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September 1978

Wilson's Disease and Monoamines

Author Affiliations

Dept of Neurology Hospital Ziekenzorg De Ruyterlaan 5 Enschede, Netherlands
Dept of Biological Psychiatry Psychiatric University Clinic Oostersingel 59 Groningen, Netherlands

Arch Neurol. 1978;35(9):617-618. doi:10.1001/archneur.1978.00500330065014

To the Editor.—  Patients suffering from Wilson's disease frequently have extrapyramidal symptoms, which are ameliorated by treatment with penicillamine.1 Apparently, the storage of copper2 in this disease gives rise to disturbances of function and later to observable damage of central neurons,3 which can be reversed to some extent after appropriate treatment. Moreover, treatment with levodopa has beneficial effects in Wilson's disease.4.5 Taking 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