Decreased dopamine transporter binding in Machado-Joseph disease

Tzu Chen Yen*, Chin Song Lu, Kai Yuan Tzen, Shiaw Pyng Wey, Yah Huei Wu Chou, Yi Hsin Weng, Pan Fu Kao, Gann Ting

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

68 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)994-998
Number of pages5
JournalJournal of Nuclear Medicine
Volume41
Issue number6
StatePublished - 06 2000

Keywords

  • Machado-Joseph disease
  • SPECT
  • TRODAT-1

Fingerprint

Dive into the research topics of 'Decreased dopamine transporter binding in Machado-Joseph disease'. Together they form a unique fingerprint.

Cite this