Decreased dopamine transporter binding in Machacio-Joseph disease

Citation
Tc. Yen et al., Decreased dopamine transporter binding in Machacio-Joseph disease, J NUCL MED, 41(6), 2000, pp. 994-998
Citations number
38
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Radiology ,Nuclear Medicine & Imaging","Medical Research Diagnosis & Treatment
Journal title
JOURNAL OF NUCLEAR MEDICINE
ISSN journal
0161-5505 → ACNP
Volume
41
Issue
6
Year of publication
2000
Pages
994 - 998
Database
ISI
SICI code
0161-5505(200006)41:6<994:DDTBIM>2.0.ZU;2-A
Abstract
The aim of this study was to use Tc-99m-TRODAT-1 brain SPECT for investigat ion of the binding of dopamine transporter (DAT) in the nigrostriatal dopam inergic pathway of symptomatic Machado-Joseph disease (MJD) and to compare the results with the abnormal cytidylate, adenylate, and guanylate (CAG) ex pansion in the MJD1 gene and other clinical factors. Methods: Ten symptomat ic 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 volunte ers (age range, 24-71 y; mean age, 47.6 +/- 20.1 y) were examined. Brain SP ECT images were acquired 4 h after injection. The ratio of specific to nons pecific nigrostriatal Tc-99m-TRODAT-1 binding was measured and compared wit h the clinical symptoms, duration of illness, and size of abnormal expanded GAG repeats. Results: All nigrostriatal Tc-99m-TRODAT-1 ratios were signif icantly lower in MJD patients than in healthy Volunteers (P < 0.05). Discri minant function analysis of all MJD patients showed that the decreased bind ing of Tc-99m-TRODAT-1 in the putamen was not significantly different from that in the caudate nucleus. Eight of 10 MJD patients had significantly dec reased Tc-99m-TRODAT-1 uptake. Of these 8, 2 had extrapyramidal signs and 6 had no obvious extrapyramidal signs. The other 2 patients, who had normal Tc-99m-TRODAT-1 uptake, had no obvious extrapyramidal signs. Conclusion: Ou r findings indicate that Tc-99m-TRODAT-1 brain SPECT is an appropriate meth od for evaluating damage to the nigrostriatal DAT in symptomatic MJD patien ts with and without extrapyramidal signs.The decreased binding of (TC)-T-99 m-TRODAT-1 in the nigrostriatal dopaminergic pathway in symptomatic MJD pat ients correlates with the phenotype of extrapyramidal signs but not with th e abnormal CAG repeat length ,age at disease onset, or disease duration.