Medical management of Lennox-Gastaut syndrome

Abstract

Lennox-Gastaut syndrome occurs in 3% of children with epilepsy and is characterized by multiple seizure types, slow spike-and-wave discharges and a poor prognosis for seizure control and cognitive development. Although randomized controlled trials of adjunctive felbamate, lamotrigine, topiramate and rufinamide have demonstrated a > or =50% reduction in seizure frequency, very few children achieve complete seizure control and a Cochrane review of the treatment of Lennox-Gastaut syndrome concluded that the optimum treatment was uncertain and that no drug has been shown to be highly efficacious. Valproate, lamotrigine and topiramate were considered recently by expert panels in the US and Europe to be the first-line drugs. The ketogenic diet may be more effective than antiepileptic drugs and should be considered early in treatment. An improvement in the management of Lennox-Gastaut syndrome requires a better understanding of the pathophysiology of this disorder and the development of animal models in which to test new compounds.