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Abstract Details

Structural Magnetic Resonance Imaging Correlates of Neurodegeneration in a Spinocerebellar Ataxia Type 7 Cohort
Movement Disorders
P6 - Poster Session 6 (11:30 AM-1:00 PM)
15-024

Precisely localize and quantify brain atrophy occurring during disease progression in spinocerebellar ataxia type 7 (SCA7) patients using structural magnetic resonance imaging (MRI) metrics.

SCA7 is a neurodegenerative disease characterized by progressive cerebellar ataxia, cone-rod dystrophy, ophthalmoplegia, and spasticity. Postmortem analysis and standard MRI reveal a classic pattern of olivopontocerebellar atrophy in SCA7 patients, but our understanding of the evolution of this pathology is limited. Methods such as voxel-based morphometry (VBM) and diffusion tensor imaging (DTI) can be used to quantify these neurodegenerative changes in vivo with high spatial specificity.

T1w, T2w, and diffusion weighted images were acquired from a cohort of 14 SCA7 patients (10 female) and 14 healthy controls (9 female). In patients, ataxia was quantified using the scale for the assessment and rating of ataxia (SARA). Motor dysfunction was measured with the 9-hole pegboard task, a keyboard dexterity task, and the Timed Up and Go test. Spasticity was quantified using the vibration inhibition index, a measure of H-reflex suppression. VBM was used to quantify differences in cortical and cerebellar gray matter (GM) volume between patients and controls. DTI tractography was used to calculate the fractional anisotropy in the corticospinal (CST), corticoreticulospinal (CRST), dentatothalamocortical (DTCT), and corticopontocerebellar (CPCT) tracts.

No differences in cortical GM volume were found. Bonferroni-corrected two-sample t-tests revealed a significant decrease in the GM volume of 21 out of 28 cerebellar ROIs in SCA7 patients. The FA of all tracts measured (CST, CRST, DTCT, and CPCT) were found to be lower in patients through Bonferroni-corrected two-sample t-tests.

Degeneration of the cerebellar cortex and tracts such as the CST, DTCT, CPCT, and CRST may be possible biomarkers of SCA7 progression. A longitudinal analysis is planned to confirm biomarkers of disease progression and correlate the degeneration of specific areas with the clinical measures.
Authors/Disclosures
Jacob Parker
PRESENTER 		No disclosure on file
No disclosure on file
Sanaz Attaripour, MD, FAAN (University of California, Irvine) Dr. Attaripour has nothing to disclose.
Shabbir Hussain I. Merchant, MD (BIDMC) Dr. Merchant has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Medtronic . Dr. Merchant has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Stanford Health. The institution of Dr. Merchant has received research support from Biogen. The institution of Dr. Merchant has received research support from Cerevance.
No disclosure on file
Mark Hallett, MD, FAAN (National Institutes of Health) Dr. Hallett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Hallett has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurocrine. Dr. Hallett has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Brainsway. Dr. Hallett has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for VoxNeuro. Dr. Hallett has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for QuantalX. Dr. Hallett has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. Dr. Hallett has received intellectual property interests from a discovery or technology relating to health care. Dr. Hallett has received publishing royalties from a publication relating to health care. Dr. Hallett has received publishing royalties from a publication relating to health care. Dr. Hallett has received publishing royalties from a publication relating to health care. Dr. Hallett has received publishing royalties from a publication relating to health care. Dr. Hallett has received personal compensation in the range of $500-$4,999 for serving as a Speaker with International Parkinson and Movement Disorder Society. Dr. Hallett has a non-compensated relationship as a Past-President with Functional Neurological Disorder Society that is relevant to AAN interests or activities.