Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features
John Best, Marianne Chapleau, Gil D. Rabinovici
Abstract
INTRODUCTION: Posterior Cortical Atrophy (PCA) is a neurodegenerative disorder characterized by impairment of higher-order visual processing in the setting of progressive atrophy of the parietal and occipital lobes. The underlying pathology is variable but most commonly Alzheimer's disease. The majority of individuals develop symptoms before 65 years of age; however, delayed diagnosis is common due to misattribution of symptoms to ocular rather than cortical pathology. AREAS COVERED: The purpose of this review is to provide readers with an in-depth analysis of Posterior Cortical Atrophy syndrome, including clinical, imaging, pathological, and genetic features, management, and treatments. EXPERT OPINION: Most patients present initially with a relatively pure visuoperceptual-visuospatial syndrome, though other cognitive domains become affected over time. Structural neuroimaging demonstrates parieto-occipital or temporo-occipital predominant atrophy. Cerebrospinal fluid Alzheimer's disease biomarkers, or amyloid/tau PET imaging can help evaluate for underlying Alzheimer's disease, which is the most common underlying neuropathology. The cornerstone of management is focused on nonpharmacologic measures. Early etiologic diagnosis is important with the arrival of disease-modifying therapies, especially for Alzheimer's disease.