Microvasculopathy is associated with the number of cerebrovascular lesions in hereditary cerebral hemorrhage with amyloidosis, Dutch type
Microvasculopathy is associated with the number of cerebrovascular lesions in hereditary cerebral hemorrhage with amyloidosis, Dutch type
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DOI：
10.1161/01.str.29.8.1588
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发表时间：
1998-08-01
期刊：
影响因子：
8.3
通讯作者：
van Duinen, SG
中科院分区：
文献类型：
--
作者：
Natté, R;Vinters, HV;van Duinen, SG
背景和目的--脑淀粉样血管病(CAA)可引起微血管改变，如微动脉瘤和纤维蛋白样坏死。这些CAA相关微血管病变(CAA-AM)可能导致CAA相关出血和/或脑梗塞的发展，以下称为“脑血管病变”。遗传性脑出血伴淀粉样变性，荷兰型(HCHWA-D)是一种常染色体显性遗传性CAA，其淀粉样血管病在病理和生化上类似于与衰老和阿尔茨海默病相关的散发性CBA。为了确定CAA-AM在CAA相关脑血管并发症中的意义，我们研究了CAA-AM与HCHWA-D患者脑血管病变的关系。方法：在先前的尸检研究中，我们对29例HCHWA-D患者的CAA-AM进行了半定量评分。在本研究中，我们回顾了这些患者的临床图表和尸检方案。我们调查了CAA-AM是否与死亡年龄、脑血管病变数目、临床病程、高血压和动脉粥样硬化相关。结果：CAA-AM与脑血管病变数目相关(P=0.009)。微动脉瘤样变性的出现与脑血管病变的数目密切相关(P
Background and Purpose-Microvascular changes such as microaneurysms and fibrinoid necrosis have been found in the presence of cerebral amyloid angiopathy (CAA). These CAA-associated microvasculopathies (CAA-AM) may contribute to the development of CAA-associated hemorrhages and/or infarcts, hereafter referred to as "cerebrovascular lesions." Hereditary cerebral hemorrhage with amyloidosis, Dutch type (HCHWA-D) is an autosomal dominant form of CAA, in which the amyloid angiopathy is pathologically and biochemically similar to sporadic CBA associated with aging and Alzheimer disease. To determine the significance of CAA-AM for CAA-associated cerebrovascular complications, we investigated the association between CAA-AM and cerebrovascular lesions in HCHWA-D patients.Methods-In a previous autopsy study we semiquantitatively scored CAA-AM in 29 HCHWA-D patients. In the present study we reviewed clinical charts and autopsy protocols of these same patients. We investigated whether CAA-AM was associated with age at death, number of cerebrovascular lesions, duration of clinical illness, hypertension, and atherosclerosis.Results-An association was found between CAA-AM and the number of cerebrovascular lesions (P=0.009). The presence of microaneurysmal degeneration was most strongly associated with the number of cerebrovascular lesions (P