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2 results

  • Dopamine Transporter Binding in Wilson's Disease

  • Chin-Chang Huang, Nai-Shin Chu, Tzu-Chen Yen, Yau-Yau Wai, Chin-Song Lu
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 30 / Issue 2 / May 2003
    Published online by Cambridge University Press:
    16 December 2016, pp. 163-167
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  • Introduction:

    In Wilson's disease (WD), brain magnetic resonance images (MRI) show increased signal intensity in T2 weighted images in the lenticular nuclei, thalamus and brainstem, including the substantia nigra. A poor therapeutic response to levodopa in WD suggests the mechanism of a postsynaptic abnormality. However positron emission tomography studies show an involvement of the nigrostriatal presynaptic dopaminergic pathway.

    Case report:

    We report the clinical manifestations in a case of WD with akinetic-rigid syndrome and initial hesitation. The brain MRI showed an increased signal intensity lesion in the substantia nigra region, in addition to basal ganglion and thalamic lesions. However, dopamine transporter (DAT) imaging with 99mTc-TRODAT-1 revealed a nonsignificantly increased DAT uptake, suggesting a normal presynaptic nigrostriatal dopaminergic terminal.

    Conclusion:

    We suggest that significant heterogeneity can be found in WD patients and a normal presynaptic dopaminergic pathway may occur in some patients, even those with typical akinetic-rigid syndrome and evidence of substantia nigra involvement in the brain on MRI.