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脑淀粉样血管病 Cerebral Amyloid Angiopathy

脑淀粉样血管病 Cerebral Amyloid Angiopathy


脑淀粉样血管病:CT和MR影像学表现 Cerebral Amyloid Angiopathy: CT and MR Imaging Findings

脑淀粉样血管病(cerebral amyloid angiopathy,CAA)是以软脑膜和皮质中、小动脉中膜和外膜出现β-淀粉样蛋白(β-amyloid peptide,Aβ)沉积为特征的一种脑血管病变。散发性CAA通常见于60岁以上的老年人,其发病率随着年龄的增长而升高,65~74岁为2.3%,80岁以上为100%。由于70%~97.6%的阿尔茨海默病(Alzheimer's disease,AD)患者存在CAA,因此被认为是AD的形态学标志,但也可见于无临床症状的健康老年人。多数CAA患者临床表现为痴呆、脑出血或短暂性脑缺血发作,其中脑出血是CAA最常见的临床表现。

CAA在组织病理学上可分3级:(1)轻度:淀粉样蛋白局限于血管中膜,但血管平滑肌细胞无明显受损;(2)中度:血管中膜为淀粉样蛋白取代,且较正常变薄;(3)重度:淀粉样蛋白广泛沉积于局部节段血管壁甚至呈双环,微小动脉瘤形成,可见纤维蛋白样坏死,血液经血管壁漏出。也有人根据分布范围将CAA分为3个阶段:(1)起始阶段:主要分布于大脑皮质和软脑膜中、小动脉壁;(2)第二阶段:可见于旧皮质区和小脑血管;(3)最终阶段:可发展到皮质下结构如丘脑、基底节、白质以及脑干。

The Boston Criteria for Diagnosis of CAA


Figure 1.  Histologic appearance of β-amyloid deposition in cerebral cortical vessels. (a) Photomicrograph (original magnification, ×100; Congo red stain) shows highlighted β-amyloid deposits along the vessel walls. (b) Photomicrograph (original magnification, ×100; Congo red stain) obtained with polarized light shows the classic yellow-green birefringence of the β-amyloid deposits.

74岁,男,急性发作的失语,精神错乱,右侧面瘫

Figure 2.  Determination of ICH location in a 74-year-old man with acute onset of expressive aphasia, confusion, and a right-sided facial droop. Axial nonen-hanced CT scan shows a left-sided frontal cortical ICH, a finding most consistent with CAA-related ICH. Pathologic tissue obtained at hematoma evacuation was positive for CAA. The location of an ICH is helpful in determining the cause of the ICH in a patient with a sudden neurologic deficit.


80岁,男,四年来进行性老年痴呆,GRE序列灵敏显示含铁血黄素沉着。

Figure 3.  Sensitivity of GRE imaging for hemosiderin in an 80-year-old man with dementia that has progressed over the past 4 years. (a) Axial GRE MR image shows multiple foci of signal loss in cortical-subcortical locations. In a patient with a diagnosis of probable CAA, these foci are consistent with chronic microhemorrhages. (b) Axial T2-weighted fast spin-echo MR image does not show the foci of chronic microhemorrhage.



65岁,女,复发CAA相关性脑出血,进行性失语,右侧视野缺损,头痛

Figure 4.  Recurrent CAA-related ICH in a 65-year-old woman with progressive aphasia, right visual field deficits, and headache. (a) Axial nonenhanced scan from the initial CT study shows a discrete, ovoid, left-sided occipital ICH. (b) Axial GRE MR image obtained the same day shows numerous cortical-subcortical microhemorrhages, a finding most compatible with a diagnosis of probable CAA. One month later, the patient returned to the emergency department with an increasing level of confusion. (c) Axial nonenhanced CT scan obtained at that time shows a larger, more devastating, left-sided parieto-occipital hemorrhage. Owing to the presence of multiple cortical-subcortical microhemorrhages, which are highly suggestive of CAA, the larger ICH was thought to represent recurrent hemorrhage rather than a hemorrhagic infarction. The patient was not a surgical candidate and was discharged to a hospice 1 week later, where she died after a few days.

81岁,男,CAA相关脑出血、蛛网膜下腔出血,急性失语,躁动

Figure 5.  CAA-related macrohemorrhage with associated subarachnoid hemorrhage in an 81-year-old man with acute dysphasia and agitation. Axial nonenhanced CT scan shows an irregular, 4 × 5-cm, left-sided frontoparietal cortical ICH. The high attenuation in adjacent sulci (arrowheads) is consistent with subarachnoid hemorrhage. The patient had a diagnosis of probable CAA on the basis of a history of two spontaneous right-sided frontal ICHs.

77岁,男,CAA相关脑出血、硬膜下出血,严重的头痛和行走困难

Figure 6.  CAA-related macrohemorrhage with associated subdural hemorrhage in a 77-year-old man with severe headache and difficulty walking. Axial nonenhanced CT scan shows a large right-sided posterior parietal ICH with irregular borders in a cortical location. There is a small right-sided posterior parafalcine subdural hemorrhage (arrow). The large hematoma causes marked effacement of right cerebral sulci and approximately 9 mm of subfalcine herniation. The patient underwent emergency hematoma evacuation; CAA was demonstrated at histologic analysis.



81岁,男,反应迟钝,CAA相关脑出血、脑室内出血

Figure 7.  CAA-related macrohemorrhage with associated intraventricular hemorrhage in an obtunded 81-year-old man. (a) Sagittal nonenhanced T1-weighted MR image shows a large frontal cortical ICH. (b) Axial GRE MR image shows that the right-sided frontal cortical ICH extends to the right lateral ventricle. GRE images also revealed multiple cortical-subcortical microhemorrhages, a finding most consistent with a diagnosis of probable CAA. (c) Axial fluid-attenuated inversion-recovery (FLAIR) MR image shows the more rarely associated intraventricular hemorrhage (arrows) as well as subarachnoid hemorrhage (arrowhead).

76岁,女,记忆力减退,癫痫,头痛,CAA相关的微出血

Figure 8.  CAA-related microhemorrhage in a 76-year-old woman with memory loss, seizures, and headaches. CAA was diagnosed with biopsy at another institution. Axial GRE MR image shows multiple cortical-subcortical microhemorrhages, a finding consistent with CAA.



脑白质病,79岁,女,缓慢进行性痴呆,类似Alzheimer老年痴呆症

Figure 9.  Leukoencephalopathy in a 79-year-old woman with slowly progressive dementia similar to Alzheimer dementia. (a, b) Axial nonenhanced CT scan(a) and FLAIR MR image (b) show symmetric periventricular leukoencephalopathy with sparing of the U fibers, corpus callosum, and internal capsules. The FLAIR image also shows encephalomalacia and hemosiderin from prior macrohemorrhage in the left frontal lobe. (c) Axial GRE MR image shows multiple bilateral cortical foci of hemosiderin, thus increasing the specificity for a diagnosis of probable CAA. The encephalomalacia and hemosiderin in the left frontal lobe are also seen.



脑白质病变,61岁,女,快速进展的认知能力下降

Figure 10c.  Leukoencephalopathy in a 61-year-old woman with rapidly progressive cognitive decline. (a) Axial FLAIR MR image shows asymmetric lobar leukoencephalopathy extending to involve the U fibers and exerting mass effect on the adjacent sulci, most prominently in the posterior left parietal lobe. The absence of signal abnormality at diffusion-weighted MR imaging made an ischemic process or acute infarction unlikely. CAA was diagnosed with biopsy. (b)Axial GRE MR image obtained after biopsy shows a few cortical microhemorrhages (arrows). The patient was treated with a short course of prednisone taper therapy, which started at 40 mg and produced clinical improvement. (c) Follow-up axial FLAIR MR image obtained 1 year later shows near-complete resolution of the leukoencephalopathy. CAA patients with subacute cognitive decline and leukoencephalopathy may respond to immunosuppressive therapy.


72岁,女,言语困难和记忆丧失,可能CAA

Figure 11.  Probable CAA in a 72-year-old woman with speech difficulties and waxing and waning memory loss. (a) Axial FLAIR MR image shows nonspecific atrophy as well as periventricular leukoencephalopathy and prominent left-sided parieto-occipital leukoencephalopathy. (b) Axial GRE MR image shows cortical-subcortical microhemorrhages and a small left-sided parietal cortical-subcortical macrohemorrhage. These findings increase suspicion for probable CAA.

80岁,女,高血压相关脑出血,右侧肢体无力,血压160/85 mmHg

Figure 12.  Hypertension-related macrohemorrhage in an 80-year-old woman with right-sided weakness and a blood pressure of 160/85 mm Hg. Axial nonenhanced CT scan shows an area of increased attenuation in the left thalamus, a finding most consistent with an acute hypertensive ICH.

91岁,女,高血压相关的微出血,患有不稳定性高血压

Figure 13.  Hypertension-related microhemorrhages in a 91-year-old woman with hypertension and unsteadiness. Axial GRE MR image shows multiple small foci of hemosiderin in both basal ganglia and thalami, locations more consistent with a hypertensive cause.


66岁,男,活检提示小细胞肺癌脑转移,头痛,头晕,行走困难。

Figure 14.  Large macrohemorrhage in a 66-year-old man with biopsyproved brain metastases from small cell lung cancer who presented with headache, light-headedness, and difficulty walking. (a) Axial FLAIR MR image shows a large right-sided frontal cortical hematoma with surrounding vasogenic edema. A fluid-fluid level is present, as is often seen in patients undergoing anticoagulation therapy. This patient was taking clopidogrel for a coronary stent. (b) Axial contrast-enhanced T1-weighted MR image shows a second, nonhemorrhagic metastatic lesion in the right temporal lobe (arrow).

http://pubs.rsna.org/doi/full/10.1148/rg.265055090


重庆三峡中心医院放射科

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