Stefanos Ioannidis
Royal Free Hospital, UK
Title: Posterior Cortical Atrophy: A case of late-onset disease presenting with memory loss
Biography
Biography: Stefanos Ioannidis
Abstract
Introduction: Posterior cortical atrophy (PCA) is a neurodegenerative disorder of progressive dementia, preceded by visuospatial, visuoperceptual deficits, and visual agnosia. It has an age of onset at 50 to 65 years. Here, we describe a case of PCA presenting at an older age with predominant complaint of memory loss.
Case Presentation: 80-year-old, right-handed man with education level at 14 years of age, presented to the memory clinic with 9-month history of episodic memory loss. This was reflected in his Addenbrooke’s cognitive examination III (52/100). He also reported visual impairment for 1 year and inability to judge distances when climbing stairs despite lack of finding in his ophthalmology assessment. He retained his ability to read albeit relying on newspapers with contextual cues such as colorful illustrations. Clinical examination and neuropsychometric evaluation showed optic ataxia, simultanagnosia, visuospatial and visuoperceptual impairment (Rey Complex figure) as well as impaired praxis (Rivermead Behavioural Memory Test). Computed tomography (CT) showed posterior parietal lobe atrophy with preserved hippocampus (Fig A). Positron emission tomography (PET) showed reduction of uptake in the posterior cortex (Fig B), in keeping with a diagnosis of PCA.
He was treated with donepezil and referred to PCA support groups.
Discussion: In elderly patients presenting with impaired memory, the diagnosis of PCA needs to be considered. Patients often develop a compensatory strategy with the visual deficit while disabling cognitive difficulties take hold, which mask the presence of PCA. Moreover, the emphasis on age as part of the diagnostic picture may inadvertently exclude older patients given that younger, working age patients are more likely to be acutely aware of visual problems and thus present to physicians. PCA may therefore be more common in older adults than previously suspected. Correctly identifying the disease process is crucial for patient management including provision of help in perceptual activities.