Abstracts

Summary: This workshop will discuss clinical and molecular approaches for study of the mechanisms of epileptogenesis in focal epilepsies, using research on the leucine-rich, glioma inactivated 1 gene (LGI1) as a model. Mutations in LGI1 have been identified in up to half of families with autosomal dominant partial epilepsy with auditory features (ADPEAF), a genetic focal epilepsy syndrome with auditory symptoms and receptive aphasia as major ictal manifestations. These symptoms strongly suggest localization to the lateral temporal lobe; hence the syndrome is also called autosomal dominant lateral temporal lobe epilepsy (ADLTE). Unlike most other genes identified in Mendelian forms of epilepsy, LGI1 has no homology to any ion channel. The mechanism by which mutations influence susceptibility to epilepsy has been unclear, but recent studies have made important progress in elucidating this mechanism. In the workshop we will present the latest findings and discuss the experimental approaches used in these investigations.