Cerebral Amyloid Angiopathy: Emerging Concepts
Open Access
- 1 January 2015
- journal article
- review article
- Published by Korean Stroke Society in Journal of Stroke
- Vol. 17 (1), 17-30
- https://doi.org/10.5853/jos.2015.17.1.17
Abstract
Cerebral amyloid angiopathy (CAA) involves cerebrovascular amyloid deposition and is classified into several types according to the amyloid protein involved. Of these, sporadic amyloid β-protein (Aβ)-type CAA is most commonly found in older individuals and in patients with Alzheimer's disease (AD). Cerebrovascular Aβ deposits accompany functional and pathological changes in cerebral blood vessels (CAA-associated vasculopathies). CAA-associated vasculopathies lead to development of hemorrhagic lesions [lobar intracerebral macrohemorrhage, cortical microhemorrhage, and cortical superficial siderosis (cSS)/focal convexity subarachnoid hemorrhage (SAH)], ischemic lesions (cortical infarction and ischemic changes of the white matter), and encephalopathies that include subacute leukoencephalopathy caused by CAA-associated inflammation/angiitis. Thus, CAA is related to dementia, stroke, and encephalopathies. Recent advances in diagnostic procedures, particularly neuroimaging, have enabled us to establish a clinical diagnosis of CAA without brain biopsies. Sensitive magnetic resonance imaging (MRI) methods, such as gradient-echo T2(*) imaging and susceptibility-weighted imaging, are useful for detecting cortical microhemorrhages and cSS. Amyloid imaging with amyloid-binding positron emission tomography (PET) ligands, such as Pittsburgh Compound B, can detect CAA, although they cannot discriminate vascular from parenchymal amyloid deposits. In addition, cerebrospinal fluid markers may be useful, including levels of Aβ40 for CAA and anti-Aβ antibody for CAA-related inflammation. Moreover, cSS is closely associated with transient focal neurological episodes (TFNE). CAA-related inflammation/angiitis shares pathophysiology with amyloid-related imaging abnormalities (ARIA) induced by Aβ immunotherapies in AD patients. This article reviews CAA and CAA-related disorders with respect to their epidemiology, pathology, pathophysiology, clinical features, biomarkers, diagnosis, treatment, risk factors, and future perspectives.Keywords
This publication has 123 references indexed in Scilit:
- Genetic variation at CR1 increases risk of cerebral amyloid angiopathyNeurology, 2012
- Differential recognition of vascular and parenchymal beta amyloid depositionNeurobiology of Aging, 2011
- Distribution of white matter hyperintensity in cerebral hemorrhage and healthy agingZeitschrift für Neurologie, 2011
- APOE genotype and extent of bleeding and outcome in lobar intracerebral haemorrhage: a genetic association studyLancet Neurology, 2011
- Genetic associations with brain microbleedsNeurology, 2011
- Cerebral amyloid angiopathy pathology and cognitive domains in older personsAnnals of Neurology, 2010
- Spatial relation between microbleeds and amyloid deposits in amyloid angiopathyAnnals of Neurology, 2010
- Silent ischemic infarcts are associated with hemorrhage burden in cerebral amyloid angiopathyNeurology, 2009
- Genetics and molecular pathogenesis of sporadic and hereditary cerebral amyloid angiopathiesActa Neuropathologica, 2009
- Impaired visual evoked flow velocity response in cerebral amyloid angiopathyNeurology, 2008