cerebral amyloid angiopathy
Definition: Cerebral amyloid angiopathy (CAA) is a histopathological term referring to the deposition of protein A? in the blood vessel walls of the brain (angiopathy), thereby predisposing individuals to brain bleeding (haemorrhagic strokes).
Severe CAA and haemorrhagic stroke are characteristic features of mutations affecting the ?-secretase-cleavage site of APP. CAA is a frequent observation in AD brains but usually does not result in strokes.
Etiology
duplication of the APP locus on chromosome 21 in autosomal dominant early-onset Alzheimer disease (ADEOAD) and cerebral amyloid angiopathy (CAA)
- Brains from individuals with APP duplication showed abundant parenchymal and vascular deposits of amyloid-beta peptides.
- Duplication of the APP locus, resulting in accumulation of amyloid-beta peptides, causes ADEOAD with CAA.
References
Rovelet-Lecrux A, Hannequin D, Raux G, Le Meur N, Laquerriere A, Vital A, Dumanchin C, Feuillette S, Brice A, Vercelletto M, Dubas F, Frebourg T, Campion D. APP locus duplication causes autosomal dominant early-onset Alzheimer disease with cerebral amyloid angiopathy. Nat Genet. 2006 Jan;38(1):24-6. PMID: 16369530
Revesz T, Ghiso J, Lashley T, Plant G, Rostagno A, Frangione B, Holton JL. Cerebral amyloid angiopathies: a pathologic, biochemical, and genetic view. J Neuropathol Exp Neurol. 2003 Sep;62(9):885-98. PMID: 14533778