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  A language-based sum score for the course and therapeutic intervention in primary progressive aphasia

Semler, E., Anderl-Straub, S., Uttner, I., Diehl-Schmid, J., Danek, A., Einsiedler, B., et al. (2018). A language-based sum score for the course and therapeutic intervention in primary progressive aphasia. Alzheimer's Research & Therapy, 10: 41. doi:10.1186/s13195-018-0345-3.

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Item Permalink: http://hdl.handle.net/21.11116/0000-0001-F73D-5 Version Permalink: http://hdl.handle.net/21.11116/0000-0005-4CA7-A
Genre: Journal Article


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Semler, Elisa 1, Author
Anderl-Straub, Sarah1, Author
Uttner, Ingo1, Author
Diehl-Schmid, Janine2, Author
Danek, Adrian3, Author
Einsiedler, Beate4, Author
Fassbender, Klaus5, Author
Fliessbach, Klaus6, Author
Huppertz, Hans-Jürgen7, Author
Jahn, Holger8, Author
Kornhuber, Johannes9, Author
Landwehrmeyer, Bernhard1, Author
Lauer, Martin10, Author
Muche, Rainer4, Author
Prudlo, Johannes11, 12, Author
Schneider, Anja13, Author
Schroeter, Matthias L.14, 15, Author              
Ludolph, Albert C.1, Author
Otto, Markus1, Author
FTLD consortium, Author              
1Department of Neurology, Ulm University, Germany, ou_persistent22              
2Department of Psychiatry and Psychotherapy, TU Munich, Germany, ou_persistent22              
3Department of Neurology, Ludwig Maximilians University Munich, Germany, ou_persistent22              
4Institute of Epidemiology and Medical Biometry, Ulm University, Germany, ou_persistent22              
5Department of Neurology, Saarland University Homburg, Germany, ou_persistent22              
6Department of Psychiatry and Psychotherapy, University Bonn, Germany, ou_persistent22              
7Swiss Epilepsy Centre, Zurich, Switzerland, ou_persistent22              
8Department of Psychiatry and Psychotherapy, University Medical Center Hamburg-Eppendorf, Germany, ou_persistent22              
9Department of Psychology and Psychotherapy, Friedrich Alexander University Erlangen, Germany, ou_persistent22              
10Department of Psychiatry, Psychosomatics and Psychotherapy, University Hospital Würzburg, Germany, ou_persistent22              
11Department of Neurology, University Medicine Rostock, Germany, ou_persistent22              
12German Center for Neurodegenerative Diseases, Rostock, Germany, ou_persistent22              
13Department of Psychiatry and Psychotherapy, Georg August University, Göttingen, Germany, ou_persistent22              
14Department Neurology, MPI for Human Cognitive and Brain Sciences, Max Planck Society, ou_persistent22              
15Clinic for Cognitive Neurology, University of Leipzig, Germany, ou_persistent22              


Free keywords: Aphasia; Assessment of cognitive disorders/dementia; Cognitive neuropsychology in dementia; Frontotemporal dementia; Volumetric MR
 Abstract: 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.


Language(s): eng - English
 Dates: 2017-11-042018-01-222018-04-25
 Publication Status: Published online
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Method: -
 Identifiers: DOI: 10.1186/s13195-018-0345-3
PMID: 29695300
PMC: PMC5922300
 Degree: -



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Project information

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Project name : German Consortium for Frontotemporal Lobar Degeneration
Grant ID : O1GI1007A
Funding program : -
Funding organization : German Federal Ministry of Education and Research (BMBF)
Project name : SOPHIA
Grant ID : -
Funding program : -
Funding organization : Joint Programme – Neurodegenerative Disease Research (JPND)
Project name : BiomarkAPD
Grant ID : -
Funding program : -
Funding organization : Joint Programme – Neurodegenerative Disease Research (JPND)
Project name : PreFrontAls
Grant ID : 01ED1512
Funding program : -
Funding organization : oint Programme – Neurodegenerative Disease Research (JPND)
Project name : Conservative iron chelation as a disease-modifying strategy in Parkinson’s disease: a multicentric, parallel-group, placebo-controlled, randomized clinical trial of deferiprone / FAIR-PARK II
Grant ID : 633190
Funding program : Horizon 2020
Funding organization : European Commission (EC)
Project name : -
Grant ID : D.3830 ; D.5009
Funding program : -
Funding organization : Foundation of the state of Baden-Württemberg

Source 1

Title: Alzheimer's Research & Therapy
Source Genre: Journal
Publ. Info: BioMed Central
Pages: - Volume / Issue: 10 Sequence Number: 41 Start / End Page: - Identifier: ISSN: 1758-9193
CoNE: https://pure.mpg.de/cone/journals/resource/1758-9193