Abstract

BackgroundCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a subcortical, inherited, cerebral small vessel disease. Several studies have revealed the involvement of specific cortical regions. However, the structural brain alterations and their clinical correlations remain largely undetermined. MethodsWe evaluated 22 CADASIL patients and 22 age- and sex-matched healthy controls. We used surface- and voxel-based morphometric data derived from 3.0-T magnetic resonance imaging (MRI) to explore structural changes in gray and white matter. We calculated Pearson correlations between such data and clinical and MRI metrics. ResultsCompared with controls, CADASIL patients exhibited significantly decreased cortical thickness in the left supramarginal gyrus, superior temporal gyrus, transverse temporal gyrus, insula, lateral orbitofrontal gyrus, isthmus cingulate gyrus and precentral gyrus. An extensive decrease in the white (but not gray) matter volume was also evident, predominantly in the frontal, parietal, temporal, and occipital lobes. The number of previous strokes or transient ischemic attacks was negatively associated with the cortical thickness of the left pars opercularis and right posterior cingulate gyrus. ConclusionReductions in cortical thickness and white matter volume were evident in CADASIL patients compared with controls, and higher numbers of strokes and transient ischemic attacks were associated with regional cortical thinning.

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