Document Detail

Decreased dopamine transporter binding in Machado-Joseph disease.
MedLine Citation:
PMID:  10855623     Owner:  NLM     Status:  MEDLINE    
The aim of this study was to use 99mTc-TRODAT-1 brain SPECT for investigation of the binding of dopamine transporter (DAT) in the nigrostriatal dopaminergic pathway of symptomatic Machado-Joseph disease (MJD) and to compare the results with the abnormal cytidylate, adenylate, and guanylate (CAG) expansion in the MJD1 gene and other clinical factors. METHODS: Ten symptomatic MJD patients (8 women, 2 men; age range, 20-71 y; mean age +/- SD, 36.4 +/- 10.6 y; mean duration of illness, 9.8 +/- 5.4 y) and 21 healthy volunteers (age range, 24-71 y; mean age, 47.6 +/- 20.1 y) were examined. Brain SPECT images were acquired 4 h after injection. The ratio of specific to nonspecific nigrostriatal 99mTc-TRODAT-1 binding was measured and compared with the clinical symptoms, duration of illness, and size of abnormal expanded CAG repeats. RESULTS: All nigrostriatal 99mTc-TRODAT-1 ratios were significantly lower in MJD patients than in healthy volunteers (P < 0.05). Discriminant function analysis of all MJD patients showed that the decreased binding of 99mTc-TRODAT-1 in the putamen was not significantly different from that in the caudate nucleus. Eight of 10 MJD patients had significantly decreased 99mTc-TRODAT-1 uptake. Of these 8, 2 had extrapyramidal signs and 6 had no obvious extrapyramidal signs. The other 2 patients, who had normal 99mTc-TRODAT-1 uptake, had no obvious extrapyramidal signs. CONCLUSION: Our findings indicate that 99mTc-TRODAT-1 brain SPECT is an appropriate method for evaluating damage to the nigrostriatal DAT in symptomatic MJD patients with and without extrapyramidal signs. The decreased binding of 99mTc-TRODAT-1 in the nigrostriatal dopaminergic pathway in symptomatic MJD patients correlates with the phenotype of extrapyramidal signs but not with the abnormal CAG repeat length, age at disease onset, or disease duration.
T C Yen; C S Lu; K Y Tzen; S P Wey; Y H Chou; Y H Weng; P F Kao; G Ting
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of nuclear medicine : official publication, Society of Nuclear Medicine     Volume:  41     ISSN:  0161-5505     ISO Abbreviation:  J. Nucl. Med.     Publication Date:  2000 Jun 
Date Detail:
Created Date:  2000-07-07     Completed Date:  2000-07-07     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  0217410     Medline TA:  J Nucl Med     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  994-8     Citation Subset:  IM    
Department of Nuclear Medicine, Chang Gung Memorial Hospital, Taipei Medical Center and School of Medicine, Chang Gung University, Taiwan.
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MeSH Terms
Brain / metabolism*,  radionuclide imaging
Carrier Proteins / metabolism*
Caudate Nucleus / metabolism,  radionuclide imaging
Dopamine / metabolism*
Dopamine Plasma Membrane Transport Proteins
Machado-Joseph Disease / metabolism*,  radionuclide imaging
Membrane Glycoproteins*
Membrane Transport Proteins*
Middle Aged
Nerve Tissue Proteins*
Organotechnetium Compounds / diagnostic use
Putamen / metabolism,  radionuclide imaging
Substantia Nigra / metabolism
Tomography, Emission-Computed, Single-Photon*
Trinucleotide Repeat Expansion
Tropanes / diagnostic use
Reg. No./Substance:
0/Carrier Proteins; 0/Dopamine Plasma Membrane Transport Proteins; 0/Membrane Glycoproteins; 0/Membrane Transport Proteins; 0/Nerve Tissue Proteins; 0/Organotechnetium Compounds; 0/SLC6A3 protein, human; 0/Tropanes; 0/technetium Tc 99m TRODAT-1

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