Sporadic cerebral amyloid angiopathy

Sporadic cerebral amyloid angiopathy (CAA) is a small vessel disease caused by vascular deposits of As-amyloid peptides in the walls of cortical and leptomeningeal vessels. Advancing age is the strongest known clinical risk factor for developing CAA. This devastating disease occurs frequently in elderly people, and is a frequent cause of symptomatic lobar intracerebral hemorrhage (ICH), cognitive impairment and transient focal neurological episodes. Typical cerebral MRI findings include lobar intracerebral hemorrhages and neuroimaging biomarkers of CAA (lobar cerebral microbleeds, cortical superficial siderosis, white matter hyperintensities, dilated perivascular spaces and microinfarcts). In the absence of direct neuropathological examination, the most commonly used criteria for CAA diagnosis are the modified Boston criteria based on clinical and MRI data. To date, no specific treatment is available to prevent bleeding or cognitive decline. Thus, management is mainly based on strict blood pressure control. Anticoagulation should usually be avoided in patients with a diagnosis of CAA and symptomatic lobar ICH. The risk/benefit ratio evaluation at individual level of antiplatelet agents is also required.