Medical Dictionary
Main Entry:
Wil·son's disease Pronunciation:
'wil-s&nz- Function:
noun : a hereditary disease that is characterized by the accumulation of copperin 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 markedespecially by liver dysfunction and disease and neurologic or psychiatric symptoms (as tremors, slowness of speech, inappropriate behaviors, or personality changes) called also
hepatolenticulardegeneration; —see
KAYSER-FLEISCHER RING 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. Hisneurological studies covered such diverse topics as epilepsy, narcolepsy, speech disorders, apraxia, and pathological laughing and crying. In 1912 he published a monograph on progressive lenticulardegeneration, which is now known as Wilson's disease. He was the first to detect the relationship between liver disease and putaminous destruction.
