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Journal of Neurology

, Volume 266, Issue 2, pp 330–338 | Cite as

The applause sign in frontotemporal lobar degeneration and related conditions

  • Sonja SchöneckerEmail author
  • Franz Hell
  • Kai Bötzel
  • Elisabeth Wlasich
  • Nibal Ackl
  • Christine Süßmair
  • German FTLD Consortium
  • Markus Otto
  • Sarah Anderl-Straub
  • Albert Ludolph
  • Jan Kassubek
  • Hans-Jürgen Huppertz
  • Janine Diehl-Schmid
  • Lina Riedl
  • Carola Roßmeier
  • Klaus Fassbender
  • Epameinondas Lyros
  • Johannes Kornhuber
  • Timo Jan Oberstein
  • Klaus Fliessbach
  • Anja Schneider
  • Matthias L. Schroeter
  • Johannes Prudlo
  • Martin Lauer
  • Holger Jahn
  • Johannes Levin
  • Adrian Danek
Original Communication

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.

Keywords

Applause sign Frontotemporal lobar degeneration Progressive supranuclear palsy Atlas-based MRI volumetry Subthalamic nucleus Pallidum 

Abbreviations

ALS/FTD

Amyotrophic lateral sclerosis with frontotemporal dementia

bvFTD

Behavioral variant frontotemporal dementia

CDR-SOB

Clinical Dementia Rating Scale Sum of Boxes

CBS

Corticobasal syndrome

FTLD

Frontotemporal lobar degeneration

HC

Healthy control

LI

Laterality index

PPA

Primary progressive aphasia

PSP

Progressive supranuclear palsy

SPM12

Statistical parametric mapping 12

STN

Subthalamic nucleus

TCT

Three clap test

Notes

Funding

The study was supported by grants from the German Federal Ministry of Education and Research (project: FTLDc 01GI1007A), the JPND network PreFrontAls (01ED1512), the EU (FAIR-PARK II 633190), the German Research Foundation/DFG (SFB1279), the foundation of the state Baden-Württemberg (D.3830), Boehringer Ingelheim Ulm University BioCenter (D.5009) and the Thierry Latran Foundation.

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Sonja Schönecker
    • 1
    Email author
  • Franz Hell
    • 1
  • Kai Bötzel
    • 1
  • Elisabeth Wlasich
    • 1
  • Nibal Ackl
    • 1
  • Christine Süßmair
    • 1
  • German FTLD Consortium
  • Markus Otto
    • 2
  • Sarah Anderl-Straub
    • 2
  • Albert Ludolph
    • 2
  • Jan Kassubek
    • 2
  • Hans-Jürgen Huppertz
    • 3
  • Janine Diehl-Schmid
    • 4
  • Lina Riedl
    • 4
  • Carola Roßmeier
    • 4
  • Klaus Fassbender
    • 5
  • Epameinondas Lyros
    • 5
  • Johannes Kornhuber
    • 6
  • Timo Jan Oberstein
    • 6
  • Klaus Fliessbach
    • 7
    • 8
  • Anja Schneider
    • 7
    • 8
  • Matthias L. Schroeter
    • 9
    • 10
  • Johannes Prudlo
    • 11
    • 12
  • Martin Lauer
    • 13
  • Holger Jahn
    • 14
    • 15
  • Johannes Levin
    • 1
    • 16
  • Adrian Danek
    • 1
  1. 1.Neurologische Klinik und PoliklinikLudwig Maximilians Universität MünchenMunichGermany
  2. 2.Department of NeurologyUniversity of UlmUlmGermany
  3. 3.Swiss Epilepsy ClinicZurichSwitzerland
  4. 4.Department of Psychiatry and PsychotherapyTechnical University of MunichMunichGermany
  5. 5.Department of NeurologySaarland UniversityHomburgGermany
  6. 6.Department of Psychiatry and PsychotherapyFriedrich-Alexander University Erlangen-NürnbergErlangenGermany
  7. 7.Department for Neurodegenerative Diseases and Geriatric PsychiatryUniversity Hospital BonnBonnGermany
  8. 8.German Center for Neurodegenerative Diseases (DZNE)BonnGermany
  9. 9.Max Planck Institute of Human Cognitive and Brain Sciences (MPG)LeipzigGermany
  10. 10.Clinic for Cognitive NeurologyUniversity Hospital LeipzigLeipzigGermany
  11. 11.Department of NeurologyRostock University Medical CenterRostockGermany
  12. 12.German Center for Neurodegenerative Diseases (DZNE)RostockGermany
  13. 13.Department of Psychiatry and PsychotherapyUniversity of WuerzburgWuerzburgGermany
  14. 14.Department of Psychiatry and PsychotherapyUniversity Medical Center Hamburg-EppendorfHamburgGermany
  15. 15.AMEOS Klinikum HeiligenhafenHeiligenhafenGermany
  16. 16.German Center for Neurodegenerative Diseases (DZNE)MunichGermany

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