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Executive dysfunction is a predominant cognitive symptom in cerebral small vessel disease (SVD). The Institute of Cognitive Neurology Frontal Screening (IFS) is a well-validated screening tool allowing the rapid assessment of multiple components of executive function in Spanish-speaking individuals. In this study, we examined performance on the IFS in subjects with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), an inherited condition leading to the early onset of SVD. We further explored associations between performance on the IFS and magnetic resonance imaging (MRI) markers of SVD.
Methods:
We recruited 24 asymptomatic CADASIL subjects and 23 noncarriers from Colombia. All subjects underwent a research MRI and a neuropsychological evaluation, including the IFS. Structural MRI markers of SVD were quantified in each subject, together with an SVD Sum Score representing the overall burden of cerebrovascular alterations. General linear model, correlation, and receiver operating characteristic curve analyses were used to explore group differences on the IFS and relationships with MRI markers of SVD.
Results:
CADASIL subjects had a significantly reduced performance on the IFS Total Score. Performance on the IFS correlated with all quantified markers of SVD, except for brain atrophy and perivascular spaces enlargement. Finally, while the IFS Total Score was not able to accurately discriminate between carriers and noncarriers, it showed adequate sensitivity and specificity in detecting the presence of multiple MRI markers of SVD.
Conclusions:
These results suggest that the IFS may be a useful screening tool to assess executive function and disease severity in the context of SVD.
This chapter describes the vascular pathologies that underlie cerebral microbleeds (CMBs), concentrating on the two commonest disorders: hypertensive small vessel disease (SVD) and cerebral amyloid angiopathy (CAA). It also describes the process of blood degradation, and the correlation of imaging with histological findings. The chapter concentrates on hypertensive arteriopathy and CAA, which is usually diagnosed following symptomatic lobar intracerebral hemorrhage (ICH). Hypertensive arteriopathy is thought to be caused by a forced dilation of resistance vessels: that is, those vessels that regulate the blood supply volume to the distal capillary bed. The first event in a hemorrhage is the extravasation of all constituents of blood along with plasma. Extravasation may occur by rhexis (rupture of a vessel wall) or by diapedesis affecting arterioles, veins or capillaries. From the perspective of neuroimaging, CMBs are focal deposits of hemosiderin and can be visualized with MRI.
Cerebral microbleeds (CMB) are considered a marker of an underlying hemorrhage-prone small vessel vasculopathy. The brain changes that accompany CMB may be a direct consequence of the CMB, of an underlying vasculopathy, or of risk factors associated with CMB or the underlying vasculopathies that cause CMB. This chapter reviews data associating CMBs with other neuroimaging findings. The relationships between CMBs and brain infarcts, hemorrhage and brain atrophy is discussed in general and in the context of specific small vessel diseases. CMBs are more frequent in patients with neuroimaging findings of white matter lesions (WML) and lacunar infarctions. By contrast, CMBs are not independently associated with brain atrophy, probably because the actual tissue damage directly caused by CMBs is minimal. These observations are consistent with the concept that CMBs are one of several neuroimaging markers of small vessel arterial disease.
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