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Abstract #0976

Impaired oxygen metabolism in the brain during visual stimulation in premanifest Huntington's Disease patients detected by 3D-TRIP MRI at 7T

Peter Klinkmueller1,2,3, Martin Kronenbuerger4,5, Xinyuan Miao2,3, Russell L. Margolis5, Peter C. M. van Zijl2,3, Christopher A. Ross4,5,6, and Jun Hua2,3

1Department of Electrical and Computer Engineering, Johns Hopkins University, Baltimore, MD, United States, 2F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, United States, 3Division of MRI Research, Department of Radiology, Johns Hopkins University School of Medicine, Baltimore, MD, United States, 4Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, United States, 5Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, MD, United States, 6Department of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, MD, United States

Huntington’s disease (HD) is a neurodegenerative disease caused by a single genetic mutation. Neurovascular abnormalities have been implicated in the pathophysiology of HD. Here, dynamic responses in BOLD, cerebral-blood-flow (CBF) and -volume (CBV) during visual stimulation were measured using 3D-TRiple-acquisition-after-Inversion-Preparation (3D-TRIP) MRI in premanifest HD patients and healthy controls, from which cerebral-metabolic-rate-of-oxygen (CMRO2) response was estimated. Decreased ΔCMRO2 and increased ΔCBV were observed in HD patients compared to controls, which correlated with genetic measures. The results suggested potential value of ΔCMRO2 as a biomarker for HD, and may shed light on the pathophysiology in HD in terms of mitochondrial deficiency.

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