Wilson''s Disease in the Adult

Abstract

We reported three cases of Wilson's disease in the adult. Onset of Wilson's disease occurs between 4 and 40 years of age, but is relatively rare in adults. In a typically progressed stage the disease may easily be diagnosed from findings of Kayser-Fleischer's corneal ring in addition to the clinical features as neurologic manifestation and liver involvement, but there still remain many unknown problems concerning abnormal copper metabolism in Wilson's disease. The retention of radioactive copper (64Cu) was measured with a whole body counter in three patients with Wilson's disease, four of their family members and two control subjects. The 64Cu retained in the body showed high value in the patients with Wilson's disease, which may reflect a larger copper pool than in normal subjects. The applicability of retention measurement with 64Cu as a screening test is discussed. Study of the body retention of 64Cu may be useful as a screening test to differentiate the carrier from normal individuals in the family of Wilson's disease. In Wilson's disease, excretion of urinary copper and body retention of radiocopper increased. Urinary copper in Wilson's disease may be excreted via the copper pool, rather than directly after absorption through the intestine.