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Dopamine D2 receptor binding and cerebral glucose metabolism recover afterd-penicillamine-therapy in Wilson's disease

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Abstract

Regional cerebral glucose metabolism (rCMRGlc) and dopamine D2 receptor binding were measured in a 31-year-old, severely affected, untreated patient with Wilson's disease of 3 years' duration using positron emission tomography and18F-deoxyglucose and18F-methylspiperone ([18F]MSP), respectively. There was a severe reduction of striatal and extrastriatal rCMRGlc as well as of striatal [18F]MSP accumulation rate. After 1 year of treatment withd-penicillamine, striatal and extrastriatal rCMRGlc and striatal [18F]MSP accumulation rate reached almost normal levels. It is hypothesized that recovery of motor functions due to copper trapping therapy was associated with an increase in basal ganglia activity and a re-expression or upregulation of dopamine D2 receptors.

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Authors and Affiliations

  1. Department of Neurology, Heinrich Heine University, P. O. Box 10 10 07, D-40001, Düsseldorf, Germany

    G. Schlaug, H. Hefter, P. Weiss & R. J. Seitz

  2. Institute of Nuclear Chemistry, Research Centre, Jülich, Germany

    B. Nebeling & G. Stöcklin

  3. Department of Diagnostic Radiology, Heinrich Heine University, D-40001, Düsseldorf, Germany

    V. Engelbrecht

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  1. G. Schlaug
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  2. H. Hefter
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  5. P. Weiss
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Schlaug, G., Hefter, H., Nebeling, B. et al. Dopamine D2 receptor binding and cerebral glucose metabolism recover afterd-penicillamine-therapy in Wilson's disease. J Neurol 241, 577–584 (1994). https://doi.org/10.1007/BF00920620

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  • DOI: https://doi.org/10.1007/BF00920620

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