The state-of-the-art of cerebral amyloid angiopathy

doi:10.11817/j.issn.1673-9248.2021.04.002

Abstract

Abstract:

Cerebral amyloid angiopathy (CAA) is characterized by the pathological deposition of amyloid β-protein in the media and adventitia of small arteries in the cortex and leptomeninges, which often causes vascular dysfunction and brain parenchyma damage. CAA is common in the elderly. This article reviews the progress in the epidemiology, pathogenesis, pathological changes, clinical manifestations, clinical imaging manifestations, diagnostic criteria, and treatment of CAA, to improve the clinical recognition and rational therapy.

Key words: Cerebral amyloid angiopathy, Amyloid β-protein, Small vessel disease, Lobar intracerebral hemorrhage

Nuo Wang, Yonghua Huang. The state-of-the-art of cerebral amyloid angiopathy[J]. Chinese Journal of Cerebrovascular Diseases(Electronic Edition), 2021, 15(04): 205-211.

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诊断	标准
确诊的CAA	全面尸检显示：（1）脑叶、皮层或皮层-皮层下出血；（2）严重的CAA血管病变；（3）无其他诊断病灶
病理支持的很可能CAA	临床资料和病理组织（血肿清除术或皮层活检）显示：（1）脑叶、皮层或皮层-皮层下出血（包括脑出血，脑微出血或cSS）；（2）活检组织有一定程度的CAA病理改变；（3）无其他诊断病灶
很可能CAA	临床资料和MRI或CT显示：（1）局限脑叶、皮层或皮层-皮层下的多发出血（脑出血，脑微出血；包括小脑出血）或单个脑叶、皮层或皮层-皮层下出血与局限或弥漫的cSS；（2）年龄≥55岁；（3）排除其他导致出血的原因
可能CAA	临床资料和MRI或CT显示：（1）单个脑叶、皮层或皮层-皮层下出血\脑微出血或cSS（局限或弥漫的）；（2）年龄≥55岁；（3）排除其他导致出血的原因

诊断	标准
确诊的CAA	全面尸检显示：（1）脑叶、皮层或皮层-皮层下出血；（2）严重的CAA血管病变；（3）无其他诊断病灶
病理支持的很可能CAA	临床资料和病理组织（血肿清除术或皮层活检）显示：（1）脑叶、皮层或皮层-皮层下出血（包括脑出血，脑微出血或cSS）；（2）活检组织有一定程度的CAA病理改变；（3）无其他诊断病灶
很可能CAA	临床资料和MRI或CT显示：（1）局限脑叶、皮层或皮层-皮层下的多发出血（脑出血，脑微出血；包括小脑出血）或单个脑叶、皮层或皮层-皮层下出血与局限或弥漫的cSS；（2）年龄≥55岁；（3）排除其他导致出血的原因
可能CAA	临床资料和MRI或CT显示：（1）单个脑叶、皮层或皮层-皮层下出血\脑微出血或cSS（局限或弥漫的）；（2）年龄≥55岁；（3）排除其他导致出血的原因

中重度CAA的概率	蛛网膜下腔出血	携带ApoEε4	指状出血
低（≤7%）	－	－	－
中（44%~64%）	＋－	－＋	－－
高（≥95%）	＋＋＋	＋－＋	－＋＋

中重度CAA的概率	蛛网膜下腔出血	携带ApoEε4	指状出血
低（≤7%）	－	－	－
中（44%~64%）	＋－	－＋	－－
高（≥95%）	＋＋＋	＋－＋	－＋＋

诊断	标准
确诊CAA	满足很可能CAA-ri诊断标准，且组织病理显示：（1）血管周围、跨血管壁及血管壁内炎症；（2）受累区域的皮层和软脑膜血管淀粉样物质沉积
很可能CAA	（1）年龄≥40岁；（2）具有≥1项下列临床症状：头痛、意识水平下降、行为改变、局灶性神经症状、癫痫，以上症状不是由急性脑出血直接导致；（3）MRI显示单发或多发白质高信号病灶（皮层-皮层下或深部），呈非对称性，且快速进展至皮层下白质（这种不对称性不是因为既往脑出血）；（4）显示≥1个下列皮层-皮层下出血病灶：脑出血、脑微出血、cSS；（5）排除肿瘤、感染或其他病因
可能CAA	（1）年龄≥40岁；（2）具有≥1项下列临床症状：头痛、意识水平下降、行为改变、局灶性神经症状、癫痫，以上症状不是由急性脑出血直接导致；（3）MRI显示白质高信号病灶快速进展至皮层下白质；（4）显示≥1个下列皮层-皮层下出血病灶：脑出血、脑微出血、cSS；（5）排除肿瘤、感染或其他病因

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