2个CADASIL患者的弥散张量成像与20例正常人的比较

目的　分析2例CADASIL患者的MR特点及弥散张量指标的变化。方法　收集2例通过病理和基因检查确诊为CA DASIL的先证者的临床资料,对其进行常规MR扫描和弥散张量成像,将弥散张量成像的指标与20例正常志愿者的指标进行比较。结果　2例CADASIL患者的MR主要表现为双侧额顶叶白质内多发腔梗、脱髓鞘改变和双侧颞叶前部白质脱髓鞘。1例患者双侧外囊、内囊后肢、胼胝体膝部和压部的部分各向异性(FA)值均小于正常组的平均值减去2倍标准差,另1例患者左侧外囊的FA值小于对照组的平均值减去2倍标准差。结论　常规MR表现和弥散张量成像指标的测量均反映了CADASIL患者中存在严重的白质病变。

Diffusion Tensor Imaging of Two Patients with Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy ( CADASIL ) Comparison with Normal Volunteers

Objective To detect abnormalities of the brain in 2 patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) on conventional MR and diffusion tensor imaging(DTI).Methods Conventional MR and diffusion tensor imaging were obtained in 2 patinets with CADASIL confirmed by pathology and genetical investigation.The fractional anisotropy (FA) values of different regions of brain were measured and compared with those of 20 normal subjects. Results Conventional MR revealed multiple lacular infarcts and demyelination in bilateral white matter of frontal lobes and parietal lobes. Bilateral temporal lobes showed signs of demyelination in the anterior part. FA values of bilateral posterior limbs of internal capsule, lateral capsules, genu and splenium of corpus callosum were significantly lower in one patient than that of normal subjects.Conclusion The abnormalities revealed by conventional MR and DTI were consistent with severe pathological changes of white matter in CADASIL patients.