TY  - JOUR
A1  - Semler, Elisa
A1  - Anderl-Straub, Sarah
A1  - Uttner, Ingo
A1  - Diehl-Schmid, Janine
A1  - Danek, Adrian
A1  - Einsiedler, Beate
A1  - Fassbender, Klaus
A1  - Fliessbach, Klaus
A1  - Huppertz, Hans-Jürgen
A1  - Jahn, Holger
A1  - Kornhuber, Johannes
A1  - Landwehrmeyer, Bernhard
A1  - Lauer, Martin
A1  - Muche, Rainer
A1  - Prudlo, Johannes
A1  - Schneider, Anja
A1  - Schroeter, Matthias L.
A1  - Ludolph, Albert C.
A1  - Otto, Markus
T1  - A language-based sum score for the course and therapeutic intervention in primary progressive aphasia
JF  - Alzheimer's Research & Therapy
N2  - Background
With upcoming therapeutic interventions for patients with primary progressive aphasia (PPA), instruments for the follow-up of patients are needed to describe disease progression and to evaluate potential therapeutic effects. So far, volumetric brain changes have been proposed as clinical endpoints in the literature, but cognitive scores are still lacking. This study followed disease progression predominantly in language-based performance within 1 year and defined a PPA sum score which can be used in therapeutic interventions.

Methods
We assessed 28 patients with nonfluent variant PPA, 17 with semantic variant PPA, 13 with logopenic variant PPA, and 28 healthy controls in detail for 1 year. The most informative neuropsychological assessments were combined to a sum score, and associations between brain atrophy were investigated followed by a sample size calculation for clinical trials.

Results
Significant absolute changes up to 20% in cognitive tests were found after 1 year. Semantic and phonemic word fluency, Boston Naming Test, Digit Span, Token Test, AAT Written language, and Cookie Test were identified as the best markers for disease progression. These tasks provide the basis of a new PPA sum score. Assuming a therapeutic effect of 50% reduction in cognitive decline for sample size calculations, a number of 56 cases is needed to find a significant treatment effect. Correlations between cognitive decline and atrophy showed a correlation up to r = 0.7 between the sum score and frontal structures, namely the superior and inferior frontal gyrus, as well as with left-sided subcortical structures.

Conclusion
Our findings support the high performance of the proposed sum score in the follow-up of PPA and recommend it as an outcome measure in intervention studies.
KW  - frontotemporal dementia
KW  - cognitive neuropsychology in dementia
KW  - assessment of cognitive disorders/dementia
KW  - volumetric MRI
KW  - aphasia
Y1  - 2018
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-236277
VL  - 10
ER  - 
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  -