An overview on Primary Progressive Aphasia and its variants

Serena Amici, Maria Luisa Gorno-Tempini, Jennifer M. Ogar, Nina Dronkers, Bruce L. Miller

Research output: Contribution to journalArticle

53 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)77-87
Number of pages11
JournalBehavioural Neurology
Volume17
Issue number2
StatePublished - 2006

Keywords

  • Language symptoms
  • Logopenic progressive aphasia
  • Neuroimaging studies
  • Nonfluent progressive aphasia
  • Primary progressive aphasia
  • Semantic dementia

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Neuroscience(all)

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    Amici, S., Gorno-Tempini, M. L., Ogar, J. M., Dronkers, N., & Miller, B. L. (2006). An overview on Primary Progressive Aphasia and its variants. Behavioural Neurology, 17(2), 77-87.