中文摘要：

目的分析常染色体显性遗传性脑动脉病伴皮层下梗死和白质脑病（ cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, CADASIL）患者的弥散张量成像特点及与临床的关系。方法患者和同龄健康志愿者各14例，进行头颅MRI常规扫描和弥散张量成像扫描，测量两组受试者各脑叶、内囊后肢、外囊以及胼胝体的部分各向异性（fractional anisotropy，FA）值及总和，比较两组间FA值的差异。对患者的认知功能和卒中后神经功能状态分别进行MMSE和NIHSS评分。校正年龄影响，分析患者各个脑白质区域FA值及总和与MMSE分值、NIHSS评分的相关关系。结果患者组各脑区的FA值均显著低于对照组。FA值总和、右额叶、右颞叶、左顶叶、左枕叶、双侧内囊后肢以及外囊白质的FA值和MMSE分值存在相关性（P〈0．05）。FA值总和及各脑区FA值与NIHSS评分无相关性。结论CADASIL患者不同脑白质区域的FA值显著下降，部分区域的白质损害与患者的认知功能减退有关。

英文摘要：

Objective Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy （CADASIL） is a systemic small vessel disease. Here we studied the change of fractional anisotropy（FA） of diffusion tensor imaging （DTI） and the relationship between FA values and MMSE and NIHSS scores in CADASIL patients. Methods Cranial DTI and conventional MRI scans were obtained from 14 CADASIL patients and 14 age-matched controls. MMSE and NIHSS scores were measured and calculated for all patients. Using DTI, FA values were calculated for the white matter in each cerebral lobe, the posterior limbs of the internal capsule, external capsule and corpus callosum. After adjusting for age, correlations between MMSE, NIHSS scores and the FA values were assessed using Partial correlation coefficients. Restilts Patients＇ FA values were significantly lower than the controls＇ （ P 〈 0. 05 ）. Whole FA value and FA values of the white matter of the right frontal lobe, right temporal lobe, left parietal lobes, left occipital lobe, posterior limbs of internal capsule, external capsule were significantly related to MMSE （P 〈 0. 05 ）. Whole FA value and FA values of the white matter of each measured regions were not related to NIHSS scores. Conclusions FA values decrease in the different area of white matter in CADASIL. The white matter lesions in some regions result in cognitive impairment.