Familial cerebral amyloid angiopathy related to stroke and dementia

The term cerebral amyloid angiopathy (CAA) refers to the specific depositio n of amyloid fibrils in the walls of leptomeningeal and cortical arteries, arterioles and, although less frequently, in capillaries and veins. It is c ommonly associated with Alzheimer disease, Down syndrome and normal aging, as it-ell cis with a variety, of familial conditions related to stroke and/ or dementia: hereditary, cerebral hemorrhage with amyloidosis of Icelandic type (HCHWA-I), various inherited disorders linked to A beta mutants (inclu ding the Dutch variant Of HCHWA), and the recently described chromosome 13 familial dementia in British and Danish kindreds. This review focuses on fo ur different types of hereditary, CAA, emphasizing the notion that CAA is n ot only, related to stroke but also to neurodegeneration and dementia of th e Alzheimer type.