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April 1930

HEPATOLENTICULAR DEGENERATIONA REPORT OF THREE UNUSUAL CASES

Author Affiliations

PARIS, FRANCE; BALTIMORE

Arch NeurPsych. 1930;23(4):750-760. doi:10.1001/archneurpsyc.1930.02220100134008
Abstract

The concept of progressive lenticular degeneration as a clinicopathologic entity has changed since its first delineation by Wilson1 in 1912. Characterized clinically by progressive symptoms referable to the basal ganglia, often clearly familial in incidence, usually occurring in young persons, running either an acute or a chronic course and always fatal, at autopsy every case has shown nodular hypertrophic cirrhosis of the liver, as well as degeneration of the putamen and caudate nuclei. The nervous lesions are sometimes more widespread, with cortical and cerebellar involvement. Through the work of many others, but notably Hall,2 there has developed a more liberal concept which includes Wilson's disease with the pseudosclerosis of Fleischer under the general term "hepatolenticular degeneration." Although there are points of difference between the two diseases that merit attention, still the nomenclature has been generally accepted and has been vigorously upheld by recent English writers.

One of the

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