Psychiatry Research: Neuroimaging
Volume 146, Issue 2 , Pages 191-197, 31 March 2006

Primary progressive aphasia accompanied by environmental sound agnosia: A neuropsychological, MRI and PET study

  • Ingo Uttner

      Affiliations

    • Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany
    • Corresponding Author InformationCorresponding author. Tel.: +49 731 177 5215; fax: +49 731 177 1202.
    • ,
  • Felix M. Mottaghy

      Affiliations

    • Department of Nuclear Medicine, University of Ulm, Robert-Koch-Str. 8, 89081 Ulm, Germany
    • ,
  • Herbert Schreiber

      Affiliations

    • Praxis für Neurologie, Pfauengasse 8, 89073 Ulm, Germany
    • ,
  • Axel Riecker

      Affiliations

    • Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany
    • ,
  • Albert C. Ludolph

      Affiliations

    • Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany
    • ,
  • Jan Kassubek

      Affiliations

    • Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany

Received 21 July 2005; received in revised form 20 October 2005; accepted 5 December 2005.

Abstract 

As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA may be associated with severe impairment in meaningful nonverbal sound recognition (environmental sound agnosia). These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging.

Keywords: Frontotemporal dementia, Nonverbal sound recognition, Slowly progressive aphasia

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PII: S0925-4927(05)00208-8

doi:10.1016/j.pscychresns.2005.12.003

Psychiatry Research: Neuroimaging
Volume 146, Issue 2 , Pages 191-197, 31 March 2006

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