Elevated arteriolar cerebral blood volume in prodromal Huntington's disease

Mov Disord. 2014 Mar;29(3):396-401. doi: 10.1002/mds.25591. Epub 2013 Jul 11.


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.

Keywords: atrophy; biomarker; imaging; neurodegeneration; neurovascular.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Atrophy / pathology
  • Biomarkers / blood
  • Blood Volume / physiology*
  • Female
  • Humans
  • Huntington Disease / blood*
  • Huntington Disease / pathology*
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Pilot Projects


  • Biomarkers