Charidimou Andreas, Boulouis Gregoire, Gurol M Edip, Ayata Cenk, Bacskai Brian J, Frosch Matthew P, Viswanathan Anand, Greenberg Steven M
Hemorrhagic Stroke Research Program, Department of Neurology, Massachusetts General Hospital Stroke Research Center, Harvard Medical School, Boston, MA, USA.
Neurovascular Research Laboratory, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Charlestown, MA, USA.
Brain. 2017 Jul 1;140(7):1829-1850. doi: 10.1093/brain/awx047.
Sporadic cerebral amyloid angiopathy is a common, well-defined small vessel disease and a largely untreatable cause of intracerebral haemorrhage and contributor to age-related cognitive decline. The term 'cerebral amyloid angiopathy' now encompasses not only a specific cerebrovascular pathological finding, but also different clinical syndromes (both acute and progressive), brain parenchymal lesions seen on neuroimaging and a set of diagnostic criteria-the Boston criteria, which have resulted in increasingly detected disease during life. Over the past few years, it has become clear that, at the pathophysiological level, cerebral amyloid angiopathy appears to be in part a protein elimination failure angiopathy and that this dysfunction is a feed-forward process, which potentially leads to worsening vascular amyloid-β accumulation, activation of vascular injury pathways and impaired vascular physiology. From a clinical standpoint, cerebral amyloid angiopathy is characterized by individual focal lesions (microbleeds, cortical superficial siderosis, microinfarcts) and large-scale alterations (white matter hyperintensities, structural connectivity, cortical thickness), both cortical and subcortical. This review provides an interdisciplinary critical outlook on various emerging and changing concepts in the field, illustrating mechanisms associated with amyloid cerebrovascular pathology and neurological dysfunction.
散发性脑淀粉样血管病是一种常见的、定义明确的小血管疾病，是脑出血的主要不可治疗病因，也是与年龄相关的认知衰退的一个促成因素。“脑淀粉样血管病”这一术语现在不仅涵盖了一种特定的脑血管病理发现，还包括不同的临床综合征（急性和进行性）、神经影像学上可见的脑实质病变以及一套诊断标准——波士顿标准，这些标准使得该病在生前的检出率越来越高。在过去几年中，越来越清楚的是，在病理生理层面，脑淀粉样血管病似乎部分是一种蛋白质清除失败性血管病，而且这种功能障碍是一个前馈过程，可能导致血管淀粉样β蛋白积累加剧、血管损伤途径激活以及血管生理功能受损。从临床角度来看，脑淀粉样血管病的特征是存在单个局灶性病变（微出血、皮质表面铁沉积、微梗死）以及皮质和皮质下的大规模改变（白质高信号、结构连接性、皮质厚度）。本综述对该领域各种新出现和不断变化的概念提供了跨学科的批判性观点，阐述了与淀粉样脑血管病理和神经功能障碍相关的机制。
