Amyloid angiitis and progressive cortical superficial siderosis as aggressive phenotypes of cerebral amyloid angiopathy: principles of rational management

Abstract

Cerebral amyloid angiopathy (CAA) is a common disease in the older age group with high clinical and neurovisual heterogeneity. A literature review and two clinical cases reflecting different phenotypes of CAA are presented — amyloid angiitis (CAA-related inflammation) and “hemorrhagic phenotype” (CAA associated with intracerebral hemorrhage and progressive cortical superficial siderosis). In each case, in relation to the clinical and neuroimaging picture, the directions of the disputed vectors of secondary prevention and therapy of CAA are considered: antithrombotic, antihypertensive and lipid-lowering therapy. The issues of epidemiology, pathogenesis, diagnosis and treatment of CAA are discussed.