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Primary progressive aphasia and Pick complex.

Andrew Kertesz1, David G Munoz

  • 1Department of Clinical Neurological Sciences, University of Western Ontario, St. Joseph's Health Care London, 268 Grosvenor Street, London, Ontario, Canada N6A 4V2. andrew.kertesz@sjhc.london.on.ca

Journal of the Neurological Sciences
|December 14, 2002
PubMed
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Clinically diagnosed primary progressive aphasia (PPA) often indicates underlying frontotemporal degeneration (FTD) pathologies, including Pick complex, corticobasilar degeneration (CBD), and motor neuron disease. Autopsy findings reveal shared histological features and clinical-pathological overlap in PPA.

Area of Science:

  • Neuropathology
  • Neurodegenerative Diseases
  • Clinical Neurology

Background:

  • Primary progressive aphasia (PPA) is a clinical syndrome characterized by gradual language decline.
  • Histological heterogeneity has been emphasized in PPA, complicating diagnosis.
  • Understanding the underlying pathologies is crucial for accurate diagnosis and management.

Observation:

  • Ten autopsied patients with clinically defined PPA were histologically characterized.
  • Pathologies included Pick body dementia (n=3), corticobasilar degeneration (CBD, n=4), and motor neuron disease-type inclusions (n=3).
  • All cases shared cortical spongiosis, neuronal loss, and gliosis, with initial preservation of memory and comprehension.

Findings:

  • Clinically probable PPA predicted a group of related frontotemporal degeneration (FTD) pathologies, collectively termed Pick complex.

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  • Significant clinical-pathological overlap was observed between PPA, FTD, and CBD.
  • Language areas were not the sole site of pathology at autopsy.

    • Implications:

    • PPA has predictive value for specific FTD-related pathologies.
    • This study supports the clinical and pathological overlap of PPA with FTD and CBD.
    • Findings contribute to refining the diagnostic and neuropsychological definition of PPA.