Wilson's Disease
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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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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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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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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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