ÖzetStiripentol (STP), Dravet sendromunda valproat (VPA) ve klobazama ek olarak kullanılmak üzere onay almış yeni bir antiepileptik ilaç (AEİ)'dir. Diğer taraftan özellikle tedaviye dirençli epilepsilerde sık görülen ani beklenmedik ölüm ("sudden unexpected death in epilepsy" SUDEP) riski Dravet sendromunda da yüksektir. Kullanmakta olduğu çeşitli AEİ'lere karşın nöbetleri devam etmekte olan, birkaç kez status epileptikus (SE) veya yapılan intravenöz diazepam sonrası 'kardiyopulmoner arest'ten geçmiş olan olgumuzda da tedaviye STP eklenmiş, tedaviye başlandıktan altı ay sonra akut geçici ensefalopati tablosu oluşmuştur. Bu tabloya STP'nin VPA ile etkileşimi sonucu ortaya çıkan hiperamonyeminin neden olabileceği üzerinde durulmuştur. Dravet sendromu, SUDEP ve STP hakkında son literatür eşliğinde tedavi seçe-nekleri ve koruyucu önlemler tartışılmıştır.Anahtar sözcükler: Dravet sendromu; hiperamonyemi; stiripentol; SUDEP; valproat. SummaryStiripentol (STP), a recent antiepileptic drug, has been approved for use in addition to valproat (VPA) and clobazam in Dravet syndrome.On the other hand, the incidence of sudden unexpected death in epilepsy (SUDEP) commonly seen in patients with intractable epilepsy, is high in patients with Dravet yndrome. In our case, who was resistant to polytherapy and had multiple cardiopulmonary arrest due to status epilepticus (SE) or intravenous diazepam; STP was used in combination with other antiepileptic drugs (AED). However, acute reversible encephalopathy developed after six months of therapy. It is argued that the condition may have been caused by hyperamonnemia due to pharmacokinetic interaction between VPA and STP. Treatment options and protective measures are discussed using recent literature on Dravet syndrome, SUDEP and STP.
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