Focal pachygyria with unusual vascular anomaly.

A case of focal pachygyria with an unusual vascular anomaly is reported. Preoperative magnetic resonance imaging demonstrated few and broad gyri, and an abnormally thickened cortex of the right frontal lobe. In addition, T2-weighted imaging showed a high intensity lesion beneath the thickened cortex. In the pachygyric cortex, the peripheral portions of the arteries were tortuous and irregularly dilated, and prominent deep medullary veins were found draining into the subependymal veins. Histological examination revealed a decreased number of neurons with no tendency towards lamination, and degenerative changes with gliosis in the white matter. These findings suggest that the etiology of this anomaly may be gradual perfusion failure restricted to the territory of the anomalous vessels through the period of neuronal migration to the post-migratory, perinatal stage.