The authors report no conflict of interests regarding this research. This work was supported by FAPESP.
Clinical Investigative Study
Neocortical Atrophy in Machado-Joseph Disease: A Longitudinal Neuroimaging Study
Article first published online: 23 JUN 2011
© 2011 by the American Society of Neuroimaging
Journal of Neuroimaging
Volume 22, Issue 3, pages 285–291, July 2012
How to Cite
D’Abreu, A., França Jr., M. C., Yasuda, C. L., Campos, B. A. G., Lopes-Cendes, I. and Cendes, F. (2012), Neocortical Atrophy in Machado-Joseph Disease: A Longitudinal Neuroimaging Study. Journal of Neuroimaging, 22: 285–291. doi: 10.1111/j.1552-6569.2011.00614.x
J Neuroimaging 2012;22:285-291.
- Issue published online: 11 JUL 2012
- Article first published online: 23 JUN 2011
- Acceptance: Received October 18, 2010, and in revised form February 23, 2011. Accepted for publication March 13, 2011.
- Spinocerebellar ataxia type 3;
- Machado-Joseph disease;
- voxel-based morphometry;
- cortical atrophy
Previous imaging studies in the Machado-Joseph disease (MJD/SCA3) have mostly concentrated on the cerebellum and brainstem. Our goal was to perform a whole brain longitudinal evaluation.
We included 45 patients and 51 controls, who underwent two brain magnetic resonance imaging and magnetic resonance spectroscopy (mean interval of 12.5 ± 1.5 months). We used voxel-based morphometry (VBM) and the MarsBar analysis toolbox to extract grey matter density (GMD) values from regions of interest. We used a linear regression model and a general linear model to correlate GMD with clinical markers, and paired t-test for the longitudinal evaluation.
We observed decreased GMD (P < .01) at frontal, parietal, temporal and occipital lobes, subcortical grey matter, cerebellum, and brainstem. White matter atrophy was restricted to the cerebellum. Age, CAG, and disease duration predicted GMD in different areas, but age and CAG were the most important predictors. The longitudinal analysis failed to demonstrate changes. Changes in regions other than the cerebellum appeared to contribute significantly to the final International Cooperative Ataxia Rating Scale score.
We confirmed cortical involvement in MJD/SCA3. The most important factors in predicting GMD were age and CAG. The lack of progression of atrophy may indicate floor effect and/or short duration of follow-up.