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

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.