Episodic Stupor Associated with an Eck Fistula in the Human with Particular Reference to the Metabolism of Ammoniaxs12

Abstract
In an attempt to effect a surgical cure of carcinoma of the head of the pancreas in a 69-yr.-old man, the portal vein was resected and an anastomosis was established between the superior mesenteric artery and the inferior vena cava. An Eck fistula was thus created for 1st time in a human subject who had neither pre-existing portal hypertension nor an established collateral circulation. Liver function was normal by all standard laboratory tests. The liver appeared normal histologically. Convalescence was uneventful until the 5th postoperative week when the 1st of a series of episodic stupors occurred. These consisted of mental confusion or coma, fluctuating rigidity of the limbs, reflex grasping and sucking and extensor plantar reflexes. The onset was acute and without assignable cause, the course progressive or several days, and recovery rapid and complete. This unique disorder prompted a prolonged metabolic study. During a 6-mo. period there were 16 such episodes. An elevation of blood ammonia to 50-75 [mu]g. above normal was the only metabolic disorder found. The admn. of a high protein diet, ammonium chloride, urea or an ammonia-liberating cation exchange resin reproduced the neurological disorder and the elevation of blood ammonia on 8 occasions. A low protein diet plus sulfathalidine to decrease intestinal flora effected a reduction in blood ammonia levels. In 5 mos. there occurred only 3 mild periods of confusion. This syndrome is probably the same as "meat intoxication" in Eck-fistula dogs.