.