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June 1950

HEPATOLENTICULAR DEGENERATION: Analysis of Dyskinetic Phenomena; Relation of Degree of Hepatic Damage to Course of the Disease; Nervous Disorders in Ordinary Disease of the Liver

Author Affiliations


From the Department of Neurology and Neuropathology, Columbia University College of Physicians and Surgeons, and the Neurological Institute of New York.

Arch NeurPsych. 1950;63(6):843-874. doi:10.1001/archneurpsyc.1950.02310240002001

THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1 Westphal2 and Strümpell3 and the numerous subsequent reports make it possible to delimit this disease from other pathologic entities in the majority of cases. However, a detailed analysis of the dyskinetic symptoms is still wanting and the importance of liver function tests for diagnostic consideration is still under discussion.

The specificity of the clinical picture, as well as that of the pathologic changes in the brain stem, needs to be stressed again in the light of recent observations on nervous disorders associated with various hepatic diseases in man and in animals. A comparison of these conditions with hepatolenticular degeneration can be established only after

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