SHUNT ENCEPHALOMYELOPATHY. II. OCCURRENCE OF PERMANENT MYELOPATHY

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Abstract
The occurrence of permanent spastic paraplegia is described in two patients with cirrhosis who had repeated episodes of protein coma which followed the combination of gastrectomy and portacaval shunt. Pyramidal tract demyelination in the spinal cord, but not in the brain, was demonstrated in the first case. Two other documented case reports of patients having spinal cord demyelination in association with portal-systemic shunting were found in a survey of the literature; however, these patients had the findings of hepatolenticular degeneration, but not Wilson''s disease, in addition to the cord changes. Both of our patients had marked protein sensitivity which progressed with time, so that body protein depletion and wasting were inevitable. Gastrectomy probably acted primarily as an intensifier of the effects of the portacaval shunt. The spinal cord lesion is believed to be a metabolic effect of some protein breakdown product ordinarily removed or modified by the liver but, in these cases, shunted around the hepatic cells. An attempted unification of the various neuropsychiatric syndromes associated with portal-systemic shunts or hepatic disease resulted in the segregation of the many variants into four broad groups: acute, acute recurrent, chronic recurrent, and chronic permanent encephalo-myelopathy.

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