HEPATOLENTICULAR DEGENERATION

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Abstract
Hepatolenticular degeneration is the name suggested by Barnes and Hurst1in 1925 for a rare, often familial, disease characterized by symptoms of extrapyramidal disturbance associated with a marked lobular cirrhosis of the liver. At autopsy, the extrapyramidal symptoms are accounted for by bilateral degenerative processes in the lenticular nuclei (Wilson's disease) and at times also in the thalamus, hypothalamus, pons and other regions (Westphal's pseudosclerosis). The name hepatolenticular degeneration is based on the opinion that the diseases first described by Wilson2and Westphal, often considered in textbooks as two distinct though closely related entities, are probably merely variations of the same disease. Strümpell3some years ago first grouped pseudosclerosis, Wilson's disease and paralysis agitans as the "amyostatic affections," thus indicating the close relation he believed these conditions bore to one another. Wechsler,4in his textbook, states that pseudosclerosis is probably a form of Wilson's disease. This

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