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KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
Cerebral amyloid angiopathy (CAA)
Synonyms
"Congophilic angiopathy," cerebral amyloidosis
Definitions
CAA is common cause of "spontaneous" lobar hemorrhage in elderly
Cerebral amyloid deposition occurs in 3 morphologic varieties
CAA (common)
Amyloidoma (uncommon)
Inflammatory CAA: Amyloid β-related angiitis (ABRA) with diffuse white matter (WM) inflammatory involvement (rare)
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Gatti L et al: Understanding the pathophysiology of cerebral amyloid angiopathy. Int J Mol Sci. 21(10), 2020
Gurol ME et al: Advanced neuroimaging to unravel mechanisms of cerebral small vessel diseases. Stroke. 51(1):29-37, 2020
Gurol ME et al: Multiple faces of cerebral small vessel diseases. Stroke. 51(1):9-11, 2020
Kuhn J et al: Cerebral amyloid angiopathy, 2020
Tsai HH et al: Superficial cerebellar microbleeds and cerebral amyloid angiopathy: a magnetic resonance imaging/positron emission tomography study. Stroke. 51(1):202-8, 2020
Akers C et al: Atypical clinical manifestations of cerebral amyloid angiopathy. Curr Neurol Neurosci Rep. 19(9):64, 2019
Chen SJ et al: Advances in cerebral amyloid angiopathy imaging. Ther Adv Neurol Disord. 12:1756286419844113, 2019
Greenberg SM et al: Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 49(2):491-7, 2018
Wermer MJH et al: The growing clinical spectrum of cerebral amyloid angiopathy. Curr Opin Neurol. 31(1):28-35, 2018
Beitzke M et al: Contribution of convexal subarachnoid hemorrhage to disease progression in cerebral amyloid angiopathy. Stroke. 46(6):1533-40, 2015
Charidimou A et al: Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology. 84(12):1206-12, 2015
Ding J et al: Risk factors associated with incident cerebral microbleeds according to location in older people: the age, gene/environment susceptibility (AGES)-Reykjavik study. JAMA Neurol. 72(6):682-8, 2015
Domingues R et al: Diagnostic evaluation for nontraumatic intracerebral hemorrhage. Neurol Clin. 33(2):315-328, 2015
Esiri M et al: Cerebral amyloid angiopathy, subcortical white matter disease and dementia: literature review and study in OPTIMA. Brain Pathol. 25(1):51-62, 2015
Murray ME et al: Clinicopathologic and 11C-Pittsburgh compound B implications of Thal amyloid phase across the Alzheimer's disease spectrum. Brain. 138(Pt 5):1370-81, 2015
Nayate AP et al: Use of standardized uptake value ratios decreases interreader variability of [18F] Florbetapir PET brain scan interpretation. AJNR Am J Neuroradiol. 36(7):1237-44, 2015
Shams S et al: Cerebral microbleeds: different prevalence, topography, and risk factors depending on dementia diagnosis-the Karolinska Imaging Dementia study. AJNR Am J Neuroradiol. 36(4):661-6, 2015
Milner E et al: Cerebral amyloid angiopathy increases susceptibility to infarction after focal cerebral ischemia in Tg2576 mice. Stroke. 45(10):3064-9, 2014
Shoamanesh A et al: Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy. Neurology. 83(20):1838-43, 2014
Alcalay RN et al: MRI showing white matter lesions and multiple lobar microbleeds in a patient with reversible encephalopathy. J Neuroimaging. 19(1):89-91, 2009
Greenberg SM et al: Microbleeds versus macrobleeds: evidence for distinct entities. Stroke. 40(7):2382-6, 2009
Mittal S et al: Susceptibility-weighted imaging: technical aspects and clinical applications, part 2. AJNR Am J Neuroradiol. 30(2):232-52, 2009
Nandigam RN et al: MR imaging detection of cerebral microbleeds: effect of susceptibility-weighted imaging, section thickness, and field strength. AJNR Am J Neuroradiol. 30(2):338-43, 2009
Santhosh K et al: Susceptibility weighted imaging: a new tool in magnetic resonance imaging of stroke. Clin Radiol. 2009 Jan;64(1):74-83. Epub 2008 Aug 21. Review. Erratum in: Clin Radiol. 64(6):653, 2009
Soontornniyomkij V et al: Cerebral microinfarcts associated with severe cerebral beta-amyloid angiopathy. Brain Pathol. Epub ahead of print, 2009
Tschampa HJ et al: MRI in amyloid beta--related brain angiitis. Neurology. 73(3):247, 2009
Kinnecom C et al: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 68(17):1411-6, 2007
Arboix A et al: New concepts in lacunar stroke etiology: the constellation of small-vessel arterial disease. Cerebrovasc Dis. 17 Suppl 1:58-62, 2004
Chalela JA et al: Multiple cerebral microbleeds: MRI marker of a diffuse hemorrhage-prone state. J Neuroimaging. 14(1):54-7, 2004
Eng JA et al: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 55(2):250-6, 2004
Georgiades CS et al: Amyloidosis: review and CT manifestations. Radiographics. 24(2):405-16, 2004
Tian J et al: Relationships between arteriosclerosis, cerebral amyloid angiopathy and myelin loss from cerebral cortical white matter in Alzheimer's disease. Neuropathol Appl Neurobiol. 30(1):46-56, 2004
Gandhi D et al: CT and MR imaging of intracerebral amyloidoma: case report and review of the literature. AJNR Am J Neuroradiol. 24(3):519-22, 2003
Dichgans M et al: Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study. Stroke. 33(1):67-71, 2002
Gallucci M et al: Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology. 44(4):333-7, 2002
Pfeifer LA et al: Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology. 58(11):1629-34, 2002
Yamada M: Risk factors for cerebral amyloid angiopathy in the elderly. Ann N Y Acad Sci. 977:37-44, 2002
Caulo M et al: Cerebral amyloid angiopathy presenting as nonhemorrhagic diffuse encephalopathy: neuropathologic and neuroradiologic manifestations in one case. AJNR Am J Neuroradiol. 22(6):1072-6, 2001
Fazekas F et al: Histopathologic analysis of foci of signal loss on gradient-echo T2*-weighted MR images in patients with spontaneous intracerebral hemorrhage: evidence of microangiopathy-related microbleeds. AJNR Am J Neuroradiol. 20(4):637-42, 1999
Greenberg SM et al: MRI detection of new hemorrhages: potential marker of progression in cerebral amyloid angiopathy. Neurology. 53(5):1135-8, 1999
McCarron MO et al: Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors. Stroke. 30(8):1643-6, 1999
Miller JH et al: Intracerebral haemorrhage and cerebral amyloid angiopathy: CT features with pathological correlation. Clin Radiol. 54(7):422-9, 1999
Related Anatomy
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Related Differential Diagnoses
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References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Clinical Issues
TERMINOLOGY
Abbreviations
Cerebral amyloid angiopathy (CAA)
Synonyms
"Congophilic angiopathy," cerebral amyloidosis
Definitions
CAA is common cause of "spontaneous" lobar hemorrhage in elderly
Cerebral amyloid deposition occurs in 3 morphologic varieties
CAA (common)
Amyloidoma (uncommon)
Inflammatory CAA: Amyloid β-related angiitis (ABRA) with diffuse white matter (WM) inflammatory involvement (rare)
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Angiographic Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Staging, Grading, & Classification
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Selected References
Gatti L et al: Understanding the pathophysiology of cerebral amyloid angiopathy. Int J Mol Sci. 21(10), 2020
Gurol ME et al: Advanced neuroimaging to unravel mechanisms of cerebral small vessel diseases. Stroke. 51(1):29-37, 2020
Gurol ME et al: Multiple faces of cerebral small vessel diseases. Stroke. 51(1):9-11, 2020
Kuhn J et al: Cerebral amyloid angiopathy, 2020
Tsai HH et al: Superficial cerebellar microbleeds and cerebral amyloid angiopathy: a magnetic resonance imaging/positron emission tomography study. Stroke. 51(1):202-8, 2020
Akers C et al: Atypical clinical manifestations of cerebral amyloid angiopathy. Curr Neurol Neurosci Rep. 19(9):64, 2019
Chen SJ et al: Advances in cerebral amyloid angiopathy imaging. Ther Adv Neurol Disord. 12:1756286419844113, 2019
Greenberg SM et al: Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 49(2):491-7, 2018
Wermer MJH et al: The growing clinical spectrum of cerebral amyloid angiopathy. Curr Opin Neurol. 31(1):28-35, 2018
Beitzke M et al: Contribution of convexal subarachnoid hemorrhage to disease progression in cerebral amyloid angiopathy. Stroke. 46(6):1533-40, 2015
Charidimou A et al: Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology. 84(12):1206-12, 2015
Ding J et al: Risk factors associated with incident cerebral microbleeds according to location in older people: the age, gene/environment susceptibility (AGES)-Reykjavik study. JAMA Neurol. 72(6):682-8, 2015
Domingues R et al: Diagnostic evaluation for nontraumatic intracerebral hemorrhage. Neurol Clin. 33(2):315-328, 2015
Esiri M et al: Cerebral amyloid angiopathy, subcortical white matter disease and dementia: literature review and study in OPTIMA. Brain Pathol. 25(1):51-62, 2015
Murray ME et al: Clinicopathologic and 11C-Pittsburgh compound B implications of Thal amyloid phase across the Alzheimer's disease spectrum. Brain. 138(Pt 5):1370-81, 2015
Nayate AP et al: Use of standardized uptake value ratios decreases interreader variability of [18F] Florbetapir PET brain scan interpretation. AJNR Am J Neuroradiol. 36(7):1237-44, 2015
Shams S et al: Cerebral microbleeds: different prevalence, topography, and risk factors depending on dementia diagnosis-the Karolinska Imaging Dementia study. AJNR Am J Neuroradiol. 36(4):661-6, 2015
Milner E et al: Cerebral amyloid angiopathy increases susceptibility to infarction after focal cerebral ischemia in Tg2576 mice. Stroke. 45(10):3064-9, 2014
Shoamanesh A et al: Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy. Neurology. 83(20):1838-43, 2014
Alcalay RN et al: MRI showing white matter lesions and multiple lobar microbleeds in a patient with reversible encephalopathy. J Neuroimaging. 19(1):89-91, 2009
Greenberg SM et al: Microbleeds versus macrobleeds: evidence for distinct entities. Stroke. 40(7):2382-6, 2009
Mittal S et al: Susceptibility-weighted imaging: technical aspects and clinical applications, part 2. AJNR Am J Neuroradiol. 30(2):232-52, 2009
Nandigam RN et al: MR imaging detection of cerebral microbleeds: effect of susceptibility-weighted imaging, section thickness, and field strength. AJNR Am J Neuroradiol. 30(2):338-43, 2009
Santhosh K et al: Susceptibility weighted imaging: a new tool in magnetic resonance imaging of stroke. Clin Radiol. 2009 Jan;64(1):74-83. Epub 2008 Aug 21. Review. Erratum in: Clin Radiol. 64(6):653, 2009
Soontornniyomkij V et al: Cerebral microinfarcts associated with severe cerebral beta-amyloid angiopathy. Brain Pathol. Epub ahead of print, 2009
Tschampa HJ et al: MRI in amyloid beta--related brain angiitis. Neurology. 73(3):247, 2009
Kinnecom C et al: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 68(17):1411-6, 2007
Arboix A et al: New concepts in lacunar stroke etiology: the constellation of small-vessel arterial disease. Cerebrovasc Dis. 17 Suppl 1:58-62, 2004
Chalela JA et al: Multiple cerebral microbleeds: MRI marker of a diffuse hemorrhage-prone state. J Neuroimaging. 14(1):54-7, 2004
Eng JA et al: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 55(2):250-6, 2004
Georgiades CS et al: Amyloidosis: review and CT manifestations. Radiographics. 24(2):405-16, 2004
Tian J et al: Relationships between arteriosclerosis, cerebral amyloid angiopathy and myelin loss from cerebral cortical white matter in Alzheimer's disease. Neuropathol Appl Neurobiol. 30(1):46-56, 2004
Gandhi D et al: CT and MR imaging of intracerebral amyloidoma: case report and review of the literature. AJNR Am J Neuroradiol. 24(3):519-22, 2003
Dichgans M et al: Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study. Stroke. 33(1):67-71, 2002
Gallucci M et al: Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology. 44(4):333-7, 2002
Pfeifer LA et al: Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology. 58(11):1629-34, 2002
Yamada M: Risk factors for cerebral amyloid angiopathy in the elderly. Ann N Y Acad Sci. 977:37-44, 2002
Caulo M et al: Cerebral amyloid angiopathy presenting as nonhemorrhagic diffuse encephalopathy: neuropathologic and neuroradiologic manifestations in one case. AJNR Am J Neuroradiol. 22(6):1072-6, 2001
Fazekas F et al: Histopathologic analysis of foci of signal loss on gradient-echo T2*-weighted MR images in patients with spontaneous intracerebral hemorrhage: evidence of microangiopathy-related microbleeds. AJNR Am J Neuroradiol. 20(4):637-42, 1999
Greenberg SM et al: MRI detection of new hemorrhages: potential marker of progression in cerebral amyloid angiopathy. Neurology. 53(5):1135-8, 1999
McCarron MO et al: Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors. Stroke. 30(8):1643-6, 1999
Miller JH et al: Intracerebral haemorrhage and cerebral amyloid angiopathy: CT features with pathological correlation. Clin Radiol. 54(7):422-9, 1999
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