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Brain
Diagnosis
Pathology-Based Diagnoses
Stroke
Nonatheromatous Vasculopathy
Cerebral Amyloid Disease
Cerebral Amyloid Disease
Karen L. Salzman, MD
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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

                                          1. Gatti L et al: Understanding the pathophysiology of cerebral amyloid angiopathy. Int J Mol Sci. 21(10), 2020
                                          2. Gurol ME et al: Advanced neuroimaging to unravel mechanisms of cerebral small vessel diseases. Stroke. 51(1):29-37, 2020
                                          3. Gurol ME et al: Multiple faces of cerebral small vessel diseases. Stroke. 51(1):9-11, 2020
                                          4. Kuhn J et al: Cerebral amyloid angiopathy, 2020
                                          5. 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
                                          6. Akers C et al: Atypical clinical manifestations of cerebral amyloid angiopathy. Curr Neurol Neurosci Rep. 19(9):64, 2019
                                          7. Chen SJ et al: Advances in cerebral amyloid angiopathy imaging. Ther Adv Neurol Disord. 12:1756286419844113, 2019
                                          8. Greenberg SM et al: Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 49(2):491-7, 2018
                                          9. Wermer MJH et al: The growing clinical spectrum of cerebral amyloid angiopathy. Curr Opin Neurol. 31(1):28-35, 2018
                                          10. Beitzke M et al: Contribution of convexal subarachnoid hemorrhage to disease progression in cerebral amyloid angiopathy. Stroke. 46(6):1533-40, 2015
                                          11. Charidimou A et al: Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology. 84(12):1206-12, 2015
                                          12. 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
                                          13. Domingues R et al: Diagnostic evaluation for nontraumatic intracerebral hemorrhage. Neurol Clin. 33(2):315-328, 2015
                                          14. 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
                                          15. 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
                                          16. 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
                                          17. 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
                                          18. Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke. 17(1):17-30, 2015
                                          19. Milner E et al: Cerebral amyloid angiopathy increases susceptibility to infarction after focal cerebral ischemia in Tg2576 mice. Stroke. 45(10):3064-9, 2014
                                          20. Shoamanesh A et al: Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy. Neurology. 83(20):1838-43, 2014
                                          21. 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
                                          22. Greenberg SM et al: Microbleeds versus macrobleeds: evidence for distinct entities. Stroke. 40(7):2382-6, 2009
                                          23. Mittal S et al: Susceptibility-weighted imaging: technical aspects and clinical applications, part 2. AJNR Am J Neuroradiol. 30(2):232-52, 2009
                                          24. 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
                                          25. 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
                                          26. Soontornniyomkij V et al: Cerebral microinfarcts associated with severe cerebral beta-amyloid angiopathy. Brain Pathol. Epub ahead of print, 2009
                                          27. Tschampa HJ et al: MRI in amyloid beta--related brain angiitis. Neurology. 73(3):247, 2009
                                          28. Kinnecom C et al: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 68(17):1411-6, 2007
                                          29. 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
                                          30. Chalela JA et al: Multiple cerebral microbleeds: MRI marker of a diffuse hemorrhage-prone state. J Neuroimaging. 14(1):54-7, 2004
                                          31. Eng JA et al: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 55(2):250-6, 2004
                                          32. Georgiades CS et al: Amyloidosis: review and CT manifestations. Radiographics. 24(2):405-16, 2004
                                          33. 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
                                          34. 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
                                          35. Dichgans M et al: Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study. Stroke. 33(1):67-71, 2002
                                          36. Gallucci M et al: Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology. 44(4):333-7, 2002
                                          37. Pfeifer LA et al: Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology. 58(11):1629-34, 2002
                                          38. Yamada M: Risk factors for cerebral amyloid angiopathy in the elderly. Ann N Y Acad Sci. 977:37-44, 2002
                                          39. 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
                                          40. 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
                                          41. Greenberg SM et al: MRI detection of new hemorrhages: potential marker of progression in cerebral amyloid angiopathy. Neurology. 53(5):1135-8, 1999
                                          42. McCarron MO et al: Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors. Stroke. 30(8):1643-6, 1999
                                          43. 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

                                                                                  1. Gatti L et al: Understanding the pathophysiology of cerebral amyloid angiopathy. Int J Mol Sci. 21(10), 2020
                                                                                  2. Gurol ME et al: Advanced neuroimaging to unravel mechanisms of cerebral small vessel diseases. Stroke. 51(1):29-37, 2020
                                                                                  3. Gurol ME et al: Multiple faces of cerebral small vessel diseases. Stroke. 51(1):9-11, 2020
                                                                                  4. Kuhn J et al: Cerebral amyloid angiopathy, 2020
                                                                                  5. 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
                                                                                  6. Akers C et al: Atypical clinical manifestations of cerebral amyloid angiopathy. Curr Neurol Neurosci Rep. 19(9):64, 2019
                                                                                  7. Chen SJ et al: Advances in cerebral amyloid angiopathy imaging. Ther Adv Neurol Disord. 12:1756286419844113, 2019
                                                                                  8. Greenberg SM et al: Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke. 49(2):491-7, 2018
                                                                                  9. Wermer MJH et al: The growing clinical spectrum of cerebral amyloid angiopathy. Curr Opin Neurol. 31(1):28-35, 2018
                                                                                  10. Beitzke M et al: Contribution of convexal subarachnoid hemorrhage to disease progression in cerebral amyloid angiopathy. Stroke. 46(6):1533-40, 2015
                                                                                  11. Charidimou A et al: Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology. 84(12):1206-12, 2015
                                                                                  12. 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
                                                                                  13. Domingues R et al: Diagnostic evaluation for nontraumatic intracerebral hemorrhage. Neurol Clin. 33(2):315-328, 2015
                                                                                  14. 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
                                                                                  15. 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
                                                                                  16. 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
                                                                                  17. 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
                                                                                  18. Yamada M: Cerebral amyloid angiopathy: emerging concepts. J Stroke. 17(1):17-30, 2015
                                                                                  19. Milner E et al: Cerebral amyloid angiopathy increases susceptibility to infarction after focal cerebral ischemia in Tg2576 mice. Stroke. 45(10):3064-9, 2014
                                                                                  20. Shoamanesh A et al: Interrelationship of superficial siderosis and microbleeds in cerebral amyloid angiopathy. Neurology. 83(20):1838-43, 2014
                                                                                  21. 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
                                                                                  22. Greenberg SM et al: Microbleeds versus macrobleeds: evidence for distinct entities. Stroke. 40(7):2382-6, 2009
                                                                                  23. Mittal S et al: Susceptibility-weighted imaging: technical aspects and clinical applications, part 2. AJNR Am J Neuroradiol. 30(2):232-52, 2009
                                                                                  24. 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
                                                                                  25. 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
                                                                                  26. Soontornniyomkij V et al: Cerebral microinfarcts associated with severe cerebral beta-amyloid angiopathy. Brain Pathol. Epub ahead of print, 2009
                                                                                  27. Tschampa HJ et al: MRI in amyloid beta--related brain angiitis. Neurology. 73(3):247, 2009
                                                                                  28. Kinnecom C et al: Course of cerebral amyloid angiopathy-related inflammation. Neurology. 68(17):1411-6, 2007
                                                                                  29. 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
                                                                                  30. Chalela JA et al: Multiple cerebral microbleeds: MRI marker of a diffuse hemorrhage-prone state. J Neuroimaging. 14(1):54-7, 2004
                                                                                  31. Eng JA et al: Clinical manifestations of cerebral amyloid angiopathy-related inflammation. Ann Neurol. 55(2):250-6, 2004
                                                                                  32. Georgiades CS et al: Amyloidosis: review and CT manifestations. Radiographics. 24(2):405-16, 2004
                                                                                  33. 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
                                                                                  34. 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
                                                                                  35. Dichgans M et al: Cerebral microbleeds in CADASIL: a gradient-echo magnetic resonance imaging and autopsy study. Stroke. 33(1):67-71, 2002
                                                                                  36. Gallucci M et al: Neuroradiological findings in two cases of isolated amyloidoma of the central nervous system. Neuroradiology. 44(4):333-7, 2002
                                                                                  37. Pfeifer LA et al: Cerebral amyloid angiopathy and cognitive function: the HAAS autopsy study. Neurology. 58(11):1629-34, 2002
                                                                                  38. Yamada M: Risk factors for cerebral amyloid angiopathy in the elderly. Ann N Y Acad Sci. 977:37-44, 2002
                                                                                  39. 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
                                                                                  40. 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
                                                                                  41. Greenberg SM et al: MRI detection of new hemorrhages: potential marker of progression in cerebral amyloid angiopathy. Neurology. 53(5):1135-8, 1999
                                                                                  42. McCarron MO et al: Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors. Stroke. 30(8):1643-6, 1999
                                                                                  43. 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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