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Primary progressive aphasia: diagnosis, varieties, evolution.

Andrew Kertesz1, Wilda Davidson, Patricia McCabe

  • 1Department of Clinical Neurological Sciences, St. Joseph's Health Care London, University of Western Ontario, London, Ontario, Canada. andrew.kertesz@sjhc.london.on.ca

Journal of the International Neuropsychological Society : JINS
|August 7, 2003
PubMed
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Primary progressive aphasia (PPA) patients often start fluent but become nonfluent, unlike Alzheimer's disease (AD) patients. Neuroimaging and language tests help distinguish these conditions.

Area of Science:

  • Neurology
  • Cognitive Science
  • Neuroimaging

Background:

  • Primary progressive aphasia (PPA) and Alzheimer's disease (AD) are neurodegenerative conditions affecting cognition and language.
  • Differentiating PPA subtypes and AD is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To compare language and cognitive profiles of PPA and AD patients.
  • To investigate the utility of neuroimaging in differentiating PPA from AD.
  • To examine the progression of language deficits in PPA.

Main Methods:

  • A case-control study comparing 67 PPA patients and 99 AD patients using formal language and nonverbal cognitive tests.
  • Longitudinal assessment of language fluency.
  • Quantification of sulcal and ventricular atrophy via MRI in a subset of patients.

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Main Results:

  • Initially, most PPA patients (57%) were fluent with preserved visuospatial and memory functions, contrasting with AD patients who showed better overall language but lower comprehension.
  • Over time, PPA patients tended to become nonfluent.
  • MRI revealed significant left-sided atrophy in most PPA patients.
  • A substantial clinical overlap was observed, with some PPA patients developing frontotemporal dementia or corticobasal degeneration syndrome.

Conclusions:

  • Language testing, particularly fluency, combined with neuroimaging, aids in distinguishing PPA from AD.
  • The fluent-nonfluent dichotomy in PPA is largely stage-dependent.
  • While PPA subtypes (aphemic, logopenic, agrammatic, semantic) are useful distinctions, their long-term outcomes can converge.