【摘要】 目的观察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)患者软脑膜动脉管壁结构及血管弹性指数的变化。方法对6例有基因突变的CADASIL患者组成的观察组的额叶、颞叶皮层脑组织切片与对照组的脑组织切片进行penta-chrome染色,对所有切片内的软脑膜血管的内膜、中膜、外膜厚度、血管腔内径和外径进行测量,计算血管弹性指数(SI)。结果 CADASIL患者的软脑膜血管的内膜与对照组相比显著增厚(P<0.0001),中膜厚度与对照组相比明显变薄(P<0.01),外膜与对照组相比差别无显著性,CADASIL的血管腔内径与对照组相比并无狭窄,血管弹性指数SI与对照组相比显著增高(P<0.00001)。结论 CADASIL的软脑膜动脉的主要病理性改变是内膜显著增厚和中膜变薄,但血管内径无明显改变。更多还原

【Abstract】 Objective Observe the CADASIL patients with soft meningeal artery wall structure and the change of blood vessel elasticity index. Method Frontal and temporal cortex of six genetically proven CADASIL brains,six controls without symptoms of cerebrovascular disease,were examined using penta-chrome staining. We determined intimal,medial,and adventitial thickness,inner diameter,and sclerotic index( SI) for all leptomeningeal arteries within tissue examined. Result The intima of CADASIL arteries was significantly thickened compared to controls( P< 0. 0001). Medial thickness was lower in CADASIL compared to controls( P < 0. 01). The adventitia was not significantly increased in CADASIL compared to age-matched controls. Arterial diameters were not smaller in CADASIL compared to controls. SI was significantly increased in CADASIL compared to other groups( P < 0. 00001).Conclusion Principle changes of leptomeningeal arteries in CADASIL include intimal thickening and medial thinning,but not luminal narrowing.更多还原