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朗读老师：朱勤勤 衢州市人民医院 
翻译老师：冯璐霏 诸暨市中心医院 
审校老师：姜春雷 青岛市第九人民医院
History: A 73-year-old woman presented with three months of worsening cognitive decline and memory problems. Her cognitive impairment notwithstanding, her physical exam and laboratory results were unremarkable. An electroencephalogram revealed periodic left cerebral epileptiform discharges. An unenhanced CT scan was performed. Click images below to enlarge. 病史：73岁女性，三个月的认知减退和记忆力下降问题。尽管有认知障碍，但体格检查和实验室结果无特殊。脑电图显示左脑有周期性癫痫样放电。行CT平扫 
MR images The patient subsequently underwent a contrast-enhanced MRI scan of the brain. Select images from axial T2-weighted, axial fluid-attenuated inversion-recovery (FLAIR), axial gradient-recalled echo (GRE), axial diffusion-weighted, and axial T1-weighted postcontrast sequences are shown below. Click images to enlarge. 患者随后接受了脑部增强MRI扫描。选取轴位T2加权、T2flair、梯度回波(GRE)、DWI和T1加权序列图像如下所示。 
Findings CT: MRI: Numerous sub-5-mm foci of susceptibility, greatest at the periphery of the cerebral hemispheres Extensive, confluent T2/FLAIR hyperintense signal in the bilateral cerebral hemispheres No abnormal restricted diffusion No abnormal enhancement 无数小于5mm的局灶性磁敏感信号，以大脑半球边缘多见 双侧大脑半球广泛融合的T2/flair高信号 无弥散受限，无异常强化
Differential diagnosis 鉴别诊断 Cerebral amyloid angiopathy (CAA) Inflammatory cerebral amyloid angiopathy (inflammatory CAA)
Cerebral amyloid angiopathy-related inflammation (CAA-RI) Aβ-related angiitis (ABRA) Vasculitis Progressive multifocal leukoencephalopathy (PML) Posterior reversible encephalopathy syndrome (PRES) Neoplasm Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) 脑淀粉样性血管病(CAA) 炎性脑淀粉样血管病（炎性CAA） 脑淀粉样血管病相关炎症（CAA-RI） β淀粉样蛋白相关性血管炎(ABRA) 血管炎 进行性多灶性脑白质病(PML) 可逆性后部脑病综合征(PRES) 肿瘤 伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL)
Diagnosis: Inflammatory cerebral amyloid angiopathy 诊断：炎性脑淀粉样血管病(inflammatory CAA) Cerebral amyloid angiopathy (CAA) Pathophysiology In CAA, there is deposition of amyloid peptides into cerebral arterial and arteriolar walls, weakening those walls and leading to micro- and macrohemorrhages. Deposits stain positive with Congo red. If the deposition leads to inflammation and edema, which is suspected to be an autoimmune reaction to the amyloid, it constitutes inflammatory CAA. If the inflammation is perivascular, it is considered cerebral amyloid angiopathy-related inflammation (CAA-RI). The presence of apolipoprotein E (ApoE) ε4 homozygosity is associated with CAA-RI. If the inflammation is intra- or transmural, it is Aβ-related angiitis (ABRA).
病理生理学 在CAA中，淀粉样蛋白类肽沉积到脑动脉和小动脉壁，使这些壁变脆弱，导致微出血和大出血。刚果红染色阳性。 如果沉积物导致炎症和水肿，考虑为淀粉样蛋白的自身免疫性反应，那么就构成炎性CAA。 如果炎症是血管周围的，则认为是脑淀粉样血管病相关性炎症（CAA-RI）。载脂蛋白E(ApoE)ε4纯合性的存在与CAA-RI有关。 如果炎症是内壁或跨壁炎症，则为β淀粉样蛋白相关性脑血管炎(ABRA)。 Epidemiology Cerebral amyloid angiopathy occurs in patients older than 40 years old, typically the elderly. 流行病学 脑淀粉样性血管病发生在40岁以上的患者中，通常是老年人。 Clinical presentation 临床表现 CAA： 短暂性脑缺血发作样症状 痴呆症炎性CAA： 亚急性神经行为症状 头痛症 癫痫发作 脑卒中样症状伴局灶性缺陷 Imaging features CAA: Multiple micro- or macrohemorrhages, which are often “lobar” in distribution: Hemorrhages are most common in the occipital lobes. Generalized atrophy Leukoencephalopathy sparing the subcortical U-fibers
Inflammatory CAA: Symmetric or asymmetric patchy, confluent T2-hyperintense lesions Leptomeningeal enhancement can be seen 成像特征 CAA： 多发微出血或大出血，通常按“叶分布”：出血最常见于枕叶。 全身性萎缩 皮质下U-纤维不受累的白质脑病 炎性CAA： 对称或不对称的斑片状、融合的T2高信号病变 可以看到软脑膜强化
Differential diagnosis Amyloid angiopathy Cerebral amyloid angiopathy-related inflammation (CAA-RI) Aβ-related angiitis (ABRA) Cerebral amyloid angiopathy Inflammatory cerebral amyloid angiopathy
Vasculitis Progressive multifocal leukoencephalopathy (PML) Posterior reversible encephalopathy syndrome (PRES) Neoplasm Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL)
鉴别诊断 淀粉样血管病 脑淀粉样性血管病 炎性脑淀粉样血管病 脑淀粉样血管病相关性炎症（CAA-RI） β淀粉样蛋白相关性脑血管炎(ABRA) 血管炎 进行性多灶性白质脑病(PML) 可逆性后部脑病综合征(PRES) 肿瘤 伴皮质下梗死和白质脑病的常染色体显性遗传性脑动脉病(CADASIL) Treatment Inflammatory CAA: Improvement is seen with steroids. Occasionally, methotrexate, mycophenolate, cyclophosphamide, and intravenous immunoglobulin can be used.
治疗 炎性CAA： 用类固醇治疗后改善明显 偶尔，也可以使用甲氨蝶呤、霉酚酸酯、环磷酰胺和静脉注射免疫球蛋白。
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