HEPATOLENTICULAR DEGENERATION

Abstract

Wilson¹ in 1912, in an article entitled "Progressive Lenticular Degeneration: A Familial Nervous Disease Associated with Cirrhosis of the Liver," first clearly defined the condition now most widely known as hepatolenticular degeneration. His accurate description included and amplified the poorly characterized so-called pseudosclerosis, described by Westphal² and by Strumpell² in the closing years of the last century. Since the publication of Wilson's article, considerably more than 100 cases of this disease have been reported in the literature. The essential features of the syndrome are cirrhosis of the liver and a bilateral softening and degeneration of the lenticular nuclei. Although not hereditary in the sense of being passed from one generation to the next, the disease has a pronounced familial tendency and frequently affects several children in the same family. As many as five of seven siblings have been known' to have the disease. It is a disease