The presence of dystonia is associated with greater severity of ataxia in spinocerebellar ataxia (SCA)1, 2, and 3, but predictive of a slower progression in SCA6, a recent study found. Complex genetic interactions among repeat expansion genes can lead to diverse clinical symptoms and progression in SCAs. Researchers studied 334 participants with SCA1, 2, 3, and 6 from the Clinical Research Consortium for Spinocerebellar Ataxias (CRC-SCA) and compared the clinical characteristics of SCAs with and without dystonia. They repeatedly measured ataxia progression by the Scale for Assessment and Rating of Ataxia (SARA) every 6 months for 2 years and found:
- Dystonia was most commonly observed in SCA3, followed by SCA2, SCA1, and SCA6.
- Dystonia was associated with longer cytosine-adenine-guanine (CAG) repeats in SCA3.
- The presence of dystonia was associated with higher SARA scores in SCA1, 2, and 3.
- Although relatively rare in SCA6, the presence of dystonia was associated with slower progression of ataxia.
Kuo P-H, Gan S-R, Wang J, et al. Dystonia and ataxia progression in spinocerebellar ataxias. [Published online ahead of print October 23, 2017]. Parkinson Relat Disord. doi:10.1016/j.parkreldis.2017.10.007.
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