Jolina Lombardi1, Benjamin Mayer2, Elisa Semler1, Sarah Anderl-Straub1, Ingo Uttner1, Jan Kassubek1, Janine Diehl-Schmid3,4, Adrian Danek5, Johannes Levin5,6,4, Klaus Fassbender7, Klaus Fliessbach8,9, Anja Schneider8,9, Hans-Jürgen Huppertz10, Holger Jahn11, Alexander Volk12, Johannes Kornhuber13, Bernhard Landwehrmeyer1, Martin Lauer14, Johannes Prudlo15,16, Jens Wiltfang17, Matthias L Schroeter18, Albert Ludolph1, Markus Otto1. 1. Department of Neurology, University Hospital Ulm, Ulm, Germany. 2. Institute for Epidemiology and Medical Biometry, University of Ulm, Ulm, Germany. 3. Department of Psychiatry and Psychotherapy, Technical University of Munich, Munich, Germany. 4. Munich Cluster for Systems Neurology (SyNergy), Munich, Germany. 5. Department of Neurology, Ludwig-Maximilians-Universität München, Munich, Germany. 6. German Center for Neurodegenerative Diseases (DZNE), Munich, Germany. 7. Department of Neurology, Saarland University Hospital, Homburg, Germany. 8. Department of Psychiatry and Psychotherapy, University Hospital Bonn, Bonn, Germany. 9. German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany. 10. Swiss Epilepsy Center, Zurich, Switzerland. 11. Department of Psychiatry and Psychotherapy, University Hospital Hamburg Eppendorf, Hamburg, Germany. 12. Institute for Human Genetics, University Hospital Hamburg Eppendorf, Hamburg, Germany. 13. Department of Psychiatry and Psychotherapy, University Hospital Erlangen, Erlangen, Germany. 14. Department of Psychiatry and Psychotherapy, University Hospital Würzburg, Würzburg, Germany. 15. Department of Neurology, University Medicine Rostock, Rostock, Germany. 16. German Center for Neurodegenerative Diseases (DZNE), Rostock, Germany. 17. Department of Psychiatry and Psychotherapy, Medical University Göttingen, Göttingen, Germany. 18. Max-Planck-Institute for Human Cognitive and Brain Sciences and Clinic for Cognitive Neurology, University Hospital Leipzig, Leipzig, Germany.
Abstract
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.
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.