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  The applause sign in frontotemporal lobar degeneration and related conditions

Schönecker, S., Hell, F., Bötzel, K., Wlasich, E., Ackl, N., Süßmair, C., et al. (2019). The applause sign in frontotemporal lobar degeneration and related conditions. Journal of Neurology, 266(2), 330-338. doi:10.1007/s00415-018-9134-y.

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Schönecker, Sonja 1, Author
Hell, Franz 1, Author
Bötzel, Kai 1, Author
Wlasich, Elisabeth 1, Author
Ackl, Nibal 1, Author
Süßmair, Christine 1, Author
German FTLD Consortium, Author              
Otto, Markus 2, Author
Anderl-Straub, Sarah 2, Author
Ludolph, Albert 2, Author
Kassubek, Jan 2, Author
Huppertz, Hans-Jürgen 3, Author
Diehl-Schmid, Janine 4, Author
Riedl, Lina 4, Author
Roßmeier, Carola 4, Author
Fassbender, Klaus 5, Author
Lyros, Epameinondas 5, Author
Kornhuber, Johannes 6, Author
Oberstein, Timo Jan 6, Author
Fliessbach, Klaus 7, 8, Author
Schneider, Anja7, 8, AuthorSchroeter, Matthias L.9, 10, Author              Prudlo, Johannes 11, 12, AuthorLauer, Martin 13, AuthorJahn, Holger14, 15, AuthorLevin, Johannes 1, 16, AuthorDanek, Adrian 1, Author more..
1Neurologische Klinik und Poliklinik, Ludwig Maximilians University Munich, Germany, ou_persistent22              
2Department of Neurology, Ulm University, Germany, ou_persistent22              
3Swiss Epilepsy Centre, Zurich, Switzerland, ou_persistent22              
4Department of Psychiatry and Psychotherapy, TU Munich, Germany, ou_persistent22              
5Department of Neurology, Saarland University Homburg, Germany, ou_persistent22              
6Department of Psychology and Psychotherapy, Friedrich Alexander University Erlangen, Germany, ou_persistent22              
7Department for Neurodegenerative Diseases and Geriatric Psychiatry, University Hospital Bonn, Germany, ou_persistent22              
8German Center for Neurodegenerative Diseases, Bonn, Germany, ou_persistent22              
9Department Neurology, MPI for Human Cognitive and Brain Sciences, Max Planck Society, ou_634549              
10Clinic for Cognitive Neurology, University of Leipzig, Germany, ou_persistent22              
11Department of Neurology, University Medicine Rostock, Germany, ou_persistent22              
12German Center for Neurodegenerative Diseases, Rostock, Germany, ou_persistent22              
13Clinic for Psychiatry, Psychosomatic Medicine and Psychotherapy, Julius Maximilian University, Würzburg, Germany, ou_persistent22              
14Department of Psychiatry and Psychotherapy, University Medical Center Hamburg-Eppendorf, Germany, ou_persistent22              
15AMEOS Klinikum Heiligenhafen, Germany, ou_persistent22              
16German Center for Neurodegenerative Diseases, Munich, Germany, ou_persistent22              


Free keywords: Applause sign; Frontotemporal lobar degeneration; Progressive supranuclear palsy; Atlas-based MRI volumetry; Subthalamic nucleus; Pallidum
 Abstract: The applause sign, i.e., the inability to execute the same amount of claps as performed by the examiner, was originally reported as a sign specific for progressive supranuclear palsy (PSP). Recent research, however, has provided evidence for the occurrence of the applause sign in various conditions. The aim of this study was to determine the prevalence of the applause sign and correlate its presence with neuropsychological and MRI volumetry findings in frontotemporal lobar degeneration and related conditions. The applause sign was elicited with the three clap test (TCT), with a higher score indicating poorer performance. Data were recorded from 272 patients from the cohort of the German consortium for frontotemporal lobar degeneration (FTLDc): 111 with behavioral variant frontotemporal dementia (bvFTD), 98 with primary progressive aphasia (PPA), 30 with progressive supranuclear palsy Richardson’s syndrome, 17 with corticobasal syndrome (CBS) and 16 with amyotrophic lateral sclerosis with frontotemporal dementia (ALS/FTD). For comparison, 29 healthy elderly control subjects (HC) were enrolled in the study. All subjects underwent detailed language and neuropsychological assessment. In a subset of 156 subjects, atlas-based volumetry was performed. The applause sign occurred in all patient groups (40% in PSP, 29.5% in CBS, 25% in ALS/FTD, 13.3% in PPA and 9.0% in bvFTD) but not in healthy controls. The prevalence was highest in PSP patients. It was significantly more common in PSP as compared to bvFTD, PPA and HC. The comparison between the other groups failed to show a significant difference regarding the occurrence of the applause sign. The applause sign was highly correlated to a number of neuropsychological findings, especially to measures of executive, visuospatial, and language function as well as measures of disease severity. TCT scores showed an inverse correlation with the volume of the ventral diencephalon and the pallidum. Furthermore the volume of the ventral diencephalon and pallidum were significantly smaller in patients displaying the applause sign. Our study confirms the occurrence of the applause sign in bvFTD, PSP and CBS and adds PPA and ALS/FTD to these conditions. Although still suggestive of PSP, clinically it must be interpreted with caution. From the correlation with various cognitive measures we suggest the applause sign to be indicative of disease severity. Furthermore we suggest that the applause sign represents dysfunction of the pallidum and the subthalamic nucleus, structures which are known to play important roles in response inhibition.


Language(s): eng - English
 Dates: 2018-11-092018-07-282018-11-192018-12-012019-02
 Publication Status: Published in print
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
 Identifiers: DOI: 10.1007/s00415-018-9134-y
PMID: 30506397
Other: Epub ahead of print
 Degree: -



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Project name : -
Grant ID : O1GI1007A
Funding program : German Consortium for Frontotemporal Lobar Degeneration
Funding organization : German Federal Ministry of Education and Research (BMBF)
Project name : PreFrontAls
Grant ID : 01ED1512
Funding program : -
Funding organization : Joint 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 : Nutzung des menschlichen Peptidoms für die Entwicklung neuer antimikrobieller und anti-Krebs Therapeutika / SFB 1279
Grant ID : -
Funding program : -
Funding organization : German Research Foundation (DFG)
Project name : -
Grant ID : D.3830
Funding program : -
Funding organization : Foundation of the State Baden-Württemberg
Project name : -
Grant ID : D.5009
Funding program : -
Funding organization : Boehringer Ingelheim Ulm University BioCenter
Project name : -
Grant ID : -
Funding program : -
Funding organization : Thierry Latran Foundation

Source 1

Title: Journal of Neurology
Source Genre: Journal
Publ. Info: Berlin [etc.] : Springer
Pages: - Volume / Issue: 266 (2) Sequence Number: - Start / End Page: 330 - 338 Identifier: ISSN: 0340-5354
CoNE: https://pure.mpg.de/cone/journals/resource/110978979590419