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April 1953

HEPATOLENTICULAR DEGENERATION: Review of the Literature and Report of a Case with Dimercaprol (BAL) Therapy

Author Affiliations


From the Veterans Administration Hospital, New Orleans; Resident in Medicine (Dr. Schechter); Chief, Medical Service (Dr. Jones).

AMA Arch Intern Med. 1953;91(4):541-549. doi:10.1001/archinte.1953.00240160127009

PROGRESSIVE lenticular degeneration (hepatolenticular degeneration, Wilson's disease) has been described as a rare disease involving the basal ganglion system and characterized by a chronic progressive fatal course. This disease is probably not so rare as was previously believed. One or two patients may be found in almost every city, and frequently in such cases the erroneous diagnosis of multiple sclerosis or paralysis agitans (Parkinsonism) is made. Recently, interest has been revived in this disease because of the possibility of a new and effective therapeutic agent. Occasional reports of small groups of patients treated with dimercaprol (Bal— 2,3-dimercaptopropanol) have been published in recent years. The most prominent of such reports is the one of Denny-Brown and Porter,1 in which they observed improvement in five patients who were subjected to repeated courses of dimercaprol. Our purpose in this report is to add an additional case report of a patient treated with

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