Hepatolenticular Degeneration (Wilson's Disease) as a Form of Idiopathic Cirrhosis

THE appearance of symptoms and signs of liver disease before the onset of neurologic abnormalities in hepatolenticular degeneration has been noted many times in the past,1 2 3 4 5 and there are several reported cases of siblings of patients with known Wilson's disease who died of liver failure without ever having neurologic abnormalities. These have been termed examples of "abdominalle" Wilson's disease by Kehrer6 or "les formes splanchniques" by André.7 It has been customary to refer to this group as the "formes frustes" of the disease, on the assumption that in these patients neurologic abnormalities would have developed if their lives had not . . .