Portal-Systemic Encephalopathy Due to Congenital Intrahepatic Shunts

Abstract

IN the course of chronic liver disease the development of portal hypertension and the subsequent formation of portal collateral circulation may permit certain nitrogen-containing substances in the portal blood to bypass the extraction mechanism of the hepatic parenchyma. A cerebral intoxication, which Sherlock and her co-workers1 have termed portal–systemic encephalopathy, has been observed in some of these cases. This phenomenon has been known since 1893, when Hahn et al.2 described the "meat-intoxication" syndrome — that is, stupor, ataxia, convulsions and coma — in animals with an Eck fistula that were fed high-protein diets. Recently, there have been a few patients . . .