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Foreign accent syndrome as the initial sign of primary progressive aphasia
  1. Simona Luzzi (simonaluzzi{at}yahoo.it)
  1. Department of Neuroscience, University of Ancona, Italy
    1. Giovanna Viticchi
    1. Department of Neuroscience, University of Ancona, Italy
      1. Massimo Piccirilli
      1. Unit of Cognitive Rehabilitation, University of Perugia, Italy
        1. Katia Fabi
        1. Department of Neuroscience, University of Ancona, Italy
          1. Martina Pesallaccia
          1. Department of Neuroscience, University of Ancona, Italy
            1. Marco Bartolini
            1. Department of Neuroscience, University of Ancona, Italy
              1. Leandro Provinciali
              1. Department of Neuroscience, University of Ancona, Italy
                1. Julie Snowden
                1. Cerebral Function Unit, Great Manchester Neuroscience Centre, United Kingdom

                  Abstract

                  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 foreign accent syndrome, 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. A one year follow-up 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 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.

                  • foreign accent syndrome
                  • language
                  • primary progressive aphasia

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