The Pallial Component in Hepato-Lenticular Degeneration

Publisher Website
Google Scholar
Abstract
The author presents the case of a boy, aged 12, with characteristic Wilson''s disease. There was symmetrical degeneration of the lenticular nuclei combined with the widespread presence of Alzheimer glia cells and lobular cirrhosis of the liver. The destruction of the brain was not limited to the lenticular nuclei, however, since there was marked change in the pallium of the cerebral hemispheres. The great frequency and profound degree to which the cerebral hemispheres participate in the destructive process of hepato-lenticular degeneration suggest that the disease be renamed hepato-cerebral degeneration. The author postulates that the liver damage and cerebral changes are parallel expressions of the action of some specific endotoxin. Usually the liver is more vulnerable and is affected first, or may be affected alone, but on rare occasions the brain is the more vulnerable organ and is the only one to be damaged.