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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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Datensatz-Permalink: https://hdl.handle.net/21.11116/0000-0002-AF48-9 Versions-Permalink: https://hdl.handle.net/21.11116/0000-0003-A31D-5
Genre: Zeitschriftenartikel

Externe Referenzen

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Urheber

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 Urheber:
Schönecker, Sonja 1, Autor
Hell, Franz 1, Autor
Bötzel, Kai 1, Autor
Wlasich, Elisabeth 1, Autor
Ackl, Nibal 1, Autor
Süßmair, Christine 1, Autor
German FTLD Consortium, Autor              
Otto, Markus 2, Autor
Anderl-Straub, Sarah 2, Autor
Ludolph, Albert 2, Autor
Kassubek, Jan 2, Autor
Huppertz, Hans-Jürgen 3, Autor
Diehl-Schmid, Janine 4, Autor
Riedl, Lina 4, Autor
Roßmeier, Carola 4, Autor
Fassbender, Klaus 5, Autor
Lyros, Epameinondas 5, Autor
Kornhuber, Johannes 6, Autor
Oberstein, Timo Jan 6, Autor
Fliessbach, Klaus 7, 8, Autor
Schneider, Anja7, 8, AutorSchroeter, Matthias L.9, 10, Autor           Prudlo, Johannes 11, 12, AutorLauer, Martin 13, AutorJahn, Holger14, 15, AutorLevin, Johannes 1, 16, AutorDanek, Adrian 1, Autor mehr..
Affiliations:
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              

Inhalt

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Schlagwörter: Applause sign; Frontotemporal lobar degeneration; Progressive supranuclear palsy; Atlas-based MRI volumetry; Subthalamic nucleus; Pallidum
 Zusammenfassung: 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.

Details

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Sprache(n): eng - English
 Datum: 2018-11-092018-07-282018-11-192018-12-012019-02
 Publikationsstatus: Erschienen
 Seiten: -
 Ort, Verlag, Ausgabe: -
 Inhaltsverzeichnis: -
 Art der Begutachtung: Expertenbegutachtung
 Identifikatoren: DOI: 10.1007/s00415-018-9134-y
PMID: 30506397
Anderer: Epub ahead of print
 Art des Abschluß: -

Veranstaltung

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Entscheidung

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Projektinformation

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Projektname : -
Grant ID : O1GI1007A
Förderprogramm : German Consortium for Frontotemporal Lobar Degeneration
Förderorganisation : German Federal Ministry of Education and Research (BMBF)
Projektname : PreFrontAls
Grant ID : 01ED1512
Förderprogramm : -
Förderorganisation : Joint Programme – Neurodegenerative Disease Research (JPND)
Projektname : 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
Förderprogramm : Horizon 2020
Förderorganisation : European Commission (EC)
Projektname : Nutzung des menschlichen Peptidoms für die Entwicklung neuer antimikrobieller und anti-Krebs Therapeutika / SFB 1279
Grant ID : -
Förderprogramm : -
Förderorganisation : German Research Foundation (DFG)
Projektname : -
Grant ID : D.3830
Förderprogramm : -
Förderorganisation : Foundation of the State Baden-Württemberg
Projektname : -
Grant ID : D.5009
Förderprogramm : -
Förderorganisation : Boehringer Ingelheim Ulm University BioCenter
Projektname : -
Grant ID : -
Förderprogramm : -
Förderorganisation : Thierry Latran Foundation

Quelle 1

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Titel: Journal of Neurology
Genre der Quelle: Zeitschrift
 Urheber:
Affiliations:
Ort, Verlag, Ausgabe: Berlin [etc.] : Springer
Seiten: - Band / Heft: 266 (2) Artikelnummer: - Start- / Endseite: 330 - 338 Identifikator: ISSN: 0340-5354
CoNE: https://pure.mpg.de/cone/journals/resource/110978979590419