ABSTRACT

Epilepsy is one of the most common neurological disorders affecting ~3% of the world’s population during their lifetime (Hauser et al. 1996), of which at least a third is primarily genetically determined. About 2% of the hereditary epilepsies are monogenic, of which most of the identi–ed defects are related to pathologic channel function. Using a total prevalence of 0.9% for all forms of epilepsies, the prevalence of channel-related epilepsies is estimated to be fewer than 15:100,000 of the population, thus ful–lling the criterion for a chronic rare disease. In addition, ion channel genes may be involved as susceptibility genes in the pathogenesis of polygenic epilepsies.