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Coronary microvascular function is impaired in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

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Title: Coronary microvascular function is impaired in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
Authors: Argirò, Alessia (AUTHOR), Sciagrà, Roberto (AUTHOR), Marchi, Alberto (AUTHOR), Beltrami, Matteo (AUTHOR), Spinelli, Enrico (AUTHOR), Salvadori, Emilia (AUTHOR), Bianchi, Andrea (AUTHOR), Mascalchi, Mario (AUTHOR), Poggesi, Anna (AUTHOR), Olivotto, Iacopo (AUTHOR), Pescini, Francesca (AUTHOR)
Source: European Journal of Neurology. Nov2021, Vol. 28 Issue 11, p3809-3813. 5p.
Subjects: Magnetic resonance imaging, Cerebral infarction, Leukoencephalopathies, Positron emission tomography, Watchful waiting, Arterial diseases
Abstract: Background and purpose: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare inherited disease caused by NOTCH3 gene mutations. Although the main clinical features reflect brain injury, CADASIL is a systemic microangiopathy, and cardiac involvement has been observed but not systematically assessed. We aimed to study the prevalence and severity of coronary microvascular dysfunction (CMD) in CADASIL patients. Methods: Seventeen patients with genetically confirmed CADASIL, aged <60 years (mean age 40 ± 9 years), with ≤1 cardiovascular risk factor underwent neurological and neuropsychological evaluation, 3T brain magnetic resonance imaging (MRI), 12‐lead electrocardiography (ECG), standard echocardiography, and measurement of myocardial blood flow at rest (resting MBF) and of maximal myocardial blood flow following Regadenoson infusion (Reg‐MBF) by 13NH3 positron emission tomography (PET). Coronary flow reserve (CFR) was defined as Reg‐MBF/resting MBF. PET results were compared to those of 15 healthy controls who were age and sex matched. Results: Twelve patients (71%) presented migraine, none (53%) had psychiatric disturbances, and one (6%) had a previous stroke. None had cognitive impairment or ECG or echocardiography abnormalities. Both Reg‐MBF and CFR were blunted in CADASIL patients compared with controls (Reg‐MBF 2.46 ± 0.54 vs. 3.09 ± 0.44 ml/g/min, respectively; p < 0.01; CFR 2.74 ± 0.36 vs. 3.28 ± 0.66, respectively, p < 0.01). No correlations were found between Reg‐MBF values and neuropsychological performance or cerebral lesion burden on MRI. Conclusions: CADASIL patients exhibit blunted CFR due to CMD, which can be severe and is independent of the severity of brain lesion load and cognitive performances. CADASIL is a systemic microcirculation disease, and active surveillance of cardiac symptoms should be considered in these patients. [ABSTRACT FROM AUTHOR]
Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)
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  Label: Title
  Group: Ti
  Data: Coronary microvascular function is impaired in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
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  Data: &lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Argir&#242;%2C+Alessia%22&quot;&gt;Argir&#242;, Alessia&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Sciagr&#224;%2C+Roberto%22&quot;&gt;Sciagr&#224;, Roberto&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Marchi%2C+Alberto%22&quot;&gt;Marchi, Alberto&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Beltrami%2C+Matteo%22&quot;&gt;Beltrami, Matteo&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Spinelli%2C+Enrico%22&quot;&gt;Spinelli, Enrico&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Salvadori%2C+Emilia%22&quot;&gt;Salvadori, Emilia&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Bianchi%2C+Andrea%22&quot;&gt;Bianchi, Andrea&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Mascalchi%2C+Mario%22&quot;&gt;Mascalchi, Mario&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Poggesi%2C+Anna%22&quot;&gt;Poggesi, Anna&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Olivotto%2C+Iacopo%22&quot;&gt;Olivotto, Iacopo&lt;/searchLink&gt; (AUTHOR)&lt;br /&gt;&lt;searchLink fieldCode=&quot;AR&quot; term=&quot;%22Pescini%2C+Francesca%22&quot;&gt;Pescini, Francesca&lt;/searchLink&gt; (AUTHOR)
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  Data: &lt;searchLink fieldCode=&quot;JN&quot; term=&quot;%22European+Journal+of+Neurology%22&quot;&gt;European Journal of Neurology&lt;/searchLink&gt;. Nov2021, Vol. 28 Issue 11, p3809-3813. 5p.
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  Group: Su
  Data: &lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Magnetic+resonance+imaging%22&quot;&gt;Magnetic resonance imaging&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Cerebral+infarction%22&quot;&gt;Cerebral infarction&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Leukoencephalopathies%22&quot;&gt;Leukoencephalopathies&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Positron+emission+tomography%22&quot;&gt;Positron emission tomography&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Watchful+waiting%22&quot;&gt;Watchful waiting&lt;/searchLink&gt;&lt;br /&gt;&lt;searchLink fieldCode=&quot;DE&quot; term=&quot;%22Arterial+diseases%22&quot;&gt;Arterial diseases&lt;/searchLink&gt;
– Name: Abstract
  Label: Abstract
  Group: Ab
  Data: Background and purpose: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare inherited disease caused by NOTCH3 gene mutations. Although the main clinical features reflect brain injury, CADASIL is a systemic microangiopathy, and cardiac involvement has been observed but not systematically assessed. We aimed to study the prevalence and severity of coronary microvascular dysfunction (CMD) in CADASIL patients. Methods: Seventeen patients with genetically confirmed CADASIL, aged &lt;60 years (mean age 40 &#177; 9 years), with ≤1 cardiovascular risk factor underwent neurological and neuropsychological evaluation, 3T brain magnetic resonance imaging (MRI), 12‐lead electrocardiography (ECG), standard echocardiography, and measurement of myocardial blood flow at rest (resting MBF) and of maximal myocardial blood flow following Regadenoson infusion (Reg‐MBF) by 13NH3 positron emission tomography (PET). Coronary flow reserve (CFR) was defined as Reg‐MBF/resting MBF. PET results were compared to those of 15 healthy controls who were age and sex matched. Results: Twelve patients (71%) presented migraine, none (53%) had psychiatric disturbances, and one (6%) had a previous stroke. None had cognitive impairment or ECG or echocardiography abnormalities. Both Reg‐MBF and CFR were blunted in CADASIL patients compared with controls (Reg‐MBF 2.46 &#177; 0.54 vs. 3.09 &#177; 0.44 ml/g/min, respectively; p &lt; 0.01; CFR 2.74 &#177; 0.36 vs. 3.28 &#177; 0.66, respectively, p &lt; 0.01). No correlations were found between Reg‐MBF values and neuropsychological performance or cerebral lesion burden on MRI. Conclusions: CADASIL patients exhibit blunted CFR due to CMD, which can be severe and is independent of the severity of brain lesion load and cognitive performances. CADASIL is a systemic microcirculation disease, and active surveillance of cardiac symptoms should be considered in these patients. [ABSTRACT FROM AUTHOR]
– Name: AbstractSuppliedCopyright
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  Data: &lt;i&gt;Copyright of European Journal of Neurology is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder&#39;s express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.&lt;/i&gt; (Copyright applies to all Abstracts.)
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        Value: 10.1111/ene.14678
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        Text: English
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      – SubjectFull: Magnetic resonance imaging
        Type: general
      – SubjectFull: Cerebral infarction
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      – SubjectFull: Leukoencephalopathies
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      – TitleFull: Coronary microvascular function is impaired in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.
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              Text: Nov2021
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