Please cite this article in press as: Somme J, et al. The applause sign and neuropsychological profile in progressive supranuclear palsy and Parkinson’s disease. Clin Neurol Neurosurg (2012), http://dx.doi.org/10.1016/j.clineuro.2012.11.013 ARTICLE IN PRESS G Model CLINEU-3175; No. of Pages 4 Clinical Neurology and Neurosurgery xxx (2012) xxx–xxx Contents lists available at SciVerse ScienceDirect Clinical Neurology and Neurosurgery journa l h omepage: www.elsevier.com/locate/clineuro The applause sign and neuropsychological profile in progressive supranuclear palsy and Parkinson’s disease Johanne Somme a,b,* , Juan Carlos Gómez Esteban a , Beatriz Tijero a , Koldo Berganzo a , Elena Lezcano a , Juan Jose Zarranz a a Movement Disorders Unit, Department of Neurology, Cruces University Hospital, University of the Basque Country, Baracaldo, Spain b Department of Neurology, Alava University Hospital, University of the Basque Country, Vitoria-Gasteiz, Spain a r t i c l e i n f o Article history: Received 15 May 2012 Received in revised form 10 September 2012 Accepted 18 November 2012 Available online xxx Keywords: Progressive supranuclear palsy Parkinson’s disease Applause sign Frontal assessment battery Neuropsychology a b s t r a c t Background: The applause sign has been associated with various neurodegenerative diseases. We inves- tigate its validity in the differential diagnosis of progressive supranuclear palsy and Parkinson’s disease, and its relationship with neuropsychological tests. Patients and methods: 23 patients with progressive supranuclear palsy and 106 patients with Parkin- son’s disease were included and administered the following scales: progressive supranuclear palsy rating scale, unified Parkinson’s disease rating scale (UPDRS), mini-mental state examination (MMSE), frontal assessment battery (FAB), neuropsychiatric inventory and three-clap test. Results: 73.9% with progressive supranuclear palsy and 21.7% with Parkinson’s disease showed a posi- tive applause sign. Only a positive applause sign, UPDRS II score and disease duration were found to be predictors of progressive supranuclear palsy. Both patient-groups showed statistically significant cor- relations between the applause sign and neuropsychological tests: in progressive supranuclear palsy patients MMSE correlation coefficient: 0.62 (p: 0.002) and FAB correlation coefficient: 0.48 (p: 0.02), and in Parkinson’s disease patients MMSE correlation coefficient: 0.47 (p < 0.001) and FAB correlation coef- ficient: 0.43 (p < 0.001). Verbal fluency and inhibitory control (FAB) and writing and orientation in time (MMSE) discriminated between patients with normal and positive applause sign. Conclusions: A positive applause sign is not specific to progressive supranuclear palsy and may also be observed in Parkinson’s disease patients with altered cognition, and it’s related to cortical frontal abnormalities such as language disorders and inhibitory control. © 2012 Elsevier B.V. All rights reserved. 1. Introduction An impaired three-clap test (positive applause sign) is consid- ered indicative of neurodegenerative disease [1,2]. It is believed to be due to an executive dysfunction that can be observed in lesions on both frontal lobes as well as in basal ganglia disorders. Some publications point to its utility in differential diagnosis between progressive supranuclear palsy (PSP) and Parkinson’s disease (PD) [2]. Other research, however, records no difference in frequency between the different atypical parkinsonisms [3]. The aim of the present work is to analyze the utility of the applause sign (AS) in the differential diagnosis between PSP and PD, and as part of this to identify the characteristics of PD patients with * Corresponding author at: Department of Neurology, Alava University Hospital (Txagorritxu), Jose Atxotegui s.n., 01009 Vitoria-Gasteiz, Alava, Spain. Tel.: +34 945007000. E-mail addresses: johannehatteland.somme@osakidetza.net, johanne somme@hotmail.com (J. Somme). abnormal AS. We also study the relationship of AS with neuropsy- chiatric symptoms and neuropsychological test scores, in particular the frontal assessment battery (FAB), which is considered a useful tool in detecting frontal lobe disorders [4]. 2. Methods 2.1. Patient selection Twenty-three patients with a diagnosis of PSP were studied (12 women, 11 men) with a mean age of 72.9 ± 6.9 and a mean of 3.3 ± 2.3 years of disease duration at the time of the study; 78% (18 patients) had a diagnosis of probable PSP and the remaining 22% (5 patients) of possible PSP, according to the clinical criteria set out by the NINDS-PSP international workshop [5]. The two diagnostic groups of PSP were separated for the analysis of general character- istics and were thereafter analyzed as a whole. The control group was a sample of 106 patients (50 men, 56 women) with a diagnosis of PD according to the London Brain Bank criteria [6]. The mean age 0303-8467/$ – see front matter © 2012 Elsevier B.V. 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