A multimodal evaluation of microstructural white matter damage in spinocerebellar ataxia type 3

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dc.contributor.author Guimaraes, Rachel P.
dc.contributor.author D'Abreu, Anelyssa
dc.contributor.author Yasuda, Clarissa L.
dc.contributor.author Franca, Marcondes C.
dc.contributor.author Silva, Beatriz H. B.
dc.contributor.author Cappabianco, Fabio A. M. [UNIFESP]
dc.contributor.author Bergo, Felipe P. G.
dc.contributor.author Lopes-Cendes, Iscia T.
dc.contributor.author Cendes, Fernando
dc.date.accessioned 2016-01-24T14:31:56Z
dc.date.available 2016-01-24T14:31:56Z
dc.date.issued 2013-07-01
dc.identifier http://dx.doi.org/10.1002/mds.25451
dc.identifier.citation Movement Disorders. Hoboken: Wiley-Blackwell, v. 28, n. 8, p. 1125-1132, 2013.
dc.identifier.issn 0885-3185
dc.identifier.uri http://repositorio.unifesp.br/handle/11600/36469
dc.description.abstract Although white matter damage may play a major role in the pathogenesis of spinocerebellar ataxia 3 (SCA3), available data rely exclusively upon macrostructural analyses. in this setting we designed a study to investigate white matter integrity. We evaluated 38 genetically-confirmed SCA3 patients (mean age, 52.76 +/- 12.70 years; 21 males) with clinical scales and brain magnetic resonance imaging (MRI) and 38 healthy subjects as a control group (mean age, 48.86 +/- 12.07 years, 20 male). All individuals underwent the same protocol for high-resolution T1 and T2 images and diffusion tensor imaging acquisition (32 directions) in a 3-T scanner. We used Tract-Based Spatial Statistics (FSL 4.1.4) to analyze diffusion data and SPM8/DARTEL for voxel-based morphometry of infratentorial structures. T2-relaxometry of cerebellum was performed with in-house-developed software Aftervoxel and Interactive Volume Segmentation (IVS). Patients' mean age at onset was 40.02 +/- 11.48 years and mean duration of disease was 9.3 +/- 2.7 years. Mean International Cooperative Ataxia Rating Scale (ICARS) and Scale for Assessment and Rating of Ataxia (SARA) scores were 32.08 +/- 4.01 and 14.65 +/- 7.33, respectively. Voxel-based morphometry demonstrated a volumetric reduction of gray and white matter in cerebellum and brainstem (P <.001). We found reduced fractional anisotropy (P <.05) in the cerebellum and brainstem. There were also areas of increased radial diffusivity (P <.05) in the cerebellum, brainstem, thalamus, frontal lobes, and temporal lobes. in addition, we found decreased T2-relaxation values in the white matter of the right cerebellar hemisphere. Microstructural white matter dysfunction, not previously reported, occurs in the cerebellum and brainstem of SCA3 patients. (c) 2013 Movement Disorder Society en
dc.description.sponsorship Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)
dc.format.extent 1125-1132
dc.language.iso eng
dc.publisher Wiley-Blackwell
dc.relation.ispartof Movement Disorders
dc.rights Acesso restrito
dc.subject Machado-Joseph disease en
dc.subject MRI en
dc.subject DTI en
dc.subject VBM en
dc.title A multimodal evaluation of microstructural white matter damage in spinocerebellar ataxia type 3 en
dc.type Artigo
dc.rights.license http://olabout.wiley.com/WileyCDA/Section/id-406071.html
dc.contributor.institution Universidade Estadual de Campinas (UNICAMP)
dc.contributor.institution Universidade Federal de São Paulo (UNIFESP)
dc.description.affiliation Univ Campinas UNICAMP, Dept Neurol, Fac Med, São Paulo, Brazil
dc.description.affiliation Univ Campinas UNICAMP, Neuroimaging Lab, Fac Med, São Paulo, Brazil
dc.description.affiliation Fed Univ São Paulo UNIFESP, Inst Sci & Technol, São Paulo, Brazil
dc.description.affiliation Univ Campinas UNICAMP, Fac Med, Dept Med Genet, São Paulo, Brazil
dc.description.affiliationUnifesp Fed Univ São Paulo UNIFESP, Inst Sci & Technol, São Paulo, Brazil
dc.identifier.doi 10.1002/mds.25451
dc.description.source Web of Science
dc.identifier.wos WOS:000322960800023


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