Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA.
Curr Opin Neurol. 2018 Feb;31(1):28-35. doi: 10.1097/WCO.0000000000000510.
Cerebral amyloid angiopathy (CAA) is diagnosed primarily as a cause of lobar intracerebral hemorrhages (ICH) in elderly patients. With improving MRI techniques, however, the role of CAA in causing other symptoms has become clear. Recognizing the full clinical spectrum of CAA is important for diagnosis and treatment. In this review we summarize recent insights in clinical CAA features, MRI biomarkers, and management.
The rate of ICH recurrence in CAA is among the highest of all stroke subtypes. Cortical superficial siderosis (cSS) and cortical subarachnoid hemorrhage (cSAH) are important imaging predictors for recurrent ICH. CAA also causes cognitive problems in multiple domains. In patients with nondemented CAA, the risk of developing dementia is high especially after ICH. CAA pathology probably starts years before the first clinical manifestations. The first signs in hereditary CAA are white matter lesions, cortical microinfarcts, and impaired occipital cerebral vasoreactivity. Visible centrum semiovale perivascular spaces, lobar located lacunes, and cortical atrophy are new nonhemorrhagic MRI markers.
CAA should be in the differential diagnosis of elderly patients with lobar ICH but also in those with cognitive decline and episodic transient neurological symptoms. Physicians should be aware of the cognitive effects of CAA. In patients with a previous ICH, cSS, or cSAH, anticoagulation should be considered risky. The increasing number of MRI markers may help to discriminate CAA from other small vessel diseases and dementia subtypes.
脑淀粉样血管病（Cerebral Amyloid Angiopathy，CAA）主要被诊断为老年患者脑叶内出血（Intracerebral Hemorrhages，ICH）的病因。然而，随着 MRI 技术的进步，CAA 引起其他症状的作用已变得清晰。认识到 CAA 的全部临床谱对于诊断和治疗很重要。在这篇综述中，我们总结了 CAA 临床特征、MRI 生物标志物和治疗的最新见解。
CAA 患者的 ICH 复发率在所有中风亚型中最高。皮质表面铁沉积（Cortical Superficial Siderosis，cSS）和皮质下蛛网膜下腔出血（Cortical Subarachnoid Hemorrhage，cSAH）是预测 ICH 复发的重要影像学标志物。CAA 还会导致多个领域的认知问题。在非痴呆性 CAA 患者中，发生痴呆的风险很高，尤其是在发生 ICH 后。CAA 病理学可能在首次临床表现前数年就已开始。遗传性 CAA 的最初迹象是白质病变、皮质微梗死和枕叶脑血管反应受损。可见的半卵圆中心血管周围间隙、位于脑叶的腔隙和皮质萎缩是新的非出血性 MRI 标志物。
CAA 应列入老年患者脑叶 ICH 的鉴别诊断，但也应列入认知能力下降和短暂性局灶性神经症状的患者。医生应该意识到 CAA 的认知影响。在有既往 ICH、cSS 或 cSAH 的患者中，应考虑抗凝治疗存在风险。越来越多的 MRI 标志物可能有助于将 CAA 与其他小血管疾病和痴呆亚型区分开来。
