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Decreased Dopamine Transporter Binding in Machado-Joseph Disease

Departments of Nuclear Medicine and Neurology and the Human Molecular Genetics Laboratory, Chang Gung Memorial Hospital, Taipei Medical Center and School of Medicine, Chang Gung University, Taipei
Institute of Nuclear Energy Research, Lung-Tan, Taiwan

Correspondence: For correspondence or reprints contact: Tzu-Chen Yen, MD, PhD, 9/F, No. 0.4, Fu-Quei First Rd., Taipei 112, Taiwan.

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 Machadc-Joseph disease (MJD) and to compare the results with the abnormal cyttdylate, 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.

Key Words: TRODAT-1 • SPECT • Machado-Joseph disease

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