| Wilson Disease (Hepatolenticular Degeneration) This disease is caused by the inheritance of a mutation on chromosome 13. The mutation prevents the body from eliminating excess copper. Ceruloplasmin is the protein that binds and removes excess copper and its levels are greatly reduced in this disorder. Too much copper in the system damages the cells of the liver and leads to cirrhosis. Neurological damage primarily occurs in the putamen and globus pallidus, collectively known as the lenticular nucleus. Symptoms of the disease include a peculiar type of tremor in the upper extremities, slowness of movement and changes in temperament. Persons may become exceptionally argumentative, overly emotional or may experience a decrease in mental capabilities. Kayser-Fleischer rings (a rusty brown discoloration at the rims of the corneas) become evident as the copper begins to affect the nervous system. The disease first affects the liver, and if treatment is administered early enough, damage to the nervous system is dramatically reduced. Treatment includes the elimination of copper containing foods such as chocolate and mushrooms. Patients are also given drugs to chelate excess copper and eliminate it from the body. |
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 Wilson, Samuel 1878-1937 |