Department of Psychiatry, Massachusetts General Hospital, Harvard Medical School, Boston, MA02114, USA.
Department of Ophthalmology, Schepens Eye Research Institute, Harvard Medical School, Boston, MA02114, USA.
J Int Neuropsychol Soc. 2020 Nov;26(10):1006-1018. doi: 10.1017/S1355617720000533. Epub 2020 Jun 3.
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病（SVD）的主要认知症状。认知神经病学研究所执行功能快速筛查（IFS）是一种经过充分验证的筛查工具，可在西班牙语人群中快速评估执行功能的多个组成部分。在这项研究中，我们检查了具有脑常染色体显性动脉病伴皮质下梗死和白质脑病（CADASIL）的受试者在 IFS 上的表现，CADASIL 是一种导致 SVD 早期发病的遗传性疾病。我们进一步探讨了 IFS 表现与 SVD 的磁共振成像（MRI）标志物之间的关联。
我们招募了来自哥伦比亚的 24 名无症状 CADASIL 受试者和 23 名非携带者。所有受试者均接受了研究性 MRI 和神经心理学评估，包括 IFS。对每个受试者的 SVD 结构性 MRI 标志物进行量化，同时还量化了代表脑血管改变总体负担的 SVD 总分。使用一般线性模型、相关性和受试者工作特征曲线分析来探讨 IFS 上的组间差异以及与 SVD MRI 标志物的关系。
CADASIL 受试者的 IFS 总分明显降低。IFS 的表现与所有量化的 SVD 标志物相关，除了脑萎缩和血管周围间隙扩大。最后，虽然 IFS 总分不能准确区分携带者和非携带者，但它在检测多个 SVD MRI 标志物的存在方面具有足够的敏感性和特异性。
这些结果表明，IFS 可能是一种有用的筛查工具，可用于评估 SVD 背景下的执行功能和疾病严重程度。
