Amyloid Deposition and Angiitis: Spectrum of Radiologic Manifestations

Cerebral amyloid angiopathy is defined as cerebrovascular amyloid deposition and is classified according to the amyloid protein involved. Cerebral amyloid angiopathy with β-amyloid protein deposition in the sporadic form of presentation is by far the most common form of cerebral amyloid angiopathy seen in elderly individuals who are asymptomatic, as well as in patients with acute intracranial hemorrhage and Alzheimer disease. β-amyloid‐type cerebral amyloid angiopathy has a broad spectrum of radiologic manifestations and a high prevalence among the elderly population. Neuroimaging plays an important role in the diagnosis and management of the common clinical presentations of cerebral amyloid angiopathy, for example, the classic lobar intracerebral hemorrhage, and is also useful in detecting clinically silent microbleeds and microinfarctions. β-amyloid‐type cerebral amyloid angiopathy can present with associated inflammation, an infrequent form called cerebral amyloid angiopathy‐related inflammation, in which patients present with acute cognitive decline. Cerebral amyloid angiopathy‐related inflammation can demonstrate asymmetric white matter changes and leptomeningeal enhancement, and its recognition is critical to establish early treatment, given that these changes are reversible and respond well to steroids and other immunosuppressant therapies.

Learning Objective: Assess the main imaging findings of the cerebral amyloid angiopathy forms, including the inflammatory form, and describe the clinical-radiologic criteria for the diagnosis.