Dopamine transporter binding in Wilson's disease.
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
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.
Huang
Chin-Chang
CC
Department of Neurology, Chang Gung Memorial Hospital and University, Taipei, Taiwan.
Chu
Nai-Shin
NS
Yen
Tzu-Chen
TC
Wai
Yau-Yau
YY
Lu
Chin-Song
CS
D000328:Adult; D001479:Basal Ganglia / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D004298:Dopamine / Q000172:deficiency*; D050483:Dopamine Plasma Membrane Transport Proteins; D005260:Female; D006527:Hepatolenticular Degeneration / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008562:Membrane Glycoproteins; D026901:Membrane Transport Proteins / Q000172:deficiency*; D009127:Muscle Rigidity / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D009419:Nerve Tissue Proteins; D009434:Neural Pathways / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D015609:Organotechnetium Compounds; D017729:Presynaptic Terminals / Q000378:metabolism; D011485:Protein Binding / Q000502:physiology; D013378:Substantia Nigra / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon; D014326:Tropanes
D050483:Dopamine Plasma Membrane Transport Proteins; D008562:Membrane Glycoproteins; D026901:Membrane Transport Proteins; D009419:Nerve Tissue Proteins; D015609:Organotechnetium Compounds; C493214:SLC6A3 protein, human; D014326:Tropanes; C104196:technetium Tc 99m TRODAT-1; D004298:Dopamine