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Progranulin-associated PiB-negative logopenic primary progressive aphasia.

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Area of Science:

  • Neurodegenerative diseases
  • Neurology
  • Genetics

Background:

  • Logopenic variant primary progressive aphasia (lvPPA) is often associated with Alzheimer's disease (AD) pathology.
  • However, lvPPA can also occur in the absence of β-amyloid deposition, suggesting alternative underlying mechanisms.
  • Frontotemporal lobar degeneration (FTLD) is a potential alternative diagnosis in such cases.

Purpose of the Study:

  • To determine the frequency of lvPPA in patients with speech and language disorders but no β-amyloid deposition.
  • To conduct detailed neuroimaging and genetic analyses in these identified lvPPA patients.
  • To investigate the role of progranulin (GRN) gene mutations in lvPPA without β-amyloid.

Main Methods:

  • Analysis of 76 patients with neurodegenerative speech and language disorders and negative β-amyloid PET scans.
  • Identification of lvPPA cases and subsequent GRN gene testing.
  • Utilized MRI and 18-F fluorodeoxyglucose (FDG) PET with advanced analysis (Structural Abnormality Index Maps, Cortex ID, SPM) to assess grey matter atrophy and hypometabolism.

Main Results:

  • Six lvPPA patients (8%) were identified among the cohort.
  • All six patients exhibited left temporoparietal atrophy and hypometabolism.
  • Fifty percent (3/6) of these lvPPA patients were found to be GRN-positive, with distinct neuroimaging patterns in GRN-positive versus GRN-negative groups.

Conclusions:

  • Logopenic PPA represents a small subset of neurodegenerative speech and language disorders unrelated to β-amyloid.
  • The presence of lvPPA in patients without β-amyloid deposition warrants GRN mutation testing.
  • GRN-positive patients, despite lacking unique clinical signs, constitute a significant portion (50%) of this specific lvPPA subgroup.