The Cerebellum

, Volume 16, Issue 4, pp 792–796 | Cite as

Spinal Cord Damage in Spinocerebellar Ataxia Type 1

  • Carlos Roberto MartinsJr
  • Alberto Rolim Muro Martinez
  • Thiago Junqueira Ribeiro de Rezende
  • Lucas Melo Teixeira Branco
  • José Luiz Pedroso
  • Orlando G. P. Barsottini
  • Iscia Lopes-Cendes
  • Marcondes C. FrançaJr
Original Paper

Abstract

Spinocerebellar ataxia type 1 (SCA1) is an autosomal dominant disorder caused by a CAG repeat expansion, characterized by progressive cerebellar ataxia and pyramidal signs. Non-motor and extracerebellar symptoms may occur. MRI-based studies in SCA1 focused in the cerebellum and connections, but there are no data about cord damage in the disease and its clinical relevance. To evaluate in vivo spinal cord damage in SCA1, a group of 31 patients with SCA1 and 31 age- and gender-matched healthy controls underwent MRI on a 3T scanner. We used T1-weighted 3D images to estimate the cervical spinal cord area (CA) and eccentricity (CE) at three C2/C3 levels based on a semi-automatic image segmentation protocol. The scale for assessment and rating of ataxia (SARA) was used to quantify disease severity. The groups were significantly different regarding CA (47.26 ± 7.4 vs. 68.8 ± 5.7 mm2, p < 0.001) and CE values (0.803 ± 0.044 vs. 0.774 ± 0.043, p < 0.05). Furthermore, in the patient group, CA presented significant correlation with SARA scores (R = −0.633, p < 0.001) and CAGn expansion (R = −0.658, p < 0.001). CE was not associated with SARA scores (p = 0.431). In the multiple variable regression, CA was strongly associated with disease duration (coefficient −0.360, p < 0.05) and CAGn expansion (coefficient −1.124, p < 0.001). SCA1 is characterized by cervical cord atrophy and anteroposterior flattening. Morphometric analyses of the spinal cord MRI might be a useful biomarker in the disease.

Keywords

Spinal cord MRI Ataxia Spinocerebellar ataxia type 1 Polyglutamine disorders Biomarker 

Notes

Compliance with Ethical Standards

Conflicts of Interest

The authors report no conflict of interests regarding this research.

Funding

This work was supported by Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)—grant 2013/01766-7.

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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Carlos Roberto MartinsJr
    • 1
  • Alberto Rolim Muro Martinez
    • 1
  • Thiago Junqueira Ribeiro de Rezende
    • 1
  • Lucas Melo Teixeira Branco
    • 1
  • José Luiz Pedroso
    • 2
  • Orlando G. P. Barsottini
    • 2
  • Iscia Lopes-Cendes
    • 3
  • Marcondes C. FrançaJr
    • 1
    • 4
  1. 1.Department of Neurology and Neuroimaging Laboratory, School of Medical SciencesUniversity of Campinas—UNICAMPCampinasBrazil
  2. 2.Department of Neurology, Ataxia UnitFederal University of São Paulo—UNIFESPSão PauloBrazil
  3. 3.Department of Medical Genetics, School of Medical SciencesUniversity of Campinas—UNICAMPCampinasBrazil
  4. 4.Department of NeurologyUniversity of Campinas—UNICAMPCampinasBrazil

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