title: Wilson's disease with extensive degeneration of cerebral white matter and cortex.
abstract: 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.
authors.affiliation: Department of Neuropsychiatry, Kumamoto Medical School, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Shimoji
authors.forename: T
authors.initials: T
authors.lastname: Miyakawa
authors.forename: K
authors.initials: K
authors.lastname: Watanabe
authors.forename: K
authors.initials: K
authors.lastname: Yamashita
authors.forename: S
authors.initials: S
authors.lastname: Katsuragi
authors.forename: K
authors.initials: K
authors.lastname: Kabashima
mesh_terms: 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
chemical_list: D003300:Copper