Hepatocerebral degeneration

Abstract

Two cases of hepatocerebral degeneration are presented, in one of which the presence of an abnormality in copper metabolism was established biochemically, while in the other such abnormality was excluded. The literature on similar cases was reviewed. The presence of Alzheimer I cells, of Opalski cells, and of widespread bizarre changes in most astrocytes in the corpus striatum visible with metallic impregnation was noted in the former case but not in the latter. These changes are thought to be a specific consequence of the copper metabolism abnormality. In both cases, a series of pathological changes were observed, which are thought to be a consequence of liver damage, perhaps due to an error of copper metabolism or to another nonspecific cause. These changes included neuronal degeneration, foci of spongy change in the cortex and corpus striatum, and diffuse degeneration of the white matter. The white matter changes are thought to be due to cerebral edema. The pathogenesis of the other changes is less clear.