TY  - JOUR
A1  - Lombardi, Jolina
A1  - Mayer, Benjamin
A1  - Semler, Elisa
A1  - Anderl‐Straub, Sarah
A1  - Uttner, Ingo
A1  - Kassubek, Jan
A1  - Diehl‐Schmid, Janine
A1  - Danek, Adrian
A1  - Levin, Johannes
A1  - Fassbender, Klaus
A1  - Fliessbach, Klaus
A1  - Schneider, Anja
A1  - Huppertz, Hans‐Jürgen
A1  - Jahn, Holger
A1  - Volk, Alexander
A1  - Kornhuber, Johannes
A1  - Landwehrmeyer, Bernhard
A1  - Lauer, Martin
A1  - Prudlo, Johannes
A1  - Wiltfang, Jens
A1  - Schroeter, Matthias L.
A1  - Ludolph, Albert
A1  - Otto, Markus
T1  - Quantifying progression in primary progressive aphasia with structural neuroimaging
JF  - Alzheimer's & Dementia
N2  - 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.
KW  - atlas‐based volumetry
KW  - disease progression
KW  - frontotemporal dementia
KW  - longitudinal magnetic resonance imaging
KW  - primary progressive aphasia
KW  - sample size calculation
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-262605
VL  - 17
IS  - 10
SP  - 1595
EP  - 1609
ER  - 
TY  - JOUR
A1  - Diehl-Schmid, Janine
A1  - Licata, Abigail
A1  - Goldhardt, Oliver
A1  - Förstl, Hans
A1  - Yakushew, Igor
A1  - Otto, Markus
A1  - Anderl-Straub, Sarah
A1  - Beer, Ambros
A1  - Ludolph, Albert Christian
A1  - Landwehrmeyer, Georg Bernhard
A1  - Levin, Johannes
A1  - Danek, Adrian
A1  - Fliessbach, Klaus
A1  - Spottke, Annika
A1  - Fassbender, Klaus
A1  - Lyros, Epameinondas
A1  - Prudlo, Johannes
A1  - Krause, Bernd Joachim
A1  - Volk, Alexander
A1  - Edbauer, Dieter
A1  - Schroeter, Matthias Leopold
A1  - Drzezga, Alexander
A1  - Kornhuber, Johannes
A1  - Lauer, Martin
A1  - Grimmer, Timo
T1  - FDG-PET underscores the key role of the thalamus in frontotemporal lobar degeneration caused by C9ORF72 mutations
JF  - Translational Psychiatry
N2  - C9ORF72 mutations are the most common cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). MRI studies have investigated structural changes in C9ORF72-associated FTLD (C9FTLD) and provided first insights about a prominent involvement of the thalamus and the cerebellum. Our multicenter, 18F-fluorodeoxyglucose positron-emission tomography study of 22 mutation carriers with FTLD, 22 matched non-carriers with FTLD, and 23 cognitively healthy controls provided valuable insights into functional changes in C9FTLD: compared to non-carriers, mutation carriers showed a significant reduction of glucose metabolism in both thalami, underscoring the key role of the thalamus in C9FTLD. Thalamic metabolism did not correlate with disease severity, duration of disease, or the presence of psychotic symptoms. Against our expectations we could not demonstrate a cerebellar hypometabolism in carriers or non-carriers. Future imaging and neuropathological studies in large patient cohorts are required to further elucidate the central role of the thalamus in C9FTLD.
KW  - diagnostic markers
KW  - psychiatric disorders
Y1  - 2019
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-225308
VL  - 9
ER  -