CURRENT OVERVIEW OF CLINICAL AND RADIOLOGICAL FINDINGS ASSOCIATED WITH CEREBRAL AMYLOID ANGIOPATHY

Year 2024, Volume: 5 Issue: 2, 104 - 113, 31.05.2024

Betül Kızıldağ , Nursel Yurttutan

https://doi.org/10.51972/tfsd.1448781

Abstract

Cerebral amyloid angipopathy (CAA), the second most common cause of spontaneous acute intracerebral hemorrhage after hypertension in the elderly population is characterized by brain parenchymal damage secondary to hemorrhage and ischemia caused by the accumulation of Aβ protein in the walls of small arteries and arterioles. Advanced age is the most significant risk factor for CAA. While the definitive diagnosis requires histopathological examination through autopsy/biopsy, the probable or possible diagnosis of CAA is based on clinical features as well as characteristic neuroimaging findings.
With the increasing elderly population and the growing prevalence of succesibility-weighted magnetic resonance imaging sequences in routine, it becomes imperative to have a thorough understanding of the imaging spectrum associated with CAA. Early diagnosis is extremely critical in patients with CAA who have not yet developed intracranial hemorrhage. Furthermore, patients with CAA may present clinically transient focal neurological episodes or cognitive impairment, which can be mistaken for transient ischemic attacks caused by convexity subarachnoid hemorrhage. Additionally, before initiating newly introduced anti-amyloid monoclonal antibody drugs in Alzheimer's disease, it is necessary to exclude signs of CAA. Moreover, the anticipated side effects of these drugs often manifest imaging abnormalities resembling inflammation or bleeding associated with CAA, necessitating familiarity with the imaging findings of CAA. In this review, the pathophysiology, clinical manifestations, and radiological findings of sporadic CAA will be detailed.

Keywords

cerebral amyloid angiopathy, suscebility weighted imaging, cerebral bleeding

References

• Biffi, A., Halpin, A., Towfighi, A., Gilson, A., Busl, K., Rost, N., ... & Viswanathan, A. (2010). Aspirin and recurrent intracerebral hemorrhage in cerebral amyloid angiopathy. Neurology, 75(8), 693-698. DOI: 10.1212/WNL.0b013e3181eee40f
• Biffi, A., & Greenberg, S. M. (2011). Cerebral amyloid angiopathy: a systematic review. Journal of clinical neurology (Seoul, Korea), 7(1), 1. DOI: 10.3988/jcn.2011.7.1.1
• Brashear, H. R., Ketter, N., Bogert, J., Di, J., Salloway, S. P., & Sperling, R. (2018). Clinical evaluation of amyloid-related imaging abnormalities in bapineuzumab phase III studies. Journal of Alzheimer's Disease, 66(4), 1409-1424. DOI: 10.3233/JAD-180675
• Charidimou, A., Gang, Q., & Werring, D. J. (2012a). Sporadic cerebral amyloid angiopathy revisited: recent insights into pathophysiology and clinical spectrum. Journal of Neurology, Neurosurgery & Psychiatry, 83(2), 124-137. DOI: 10.1136/jnnp-2011-301308
• Charidimou, A., Peeters, A., Fox, Z., Gregoire, S. M., Vandermeeren, Y., Laloux, P., ... & Werring, D. J. (2012b). Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre magnetic resonance imaging cohort study and meta-analysis. Stroke, 43(9), 2324-2330. DOI: 10.1161/STROKEAHA.112.657759
• Charidimou, A., Jaunmuktane, Z., Baron, J. C., Burnell, M., Varlet, P., Peeters, A., ... & Werring, D. J. (2014). White matter perivascular spaces: an MRI marker in pathology-proven cerebral amyloid angiopathy?. Neurology, 82(1), 57-62. DOI: 10.1212/01.wnl.0000438225.02729.04
• Charidimou, A., Martinez-Ramirez, S., Shoamanesh, A., Oliveira-Filho, J., Frosch, M., Vashkevich, A., ... & Viswanathan, A. (2015a). Cerebral amyloid angiopathy with and without hemorrhage: evidence for different disease phenotypes. Neurology, 84(12), 1206-1212. DOI: 10.1212/WNL.0000000000001398
• Charidimou, A., Linn, J., Vernooij, M. W., Opherk, C., Akoudad, S., Baron, J. C., ... & Werring, D. J. (2015b). Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. Brain, 138(8), 2126-2139. DOI: 10.1093/brain/awv162
• Charidimou, A., Boulouis, G., Frosch, M. P., Baron, J. C., Pasi, M., Albucher, J. F., ... & Greenberg, S. M. (2022). The Boston criteria version 2.0 for cerebral amyloid angiopathy: a multicentre, retrospective, MRI–neuropathology diagnostic accuracy study. The Lancet Neurology, 21(8), 714-725. DOI: 10.1016/S1474-4422(22)00208-3
• Ellis, R. J., Olichney, J. M., Thal, L. J., Mirra, S. S., Morris, J. C., Beekly, D., & Heyman, A. (1996). Cerebral amyloid angiopathy in the brains of patients with Alzheimer's disease: the CERAD experience, Part XV. Neurology, 46(6), 1592-1596.doi:10.1212/wnl.46.6.1592.
• Eng, J. A., Frosch, M. P., Choi, K., Rebeck, G. W., & Greenberg, S. M. (2004). Clinical manifestations of cerebral amyloid angiopathy–related inflammation. Annals of Neurology: Official Journal of the American Neurological Association and the Child Neurology Society, 55(2), 250-256. doi: 10.1002/ana.10810.
• Greenberg, S. M., & Vonsattel, J. P. G. (1997). Diagnosis of cerebral amyloid angiopathy: sensitivity and specificity of cortical biopsy. Stroke, 28(7), 1418-1422. doi: 10.1161/01.str.28.7.1418.
• Greenberg, C. H., Frosch, M. P., Goldstein, J. N., Rosand, J., & Greenberg, S. M. (2012). Modeling intracerebral hemorrhage growth and response to anticoagulation. PLoS One, 7(10), e48458. doi: 10.1371/journal.pone.0048458.
• Greenberg, S. M., & Charidimou, A. (2018). Diagnosis of cerebral amyloid angiopathy: evolution of the Boston criteria. Stroke, 49(2), 491-497. DOI: 10.1161/STROKEAHA.117.016990
• Greenberg, S. M., Bacskai, B. J., Hernandez-Guillamon, M., Pruzin, J., Sperling, R., & van Veluw, S. J. (2020). Cerebral amyloid angiopathy and Alzheimer disease—one peptide, two pathways. Nature Reviews Neurology, 16(1), 30-42. DOI: 10.1038/s41582-019-0281-2
• Hawkes, C. A., Jayakody, N., Johnston, D. A., Bechmann, I., & Carare, R. O. (2014). Failure of perivascular drainage of β‐amyloid in cerebral amyloid angiopathy. Brain Pathology, 24(4), 396-403. DOI: 10.1111/bpa.12159
• Kimberly W.T., Gilson A, Rost N.S. et al., (2019). Silent ischemic infarcts are associated with hemorrhage burden in cerebral amyloid angiopathy. Neurology. 72(14), 1230-5. DOI: 10.1212/01.wnl.0000345666.83318.03.
• Koo, H. W., Jo, K. I., Yeon, J. Y., Kim, J. S., & Hong, S. C. (2016). Clinical features of high-degree centrum semiovale-perivascular spaces in cerebral amyloid angiopathy. Journal of the neurological sciences, 367, 89-94. DOI: 10.1016/j.jns.2016.05.040
• Lee, J. M., & Markus, H. S. (2006). Does the white matter matter in Alzheimer disease and cerebral amyloid angiopathy?. Neurology, 66(1), 6-7. . DOI: 10.1212/01.wnl.0000196863.43951.44
• Maia, L. F., Mackenzie, I. R., & Feldman, H. H. (2007). Clinical phenotypes of cerebral amyloid angiopathy. Journal of the neurological sciences, 257(1-2), 23-30. DOI: 10.1016/j.jns.2007.01.054
• Martinez-Ramirez, S., Pontes-Neto, O. M., Dumas, A. P., Auriel, E., Halpin, A., Quimby, M., ... & Viswanathan, A. (2013). Topography of dilated perivascular spaces in subjects from a memory clinic cohort. Neurology, 80(17), 1551-1556. DOI: 10.1212/WNL.0b013e31828f1876
• Morrone, C. D., Liu, M., Black, S. E., & McLaurin, J. (2015). Interaction between therapeutic interventions for Alzheimer’s disease and physiological Aβ clearance mechanisms. Frontiers in Aging Neuroscience, 7, 64. DOI: 10.3389/fnagi.2015.00064
• Poels, M. M., Ikram, M. A., van der Lugt, A., Hofman, A., Niessen, W. J., Krestin, G. P., ... & Vernooij, M. W. (2012). Cerebral microbleeds are associated with worse cognitive function: the Rotterdam Scan Study. Neurology, 78(5), 326-333. DOI: 10.1212/WNL.0b013e3182452928
• Revesz, T., Holton, J. L., Lashley, T., Plant, G., Rostagno, A., Ghiso, J., & Frangione, B. (2002). Sporadic and familial cerebral amyloid angiopathies. Brain pathology, 12(3), 343-357. doi: 10.1111/j.1750-3639.2002.tb00449.x.
• Schweser, F., Deistung, A., Lehr, B. W., & Reichenbach, J. R. (2010). Differentiation between diamagnetic and paramagnetic cerebral lesions based on magnetic susceptibility mapping. Medical physics, 37(10), 5165-5178. DOI: 10.1118/1.3481505
• Sharma, R., Dearaugo, S., Infeld, B., O'Sullivan, R., & Gerraty, R. P. (2018). Cerebral amyloid angiopathy: review of clinico‐radiological features and mimics. Journal of Medical Imaging and Radiation Oncology, 62(4), 451-463. DOI: 10.1111/1754-9485.12726
• Smith, E. E., Charidimou, A., Ayata, C., Werring, D. J., & Greenberg, S. M. (2021). Cerebral amyloid angiopathy–related transient focal neurologic episodes. Neurology, 97(5), 231-238. DOI: 10.1212/WNL.0000000000012234
• Tarasoff-Conway, J. M., Carare, R. O., Osorio, R. S., Glodzik, L., Butler, T., Fieremans, E., ... & De Leon, M. J. (2015). Clearance systems in the brain—implications for Alzheimer disease. Nature reviews neurology, 11(8), 457-470. DOI: 10.1038/nrneurol.2015.119
• Vinters, H. V. (1987). Cerebral amyloid angiopathy. A critical review. Stroke, 18(2), 311-324. https://doi.org/10.1161/01.STR.18.2.311
• Viswanathan, A., & Greenberg, S. M. (2011). Cerebral amyloid angiopathy in the elderly. Annals of neurology, 70(6), 871-880. DOI: 10.1002/ana.22516
• Withington, C. G., & Turner, R. S. (2022). Amyloid-related imaging abnormalities with anti-amyloid antibodies for the treatment of dementia due to Alzheimer's disease. Frontiers in Neurology, 13, 862369. DOI: 10.3389/fneur.2022.862369