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Behavioural Neurology
Volume 17 (2006), Issue 2, Pages 77-87

An Overview on Primary Progressive Aphasia and Its Variants

Serena Amici,1,4 Maria Luisa Gorno-Tempini,1 Jennifer M. Ogar,1,2 Nina F. Dronkers,1,2,3 and Bruce L. Miller1

1Memory and Aging Center, Department of Neurology, University of California, San Francisco, USA
2VA Northern California Health Care System, Martinez, CA, USA
3University of California, Davis, Davis, CA, USA
4Department of Neurosciences, University of Perugia, Perugia, Italy

Received 10 July 2006; Accepted 10 July 2006

Copyright © 2006 Hindawi Publishing Corporation and the authors. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


We present a review of the literature on Primary Progressive Aphasia (PPA) together with the analysis of neuropschychological and neuroradiologic profiles of 42 PPA patients. Mesulam originally defined PPA as a progressive degenerative disorder characterized by isolated language impairment for at least two years. The most common variants of PPA are: (1) Progressive nonfluent aphasia (PNFA), (2) semantic dementia (SD), (3) logopenic progressive aphasia (LPA). PNFA is characterized by labored speech, agrammatism in production, and/or comprehension. In some cases the syndrome begins with isolated deficits in speech. SD patients typically present with loss of word and object meaning and surface dyslexia. LPA patients have word-finding difficulties, syntactically simple but accurate language output and impaired sentence comprehension. The neuropsychological data demonstrated that SD patients show the most characteristic pattern of impairment, while PNFA and LPA overlap within many cognitive domains. The neuroimaging analysis showed left perisylvian region involvement. A comprehensive cognitive, neuroimaging and pathological approach is necessary to identify the clinical and pathogenetic features of different PPA variants.