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- W2806462912 abstract "OBJECTIVE: To evaluate the clinico-anatomical correlation between neuroimaging findings and SARA (Scale for the assessment and rating of ataxia) and ICARS (International Cooperative Ataxia Rating Scale) scores in patients with spinocerebellar ataxia 3 (SCA3). BACKGROUND: SCA3 is an autosomal dominant neurodegenerative disorder characterized by ataxia, ophthalmoplegia, pyramidal dysfunction and movement disorder. SARA and ICARS are widely used in the clinical assessment of patients with SCA3. However, few studies have clearly demonstrated the correspondence between clinical scores and imaging findings. DESIGN/METHODS: We included thirty-eight patients (22 male, 49.32±10.65 years) with SCA3 with molecular confirmation (mean age of onset was 40.02 + 11.48 years) and 38 healthy subjects (20 male, 46.86 + 12.07 years). Clinical evaluation included SARA and ICARS. All individuals underwent the same protocol for high resolution T1 and DTI acquisition (32 directions) in a 3T scanner (Philips Achieva). We performed analysis of FA with TBSS (FSL 4.1.4) and voxel based morphometry with SPM 8/DARTEL (http://www.fil.ion.ucl.ac.uk). RESULTS: Baseline characteristics between patients and controls were comparable for gender (p = 0.9) and age (p=0.8). ICARS mean score was 31.7± 13.79 and SARA 14.65 ± 7.33. We found a negative correlation between WM and ICARS and SARA scores in the brainstem (p CONCLUSIONS: SARA and ICARS scores are valuable clinical tools in the evaluation of SCA3 patients, since it indirectly signals the degree of cerebral and cerebellar pathology. Supported by: FAPESP. Disclosure: Dr. Guimaraes has nothing to disclose. Dr. D9Abreu has received personal compensation for activities with Boehringer Ingelheim Pharmaceuticals, Inc., EMS, Roche, and Novartis. Dr. Yasuda has nothing to disclose. Dr. Franca, Jr. has nothing to disclose. Dr. Lopes-Cendes has nothing to disclose. Dr. Cendes has nothing to disclose." @default.
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- W2806462912 date "2013-02-12" @default.
- W2806462912 modified "2023-10-17" @default.
- W2806462912 title "Clinical Scales and Brain Pathology in SCA3: A Voxel-Based Morphometry and Diffusion Tensor Imaging Study (PD2.003)" @default.
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