Background/Aims: The longitudinal course of three primary progressive aphasia (PPA) variants was examined using Addenbrooke’s Cognitive Examination-Revised (ACE-R) and the Frontotemporal dementia Rating Scale (FRS). Methods: Cases with two assessments on the ACE-R and FRS were selected. A total of 220 assessments were obtained on 55 patients: 17 Alzheimer’s disease (AD) and 38 PPA [17 semantic variant (svPPA), 12 non-fluent/agrammatic (naPPA) and 9 logopenic variant (lvPPA) cases]. Results: The annualized rate of change was greater in all PPA variants in comparison with the AD group on the ACE-R whereas only the svPPA and naPPA groups differed from AD on the FRS. Conclusions: The longitudinal profile differs across PPA syndromes on cognitive and functional measures. Findings have theoretical implications and are relevant to the care of patients with dementia.

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