Elevated Arteriolar Cerebral Blood Volume in Prodromal Huntington's Disease

BackgroundNeurovascular 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. MethodsCBV(a) 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. ResultsCortical 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. ConclusionsCBV(a) 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. (c) 2013 Movement Disorder Society