An autopsy case of the "demyelinating type" of Wilson's disease.
A 17 year-old male, who was mentally and bodily retarded from the age of 7 years. At the age of 16 years, several neurological signs appeared. Pathologically, there was widespread myelin loss and tissue sponginess in the white matter of the cerebrum and cerebellum, bilaterally and symmetrically. A great many Alzheimer type II glia and Opalski glia were widely distributed in the cerebral cortex and the basal ganglia. Despite severe destruction of the white matter, mesenchymal and gliofibre proliferation were not noticed. In the liver, Laennec's cirrhosis and a great many copper granules in the cytoplasm of the parenchymal cells were identified. From the above mentioned findings the present case, being a new type, could be called "Demyelinating type of Wilson's disease".
D000293:Adolescent; D001921:Brain / Q000473:pathology*; D003711:Demyelinating Diseases; D006527:Hepatolenticular Degeneration / Q000473:pathology*; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male