Busca avançada
Ano de início
Entree
(Referência obtida automaticamente do Web of Science, por meio da informação sobre o financiamento pela FAPESP e o número do processo correspondente, incluída na publicação pelos autores.)

Spinal Cord Damage in Spinocerebellar Ataxia Type 1

Texto completo
Autor(es):
Martins, Jr., Carlos Roberto ; Muro Martinez, Alberto Rolim ; Ribeiro de Rezende, Thiago Junqueira ; Teixeira Branco, Lucas Melo ; Pedroso, Jose Luiz ; Barsottini, Orlando G. P. ; Lopes-Cendes, Iscia ; Franca, Jr., Marcondes C.
Número total de Autores: 8
Tipo de documento: Artigo Científico
Fonte: CEREBELLUM; v. 16, n. 4, p. 792-796, AUG 2017.
Citações Web of Science: 9
Resumo

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. (AU)

Processo FAPESP: 13/01766-7 - Contribuição ao diagnóstico, à fisiopatologia e à terapêutica das neuronopatias sensitivas
Beneficiário:Marcondes Cavalcante Franca Junior
Linha de fomento: Auxílio à Pesquisa - Apoio a Jovens Pesquisadores