Abstract
An autopsy case of Huntington's chorea is reported and its psychotic features is discussed. A 38-year-old man showed chorea of extremities and gait disturbance. Six years later, personality changes developed and he became unrestrained, irritable, and aggressive. Furthermore, in addition to calculation disturbance and disabled understanding, symptoms of so called “subcortical dementia” such as forgetfullness, slowing of thought processes, and depressed drives were observed. He died of pulmonary edema at the age of 48. Neuropathologically, severe atrophy of the striatum with a marked loss of small neurons was observed. The neuronal loss of the cerebral cortex, especially in the frontal and occipital lobes, as well as atrophy and myelin pallor of the cerebral white matter were found. From the clinicopathotogical standpoint, the personality changes in this case could be attributed to the degeneration of the striatum and the frontal lobes. Furthermore, the dementia characteristic of so called “subcortical dementia” in this case might be contributed not only to the striatal lesion but also to the degeneration of the cerebral cortex and the cerebral white matter.
