Corticospinal tract involvement in spinocerebellar ataxia type 3: a diffusion tensor imaging study
- PMID: 32876704
- DOI: 10.1007/s00234-020-02528-3
Corticospinal tract involvement in spinocerebellar ataxia type 3: a diffusion tensor imaging study
Abstract
Purpose: The aim of this study was to evaluate the integrity of the corticospinal tracts (CST) in patients with SCA3 and age- and gender-matched healthy control subjects using diffusion tensor imaging (DTI). We also looked at the clinical correlates of such diffusivity abnormalities.
Methods: We assessed 2 cohorts from different Brazilian centers: cohort 1 (n = 29) scanned in a 1.5 T magnet and cohort 2 (n = 91) scanned in a 3.0 T magnet. We used Pearson's coefficients to assess the correlation of CST DTI parameters and ataxia severity (expressed by SARA scores).
Results: Two different results were obtained. Cohort 1 showed no significant between-group differences in DTI parameters. Cohort 2 showed significant between-group differences in the FA values in the bilateral precentral gyri (p < 0.001), bilateral superior corona radiata (p < 0.001), bilateral posterior limb of the internal capsule (p < 0.001), bilateral cerebral peduncle (p < 0.001), and bilateral basis pontis (p < 0.001). There was moderate correlation between CST diffusivity parameters and SARA scores in cohort 2 (Pearson correlation coefficient: 0.40-0.59).
Conclusion: DTI particularly at 3 T is able to uncover and quantify CST damage in SCA3. Moreover, CST microstructural damage may contribute with ataxia severity in the disease.
Keywords: Cerebellar ataxia; Corticospinal tract; Diffusion tensor imaging; Machado-Joseph disease; Retrograde degeneration; Spinocerebellar ataxia type 3.
References
-
- Kawaguchi Y, Okamoto T, Taniwaki M, Aizawa M, Inoue M, Katayama S, Kawakami H, Nakamura S, Nishimura M, Akiguchi I, Kimura J, Narumiya S, Kakizuka A (1994) CAG expansions in a novel gene for Machado-Joseph disease at chromosome 14q32.1. Nat Genet 8:221–228. https://doi.org/10.1038/ng1194-221 - DOI - PubMed
-
- Nakano K, Spence A, Dawson D (1972) Machado disease. Neurology 22:49–55. https://doi.org/10.1212/WNL.22.1.49 - DOI - PubMed
-
- Rosenberg RN, Fowler HL, de Magalhães J et al (1977) Azorean disease of the nervous system. N Engl J Med 297:729–730. https://doi.org/10.1056/NEJM197709292971318 - DOI
-
- Dürr A, Stevanin G, Cancel G et al (1996) Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features. Ann Neurol 39:490–499. https://doi.org/10.1002/ana.410390411 - DOI - PubMed
-
- Pedroso JL, França MC, Braga-Neto P et al (2013) Nonmotor and extracerebellar features in Machado-Joseph disease: a review. Mov Disord 28:1200–1208. https://doi.org/10.1002/mds.25513 - DOI - PubMed
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