CAJ | 학술논문

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。
목적：관찰반유피질하경사화백질뇌병적상염색체현성유전성뇌동맥병( CADASIL)환자연뇌막동맥관벽결구급혈관탄성지수적변화。방법대6례유기인돌변적CADASIL환자조성적관찰조적액협、섭협피층뇌조직절편여대조조적뇌조직절편진행penta-chrome염색,대소유절편내적연뇌막혈관적내막、중막、외막후도、혈관강내경화외경진행측량,계산혈관탄성지수( SI)。결과 CADASIL환자적연뇌막혈관적내막여대조조상비현저증후(P<0.0001),중막후도여대조조상비명현변박(P<0.01),외막여대조조상비차별무현저성,CADASIL적혈관강내경여대조조상비병무협착,혈관탄성지수SI여대조조상비현저증고(P<0.00001)。결론 CADASIL적연뇌막동맥적주요병이성개변시내막현저증후화중막변박,단혈관내경무명현개변。
Objective Observe the CADASIL patients with soft meningeal artery wall structure and the change of blood vessel elasticity index. Method③rontal and temporal cortex of six genetically proven CADASIL brains,six con-trols without symptoms of cerebrovascular disease, were examined using penta-chrome staining. We determined inti-mal, 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 signifi-cantly 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.