Acquired hepatocerebral degeneration: report of an atypical case

SUMMARY A case of acquired hepatocerebral degeneration secondary to biliary cirrhosis is described. It differs from the conventional type because of the clinical predominance of cerebellar symptomatology and because the principal neuropathological abnormalities were restricted to the pallidum and to the cerebellum. Implication of the nervous system in the acquired (non-Wilsonian) type of chronic hepatocerebral de-generation may take the form of transient or permanent psychiatric or neurological disturbances. It is generally accepted that these sequelae are due to an extensive porto-systemic collateral circulation and not to the type of liver disease or to the portal hypertension per se. The protean nature of the neuropsychiatric syndromes associated with chronic liver disease and an extensive porto-systemic collateral circulation has been reviewed by Summerskill, Davidson, Sherlock, and Steiner (1956) and by Read, Sherlock, Laidlaw, and Walker (1967). The commonest neurological disorder is a reversible encephalopathy-namely, hepatic precoma and coma. It is also recognized that there may be permanent damage to the brai