Cerebral amyloid angiopathy

BACKGROUND- Cerebral amyloid angiopathy (CAA) is increasingly recognized as an important cause of lobar intracerebral hemorrhage in the elderly and is the only major form of stroke without effective treatment. It is characterized by deposition of congophilic material in small- to medium-sized blood vessels of the brain and leptomeninges. In the most severe stages of the disease, amyloid deposits cause breakdown of the blood vessel wall with consequent hemorrhage. Presently, definitive diagnosis of CAA-related hemorrhage requires examination of the brain at autopsy. REVIEW SUMMARY- This article reviews the pathology and pathogenesis of CAA. To aid in identifying patients at risk for CAA, the range of clinical presentations is discussed. Particular attention is devoted to lobar intracerebral hemorrhage, the most common and devastating manifestation of CAA. We review recent developments in the epidemiology of CAA, particularly the lessons learned from studies of the apolipoprotein E genotype. We conclude with a discussion of antemortem diagnosis, the differential diagnosis and management of CAA, and future directions for the development of treatments. CONCLUSIONS- CAA will become increasingly common as the population ages. Its presence should be considered in any elderly patient with lobar hemorrhage or dementia. Recognition of CAA is particularly important in the setting of anticoagulant therapy, which places patients with CAA at high risk for intracerebral hemorrhage. Finally, patients with CAA-related hemorrhage are at high risk for recurrent hemorrhage.