Wilson's Disease and Monoamines

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Abstract
To the Editor.— Patients suffering from Wilson's disease frequently have extrapyramidal symptoms, which are ameliorated by treatment with penicillamine.1Apparently, the storage of copper2in this disease gives rise to disturbances of function and later to observable damage of central neurons,3which can be reversed to some extent after appropriate treatment. Moreover, treatment with levodopa has beneficial effects in Wilson's disease.4.5Taking this into consideration, it can be speculated that changes in central neuronal functioning in Wilson's disease may result inter alia in abnormalities in the metabolism of the neurotransmitters, dopamine, and serotonin. Recently, we had the opportunity to investigate this possibility in a 21-year-old patient with Wilson's disease. We measured the levels of 5-hydroxyindole acetic acid (5-HIAA, a 5-HT metabolite) and homovanillic acid (HVA, a metabolite of dopamine) in lumbar CSF of this patient, before and during treatment with penicillamine (1 g/day). In addition, we