(wil´sun) a rare progressive disease, inherited as an autosomal recessive trait and due to a defect in metabolism of copper , with accumulation of copper in the liver, brain, kidney, cornea, and other tissues. Characteristics include cirrhosis in the liver and degenerative changes in the brain, particularly the basal ganglia. Liver disease is the most likely manifestation in children; neurologic disease is most common in young adults. The characteristic ophthalmic feature is a pigmented ring ( Kayser-Fleischer ring ) at the outer margin of the cornea. Called also hepatolenticular degeneration .
