Wilson's disease


Wil·son's disease

noun \ˈwil-sənz-\

Definition of WILSON'S DISEASE

: a hereditary disease that is characterized by the accumulation of copper in the body (as in the liver, brain, or cornea) due to abnormal copper metabolism associated with ceruloplasmin deficiency, that is determined by an autosomal recessive gene, and that is marked especially by liver dysfunction and disease and neurologic or psychiatric symptoms (as tremors, slowness of speech, inappropriate behaviors, or personality changes)—called also hepatolenticular degeneration; see kayser-fleischer ring

Biographical Note for WILSON'S DISEASE

Wilson, Samuel Alexander Kinnier (1877–1937), British neurologist. Wilson enjoyed long-term associations with King's College Hospital and the National Hospital for the Paralyzed and Epileptic, both in London. His neurological studies covered such diverse topics as epilepsy, narcolepsy, speech disorders, apraxia, and pathological laughing and crying. In 1912 he published a monograph on progressive lenticular degeneration, which is now known as Wilson's disease. He was the first to detect the relationship between liver disease and putaminous destruction.

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