Abstract Details

Title Clinical Scales and Brain Pathology in SCA3: A Voxel-Based Morphometry and Diffusion Tensor Imaging Study

Topic Movement Disorders

Presentation(s) (-)

Poster/Presentation
Number 003

Objective

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<0.001) and GM in brainstem, anterior and posterior cerebellar lobe and pons (p<0.001); We also found a positive correlation between scales scores and disease duration (r=0.4, p<0.003) and a correlation between ICARS and SARA (r=0.9, p=0.003).

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.

Authors/Disclosures
Rachel P. Guimaraes, PT (Unicamp) PRESENTER	No disclosure on file
Anelyssa D'Abreu, MD, PhD, MPH, FAAN (Department of Neurology- University of Virginia)	The institution of Dr. D'Abreu has received research support from U.S. NIH Institute on Aging. The institution of Dr. D'Abreu has received research support from Biogen. The institution of Dr. D'Abreu has received research support from American College Of Radiology. The institution of Dr. D'Abreu has received research support from COGNITION THERAPEUTICS, INC.. The institution of Dr. D'Abreu has received research support from Eli Lilly. The institution of Dr. D'Abreu has received research support from Jansen. Dr. D'Abreu has received personal compensation in the range of $10,000-$49,999 for serving as a Lecturer with Pri_med.
Frederick E. Munschauer III, MD, FAAN (FEMC)	Dr. Munschauer has received personal compensation for serving as an employee of Emalex Biosciences. Dr. Munschauer has stock in Emalex Biosciences.
Clarissa L. Yasuda, MD, PhD (University of Campinas)	Prof. Yasuda has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for LIBBS. Prof. Yasuda has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ABBOTT. Prof. Yasuda has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for LIBBS.
Marcondes C. Franca, Jr., MD	Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PTC. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Dr. Franca has received research support from FARA.
Iscia Lopes-Cendes, MD, PhD (University of Campinas - UNICAMP)	No disclosure on file
Fernando Cendes, MD, PhD, FAAN (Departamento de Neurologia; FCM; UNICAMP)	Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB Biopharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for United Medical – Brazil. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Zodiac Pharma . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Eurofarma – Brazil . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Epilepsia. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers in Neurology - Epilepsy. The institution of Dr. Cendes has received research support from São Paulo Research Foundation - FAPESP. The institution of Dr. Cendes has received research support from Conselho Nacional de Desenvolvimento Científico e Tecnológico - Brazil . The institution of Dr. Cendes has received research support from NIH.

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