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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. 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. Anakinra may be considered as a treatment option in patients with RE and refractory seizures. © 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.

Citation

Abdullah Arcan, Esra Koçhan Kızılkılıç, Ayşegül Gündüz, Rümeysa Unkun, Annamaria Vezzani, Çiğdem Özkara. Rasmussen's syndrome treated with anakinra. Journal of neurology. 2024 Feb;271(2):723-726

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PMID: 37922068

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