Association between semantic dementia and progressive supranuclear palsy

Introduction

Clinical syndromes associated with frontotemporal lobar degeneration (FTLD) pathology may overlap. Progressive supranuclear palsy syndrome (PSPs) may co-occur with behavioural frontotemporal dementia (bvFTD), non-fluent aphasia (nfPPA) and corticobasal syndrome.1 This is unsurprising, given each syndrome's association with tau pathology. We describe here a less anticipated association: between PSPs and semantic dementia (SD).

Case history

A 72-year-old man presented with an 8-year history of difficulty understanding words and phrases and recognising people and places. No behavioural changes were reported. There was no relevant family history. Neurological examination was normal. Neuropsychological examination revealed a severe disorder of semantic, and to a lesser extent, episodic memory. He could not identify high-profile famous faces and names, reporting most to be unfamiliar. He named only 2/30 pictures on the Graded naming test and scored 46/52 and 38/52 on word and picture versions of the Pyramids and Palm trees test. He performed normally on perceptual...

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