Background

Cerebral 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.

Methods

We 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.

Results

Compared 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.

Conclusion

Reductions 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.

中文翻译：

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CADASIL患者的大脑结构变化：形态磁共振成像研究。

背景

大脑皮层下梗死和白质脑病（CADASIL）是常染色体显性遗传性动脉疾病，是一种皮下遗传的遗传性脑小血管疾病。多项研究表明特定皮层区域的参与。但是，大脑的结构改变及其临床相关性仍未确定。

方法

我们评估了22位CADASIL患者和22位年龄和性别相匹配的健康对照。我们使用源自3.0-T磁共振成像（MRI）的基于表面和体素的形态学数据来探索灰色和白色物质的结构变化。我们计算了此类数据与临床和MRI指标之间的Pearson相关性。

结果

与对照组相比，CADASIL患者的左上颌上回，颞上回，颞侧回，岛状，眶额外侧回，扣带回和中央前回的皮质厚度显着降低。白（而不是灰色）物质的体积也明显减少，主要是在额叶，顶叶，颞叶和枕叶。既往中风或短暂性脑缺血发作的次数与左侧胸膜和右侧后扣带回的皮层厚度呈负相关。

结论

与对照组相比，CADASIL患者的皮质厚度和白质体积明显减少，并且卒中次数增加和短暂性脑缺血发作与局部皮质变薄有关。