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Treatment of the Neurologic Manifestations of Wilson's Disease

Treatment of the Neurologic Manifestations of Wilson's Disease Abstract Because of their concern that initial treatment of neurologically manifest Wilson's disease with penicillamine can cause worsening of the neurologic symptoms, Brewer and colleagues1 have used ammonium tetrathiomolybdate as the initial treatment in 11 such patients. Six additional patients, who had been initially treated with penicillamine, were subsequently treated with ammonium tetrathiomolybdate. During 8 weeks of treatment with tetrathiomolybdate, "None of the patients suffered a loss of neurologic function."1 In the follow-up period of 1 to 5 years, during which the patients received only 150mg of zinc acetate daily, there was"... substantial improvement (of neurologic function) in almost all patients."1 In the past 35 years, we ourselves, or as consultants to other physicians, have prescribed initial treatment with penicillamine for hundreds of patients with Wilson's disease, of whom about 40% were affected neurologically. Neurologic worsening occurred in about 10% of the latter group in the weeks following References 1. Brewer GJ, Dick RD, Johnson V, et al. Treatment of Wilson's disease with ammonium tetrathiomolybdate . Arch Neurol . 1994;51:545-554.Crossref 2. Cumings JN. The effects of BAL in hepatolenticular degeneration . Brain . 1951; 74:10-22.Crossref 3. Scheinberg IH, Sternlieb I. Treatment . In: Wilson's Disease . Philadelphia, Pa: WB Saunders Co; 1984:146. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Treatment of the Neurologic Manifestations of Wilson's Disease

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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1995.00540280019007
Publisher site
See Article on Publisher Site

Abstract

Abstract Because of their concern that initial treatment of neurologically manifest Wilson's disease with penicillamine can cause worsening of the neurologic symptoms, Brewer and colleagues1 have used ammonium tetrathiomolybdate as the initial treatment in 11 such patients. Six additional patients, who had been initially treated with penicillamine, were subsequently treated with ammonium tetrathiomolybdate. During 8 weeks of treatment with tetrathiomolybdate, "None of the patients suffered a loss of neurologic function."1 In the follow-up period of 1 to 5 years, during which the patients received only 150mg of zinc acetate daily, there was"... substantial improvement (of neurologic function) in almost all patients."1 In the past 35 years, we ourselves, or as consultants to other physicians, have prescribed initial treatment with penicillamine for hundreds of patients with Wilson's disease, of whom about 40% were affected neurologically. Neurologic worsening occurred in about 10% of the latter group in the weeks following References 1. Brewer GJ, Dick RD, Johnson V, et al. Treatment of Wilson's disease with ammonium tetrathiomolybdate . Arch Neurol . 1994;51:545-554.Crossref 2. Cumings JN. The effects of BAL in hepatolenticular degeneration . Brain . 1951; 74:10-22.Crossref 3. Scheinberg IH, Sternlieb I. Treatment . In: Wilson's Disease . Philadelphia, Pa: WB Saunders Co; 1984:146.

Journal

Archives of NeurologyAmerican Medical Association

Published: Apr 1, 1995

References