Generalized cortical dysplasia manifested by diffusely thick cerebral cortex

Unilateral or bilateral rolandic macrogyria has been described as a cause of epilepsy and, in some cases, retardation. Tissue from the periphery of these lesions shows the changes of focal cortical dysplasia. Evidence reported herein suggests that cortical dysplasia may also be generalized. Two patients with intractable epilepsy and mental retardation had diffusely abnormal, thick cortex, shallow gyri, and poor demarcation of gray and white matter. One patient had an anterior callosotomy that led to considerable improvement of the epilepsy. Cortical layers 5 and 6 could not be differentiated on biopsy material. The white matter was poorly myelinated and contained clusters of heterotopic neurons. This syndrome, a congenital disorder of neuronal migration, with prolonged survival, represents a mild form of lissencephaly. It can be diagnosed during life by computed tomography or magnetic resonance scanning.