Benign rolandic epilepsy Some cases have been mapped to 15q. The mutation is known, but the pathogenic relationship to epilepsy is unexplained. Neuronal migration abnormalities Miller-Dieker syndrome Autosomal dominant. Mutation in LS1 gene on chromosome 17. Males have severe lissencephaly.
Females have milder disease.
Arrested migration of neurons between the periventricular subependymal plate and the cortical gray matter. Mutation in doublecortin, an intracellular signaling molecule, having phosphorylation motifs for signaling kinases. Family history shows reduced males in the pedigree. Females have normal neurologic development but develop partial seizures with secondary generalization. MRI shows heterotopias in the walls of the ventricles bilaterally.