ウイルソン病の一例

1) A case reported of Wilson's disease confirmed by autopsy was presented. 2) The patient was a 17-year old girl. The disease developed with dysarthria having tremors like Parkinson's disease on the left hand and after passing subacute stage, the symptoms of extrapyramidal tract aggravated. Later in the terminal stage there occurred two attacks of clouding of consciousness and finally she died in the state of coma with high fever. However, Kayser-Fleischer ring was not detected. 3) The autopsy examination disclosed coarse nodular liver cirrhosis which is typical of Wilson's disease. Macroscopically, there was observed no marked change in the lentiform nucleus, but microscopically, there were such findings as Wilson's elements and pseudosclerotic elements that corresponded to histopathological changes of Wilson's disease. 4) Further discussion was made on the results of histochemical studies on copper, iron, polysaccharides and sugars.