Wilson's disease with extensive degeneration of cerebral white matter and cortex

This is a report of an autopsy case of Wilson's disease with widespread degeneration of the cerebral cortex and white matter, the basal ganglia and thalamus and, to a lesser degree, the cerebellum and brain stem. The patient was a 28-year-old man at the time of death with the clinical course of...

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Bibliographic Details
Published inJapanese journal of psychiatry and neurology Vol. 41; no. 4; p. 709
Main Authors Shimoji, A, Miyakawa, T, Watanabe, K, Yamashita, K, Katsuragi, S, Kabashima, K
Format Journal Article
LanguageEnglish
Published Japan 01.12.1987
Subjects
Adult
Brain - pathology
Cerebral Cortex - pathology
Copper - metabolism
Epilepsy - pathology
Hepatolenticular Degeneration - pathology
Humans
Liver - pathology
Male
Nerve Degeneration
Tomography, X-Ray Computed
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Summary:This is a report of an autopsy case of Wilson's disease with widespread degeneration of the cerebral cortex and white matter, the basal ganglia and thalamus and, to a lesser degree, the cerebellum and brain stem. The patient was a 28-year-old man at the time of death with the clinical course of a 20-year duration. The lesions consisted of spongy degeneration leading to a cavity formation with insufficient glia fiber proliferation. We noted the characteristic findings of Alzheimer glia (Types I and II) and Opalski cells and the new formation in capillaries. The distribution of the changes in the hemispheres showed the typical pattern with a tendency of preferential superior and anterior localization and a relative preservation of the hippocampal formation, carcarine areas, amygdaloid nuclei and the hypothalamus. Similar cases in the literature were reviewed.
ISSN:0912-2036