CAG repeat length does not associate with the rate of cerebellar degeneration in spinocerebellar ataxia type 3

Shang Ran Huang, Yu Te Wu, Chii Wen Jao, Bing Wen Soong, Jiing Feng Lirng, Hsiu Mei Wu, Po Shan Wang*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

22 Scopus citations

Abstract

This cross-sectional study investigated the correlation between the CAG repeat length and the degeneration of cerebellum in spinocerebellar ataxia type 3 (SCA3) patients based on neuroimaging approaches. Forty SCA3 patients were recruited and classified into two subgroups according to their CAG repeat lengths (≥ 74 and < 74). We measured each patient's Scale for the Assessment and Rating of Ataxia (SARA) score, N-acetylaspartate (NAA)/creatine (Cr) ratios based on magnetic resonance spectroscopy (MRS), and 3-dimensional fractal dimension (3D-FD) values derived from magnetic resonance imaging (MRI) results. Furthermore, the 3D-FD values were used to construct structural covariance networks based on graph theoretical analysis. The results revealed that SCA3 patients with a longer CAG repeat length demonstrated earlier disease onset. However, the CAG repeat length did not significantly correlate with their SARA scores, cerebellar NAA/Cr ratios or cerebellar 3D-FD values. Network dissociation between cerebellar regions and parietal-occipital regions was found in SCA3 patients with CAG ≥ 74, but not in those with CAG < 74. In conclusion, the CAG repeat length is uncorrelated with the change of SARA score, cerebellar function and cerebellar structure in SCA3. Nevertheless, a longer CAG repeat length may indicate early structural covariance network dissociation.

Original languageEnglish
Pages (from-to)97-105
Number of pages9
JournalNeuroImage: Clinical
Volume13
DOIs
StatePublished - 2017

Keywords

  • Brain connectivity
  • Fractal dimension
  • Magnetic resonance imaging
  • Magnetic resonance spectroscopy
  • NAA
  • SCA3

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