Posterior Cortical Atrophy

Abstract

Posterior cortical atrophy (PCA), also known as Benson’s syndrome, is a neurodegenerative syndrome characterized by progressive visuospatial and visuoperceptual dysfunction. PCA most commonly occurs due to Alzheimer’s disease (AD) pathology; however, it is associated with other underlying pathologies, including dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), and prion disease. Patients often exhibit symptoms at a younger age than typical amnestic AD, often in their 50s–60s, with nonspecific visual complaints that may be misinterpreted as ophthalmologic in etiology. Imaging can support the diagnosis when regional atrophy or decreased cortical FDG avidity is present, specifically in the occipitoparietal and occipitotemporal regions, noting absence of these imaging findings does not exclude the diagnosis. This chapter reviews the unique entity of PCA and its currently recognized diagnostic criteria, clinical presentation, multimodal imaging features, histopathology, and treatment options.