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Abstract #0064

High field MRS is more sensitive to progression of neurodegeneration than clinical decline in spinocerebellar ataxia type 1 (SCA1)

Dinesh K Deelchand 1 , Uzay E Emir 1,2 , Diane Hutter 1 , Christopher M Gomez 3 , Lynn E Eberly 4 , Khalaf O Bushara 5 , and Gulin Oz 1

1 Center for Magnetic Resonance Research, University of Minnesota, Minneapolis, MN, United States, 2 FMRIB Centre, Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom, 3 Department of Neurology, University of Chicago, Chicago, IL, United States, 4 Division of Biostatistics, School of Public Health, University of Minnesota, Minneapolis, MN, United States, 5 Department of Neurology, Medical School, University of Minnesota, Minneapolis, MN, United States

In this study, MR spectroscopy was used to monitor disease progression in SCA1 patients. Subjects were scanned at baseline and after an ~18 month follow-up on 3 T. We found that in pons, [tNAA]/[Ins] was significantly reduced in SCA1 at visit #2 vs. visit #1 while no difference was detected in controls. The change in ataxia rating scale between the two visits did not reach significance suggesting that MRS is more sensitive to detect a small change due to disease progression than clinical assessment.

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