Quantifying progression in primary progressive aphasia with structural neuroimaging

  • Jolina Lombardi
  • Benjamin Mayer
  • Elisa Semler
  • Sarah Anderl-Straub
  • Ingo Uttner
  • Jan Kassubek
  • Janine Diehl-Schmid
  • Adrian Danek
  • Johannes Levin
  • Klaus Fassbender
  • Klaus Fliessbach
  • Anja Schneider
  • Hans-Jürgen Huppertz
  • Holger Jahn
  • Alexander Volk
  • Johannes Kornhuber
  • Bernhard Landwehrmeyer
  • Martin Lauer
  • Johannes Prudlo
  • Jens Wiltfang
  • Matthias L Schroeter
  • Albert Ludolph
  • Markus Otto
  • FTLD consortium


INTRODUCTION: The term primary progressive aphasia (PPA) sums up the non-fluent (nfv), the semantic (sv), and the logopenic (lv) variant. Up to now, there is only limited data available concerning magnetic resonance imaging volumetry to monitor disease progression.

METHODS: Structural brain imaging and an extensive assessment were applied at baseline and up to 4-year(s) follow-up in 269 participants. With automated atlas-based volumetry 56 brain regions were assessed. Atrophy progression served to calculate sample sizes for therapeutic trials.

RESULTS: At baseline highest atrophy appeared in parts of the left frontal lobe for nfvPPA (-17%) and of the left temporal lobe for svPPA (-34%) and lvPPA (-24%). Severest progression within 1-year follow-up occurred in the basal ganglia in nfvPPA (-7%), in the hippocampus/amygdala in svPPA (-9%), and in (medial) temporal regions in lvPPA (-6%).

CONCLUSION: PPA presents as a left-dominant, mostly gray matter sensitive disease with considerable atrophy at baseline that proceeds variant-specific.

Bibliographical data

Original languageEnglish
Publication statusPublished - 10.2021
PubMed 33787063