An inborn defect of copper metabolism in which there is a deficiency of caeruloplasmin (which normally forms a nontoxic complex with copper). The free copper may be deposited in the liver, causing jaundice and cirrhosis, or in the brain, causing mental retardation and symptoms resembling parkinsonism. There is a characteristic brown ring in the cornea (the Kayser-Fleischer ring). If the excess copper is removed from the body by regular treatment with penicillamine both mental and physical deficits tend to improve. Medical name: hepatolenticular degeneration. S. A. K. Wilson (1878–1936), British neurologist
Subjects: Medicine and Health — Science and Mathematics.