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 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.
Shimoji
A
A
Department of Neuropsychiatry, Kumamoto Medical School, Japan.
Miyakawa
T
T
Watanabe
K
K
Yamashita
K
K
Katsuragi
S
S
Kabashima
K
K
D000328:Adult; D001921:Brain / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology*; D003300:Copper / Q000378:metabolism; D004827:Epilepsy / Q000473:pathology; D006527:Hepatolenticular Degeneration / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D009410:Nerve Degeneration; D014057:Tomography, X-Ray Computed
D003300:Copper