A case study on wilson’s disease (westphal- strümpell pseudosclerosis).

ABSTRACT

Wilson disease of Progressive Lenticular Degeneration is a familial nervous disease associated with Cirrhosis of the Liver. It has been observed that there is an increased copper content of the liver and brain. Increased excretion of copper in patients with Wilson disease & increased even more after administration of the chelating agent British anti-Lewisite (BAL). Ciruloplasmin, serum protein that binds copper, is reduced. The deposition of copper in tissues is the cause of virtually all the manifestations of the disease in Liver Blood Kidney & Brain We have observed three different cases of different clinical presentations.