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Foreign accent syndrome as the initial sign of primary progressive aphasia
  1. S Luzzi1,
  2. G Viticchi1,
  3. M Piccirilli2,
  4. K Fabi1,
  5. M Pesallaccia1,
  6. M Bartolini1,
  7. L Provinciali1,
  8. J S Snowden3
  1. 1
    Department of Neuroscience, University of Ancona, Italy
  2. 2
    Unit of Cognitive Rehabilitation, University of Perugia, Italy
  3. 3
    Cerebral Function Unit, Great Manchester Neuroscience Centre, Manchester, UK
  1. S Luzzi, Clinica Neurologica, Dipartimento di Neuroscienze, Universitè Politecnica delle Marche-Ospedali Riuniti di Ancona, Via Conca, 1, Torrette di Ancona, Italy; simonaluzzi{at}


Foreign accent syndrome (FAS) is a rare speech disorder characterised by the emergence of a new accent, perceived by listeners as foreign. FAS has usually been described following focal brain insults, such as stroke. We describe the unusual case of a woman presenting with FAS as the earliest symptom of progressive degenerative brain disease. At presentation, she showed no language or other cognitive impairment, and functional and structural brain imaging were normal. Follow-up 1 year later revealed the emergence of mild expressive language problems. Repeat functional neuroimaging showed mild hypoperfusion of the perisylvian speech area of the left hemisphere, and structural imaging showed mild left perisylvian atrophy. We interpret the case as an unusual presentation of primary progressive non-fluent aphasia. The case provides further evidence of the variable and circumscribed nature of the clinical presentation of focal cerebral degeneration.

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  • Competing interests: None.