it is a rare diorder of abnormal copper metabolism and is
characterised by:
hepatolenticular degeneration.
copper get deposited in abnormal amounts in liver and lenticular
nucleus of brain leading to necrosis of liver and brain.
low level of copper and ceruloplasmin in plasma with increased
excretion of copper in urine,
copper deposition in kidneys causes renal damage.
causes:
failure in the synthesis of ceruloplasmin.
excess copper in plasma.
reduced intestinal excretion of copper.
treatment: penicillamine.