Cerebral Hemodynamics and White Matter Hyperintensities in CADASIL

Boom, Rivka van den; Oberstein, Saskia A J Lesnik; Spilt, Aart; Behloul, F.; Ferrari, Michel D.; Haan, Joost; Westendorp, Rudi G.J.; Buchem, Mark A. van

doi:10.1097/01.wcb.0000062341.61367.d3

Cited by 67 publications

(50 citation statements)

References 27 publications

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“…Participants were drawn from the Dutch cross-sectional CADASIL study, performed in 1999 and 2000, 7 which included 40 symptomatic and asymptomatic NOTCH3 mutation carriers (MCs) and 22 nonmutation carriers (nonMCs) from 15 unrelated families. All living participants were invited for a follow-up visit.…”

Section: Patientsmentioning

confidence: 99%

“…7,8 It has been suggested that these changes precede the development of WMHs and that flow changes are, thus, a key underlying factor in the pathophysiology of CADASIL. 7 However, to our knowledge, no longitudinal studies have been performed to confirm this concept.…”

mentioning

confidence: 99%

“…To measure TCBF and CVR, we used a gradient-echo phase-contrast technique before and after administration of intravenous acetazolamide. 7,12 Two localizer MR angiography scans (gradient-echo phase-contrast sequence: TR, 17.5 ms; TE, 6.7 ms; flip angle, 7.5°; section thickness, 60 mm; matrix, 256 ϫ 128; FOV, 250 mm) were placed in the region of the cervical and intracranial parts of the basilar and carotid arteries in the coronal plane and in the sagittal plane. These scans were also used to rule out concomitant large-vessel disease.…”

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confidence: 99%

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Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL

Liem¹,

Oberstein²,

Haan³

et al. 2009

AJNR Am J Neuroradiol

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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.

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Section: Patientsmentioning

confidence: 99%

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confidence: 99%

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confidence: 99%

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Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL

Liem¹,

Oberstein²,

Haan³

et al. 2009

AJNR Am J Neuroradiol

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“…Also, patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) have significantly lower baseline CBF than controls. 9 However, because these studies are cross-sectional, it could also be hypothesized that WMHs and lacunes lead to downregulation of CBF because of reduced metabolic demand, which is a normal function of blood vessels. 10 We aimed to investigate the bidirectional longitudinal relationship between WMHs and lacunes and CBF during 4 years of follow-up in patients with manifest arterial disease.…”

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confidence: 99%

Longitudinal Relationship Between Cerebral Small-Vessel Disease and Cerebral Blood Flow

Veen

Muller

Vincken

et al. 2015

Stroke

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Background and Purpose-Cerebral small-vessel disease and cerebral blood flow (CBF) are interrelated. However, the direction of the relationship is unknown, and longitudinal studies are scarce. We investigated the longitudinal relationship between CBF and white matter hyperintensities (WMHs) and lacunes, as representatives of cerebral small-vessel disease, in patients with manifest arterial disease. Methods-Within the Second Manifestations of Arterial Disease-Magnetic Resonance (SMART-MR) study, 1.5T brain magnetic resonance imaging, including an MR angiography, was obtained at baseline and after on ≈3.9 years of follow-up in 575 patients with manifest arterial disease (mean age, 57±10 years). Longitudinal associations of WMHs and lacunes with parenchymal CBF (pCBF; per 100-mL brain volume) were estimated using regression analyses, adjusted for age, sex, follow-up time, and baseline brain measures. Results-Baseline pCBF was not associated with progression of WMHs and lacunes over time. However, periventricular and deep WMHs at baseline were associated with decline in pCBF; mean (95% confidence interval) decline in pCBF per % intracranial volume increase in periventricular and deep WMH volume was −0.70 (−1.40 to −0.00) and −1.01 (−1.64 to −0.38) mL/min per 100-mL brain volume, respectively. These associations were partly explained by cardiovascular risk factors but remained significant for deep WMHs (mean decline [95% confidence interval] in pCBF per % intracranial volume increase in deep WMH volume was −0.92 [−1.56 to −0.28] mL/min per 100-mL brain volume). Lacunes were not associated with change in pCBF. Conclusions-In patients with manifest arterial disease, baseline periventricular and deep WMH volumes were associated with decline in pCBF over time, but baseline pCBF was not associated with progression of WMHs and lacunes over time. Of the 1309 patients included, 1152 patients had adequate brain segmentation and 2-dimensional (2D) MR angiography measurements. Of these 1152 patients, 1076 were still alive at follow-up and 674 participated. Of these, 575 patients had an MRI at baseline and follow-up, and adequate brain segmentation and 2D MR angiography measurements.The SMART-MR study was approved by the ethics committee of our institution and written informed consent was obtained from all patients. MRI Protocol and Brain SegmentationAt baseline and follow-up, MR investigations were performed on a 1.5T whole-body system (Gyroscan ACS-NT; Philips Medical Systems, Best, the Netherlands). The protocol consisted of transversal T1-weighted (repetition time [TR]/echo time [TE], 235/2 ms), T2-weighted (TR/TE, 2200/11 and 2200/100 ms), fast fluid-attenuated inversion recovery (TR/TE/inversion time, 6000/100/2000 ms), and inversion recovery (TR/TE/inversion time, 2900/22/410 ms) sequences. Field of view was 230×230 mm (matrix size, 180×256; slice thickness, 4.0 mm; no gap; 38 slices). 11On the basis of a localizer MR angiographic slab in the sagittal plane, we positioned a 2D phase-contrast section at the level of the ...

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“…7,8 It has been hypothesized that the structural microvessel changes lead to impaired vasoreactivity with consequent reduced cerebral perfusion and tissue damage. Cerebral hypoperfusion has been documented in CADASIL using blood flow imaging studies, 9,10 and, although not univocally, vasoreactivity changes have been reported by transcranial Doppler sonography studies. 11,12 Altered skin microvessel reactivity 13 and endothelial-dependent vasodilatation in cerebral and forearm arteries studies have been observed.…”

mentioning

confidence: 99%

Bone Marrow-Derived Progenitor Cells in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

Pescini

Cesari

Giusti

et al. 2010

Stroke

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Background and Purpose— Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited disease due to cerebral microangiopathy presenting with variable pictures, including stroke, progressive cognitive impairment, and disability. Mechanisms leading from vessel structural changes to parenchymal damage and eventually to clinical expression are not fully understood. Among pathogenic processes, endothelial dysfunction has been hypothesized. Endothelial progenitor cells and circulating progenitor cells (CPCs) derived from bone marrow participate in endothelium structure and function maintenance and contribute to ischemic area revascularization. No data are available about these cells in CADASIL. Our objective in this study was to evaluate endothelial progenitor cells and CPCs role in CADASIL. Methods— Twenty-nine patients with CADASIL and 29 sex- and age-matched control subjects were enrolled. Cells were measured in peripheral blood using flow cytometry. Endothelial progenitor cells were defined as positive for CD34/KDR, CD133/KDR, and CD34/CD133/KDR; and CPCs as positive for CD34, CD133, and CD34/CD133. Results— Endothelial progenitor cells were significantly lower in patients with CADASIL than in control subjects (CD34/KDR: 0.05 versus 0.1 cells/μL, P =0.005; CD133/KDR: 0.07 versus 0.1 cells/μL, P =0.006; CD34/CD133/KDR: 0.05 versus 0.1 cells/μL, P =0.001). The difference remained significant after adjusting for age, sex, and statin use. CPCs were not significantly lower in CADASIL, but patients with stroke or dementia had significantly reduced CPC levels than patients without (CD34: 1.68 versus 2.95 cells/μL, P =0.007; CD133: 1.40 versus 2.82 cells/μL, P =0.004; CD34/CD133: 1.44 versus 2.75 cells/μL, P =0.004). CPC levels significantly correlated with cognitive and motor performance measures. Conclusions— We have documented an association between endothelial progenitor cells and CPCs and CADASIL, extending previous data about the presence of endothelial dysfunction in this disease and its potential role in modulating phenotype.

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Cerebral Hemodynamics and White Matter Hyperintensities in CADASIL

Cited by 67 publications

References 27 publications

Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL

Cerebrovascular Reactivity Is a Main Determinant of White Matter Hyperintensity Progression in CADASIL

Longitudinal Relationship Between Cerebral Small-Vessel Disease and Cerebral Blood Flow

Bone Marrow-Derived Progenitor Cells in Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy

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