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Dopamine Transporter Binding in Wilson's Disease

Published online by Cambridge University Press:  16 December 2016

Chin-Chang Huang ,
Nai-Shin Chu ,
Tzu-Chen Yen ,
Yau-Yau Wai  and
Chin-Song Lu
Chin-Chang Huang*
Affiliation:
Department of Neurology, Chang Gung Memorial Hospital and University, Taipei, Taiwan
Nai-Shin Chu
Affiliation:
Department of Neurology, Chang Gung Memorial Hospital and University, Taipei, Taiwan
Tzu-Chen Yen
Affiliation:
Department of Nuclear Medicine, Chang Gung Memorial Hospital and University, Taipei, Taiwan
Yau-Yau Wai
Affiliation:
Department of Neuroradiology, Chang Gung Memorial Hospital and University, Taipei, Taiwan
Chin-Song Lu
Affiliation:
Department of Neurology, Chang Gung Memorial Hospital and University, Taipei, Taiwan
*
Department of Neurology, Chang Gung Memorial Hospital and University, 199 Tung Hwa North Road, Taipei, Taiwan.
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Abstract:

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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.

Résumé:

RÉSUMÉ:Introduction:

Dans la maladie de Wilson (MW), l'imagerie par résonance magnétique (IRM) du cerveau montre une augmentation de l'intensité du signal sur les images pondérées en T2 dans les noyaux lenticulaires, le thalamus et le tronc cérébral incluant la substance noire. Une réponse thérapeutique médiocre à la lévodopa dans la MW suggère que le mécanisme est une anomalie postsynaptique. Cependant la tomographie par émission de positons montre une implication de la voie dopaminergique présynaptique nigrostriée.

Observation:

Nous rapportons les manifestations cliniques observées chez un cas de MW présentant un syndrome akinéto-rigide et un retard à initier le mouvement. L'IRM du cerveau a montré une augmentation de l'intensité du signal dans la région de la substance noire ainsi que des lésions du noyau lenticulaire, du noyau caudé, de l'avant-mur, du noyau amygdalien et du thalamus. Cependant, l'imagerie du transporteur de la dopamine par le 99mTc-TRODAT-1 a montré une captation DAT augmentée de façon non significative suggérant que la terminaison dopaminergique nigrostriée présynaptique est normale.

Conclusion:

Nous suggérons qu'il existe une hétérogénéité significative chez les patients atteints de la MW et que la voie dopaminergique présynaptique peut être normale chez certains de ces patients, même en présence d'un syndrome akinéto-rigide typique et de l'observation d'une lésion de la substance noire à l'IRM.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 2 , May 2003 , pp. 163 - 167
Copyright
Copyright © The Canadian Journal of Neurological 2003

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