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DOI: 10.1148/rg.265055090
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Cerebral Amyloid Angiopathy: CT and MR Imaging Findings1

Christine P. Chao, MD, Amy L. Kotsenas, MD and Daniel F. Broderick, MD

1 From the Department of Radiology, Mayo Clinic, 4500 San Pablo Rd, Jacksonville, FL 32224. Recipient of a Certificate of Merit award for an education exhibit at the 2004 RSNA Annual Meeting. Received April 17, 2005; revision requested July 12 and received September 8; accepted September 14. All authors have no financial relationships to disclose.

Figure 1
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Figure 1a.  Histologic appearance of ß-amyloid deposition in cerebral cortical vessels. (a) Photomicrograph (original magnification, x100; Congo red stain) shows highlighted ß-amyloid deposits along the vessel walls. (b) Photomicrograph (original magnification, x100; Congo red stain) obtained with polarized light shows the classic yellow-green birefringence of the ß-amyloid deposits.

 

Figure 1
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Figure 1b.  Histologic appearance of ß-amyloid deposition in cerebral cortical vessels. (a) Photomicrograph (original magnification, x100; Congo red stain) shows highlighted ß-amyloid deposits along the vessel walls. (b) Photomicrograph (original magnification, x100; Congo red stain) obtained with polarized light shows the classic yellow-green birefringence of the ß-amyloid deposits.

 

Figure 2
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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.

 

Figure 3
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Figure 3a.  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.

 

Figure 3
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Figure 3b.  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.

 

Figure 4
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Figure 4a.  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.

 

Figure 4
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Figure 4b.  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.

 

Figure 4
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Figure 4c.  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.

 

Figure 5
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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 x 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.

 

Figure 6
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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.

 

Figure 7
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Figure 7a.  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).

 

Figure 7
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Figure 7b.  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).

 

Figure 7
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Figure 7c.  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).

 

Figure 8
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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.

 

Figure 9
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Figure 9a.  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.

 

Figure 9
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Figure 9b.  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.

 

Figure 9
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Figure 9c.  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.

 

Figure 10
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Figure 10a.  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.

 

Figure 10
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Figure 10b.  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.

 

Figure 10
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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.

 

Figure 11
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Figure 11a.  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.

 

Figure 11
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Figure 11b.  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.

 

Figure 12
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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.

 

Figure 13
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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.

 

Figure 14
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Figure 14a.  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).

 

Figure 14
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Figure 14b.  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).

 





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