An Elusive Diagnosis - Posterior Cortical Atrophy

Abstract

Posterior Cortical Atrophy (PCA) is a neurodegenerative syndrome characterized by progressive apraxia, dyslexia and most strikingly, loss of visual perceptual and visuospatial ability. In most cases, neuro-degeneration of the occipital, parietal, and posterior temporal cortices occurs as a consequence of the Visual Variant of Alzheimer disease (AD). Less frequent etiologies include Corticobasal Degeneration, Dementia with Lewy bodies (DLB), and the Heidenhain variant of Prion disease. It is unknown what predisposes these conditions to present as PCA. Given its rarity and variable presentations, misdiagnosis is common. We present a case of a 60-year-old woman with PCA who was initially undiagnosed. She measurably improved on donepezil and memantine suggesting AD as its pathogenesis.