Portal-Systemic Encephalopathy in the Noncirrhotic Patient

Abstract

A pilot multidisciplined clinical and laboratory study of 16 patients who had undergone a portal-systemic shunt in childhood disclosed evidence of central nervous system impairment to varying degrees 4 to 19 years later. Eight of the patients had normal livers and eight had abnormal livers (cirrhosis) at the time of the shunting procedure. Those patients with normal livers developed predominantly emotional manifestations and those with abnormal livers predominantly neurological manifestations. The severity of the abnormalities in both groups seemed directly related to the duration of exposure to the shunt and to the degree of liver insufficiency, if present. We felt that the observed changes could not be explained on the basis of early environmental factors alone and that the portalsystemic shunt played a significant causative role in the normal liver group.