MORBUS WILSON - KRITISCHE VERSCHLECHTERUNG UNTER HOCHDOSIERTER PARENTERALER PENICILLAMIN-THERAPIE

A 31-year-old man with Wilson's disease, not treated for the past 4 1/2 years, was admitted to hospital with brain concussion after a fall. While receiving penicillamine, 1 g i.v. four times daily, the neurological signs worsened and akinesia, mutism, tachy- and bradyarrhythmias, as well as transitory respiratory insufficiency developed. Serum copper concentration on the sixth day of treatment was markedly decreased to 28 μg/dl, rising to 60 μg/dl on the ninth day. 24-hour urinary copper excretion was at first 4500-5000 μg. Only after drastic reduction of the penicillamine dosage to 600 mg three times daily was there any improvement and after 11 weeks the patient was again able to walk and discharged. Marked, mainly hepatic, copper depletion from the high penicillamine dosage was the likely cause of the patient's initial deterioration. To avoid cerebral complications penicillamine should be administered in gradually increasing doses.