Case of Dravet Syndrome Who Developed Acute Reversible Encephalopathy That May Have Been Caused by Hyperamonnemia Due to Pharmacokinetic Interaction Between Valproate and Stiripentol

Atmaca M. M., Gurses C., Marufoglu F., BEBEK N., BAYKAL B., Gokyigit A.

EPILEPSI, vol.18, no.3, pp.25-29, 2012 (ESCI) identifier

  • Publication Type: Article / Article
  • Volume: 18 Issue: 3
  • Publication Date: 2012
  • Doi Number: 10.5505/epilepsi.2012.48658
  • Journal Name: EPILEPSI
  • Journal Indexes: Emerging Sources Citation Index (ESCI), TR DİZİN (ULAKBİM)
  • Page Numbers: pp.25-29
  • Istanbul University Affiliated: Yes


Stiripentol (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.