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Spinal cord atrophy in spinocerebellar ataxia type 3 and 6

Impact on clinical disability

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Abstract

Objective

To quantify spinal cord atrophy and its impact on clinical disability in spinocerebellar ataxia (SCA) type 3 and 6.

Methods

Atrophy of the upper spinal cord was assessed by high resolution T1-weighted MRI of patients with SCA3 (n = 14) and SCA6 (n = 10). Furthermore, two groups of age- and sex-matched healthy control subjects (n = 24,) corresponding to the two SCA groups, were studied. Images were post-processed by a semi-automated volumetry method combining a marker based segmentation and an automatic histogram method facilitating highly reliable quantification and morphometry of the upper cervical cord in vivo.

Results

We found a significant reduction of normalized mean crosssectional area of the spinal cord in SCA3 (p < 0.0005), whereas in SCA6 patients normalized mean crosssectional area was in the normal range (p = 0.379). No correlation was found between spinal cord atrophy and disease duration as well as CAG repeat length in both subtypes. In SCA6 a negative dependency between clinical disability, as expressed by the International Cooperative Ataxia Rating Scale as a well established ataxia score, and the mean cross-sectional area was found (p = 0.02). A similar correlation was observed in SCA3 but did not reach statistical significance.

Conclusion

Our results quantify for the first time in vivo spinal cord atrophy as a non-cerebellar neurodegenerative process in SCA3. Our results suggest MR volumetry of the upper cervical cord as a marker of functional importance in SCA3 and SCA6.

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Authors and Affiliations

  1. Dept. of Diagnostic and Interventional Radiology and Nuclear Medicine, St. Josef Hospital Ruhr University Bochum, Gudrunstr. 56, 44791, Bochum, Germany

    C. Lukas MD & O. Köster MD

  2. MeVis Research, Universitaetsallee 29, 28359, Bremen, Germany

    H. K. Hahn PhD

  3. Dept. of Neurology, St. Josef Hospital Ruhr University Bochum, Gudrunstr. 56, 44791, Bochum, Germany

    B. Bellenberg PhD, K. Hellwig MD & S. K. Schimrigk MD

  4. Dept. of Neurology and Hertie-Institute for Clinical Brain Research, University of Tübingen, Hoppe-Seyler-Str. 3, 72076, Tübingen, Germany

    C. Globas MD & L. Schöls MD

Authors
  1. C. Lukas MD
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  2. H. K. Hahn PhD
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  3. B. Bellenberg PhD
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  4. K. Hellwig MD
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  5. C. Globas MD
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  6. S. K. Schimrigk MD
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  7. O. Köster MD
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  8. L. Schöls MD
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Corresponding author

Correspondence to C. Lukas MD.

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Lukas, C., Hahn, H.K., Bellenberg, B. et al. Spinal cord atrophy in spinocerebellar ataxia type 3 and 6. J Neurol 255, 1244–1249 (2008). https://doi.org/10.1007/s00415-008-0907-6

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  • DOI: https://doi.org/10.1007/s00415-008-0907-6

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