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Wilson's Disease

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What is Wilson's Disease?

Wilson's disease is an inherited disorder in which excessive amounts of copper accumulate in the body. Although the accumulation of copper begins at birth, symptoms of the disorder appear later in life, between the ages of 6 and 40. The primary consequence for approximately 40 percent of patients with Wilson's is liver disease. In other patients the first symptoms are either neurological or psychiatric or both, and include tremor, rigidity, drooling, difficulty with speech, abrupt personality change, grossly inappropriate behavior and unexplicable deterioration of school work, neurosis or psychosis.

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Is there any treatment?

Treatment of Wilson's disease generally consists of anti-copper agents to remove excess copper from the body and to prevent it from reaccumulating. Most cases are treated with the drugs zinc acetate, trientine, or penicillamine. Penicillamine and trientine increase urinary excretion of copper, however, both drugs can cause serious side effects. Zinc acetate which blocks the absorption of copper, increases copper excretion in the stool, and causes no serious side affects is often considered the treatment of choice. Tetrathiomolybdate, an experimental drug, also shows promise in treating Wilson's disease. In rare cases in which there is severe liver disease, a liver transplant may be needed.

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What is the prognosis?

Without proper treatment, Wilson's disease is generally fatal, usually by the age of 30. If treatment is begun early enough, symptomatic recovery is usually complete, and a life of normal length and quality can be expected.

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What research is being done?

NINDS supports research to find ways to treat and prevent inherited disorders such as Wilson's disease. The ultimate goal of this research is to discover new treatments for the many genetic disorders that strike the brain and nervous system, including Wilson's disease.

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American Liver Foundation

March of Dimes Birth Defects Foundation

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK

Wilson's Disease Association, International

Worldwide Education & Awareness for Movement Disorders (WE MOVE)

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Wilson's Disease