Dementia associated with cortical dysplasia

Detailed neuropathologic examination was performed on a 47.5-year-old man with an unusual adult-onset dementing illness. His initial symptoms were those of depression, memory loss, and personality change. He developed progressive cognitive decline with prominent psychiatric symptoms. Seizures began approximately 11 months prior to death and he died 5.5 years after onset of symptoms. Pathologic examination of the brain at autopsy revealed organizing necrosis of the hippocampi, felt to be the result of his seizures. More significant was the finding of widespread microscopic nodular cortical dysplasia. The dysplastic nodules were composed of clusters of abnormal cells with enlarged, pleomorphic, vesicular nuclei, many of which contained nucleoli and had ballooned cytoplasm. There were no mitoses. Cortical dysplasia is most commonly associated with childhood-onset seizures. It has not, to our knowledge, been reported as a cause of dementia. Whether or not the dysplasia was the basis of the patient's dementia is difficult to say with certainty, but we discuss possible pathoetiologic mechanisms of dementia due to cortical dysplasia.