Development of Neurologic Symptoms in a Patient With Asymptomatic Wilson's Disease Treated With Penicillamine
George J. Brewer, MD;
Atac Turkay, MD;
Vilma Yuzbasiyan-Gurkan, PhD
Arch Neurol. 1994;51(3):304-305.
Abstract
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Objective
To report a case of presymptomatic Wilson's disease in a patient who became severely neurologically disabled after treatment with penicillamine and to discuss alternative initial therapy for such patients.
Design
Case report. The patient described is briefly compared with a previously studied group of 13 similar presymptomatic patients who received zinc therapy without any clinical worsening and who have had 3 to 9 years of follow-up.
Setting
Referral hospital.
Patients
The patient was referred to us.
Intervention
The patient had initially been treated with penicillamine.
Main Outcome Measure
The main outcome of interest is permanent neurologic disability, depending on type of initial treatment.
Results
The result of initial penicillamine therapy in the patient described is permanent neurologic disability. This is believed to be due to mobilization and redistribution of hepatic copper causing higher levels of copper in key areas of the brain.
Conclusion
We conclude that penicillamine used as initial therapy, even in patients with neurologically asymptomatic Wilson's disease, increases the risk of inducing permanent neurologic damage.
Author Affiliations
From the Departments of Human Genetics (Drs Brewer, Turkay, and Yuzbasiyan-Gurkan) and Internal Medicine (Dr Brewer), The University of Michigan Medical School, Ann Arbor.
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