Wilson’s disease

Wilson’s disease [Samuel A.K. Wilson, English neurologist, 1878–1937] , a rare inherited disorder whereby a decrease in ceruloplasmin causes copper to accumulate slowly in the liver, to then be released, and to be taken up in other parts of the body. Hemolysis and hemolytic anemia occur as the copper accumulates in the red blood cells. Accumulation in the brain destroys certain tissue and may cause tremors, muscle rigidity, poorly articulated speech, and dementia. Kidney function is diminished. The liver becomes cirrhotic. Treatment of Wilson’s disease includes a reduction of copper in the diet and the prescription of copper-binding agents and penicillamine. Also called hepatolenticular degeneration.