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Quantitative template for subtyping primary progressive aphasia.

Marsel Mesulam1, Christina Wieneke, Emily Rogalski

  • 1Cognitive Neurology and Alzheimer's Disease Center, Northwestern University, Chicago, Illinois 60611, USA. mmesulam@northwestern.edu

Archives of Neurology
|December 17, 2009
PubMed
Summary

A new quantitative algorithm aids in classifying primary progressive aphasia (PPA) subtypes. This method uses a 2D template to differentiate agrammatic, semantic, and logopenic variants, aiding in diagnosis.

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Area of Science:

  • Neurology
  • Neuroscience
  • Linguistics

Background:

  • Primary progressive aphasia (PPA) is a neurodegenerative syndrome characterized by gradual decline in language abilities.
  • Accurate subtyping of PPA is crucial as variants have different associations with underlying pathologies like Alzheimer disease or frontotemporal lobar degeneration.

Purpose of the Study:

  • To develop and validate a quantitative algorithm for classifying PPA into its agrammatic (PPA-G), semantic (PPA-S), and logopenic (PPA-L) variants.
  • To correlate PPA subtypes with specific patterns of brain atrophy.

Main Methods:

  • Prospective study involving 16 patients with PPA at a university medical center.
  • Neuropsychological testing, including the Northwestern Anagram Test and Peabody Picture Vocabulary Test-Fourth Edition, and magnetic resonance imaging were performed.

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  • A 2-dimensional template was created based on syntax and lexical semantics performance.

    • Main Results:

    • The 2D template successfully classified all 16 patients into PPA subtypes, aligning with prior clinical diagnoses.
    • Distinct patterns of asymmetrical atrophy in the left perisylvian language network were observed for each subtype.
    • Specific peak atrophy sites were identified: PPA-G (inferior frontal gyrus), PPA-S (anterior temporal lobe), and PPA-L (Brodmann area 37).

    Conclusions:

    • A quantitative algorithm using a 2D template based on grammar and word comprehension tasks can guide PPA subtyping.
    • This clinicoanatomical classification approach is feasible and may aid in predicting the underlying neuropathology.
    • Further prospective studies are needed to confirm the clinical utility of this subtyping method.