Cerebral amyloid angiopathy associated with inflammation: A systematic review of clinical and imaging features and outcome
- 14 November 2017
- journal article
- review article
- Published by SAGE Publications in International Journal of Stroke
- Vol. 13 (3), 257-267
- https://doi.org/10.1177/1747493017741569
Abstract
Cerebral amyloid angiopathy associated with inflammation is an increasingly recognized condition, characterized by an inflammatory response to the vascular deposits of β-amyloid within the brain that are the hallmark of cerebral amyloid angiopathy. Two main patterns of this inflammatory response have been identified to date: one involving a perivascular inflammatory cell infiltrate (cerebral amyloid angiopathy-related inflammation); the other a transmural vasculitic process (A-beta related angiitis). Unlike cerebral amyloid angiopathy itself, which predisposes to intracerebral hemorrhage and has no known treatment, cerebral amyloid angiopathy associated with inflammation typically presents in diverse ways and diagnosis may be challenging and delayed. We sought to summarize the clinical features, imaging appearances and available data on outcome and treatment responses, using information derived from a systematic review of pathologically proven cases of cerebral amyloid angiopathy associated with inflammation. We identified 213 distinct pathologically proven cases of cerebral amyloid angiopathy-related inflammation/A-beta related angiitis, from 104 publications. The clinical presentation, imaging features, pathology, treatment, and outcomes of cerebral amyloid angiopathy associated with inflammation are described. Cerebral amyloid angiopathy associated with inflammation is an important and increasingly recognized clinical condition, which affects the older patient population and presents most commonly with cognitive decline, seizures, and headaches. Future research is required to develop and validate diagnostic criteria and determine optimum treatment strategies.Keywords
This publication has 99 references indexed in Scilit:
- Immune Activation in Amyloid-β-Related Angiitis Correlates with Decreased Parenchymal Amyloid-β Plaque LoadNeurodegenerative Diseases, 2013
- Corticosteroid therapy in a patient with cerebral amyloid angiopathy-related inflammationJournal of Neuroinflammation, 2013
- SMASH-UStroke, 2012
- Amyloidβ-Related Angiitis Causing Coma Responsive to ImmunosuppressionCase Reports in Pathology, 2012
- Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literatureJournal of Neuroinflammation, 2011
- Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatmentJournal of Neuroinflammation, 2010
- MRI in amyloid β–related brain angiitisNeurology, 2009
- Immune Reactions Associated With Cerebral Amyloid AngiopathyStroke, 1996
- Cerebral amyloid angiopathy presenting as a mass lesion.Stroke, 1987
- Cerebral amyloid angiopathy associated with giant cell arteritis: a case report.Stroke, 1985