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April 1995

Treatment of the Neurologic Manifestations of Wilson's Disease

Author Affiliations

National Center for the Study of Wilson's Disease Departments of Medicine St Luke's/Roosevelt Hospital College of Physicians and Surgeons Columbia University 432 W 58th St, Suite 614 New York, NY 10019

Arch Neurol. 1995;52(4):339-340. doi:10.1001/archneur.1995.00540280019007

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

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