hepatolenticular degeneration /həpat′ōlentik″yo͝olər/ [Gk, hēpar + L, lens, lentil] , an abnormal autosomal-recessive condition associated with defective copper metabolism in the body, characterized by decreased serum ceruloplasmin and copper levels and increased secretion of urinary copper. In individuals with this condition, tissue deposits of copper associated with hepatic cirrhosis, deep marginal pigmentation of the cornea (known as Kayser-Fleischer rings), and extensive degeneration of the central nervous system, especially the basal ganglions, develop. Also called Wilson’s disease. See also Kayser-Fleischer ring.
