Abstract Details

Title Elevated Arteriolar Cerebral Blood Volume in Prodromal Huntington's Disease Patients Measured with Ultra-High Field Magnetic Resonance Imaging

Topic Movement Disorders

Presentation(s) S20 - (-)

Poster/Presentation
Number 005

Objective

Background Vascular abnormalities have been identified in many neurodegenerative disorders. Recent studies suggest that neurovascular and metabolic impairment may be involved in the pathophysiology of HD, and may be early features of the disease. As the driving source to supply and regulate tissue perfusion, arterial vessels are most responsive to changes in metabolism. Therefore, CBVa may be employed as an important indicator of cerebrovascular regulation and brain function.

Design/Methods CBVa in cortical grey matter was measured with the recently developed iVASO MRI technique in 7 prodromal HD patients and 9 age-matched controls on a 7T MRI scanner. Anatomical images were acquired with MPRAGE scans for regional brain volume measurement.

Results Cortical grey matter CBVa was significantly elevated in prodromal HD patients compared to age-matched controls, with a relative change of 38.5% and effect size of 1.48. This effect was the most significant (P=0.008) in the frontal cortex, with a relative change of 48.9% and effect size of 2.11. Furthermore, significant correlations were found between grey matter CBVa in the frontal cortex and genetic measures for disease status including the CAG-Age product (CAP) score and estimated years to onset of motor symptoms. By contrast, volumes of brain regions in these prodromal HD patients did not differ significantly from the controls, and did not correlate with cortical grey matter CBVa values.

Conclusions These results implicate the potential value of CBVa as a quantitative biomarker prior to prominent brain atrophy in the prodromal period of HD.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
	No disclosure on file
Howard Feldman, MD, FAAN (University of California San Diego)	The institution of Dr. Feldman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Novo Nordisk. The institution of Dr. Feldman has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen Research & Development LLC. The institution of Dr. Feldman has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Tau Consortium. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from Vivoryon Therapeutics. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from US National Institute of Health NIH/NIA. The institution of Dr. Feldman has received research support from Allyx Pharmaceuticals. The institution of Dr. Feldman has received research support from Canadian Institutes of Health Research . Dr. Feldman has received intellectual property interests from a discovery or technology relating to health care. Dr. Feldman has a non-compensated relationship as a Medical Advisory Council with Association for Frontotemporal Degeneration (AFTD) that is relevant to AAN interests or activities. Dr. Feldman has a non-compensated relationship as a International Scientific Advisory Board with Translating Research in Elder Care (TREC) that is relevant to AAN interests or activities. Dr. Feldman has a non-compensated relationship as a Conference, Presenter with Royal Society of Canada that is relevant to AAN interests or activities. Dr. Feldman has a non-compensated relationship as a Conference, Presenter with Banner Health that is relevant to AAN interests or activities. Dr. Feldman has a non-compensated relationship as a Conference, Presenter, Manuscript Author with University of British Columbia that is relevant to AAN interests or activities.
	No disclosure on file
Christopher Ross, MD, PhD (JHU)	Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for uniqure. Christopher Ross, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Spark. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Mitoconix. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sage. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Annexon. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuCell. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuCell. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuBase. Christopher Ross, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Huntington Study Group. Christopher Ross, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Spark. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for uniQure. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Mitoconix. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sage. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Annexon. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NeuCell. Christopher Ross, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for NeuBase. Christopher Ross, MD, PhD has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Christopher Ross, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Huntington Study Group. Christopher Ross, MD, PhD has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Mintz. Christopher Ross, MD, PhD has received intellectual property interests from a discovery or technology relating to health care. Christopher Ross, MD, PhD has a non-compensated relationship as a consultant with Huntington Study Group that is relevant to AAN interests or activities.

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