Document Detail


Decreased dopamine transporter binding in Machado-Joseph disease.
MedLine Citation:
PMID:  10855623     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
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    
Affiliation:
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
Descriptor/Qualifier:
Adolescent
Adult
Aged
Brain / metabolism*,  radionuclide imaging
Carrier Proteins / metabolism*
Caudate Nucleus / metabolism,  radionuclide imaging
Child
Dopamine / metabolism*
Dopamine Plasma Membrane Transport Proteins
Female
Humans
Machado-Joseph Disease / metabolism*,  radionuclide imaging
Male
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
Chemical
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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