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Basal Ganglia Diseases

Abstract

Wilson’s disease is an autosomal recessive disorder in which excessive amounts of copper accumulate in body tissues, particularly in the liver, kidneys, cornea, and brain. It presents initially at ages 8–12 with hepatic, neurological, or psychiatric disturbances including movement disorders (dysarthria, rigidity, tremor) and psychotic behavior. There are characteristic Kayser-Fleischer rings caused by copper in the limbus of the cornea. Serum ceruloplasmin and copper levels are low, and urinary copper is increased.

Keywords

Caudate Nucleus Globus Pallidus Dentate Nucleus Autosomal Recessive Disorder Menkes Disease 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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16 Basal Ganglia Diseases

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Copyright information

© Springer 2007

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