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Updated: Jun 25, 2026
Evaluation of the Cognitive Performance of Hypertensive Patients with Silent Cerebrovascular Lesions
Published on: April 23, 2021
Cerebrovascular reactivity is a main determinant of white matter hyperintensity progression in CADASIL
M K Liem1, S A J Lesnik Oberstein, J Haan
1Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands. m.k.liem@lumc.nl
Insights
Decreased cerebrovascular reactivity (CVR) predicts white matter hyperintensities progression in CADASIL patients. Total cerebral blood flow (TCBF) showed no association with disease progression over seven years.
Area of Science:
- Neurology
- Vascular Biology
- Medical Imaging
Background:
- Small-vessel diseases, like Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), are linked to cerebral blood flow and reactivity.
- CADASIL, a monogenetic disorder, provides a model for studying arterial small-vessel disease pathophysiology.
Purpose of the Study:
- To investigate the relationship between basal total cerebral blood flow (TCBF) and cerebrovascular reactivity (CVR) and the progression of MR imaging abnormalities in CADASIL.
- To determine if TCBF and CVR are predictors of disease advancement in individuals with CADASIL.
Main Methods:
- Basal TCBF was measured in 25 NOTCH3 mutation carriers and 13 controls.
- Cerebrovascular reactivity (CVR) was assessed post-acetazolamide administration in 14 NOTCH3 mutation carriers and 9 controls.
- Magnetic resonance imaging (MRI) was used to quantify changes in white matter hyperintensities (WMHs), lacunar infarcts, and microbleeds over a 7-year period.
Main Results:
- Lower baseline CVR was significantly associated with a greater increase in white matter hyperintensities (WMHs) over seven years (P = .001).
- Baseline TCBF did not correlate with the progression of MR imaging abnormalities, including WMHs, lacunar infarcts, or microbleeds.
- No association was found between baseline CVR and the increase in lacunar infarcts or microbleeds.
Conclusions:
- Reduced cerebrovascular reactivity (CVR) emerges as a potential predictor for the progression of white matter hyperintensities in CADASIL.
- TCBF is not identified as a predictor for disease progression in this CADASIL cohort.
- These findings highlight the importance of CVR in understanding the pathophysiology and progression of white matter changes in CADASIL.
Background And Purpose:
Basal total cerebral blood flow (TCBF) and cerebrovascular reactivity (CVR) are assumed to play an important role in the pathophysiology of small-vessel disease. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a unique monogenetic model to study the pathophysiology of arterial small-vessel disease. The aim of this study was to investigate the role of TCBF and CVR in the progression of MR imaging abnormalities in CADASIL.
Materials And Methods:
Basal TCBF was measured in 25 NOTCH3 mutation carriers and 13 control subjects at baseline. CVR after administration of acetazolamide was measured in 14 NOTCH3 mutation carriers and 9 control subjects. Increase in white matter hyperintensities (WMHs), lacunar infarcts, and microbleeds on MR imaging was measured 7 years later.
Results:
Lower CVR at baseline was associated with larger increase of WMHs (P = .001) but not with a larger increase of lacunar infarcts or microbleeds. TCBF at baseline was not associated with an increase of MR imaging abnormalities.
Conclusions:
Decreased CVR is a potential predictor of disease progression as indicated by increasing WMHs in CADASIL.
