Journal of Neurology

, Volume 255, Issue 8, pp 1244–1249

Spinal cord atrophy in spinocerebellar ataxia type 3 and 6

Impact on clinical disability
  • C. Lukas
  • H. K. Hahn
  • B. Bellenberg
  • K. Hellwig
  • C. Globas
  • S. K. Schimrigk
  • O. Köster
  • L. Schöls
ORIGINAL COMMUNICATION

DOI: 10.1007/s00415-008-0907-6

Cite this article as:
Lukas, C., Hahn, H.K., Bellenberg, B. et al. J Neurol (2008) 255: 1244. doi:10.1007/s00415-008-0907-6

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.

Key words

spinocerebellar ataxia type 3 and type 6spinal cord atrophyMRI

Copyright information

© Springer 2008

Authors and Affiliations

  • C. Lukas
    • 1
  • H. K. Hahn
    • 2
  • B. Bellenberg
    • 3
  • K. Hellwig
    • 3
  • C. Globas
    • 4
  • S. K. Schimrigk
    • 3
  • O. Köster
    • 1
  • L. Schöls
    • 4
  1. 1.Dept. of Diagnostic and Interventional Radiology and Nuclear MedicineSt. Josef Hospital Ruhr University BochumBochumGermany
  2. 2.MeVis ResearchBremenGermany
  3. 3.Dept. of NeurologySt. Josef Hospital Ruhr University BochumBochumGermany
  4. 4.Dept. of Neurology and Hertie-Institute for Clinical Brain ResearchUniversity of TübingenTübingenGermany