Primary progressive aphasia: Serial linguistic, neuropsychological and radiological findings with neuropathological results

Since Mesulam and Weintraub's (1992) original description of six patients with primary progressive aphasia (PPA), there has been considerable debate concerning the quality of the speech and language impairment, the long-term course of the disorder, the existence of syndrome subtypes, and the nature of the underlying pathological process. We report the case of a 60-year-old woman who presented with a 4-year history of word-finding difficulty, and received a diagnosis of PPA. In contrast to a relentless deterioration of word-finding, comprehension and auditory-verbal memory, all other intellectual skills remained well preserved for a considerable period before the onset of dementia, and death. Regular detailed assessments of language, mnestic, intellectual and psychological functioning were performed over a 6-year period, as well as serial radiology and post-mortem neuropathology. We report the findings and discuss them in the context of the theoretical issues.