肝脳変性疾患の一症例について

私は最近，肝脳変性疾患の1例で，特殊型に類似した興味ある症例に遭遇し，其の臨床経過,病理組織，及び若干の組織化学的検索を行つたので其の結果について述べた．

The author reported the clinical course, histopathological and histochemical findings of a interesting case of hepatolenticular degeneration which is similar to the so called Inose's specific type. The onset was in the age of 62 years. His hereditary history is unknown. 26 months after the onset he had the remarkable disturbances of Consciousness and died 43 days after. The cardinal symptoms were the disturbances of consciousness, occurred twice, progressive dement state and extrapyramidal manifestations, i. e. remarkable tremor and slight muscle rigidity. He had not Kayser-Fleischer's corneal ring or ascites. Histopathological examinations revealed the softening areas in the 3rd, 4th and 6th layers of sulci cerebri, newly formed blood vessels, ganglion cell deficiency areas of the lateral und posterior site of the globus pallidum and of nucleus thalamicus ventralis, slight spongious changes of the putamen, and appearance of many glia cells of the Alzheimer's IInd type. The remarkable liver changes were the proliferation of Glisson's capsules and those which were similar to the pseudocirrhosis of liver of the Inose's type. Histochemically, copper granules did not appear in both brain and liver, but Best carmine positive substance was not found in the Alzheimer's IInd type glia cells.