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.

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.

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.

These results suggest that the IFS may be a useful screening tool to assess executive function and disease severity in the context of SVD.

执行功能障碍是脑小血管疾病（SVD）的主要认知症状。认知神经病学研究所额叶筛查（IFS）是一种经过充分验证的筛查工具，可以快速评估说西班牙语的人的执行功能的多个组成部分。在这项研究中，我们检查了患有脑常染色体显性遗传性动脉病并伴有皮层下梗塞和白质脑病（CADASIL）（一种导致SVD早期发作的遗传病）的IFS表现。我们进一步探讨了IFS的性能与SVD的磁共振成像（MRI）标记之间的关联。

我们从哥伦比亚招募了24名无症状的CADASIL受试者和23名非携带者。所有受试者均接受了MRI研究和神经心理学评估，包括IFS。SVD的结构性MRI标记物在每个受试者中都进行了量化，并附有代表脑血管改变总体负担的SVD Sum Score。使用一般线性模型，相关性和接收器工作特性曲线分析来探讨IFS上的组差异以及与SVD的MRI标记之间的关系。

CADASIL受试者的IFS总分成绩明显下降。除脑萎缩和血管周间隙扩大外，IFS的表现与所有SVD量化指标相关。最后，尽管IFS总分无法准确区分携带者和非携带者，但它在检测SVD的多个MRI标记物时显示出足够的敏感性和特异性。

这些结果表明，IFS可能是评估SVD背景下执行功能和疾病严重性的有用筛查工具。