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April 1995

Treatment of the Neurologic Manifestations of Wilson's Disease-Reply

Author Affiliations

Departments of Human Genetics and Internal Medicine University of Michigan Medical School Ann Arbor, MI 48109

Arch Neurol. 1995;52(4):340. doi:10.1001/archneur.1995.00540280019008

In reply  The main point of the letter by Scheinberg and Sternlieb seems to be that penicillamine is an excellent therapy most of the time for patients with Wilson's disease who present with neurologic disease and, when it is not, injections with British anti-Lewisite are often helpful. The apparent implication is that, in their hands, problems with initial therapy are so few that development of an additional agent for this purpose, such as the tetrathiomolybdate therapy that we reported,1 is not worthwhile.The major problem with the assertions of Scheinberg and Sternlieb is that they are based on the notoriously inaccurate "clinical impression" rather than on rigorously established scientific fact. For example, they say that for 35 years "ourselves, or as consultants to other physicians, have prescribed initial treatment with penicillamine for hundreds of patients." Specifically, how many patients is "hundreds"?A scientific study would not say "hundreds," but would

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