Advanced intimal hyperplasia without luminal narrowing of leptomeningeal arteries in CADASIL.
Advanced intimal hyperplasia without luminal narrowing of leptomeningeal arteries in CADASIL.
复制标题
DOI：
10.1161/strokeaha.111.000721
复制
发表时间：
2013-05
期刊：
影响因子：
8.3
通讯作者：
Wang MM
中科院分区：
文献类型：
--
作者：
Dong H;Ding H;Young K;Blaivas M;Christensen PJ;Wang MM
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）中的软脑膜动脉异常尚未得到广泛表征。我们量化了CADASIL中软脑膜动脉的亚结构和直径，并与年龄匹配的对照组和老年人进行了比较;此外，我们还使用免疫组织化学表征了CADASIL中的内膜增厚。对6例经遗传学证实的CADASIL脑（平均年龄66岁）、6例无脑血管疾病症状的对照组和6例高龄脑（平均年龄89岁）的额叶和颞叶皮质进行了软脑膜动脉内膜、中膜和外膜厚度、内径、硬化指数（SI）和平滑肌标记物检查。CADASIL动脉的内膜比对照组和老年组厚5倍（p<0.0001）。CADASIL组的中膜厚度低于对照组和高龄组（p<0.01）。与年龄匹配的对照组相比，CADASIL组的外膜未显著增加。与对照组相比，CADASIL组的动脉直径并不更小。与其他组相比，CADASIL组的SI显著增加（p<0.00001）。CADASIL中的内膜细胞表达平滑肌肌动蛋白、S100 A4和波形蛋白，但不表达结蛋白。CADASIL中软脑膜动脉的主要变化包括内膜增厚和中膜变薄，但不包括管腔狭窄。平滑肌样细胞参与CADASIL动脉的新生内膜增厚。
Leptomeningeal artery abnormalities in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) have not been extensively characterized. We quantified substructure and diameter of leptomeningeal arteries in CADASIL in comparison to age matched controls and the very old; in addition, we characterized intimal thickening in CADASIL using immunohistochemistry. Frontal and temporal cortex of six genetically proven CADASIL brains (average age 66), six controls without symptoms of cerebrovascular disease, and six very old brains (average age 89) were examined for leptomeningeal artery intimal, medial, and adventitial thickness, inner diameter, and sclerotic index (SI) and for smooth muscle markers. The intima of CADASIL arteries was thickened five-fold compared to controls and the very aged (p<0.0001). Medial thickness was lower in CADASIL compared to controls and the very old (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). Intimal cells in CADASIL expressed smooth muscle actin, S100A4, and vimentin but not desmin. Principle changes of leptomeningeal arteries in CADASIL include intimal thickening and medial thinning, but not luminal narrowing. Smooth muscle-like cells participate in neointimal thickening of CADASIL arteries.