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; F. M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, Maryland, USA.
Mov Disord. 2014 Mar;29(3):396-401. doi: 10.1002/mds.25591. Epub 2013 Jul 11.
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.
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.
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.
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.
神经血管改变与亨廷顿病（HD）的病理生理学有关。由于小动脉对代谢改变最敏感，因此小动脉脑血容量（CBVa）是脑血管调节的重要指标。本初步研究的目的是研究前驱期 HD 患者潜在的神经血管（CBVa）异常，并将其与广泛使用的成像标志物：脑萎缩进行比较。
使用超高场（7.0-Telsa）磁共振成像在 7 名前驱期 HD 患者和 9 名年龄匹配的对照者中测量 CBVa 和脑容量。
与对照组相比，前驱期 HD 患者的皮质 CBVa 明显升高（相对差异，38.5％；效应大小，1.48）。在前额皮质的 CBVa 与遗传测量之间发现了显著的相关性。相比之下，前驱期 HD 患者没有发现明显的脑萎缩。
即使在发生大量脑萎缩之前，前驱期 HD 患者的 CBVa 也可能异常。值得进一步研究更大的队列和纵向随访，以确定 CBVa 是否可用作临床试验的潜在生物标志物。
