Bookmarks
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
- Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke. 17(1):17-30, 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
Loading...
Related Differential Diagnoses
Loading...
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
- Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke. 17(1):17-30, 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
