CADASIL的脑小动脉病变
北京军区总医院 (100700)
【摘要】目的：伴有皮层下梗死和脑白质病的常染色体显性遗传性脑动脉病(CADASIL)的病理特征是血管损害，首先侵及中-小动脉，以血管平滑肌细胞变性为特征并可见嗜锇酸颗粒物质沉积。患者脑部的白质和深层灰质中有多发性小梗死和弥散性脱髓鞘。本研究对CADASIL患者灰质和白质中小动脉的硬化指数(SI)进行了测量。
方法：选取4名已故的有明确C475T(R133C)Notch3基因变异的CADASIL患者。2组不同人群作为对照组。分别测量各组中灰质和白质中小动脉的硬化指数。通过CADASIL患者1000张穿动脉切片重建了9条小动脉。结果：CADASIL组白质及灰质小动脉的硬化指数明显高于对照组。CADASIL组中白质小动脉硬化程度明显高于灰质。血管内径值分布曲线可见CADASIL组内径小于对照组。当血管内径小于20-30 µm、血管外径小于100-130 µm时，SI值明显增加。
, http://www.100md.com
结论：患者供应皮质下结构的长穿动脉或分支动脉结构紊乱，血管壁增厚。本研究支持CADASIL患者脑白质中脑血流(CBF)和局部脑血流量(rCBV)减少，而皮层中没有明显减少的观点并与临床上的多发梗死的性质与部位相符合。
Cerebral arteriopathy in CADASIL patients
ZHANG Weiwei, Hannu Kalimo, ZHU Guangming
【Abstract】Objective: In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) arteriolar walls are thickened with destruction of vascular smooth muscle cells (VSMCs) and deposits of granular osmiophilic material (GOM). Small infarcts occur in the white matter (WM) and deep grey matter (GM) while the cerebral cortex is sparing. We analysed the arteriolar structural changes in CADASIL patients. Materials and Methods: The sclerotic index (SI) of arterioles in GM and WM was analysed in four patients with CADASIL caused by C475T (R133C) mutation in Notch3 and in six age-matched controls. We also reconstructed 9 arterioles from 1000 serial sections of penetrating arterioles or their branches in two CADASIL patients. Results: SI of CADASIL group was significantly higher than that of control group in both GM and WM. In CADASIL group SI was significantly higher in WM than in GM. Distribution curve of the arteriolar internal diameters in CADASIL shifted towards smaller sizes than those in the controls. In serial sections, SI of WM penetrating arterioles or their branches did not increase sharply until the internal diameters had decreased to about 20-30µm and external diameters to about 100-130µm. Conclusions: In CADASIL long penetrating arterioles or branches supplying subcortical structures are obliterated and their walls are thickened. Our present study supports the findings of reduction in cerebral blood flow (CBF) or regional cerebral blood volume (rCBV) in WM of CADASIL and conforms to clinical abundance of infarcts and primary ischemic damage in CADASIL patients., 百拇医药(张微微 Hannu Kalimo 朱光明)
【摘要】目的：伴有皮层下梗死和脑白质病的常染色体显性遗传性脑动脉病(CADASIL)的病理特征是血管损害，首先侵及中-小动脉，以血管平滑肌细胞变性为特征并可见嗜锇酸颗粒物质沉积。患者脑部的白质和深层灰质中有多发性小梗死和弥散性脱髓鞘。本研究对CADASIL患者灰质和白质中小动脉的硬化指数(SI)进行了测量。
方法：选取4名已故的有明确C475T(R133C)Notch3基因变异的CADASIL患者。2组不同人群作为对照组。分别测量各组中灰质和白质中小动脉的硬化指数。通过CADASIL患者1000张穿动脉切片重建了9条小动脉。结果：CADASIL组白质及灰质小动脉的硬化指数明显高于对照组。CADASIL组中白质小动脉硬化程度明显高于灰质。血管内径值分布曲线可见CADASIL组内径小于对照组。当血管内径小于20-30 µm、血管外径小于100-130 µm时，SI值明显增加。
, http://www.100md.com
结论：患者供应皮质下结构的长穿动脉或分支动脉结构紊乱，血管壁增厚。本研究支持CADASIL患者脑白质中脑血流(CBF)和局部脑血流量(rCBV)减少，而皮层中没有明显减少的观点并与临床上的多发梗死的性质与部位相符合。
Cerebral arteriopathy in CADASIL patients
ZHANG Weiwei, Hannu Kalimo, ZHU Guangming
【Abstract】Objective: In cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) arteriolar walls are thickened with destruction of vascular smooth muscle cells (VSMCs) and deposits of granular osmiophilic material (GOM). Small infarcts occur in the white matter (WM) and deep grey matter (GM) while the cerebral cortex is sparing. We analysed the arteriolar structural changes in CADASIL patients. Materials and Methods: The sclerotic index (SI) of arterioles in GM and WM was analysed in four patients with CADASIL caused by C475T (R133C) mutation in Notch3 and in six age-matched controls. We also reconstructed 9 arterioles from 1000 serial sections of penetrating arterioles or their branches in two CADASIL patients. Results: SI of CADASIL group was significantly higher than that of control group in both GM and WM. In CADASIL group SI was significantly higher in WM than in GM. Distribution curve of the arteriolar internal diameters in CADASIL shifted towards smaller sizes than those in the controls. In serial sections, SI of WM penetrating arterioles or their branches did not increase sharply until the internal diameters had decreased to about 20-30µm and external diameters to about 100-130µm. Conclusions: In CADASIL long penetrating arterioles or branches supplying subcortical structures are obliterated and their walls are thickened. Our present study supports the findings of reduction in cerebral blood flow (CBF) or regional cerebral blood volume (rCBV) in WM of CADASIL and conforms to clinical abundance of infarcts and primary ischemic damage in CADASIL patients., 百拇医药(张微微 Hannu Kalimo 朱光明)