Spinal Cord Damage in Spinocerebellar Ataxia Type 1

Spinal Cord Damage in Spinocerebellar Ataxia Type 1

Author Martins, Carlos Roberto, Jr. Google Scholar
Muro Martinez, Alberto Rolim Google Scholar
Ribeiro de Rezende, Thiago Junqueira Google Scholar
Teixeira Branco, Lucas Melo Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Barsottini, Orlando G. P. Autor UNIFESP Google Scholar
Lopes-Cendes, Iscia Google Scholar
Franca, Marcondes C., Jr. Google Scholar
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
Spinocerebellar ataxia type 1
Polyglutamine disorders
Language English
Sponsor Fundação de Amparo a Pesquisa do Estado de São Paulo (FAPESP)
Grant number FAPESP: 2013/01766-7
Date 2017
Published in Cerebellum. New York, v. 16, n. 4, p. 792-796, 2017.
ISSN 1473-4222 (Sherpa/Romeo, impact factor)
Publisher Springer
Extent 792-796
Origin http://dx.doi.org/10.1007/s12311-017-0854-9
Access rights Closed access
Type Article
Web of Science ID WOS:000405033000005
URI http://repositorio.unifesp.br/handle/11600/51514

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