脑淀粉样血管病的研究现状

doi:10.11817/j.issn.1673-9248.2021.04.002

脑淀粉样血管病是指淀粉样物质在大脑皮层和软脑膜小动脉及毛细血管过量沉积，导致血管功能障碍和脑实质损伤的一类神经系统疾病，在老年人群中发病率较高。本文对脑淀粉样血管病的流行病学、发病机制、病理改变、临床表现、辅助检查及相关表现、诊断标准以及治疗等方面的研究进展进行综述，希望提高该病的临床识别率以及合理治疗率。

关键词: 脑淀粉样血管病, β-淀粉样蛋白, 小血管疾病, 脑叶出血

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

表1 CAA修正版波士顿诊断标准

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

表1 CAA修正版波士顿诊断标准

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

表2 CAA爱丁堡诊断标准

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%）	＋＋＋	＋－＋	－＋＋

表2 CAA爱丁堡诊断标准

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%）	＋＋＋	＋－＋	－＋＋

表3 CAA-ri诊断标准

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

表3 CAA-ri诊断标准

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

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