Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

Authors

  • Jun Hua PhD,

    Corresponding author
    1. The Russell H. Morgan Department of Radiology and Radiological Science, Division of Magnetic Resonance Research, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    2. F. M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, Maryland, USA
    • Correspondence to: Dr. Jun Hua, Department of Radiology, Johns Hopkins University School of Medicine, F. M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, 707 N. Broadway, Baltimore, MD 21205; jhua@mri.jhu.edu

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    • Drs. Hua and Unschuld contributed equally to this work.

  • Paul G. Unschuld MD,

    1. Division of Psychiatry Research and Psychogeriatric Medicine, University of Zurich, Zurich, Switzerland
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    • Drs. Hua and Unschuld contributed equally to this work.

  • Russell L. Margolis MD,

    1. Department of Psychiatry, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    2. Department of Neurology and Program in Cellular and Molecular Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
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  • Peter C. M. van Zijl PhD,

    1. The Russell H. Morgan Department of Radiology and Radiological Science, Division of Magnetic Resonance Research, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    2. F. M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, Maryland, USA
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  • Christopher A. Ross MD, PhD

    1. Department of Psychiatry, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    2. Department of Neurology and Program in Cellular and Molecular Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
    3. Departments of Neuroscience and Pharmacology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
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  • Relevant conflicts of interest/financial disclosures: Dr. van Zijl has a patent on VASO technology licensed to Philips Healthcare.

  • Full financial disclosures and author roles may be found in the online version of this article.

ABSTRACT

Background

Neurovascular alterations have been implicated in the pathophysiology of Huntington's disease (HD). Because arterioles are most responsive to metabolic alterations, arteriolar cerebral blood volume (CBVa) is an important indicator of cerebrovascular regulation. The objective of this pilot study was to investigate potential neurovascular (CBVa) abnormality in prodromal-HD patients and compare it with the widely used imaging marker: brain atrophy.

Methods

CBVa and brain volumes were measured with ultra-high-field (7.0-Telsa) magnetic resonance imaging in seven prodromal-HD patients and nine age-matched controls.

Results

Cortical CBVa was elevated significantly in prodromal-HD patients compared with controls (relative difference, 38.5%; effect size, 1.48). Significant correlations were found between CBVa in the frontal cortex and genetic measures. By contrast, no significant brain atrophy was detected in the prodromal-HD patients.

Conclusions

CBVa may be abnormal in prodromal-HD, even before substantial brain atrophy occurs. Further investigation with a larger cohort and longitudinal follow-up is merited to determine whether CBVa could be used as a potential biomarker for clinical trials. © 2013 Movement Disorder Society

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