dc.creatorFahl, Camila N
dc.creatorBranco, Lucas Melo T
dc.creatorBergo, Felipe P G
dc.creatorD'Abreu, Anelyssa
dc.creatorLopes-Cendes, Iscia
dc.creatorFrança, Marcondes C
dc.date2015-Apr
dc.date2015-11-27T13:46:38Z
dc.date2015-11-27T13:46:38Z
dc.date.accessioned2018-03-29T01:24:07Z
dc.date.available2018-03-29T01:24:07Z
dc.identifierCerebellum (london, England). v. 14, n. 2, p. 128-32, 2015-Apr.
dc.identifier1473-4230
dc.identifier10.1007/s12311-014-0619-7
dc.identifierhttp://www.ncbi.nlm.nih.gov/pubmed/25370748
dc.identifierhttp://repositorio.unicamp.br/jspui/handle/REPOSIP/202280
dc.identifier25370748
dc.identifier.urihttp://repositorioslatinoamericanos.uchile.cl/handle/2250/1302513
dc.descriptionMachado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in the disease and its clinical relevance. To evaluate the spinal cord damage in SCA3 through quantitative analysis of MRI scans. A group of 48 patients with SCA3 and 48 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 employed to quantify disease severity. The two groups-SCA3 and controls-were significantly different regarding CA (49.5 ± 7.3 vs 67.2 ± 6.3 mm(2), p < 0.001) and CE values (0.79 ± 0.06 vs 0.75 ± 0.05, p = 0.005). In addition, CA presented a significant correlation with SARA scores in the patient group (p = 0.010). CE was not associated with SARA scores (p = 0.857). In the multiple variable regression, we found that disease duration was the only variable associated with CA (coefficient = -0.629, p = 0.025). SCA3 is characterized by cervical cord atrophy and antero-posterior flattening. In addition, the spinal cord areas did correlate with disease severity. This suggests that quantitative analyses of the spinal cord MRI might be a useful biomarker in SCA3.
dc.description14
dc.description128-32
dc.languageeng
dc.relationCerebellum (london, England)
dc.relationCerebellum
dc.rightsfechado
dc.rights
dc.sourcePubMed
dc.titleSpinal Cord Damage In Machado-joseph Disease.
dc.typeArtículos de revistas


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