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Abstract #0541

Longitudinal Assessment of Neurodegeneration in a Spinocerebellar Ataxia Type 1 (SCA1) Mouse Model by 1H MRS at 9.4 Tesla: Correlation with Histopathology

Gulin Oz1, H. Brent Clark1, Christopher D. Nelson1, Dee M. Koski1, Pierre-Gilles Henry1, Malgorzata Marjanska1, Dinesh K. Deelchand1, Harry T. Orr1

1University of Minnesota, Minneapolis, MN, USA

To assess the sensitivity of high field 1H MRS to disease onset and progression in neurodegenerative diseases, we measured cerebellar neurochemical profiles of a mouse model of spinocerebellar ataxia 1 (SCA1) and controls at 3 different ages at 9.4T using short-echo LASER. Multiple biomarkers clearly distinguished the mutant animals from controls at all ages. Multiple MRS measures were significantly different in the mutant animals already at 6 weeks, prior to significant pathological and neurological changes. These data demonstrate the ability of MRS to non-invasively detect pre-clinical disease, as well as its sensitivity to disease progression.