Distribution of cerebral lesions in acquired hepatocerebral degeneration

The brain lesions of our patients with chronic portal-systemic venous shunts were much the same regardless of the presence or absence of primary liver disease. Alzheimer's type 2 abnormality of astrocytes and demarcated areas of spongy degeneration were found, mainly in the grey matter, with more severe involvement of the basal ganglia and the deeper layers of the frontal, parietal and occipital cortex. The spongy degeneration showed an especially close correlation with the arterial blood supply, being greatest in borderland areas that fall between the regions usually supplied by one or other of the major cerebral arteries. Previous investigations have not been primarily concerned with selective vulnerability so that comparisons are difficult, but on review, there is evidence that this pattern of involvement is a feature of both Wilson's disease and acquired hepatocerebral degeneration. The toxic effect of the portal blood is greatest in the grey matter, probably because of the greater metabolic activity there, and the distribution of lesions within this area of greater vulnerability appears to be further influenced by circulatory factors.