Temporal Lobe Epilepsy: Genetic Determinants of Temporal Lobe Epilepsy

Temporal lobe epilepsy (TLE) has long been the focus of both clinical and basic laboratory investigations. Most studies have approached this common and often medically refractory form of epilepsy as a unitary disorder. TLE has been identified as perhaps the prototypical 'acquired' epilepsy, thought to arise as a result of a brain injury or insult. However, recent studies suggest that TLE may include a number of distinct disorders, some of which arise because of genetic mutations in genes that normally control patterns of brain development and/or excitability. Animal models can help to explore these possibilities. Two specific mouse models - the Kv1.1 knockout mouse and the p35 knockout mouse - show typical TLE-like seizure phenotypes and present with histopathological features also seen in human TLE. They arise, however, from very different processes; seizures in Kv1.1^-/- mice reflect a 'channelopathy'-like disorder, while seizures in p35^-/- mice arise from a disruption of normal brain development.