Affiliations: Memory and Aging Center, Department of Neurology,
University of California, San Francisco, USA | VA Northern California Health Care System, Martinez,
CA, USA | University of California, Davis, Davis, CA, USA | Department of Neurosciences, University of Perugia,
Perugia, Italy
Note: [] Corresponding author: Serena Amici, 350 Parnassus Avenue, San
Francisco, CA 94143-1207, USA. Tel.: +1 415 476 3572; E-mail:
[email protected]
Abstract: 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.
