Portal-Systemic Encephalopathy Due to Congenital Intrahepatic Shunts

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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 . . .