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Semin Neurol 1999; 19(3): 261-270
DOI: 10.1055/s-2008-1040842
© 1999 by Thieme Medical Publishers, Inc.

Diagnosis and Treatment of Wilson's Disease

George J. Brewer1 , John K. Fink2 , 3 , Peter Hedera2
  • 1University of Michigan Departments of Human Genetics and Internal Medicine, Ann Arbor Veteran's Affairs Medical Center, Ann Arbor, Michigan
  • 2University of Michigan Department of Neurology, Ann Arbor Veteran's Affairs Medical Center, Ann Arbor, Michigan
  • 3Geriatric Research Education and Clinical Center, Ann Arbor Veteran's Affairs Medical Center, Ann Arbor, Michigan
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Publication History

Publication Date:
19 March 2008 (online)

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ABSTRACT

Wilson's disease is due to an inherited defect in copper excretion into the bile by the liver. The resulting copper accumulation and copper toxicity results in liver disease, and in some patients, brain damage. Patients present, generally between the ages of 10 and 40 years, with liver disease, neurological disease of a movement disorder type, or behavioral abnormalities, and often with a combination of these. Because Wilson's disease is effectively treated, it is extremely important for physicians to learn to recognize and diagnose the disease. Treatment options have evolved rapidly in the last few years, with zinc now being the drug of choice in most situations.

Keywords

Wilson's disease - neurological disease - liver disease - ATP7B - copper