Childhood epilepsy can result from malformations of the cerebral cortex known as cortical dysplasia. The treatment of these seizures is limited by a lack of animal models with key features of the disease, such as characteristic alterations in cell morphology and structural abnormalities in the cortex. M. Cecilia Ljungberg, C. Nicole Sunnen and colleagues report that a conditional Pten knockout mouse has characteristics that support its utility as a model for cortical dysplasia, such as enlarged cortical neurons and spontaneous seizures. As Pten is part of the PI3K-mTOR pathway, they have also identified an important molecular signaling pathway in cortical dysplasia. These data suggest that pharmacological inhibitors of this pathway, specifically the mTOR inhibitor rapamycin, are a potential therapy for controlling seizures in cortical dysplasia patients.
