J Clin Neurol.  2011 Mar;7(1):1-9. 10.3988/jcn.2011.7.1.1.

Cerebral Amyloid Angiopathy: A Systematic Review

• Biffi A
• Greenberg

• KMID: 1452566
• DOI: http://doi.org/10.3988/jcn.2011.7.1.1

Abstract

Cerebral amyloid angiopathy (CAA) is a disorder characterized by amyloid deposition in the walls of leptomeningeal and cortical arteries, arterioles, and less often capillaries and veins of the central nervous system. CAA occurs mostly as a sporadic condition in the elderly, its incidence associating with advancing age. All sporadic CAA cases are due to deposition of amyloid-beta, originating from proteolytic cleavage of the Amyloid Precursor Protein. Hereditary forms of CAA are generally familial (and therefore rare in the general population), more severe and earlier in onset. CAA-related lobar intracerebral hemorrhage is the most well-studied clinical condition associated with brain amyloid deposition. Despite ever increasing understanding of CAA pathogenesis and availability of reliable clinical and diagnostic tools, preventive and therapeutic options remain very limited. Further research efforts are required in order to identify biological targets for novel CAA treatment strategies. We present a systematic review of existing evidence regarding the epidemiology, genetics, pathogenesis, diagnosis and clinical management of CAA.

Keyword

cerebral amyloid angiopathy; cerebral hemorrhage; dementia

MeSH Terms

Aged
Amyloid
Arteries
Arterioles
Brain
Capillaries
Central Nervous System
Cerebral Amyloid Angiopathy
Cerebral Hemorrhage
Congenital Abnormalities
Dementia
Ear
Humans
Incidence
Plaque, Amyloid
Veins
Amyloid
Cerebral Amyloid Angiopathy
Congenital Abnormalities
Ear

Figure

• 

  Fig. 1 Mutations in the APP gene and their relationship to the amino acid sequence of the Aβ peptide. APP: amyloid precursor protein.

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