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目的:通过meta分析评价颞极和外囊白质高信号(white matter hyperintensities, WMHs)对诊断伴皮质下梗死和白质脑病的常染色体显性遗传脑动脉病(cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL)患者的临床价值。方法:检索PubMed、Cochrane、Embase、维普中文科技期刊数据库、中国生物医学文献数据库、中国知网、万方数据服务平台等数据库,收集颞极和外囊WMHs诊断CADASIL患者的相关文献,检索时限均从建库至2020年4月1日。使用诊断准确性研究质量评价工具QUADAS-2进行文献质量评价。采用Stata 15.1软件进行统计分析,拟合综合受试者工作特征(summary receiver operating characteristic, SROC)曲线,合并诊断效应量,评估颞极和外囊WMHs对CADASIL的诊断价值。结果:纳入9篇文献共10项研究,累计880例患者。颞极和外囊WMHs的合并敏感性分别为0.67[95%置信区间(confidence interval, n CI)0.54~0.78]和0.84(95% n CI 0.72~0.91),合并特异性分别为0.64(95% n CI 0.47~0.78)和0.44(95% n CI 0.36~0.53),合并阳性似然比分别为1.9(95% n CI 1.4~2.6)和1.5(95% n CI 1.2~1.8),合并阴性似然比分别为0.51(95% n CI 0.42~0.63)和0.37(95% n CI 0.20~0.69),合并诊断优势比分别为4(95% n CI 3~5)和4(95% n CI 2~9),SROC曲线下面积分别为0.71(95% n CI 0.66~0.74)和0.62(95% n CI 0.58~0.66)。n 结论:颞极和外囊WMHs对CADASIL的诊断价值有限,在临床诊断过程中需要综合考虑其他因素。“,”Objective:To evaluate the clinical value of temporal pole and external capsule white matter hyperintensities (WMHs) on the diagnosie of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) by meta-analysis.Methods:PubMed, Cochrane, Embase, VIP database, China Biomedical Literature database, CNKI, Wanfang Data Service Platform were retrieved. The relevant literature of temporal pole and external capsule WMHs for the diagnosis of CADASIL was collected. The retrieval time limit was from the establishment of the databases to April 1, 2020. Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) was used to evaluate the quality of literature. Stata 15.1 software was used for statistical analysis. The fitted Summary Receiver Operating Characteristic (SROC) curve and combined diagnostic effect size were used to evaluate the diagnostic value of temporal pole and external capsule WMHs for CADASIL.Results:A total of 9 articles involving 10 studies were enrolled, including 880 patients. The combined sensitivities of temporal pole and external capsule WMHs for CADASIL were 0.67 (95% confidence interval [n CI] 0.54-0.78) and 0.84 (95% n CI 0.72-0.91) respectively, the combined specificities were 0.64 (95% n CI 0.47-0.78) and 0.44 (95% n CI 0.36-0.53) respectively, the combined positive likelihood ratios were 1.9 (95% n CI 1.4-2.6) and 1.5 (95% n CI 1.2-1.8) respectively, the combined negative likelihood ratios were 0.51 (95% n CI 0.42-0.63) and 0.37 (95% n CI 0.20-0.69) respectively, the odds ratios of combined diagnosis were 4 (95% n CI 3-5) and 4 (95% n CI 2-9) respectively, and the area under the SROC curves were 0.71 (95% n CI 0.66-0.74) and 0.62 (95% n CI 0.58-0.66) respectively.n Conclusions:The temporal pole and external capsule WMHs have limited diagnostic value for CADASIL, and other factors need to be comprehensively considered in the clinical diagnosis process.