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 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.