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Brain structural damage in spinocerebellar ataxia type 1

A VBM study

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Abstract

Background and objective

Neuropathological description of the brain in spinocerebellar ataxia type 1(SCA1) is limited to a few cases. Voxel-based morphometry (VBM) enables an unbiased in vivo whole-brain quantitative analysis of regional differences in gray matter (GM) and white matter (WM) volume. We assessed with VBM the structural damage in patients with genetically confirmed SCA1.

Method

Fifteen SCA1 patients and 15 age-matched healthy controls underwent MR examination with acquisition of high-resolution T1-weighted images. The results were correlated with the disease duration and severity of the clinical deficit assessed with the International Cerebellar Ataxia Rating Scale (ICARS) and Inherited Ataxia Clinical Rating Scale (IACRS).

Results

As compared to controls, patients with SCA1 showed a significant (p < 0.05 corrected for multiple comparison) symmetric loss of volume of the GM in the rostral cerebellar vermis and paramedian portions of the anterior cerebellar lobes. WM was decreased in the peridentate region and middle cerebellar peduncles but not in the pons. No GM or WM volume loss was found in the cerebral hemispheres. The cerebellar and brainstem GM and WM volume loss correlated with disease duration and the ICARS and IACRS scores.

Conclusions

VBM confirms that atrophy predominantly involves the brainstem and cerebellum in SCA1. The correlation with the clinical features indicates that VBM might be useful to monitor disease progression.

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Authors and Affiliations

  1. Radiodiagnostic Section, Dept. of Clinical Physiopathology, University of Florence, Viale Morgagni 85, 50134, Florence, Italy

    A. Ginestroni, R. Della Nave &  M. Mascalchi

  2. Radiology Unit, Versilia Hospital, Camaiore (Lucca), Italy

    C. Tessa

  3. Medical Physics, University of Pisa, Pisa, Italy

    M. Giannelli

  4. Dept. of Neurology, Rovigo Hospital, Rovigo, Italy

    D. De Grandis

  5. Division of Neurology, Bellaria Hospital, Bologna, Italy

    R. Plasmati & F. Salvi

  6. Dept. of Neurological and Psychiatric Sciences, University of Florence, Florence, Italy

    S. Piacentini

Authors
  1. A. Ginestroni
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  2. R. Della Nave
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  3. C. Tessa
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  4. M. Giannelli
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  5. D. De Grandis
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  6. R. Plasmati
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  7. F. Salvi
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  8. S. Piacentini
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  9. M. Mascalchi
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Corresponding author

Correspondence to M. Mascalchi.

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Ginestroni, A., Della Nave, R., Tessa, C. et al. Brain structural damage in spinocerebellar ataxia type 1. J Neurol 255, 1153–1158 (2008). https://doi.org/10.1007/s00415-008-0860-4

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  • DOI: https://doi.org/10.1007/s00415-008-0860-4

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