Shi YuZhi, Li ShaoWu, Li Wei, Zhang Chen, Guo LiYing, Pan YunZhu, Zhou XueMei, Wang XinGao, Niu Songtao, Yu XueYing, Tang HeFei, Chen Bin, Zhang ZaiQiang
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Department of Functional Neuroimaging, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China.
Front Neurol. 2018 Oct 16;9:862. doi: 10.3389/fneur.2018.00862. eCollection 2018.
Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is the best known and the most common monogenic small vessel disease (SVD). Cognitive impairment is an inevitable feature of CADASIL. Total SVD score and global cortical atrophy (GCA) scale were found to be good predictors of poor cognitive performance in community-dwelling adults. We aimed to estimate the association between the total SVD score, GCA scale and the cognitive performance in patients with CADASIL. We enrolled 20 genetically confirmed CADASIL patients and 20 controls matched by age, gender, and years of education. All participants underwent cognitive assessments to rate the global cognition and individual domain of executive function, information processing speed, memory, language, and visuospatial function. The total SVD score and GCA scale were rated. The CADASIL group performed worse than the controls on all cognition measures. Neither global cognition nor any separate domain of cognition was significantly different among patients grouped by total SVD score. Negative correlations between the GCA score and cognitive performance were observed. Approximately 40% of the variance was explained by the total GCA score in the domains of executive function, information processing speed, and language. The superficial atrophy score was associated with poor performance in most of the domains of cognition. Adding the superficial atrophy score decreased the prediction power of the deep atrophy score on cognitive impairment alone. The GCA score, not the total SVD score, was significantly associated with poor cognitive performance in patients with CADASIL. Adding the superficial atrophy score attenuated the prediction power of the deep atrophy score on cognitive impairment alone.
伴有皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病（CADASIL）是最知名且最常见的单基因小血管疾病（SVD）。认知障碍是CADASIL不可避免的特征。在社区居住的成年人中，总SVD评分和全脑皮质萎缩（GCA）量表被发现是认知功能不佳的良好预测指标。我们旨在评估CADASIL患者的总SVD评分、GCA量表与认知功能之间的关联。我们纳入了20例基因确诊的CADASIL患者以及20例年龄、性别和受教育年限相匹配的对照。所有参与者均接受认知评估，以评定整体认知以及执行功能、信息处理速度、记忆、语言和视觉空间功能等各个领域。评定总SVD评分和GCA量表。CADASIL组在所有认知测量方面的表现均比对照组差。按总SVD评分分组的患者之间，整体认知或任何单独的认知领域均无显著差异。观察到GCA评分与认知功能之间呈负相关。在执行功能、信息处理速度和语言领域，约40%的变异可由总GCA评分解释。浅表萎缩评分与大多数认知领域的表现不佳相关。加入浅表萎缩评分会降低深部萎缩评分单独对认知障碍的预测能力。GCA评分而非总SVD评分与CADASIL患者的认知功能不佳显著相关。加入浅表萎缩评分会减弱深部萎缩评分单独对认知障碍的预测能力。
