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Related Experiment Videos

Frontotemporal dementia: clinicopathological correlations.

Mark S Forman1, Jennifer Farmer, Julene K Johnson

  • 1Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4283, USA.

Annals of Neurology
|May 24, 2006
PubMed
Summary
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Clinical features can suggest the underlying pathology in frontotemporal lobar degeneration (FTLD). However, distinct biomarkers are needed to accurately predict FTLD pathology, which includes tauopathies, ubiquitin-positive inclusions, and Alzheimer's disease.

Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Frontotemporal lobar degeneration (FTLD) presents with social, behavioral, and/or language impairments.
  • Postmortem studies reveal diverse neuropathological entities underlie FTLD.

Purpose of the Study:

  • To determine if specific clinical features can predict the underlying neuropathology in FTLD patients.
  • To correlate clinical presentations with distinct pathological findings.

Main Methods:

  • Clinicopathological correlation in 90 FTLD patients with pathological diagnosis.
  • Comparison with 24 additional FTLD cases with non-typical pathology.

Main Results:

  • Identified tauopathies (46%), FTLD with ubiquitin-positive inclusions (29%), and Alzheimer's disease (17%) postmortem.

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  • Observed statistical associations between pathology and distinct demographic, clinical, and neuropsychological features.
  • FTLD with ubiquitin-positive inclusions linked to social/language dysfunction and motor neuron disease; tauopathies to extrapyramidal disorders; Alzheimer's disease to memory/executive deficits.

    • Conclusions:

    • Clinical and neuropsychological features aid in understanding FTLD pathology spectrum.
    • Development of biomarkers is crucial for predicting underlying neuropathology alongside clinical phenotype.