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January 1955

WILSON'S DISEASE—CHRONIC FORM: Clinical-Pathological Observations in a Brother and Sister

Author Affiliations


From the Department of Neurology and Neurological Surgery, University of Illinois College of Medicine, and Mount Sinai Hospital (Dr. Lichtenstein), and the Department of Pathology, Chicago Medical School, and Mount Sinai Hospital (Dr. Gore).

AMA Arch NeurPsych. 1955;73(1):13-21. doi:10.1001/archneurpsyc.1955.02330070015003

ALTHOUGH we deplore the general use L of eponyms to designate disease entities, under certain circumstances an eponym is more informative than a purely descriptive clinical pathological term. Such is the case with Wilson's disease, in which the classic clinical triad of cirrhosis of the liver, degeneration of the lenticular nuclei, and Kayser-Fleischer rings has, in recent years, been somewhat overshadowed by the occurrence of massive amino-aciduria and increased urinary excretion of copper. The pathological designation of hepatolenticular degeneration is, in a sense, misleading, since the changes in the brain are certainly not limited to the lenticular nuclei. Wilson's disease generally appears in an acute or a chronic form, although sharp delineations cannot always be made. The chronic form is often mistaken for multiple sclerosis or chronic encephalitic Parkinsonism until its true nature is determined by the recognition of the characteristic pigmented ring at the corneoscleral junction and the clinical

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