Chapter 4 - Cerebral amyloid angiopathy

Summary

Advanced cerebral amyloid angiopathy (CAA) consists of vascular deposition of amyloid and secondary breakdown of amyloid-laden vessel walls. This chapter focuses on the pathogenesis of CAA, clinical and genetic risk factors, presentations and diagnosis, and prospects for treatment. CAA-related intracerebral hemorrhage (ICH) accounts for a substantial proportion of all spontaneous ICH in the elderly. CAA-related lobar ICH presents similarly to other types of lobar ICH with acute onset of neurological symptoms and the variable presence of headache, seizures, or decreased consciousness according to hemorrhage size and location. CAA-related hemorrhages can also be small and clinically silent. CAA can also present with transient neurological symptoms, another syndrome where diagnosis during life is of particular practical importance. Future treatments for CAA are likely to focus on preventive or protective therapy aimed at decreasing the deposition or toxicity of vascular amyloid.