Vasoreactivity in CADASIL: Comparison to structural MRI and neuropsychology.
Vasoreactivity in CADASIL: Comparison to structural MRI and neuropsychology.
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DOI：
10.1177/0271678x17710375
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发表时间：
2018-06
期刊：
影响因子：
--
通讯作者：
Muir KW
中科院分区：
文献类型：
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作者：
Moreton FC;Cullen B;Delles C;Santosh C;Gonzalez RL;Dani K;Muir KW
在动物模型中，脑血管反应性受损先于CADASIL的组织学和临床证据。我们的目标是更全面地描述一组成年CADASIL患者的外周和脑血管功能和反应性，并探索这些与常规临床、影像和神经心理学指标的相关性。共有22名成年CADASIL患者知情同意参加CADASIL血管功能的探索性研究。进行临床评估、血管综合评估、MRI和神经心理测试。我们用经颅多普勒和动脉自旋标记MRI测量了高碳酸血症时的脑血管反应性。在MRI上评估腔隙的数量和体积、皮质下高信号体积、微出血和正常化的脑体积。分析是探索性的，并检查了不同标记之间的关联。ASL测定的脑血管反应性与血流介导的扩张法测定的外周血管反应性相关。≥5基因陷窝的受试者年龄较大，颈动脉内膜中层厚度较高，脑血管和外周血管反应性受损。有抑郁症状、残疾或加工速度延迟的受试者也表现出血管反应性受损的趋势。血管反应性受损和血管功能障碍可能在CADASIL的病理生理学中起重要作用，在纵向和临床试验中，血管评估可能是有用的严重程度的生物标志物。
Impaired cerebrovascular reactivity precedes histological and clinical evidence of CADASIL in animal models. We aimed to more fully characterise peripheral and cerebral vascular function and reactivity in a cohort of adult CADASIL patients, and explore the associations of these with conventional clinical, imaging and neuropsychological measures. A total of 22 adults with CADASIL gave informed consent to participate in an exploratory study of vascular function in CADASIL. Clinical assessment, comprehensive vascular assessment, MRI and neuropsychological testing were conducted. We measured cerebral vasoreactivity with transcranial Doppler and arterial spin labelling MRI with hypercapnia challenge. Number and volume of lacunes, subcortical hyperintensity volume, microbleeds and normalised brain volume were assessed on MRI. Analysis was exploratory and examined the associations between different markers. Cerebrovascular reactivity measured by ASL correlated with peripheral vasoreactivity measured by flow mediated dilatation. Subjects with ≥5 lacunes were older, with higher carotid intima-media thickness and had impaired cerebral and peripheral vasoreactivity. Subjects with depressive symptoms, disability or delayed processing speed also showed a trend to impaired vasoreactivity. Impaired vasoreactivity and vascular dysfunction may play a significant role in the pathophysiology of CADASIL, and vascular assessments may be useful biomarkers of severity in both longitudinal and clinical trials.