Rasmussen's syndrome treated with anakinra
JOURNAL OF NEUROLOGY, sa.2, ss.723-726, 2024 (SCI-Expanded, Scopus)
- Yayın Türü: Makale / Editöre Mektup
- Basım Tarihi: 2024
- Doi Numarası: 10.1007/s00415-023-12072-8
- Dergi Adı: JOURNAL OF NEUROLOGY
- Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, BIOSIS, CAB Abstracts, Veterinary Science Database
- Sayfa Sayıları: ss.723-726
- İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet
Özet
Background and objectiveRasmussen'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-1 beta-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 resultsWe 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.DiscussionAnakinra may be considered as a treatment option in patients with RE and refractory seizures.