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April 1957

Hepatolenticular Degeneration: Follow-Up Report of a Case Treated with Dimercaprol (BAL), Potassium Sulfide, and High-Protein Diet

Author Affiliations

New Orleans

From the Louisiana State University School of Medicine Neurology Service, Charity Hospital of Louisiana at New Orleans; Instructor in Neurology (Dr. Ferriss); Resident in Medicine (Dr. Berry).

AMA Arch Intern Med. 1957;99(4):656-659. doi:10.1001/archinte.1957.00260040156015

Since the first report of this case, in this journal (1953),1 there has been considerable progress in the elucidation of copper metabolism in man2 and the biochemical pathophysiology of Wilson's disease. The suggestion that the neurological manifestations of this condition are related to the positive copper balance, with alteration in structure and function of the brain secondary to copper deposition therein,3 has been widely, though not universally,4 accepted and is further supported by this report. Copper absorption from the gut is greatly increased.5 Transport of most of the plasma copper, loosely bound to protein, in the absence of sufficient ceruloplasmin 6-8 allows deposition of copper in the tissues. Excretion through both liver and kidneys is reflected in the high values in the bile 9 and urine.10 In the latter, it is supposed that the deposition of copper in kidney tissue precedes aminoaciduria,11 and then these amino acids and peptides in