Association between semantic dementia and progressive supranuclear palsy

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).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 and spatial tasks (Visual Object and Space Perception Battery), except where recognition of object identity was required. Sentence comprehension (Test of Reception of Grammar) and executive performance (Weigls blocks, Brixton) were preserved. Memory test scores were reduced. However, he was fully oriented in time and place raising the possibility that semantic impairment contributed to his poor scores. An initial MR brain scan showed marked anterior temporal lobe atrophy with right-sided predominance (figure 1A) and atrophy of the superior cerebellar peduncles (figure 1B). The clinical picture suggested SD, although with greater episodic memory loss than commonly found.Figure 1 Coronal T1-weighted MR brain imaging showing marked (A) anterior temporal lobe atrophy and (B) atrophic superior cerebellar peduncles (arrowhead). (C) Sagittal T1-weighted image showing midbrain atrophy (midbrain:pons ratio 0.47). Macroscopic image of brain showing temporal lobe atrophy, most marked …