HEPATOLENTICULAR DEGENERATION

Abstract

Hepatolenticular degeneration is a rare, progressive and fatal disease characterized by extrapyramidal signs, degenerative changes in the basal ganglions, cirrhosis of the liver and pigmentation of Descemet's membrane at the limbus of the cornea (Kayser-Fleischer ring). Denny-Brown¹ in a recent review of this subject has pointed out that the work of Hall² in establishing the identity of Wilson's disease (progressive lenticular degeneration) and Westphal-Strümpell's disease (pseudosclerosis) has obscured the remarkable differences between the two syndromes. There can be no doubt that the two groups are closely related and should be considered as aspects of the same disease process. To that extent Wilson's attempt to absorb pseudosclerosis into his group of "progressive lenticular degeneration" may have been justified. However, in the cases described by Wilson and most English and American authors the postural changes were prominent and cases with tremor and without postural changes were largely overlooked, although this