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...

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Bibliographic Details
Published in:Acta neuropathologica 1998-02, Vol.95 (2), p.193-198
Main Authors: BIGIO, E. H, FONTAINE, C. S, DABABO, M. A, PRAHLOW, J. A, CHASON, D. P, WEINER, M. F, WHITE, C. L
Format: Article
Language:English
Subjects:
Autopsy
Biological and medical sciences
Brain - pathology
Cerebral Cortex - pathology
Children
Cognition Disorders - pathology
Cognitive ability
Cytoplasm
Dementia
Dementia - complications
Dementia - pathology
Dementia disorders
Disorders of higher nervous function. Focal brain diseases. Central vestibular syndrome and deafness. Brain stem syndromes
Dysplasia
Hippocampus
Hippocampus - pathology
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Middle Aged
Mitosis
Necrosis
Nervous system (semeiology, syndromes)
Neurology
Neurons - pathology
Nodules
Nucleoli
Seizures
Seizures - complications
Seizures - pathology
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Summary: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.
ISSN:0001-6322
1432-0533
DOI:10.1007/s004010050786