肝脳疾患特殊型の1例

Abstract

Clinical, cerebro-pathological, histochemical and biochemical studies were carried out on a case what appeared to be the special type (Inose) of hepato-cerebral disease, and the following results were obtained.1) Clinically, there could be recognized no Kayser-Fleischer ring, and the liver disturbances were observed from early stage, edema and ascites being especially marked. In addition, characteristic tremors of the fingers, i.e. Wackel tremors, were observed. It was characteristic with this case that there occurred repeated clouding of consciousness for the four months prior to death.2) There were observed abnormalities in the copper metabolism. Namely, despite the presence of copper deposition in the cytoplasm of liver cells, no such deposition could be detected in the brain tissue. Furthermore, tbe amount of copper excreted into urine proved to be as much as twice that in normal person.3) From the pathology of the brain, Alzheimer type II glia could be seen everywhere in the brain, and globus pallidus as well as the boundary between the cerebral cortex and medullar had fallen into a marked spongy state. In addition, carmine-positive substance was found in the nuclei of liver cells and in Alzheimer type II glia nuclei. These finding correspond to those of the special type of hepato-cerebral disease as reported by Inose. However, there were also observed Opalski cells in putamen and globus pallidus, the finding said to be specific to Wilson's disease.4) Pathologically, the liver was in the state of coarse, nodular and annular cirrhosis, and the fat degeneration of liver parenchyma was marked. By the iron staining both the liver and brain gave negative results, 5) Some comments were made on hepatic coma, Wilson's disease and the special type of hepato-cerebral disease, while referring to the available literature, and also discrepancy in regard to the findings in these diseases was pointed out.6) Admitting that the abnormality in the copper metabolism, the pathological findings of the liver and the appearance of Opalski cells would raise some question of doubt, after due consideration on these points it seems more rational to classify this case as the special type (Inose) of hepato-cerebral disease.