A study of voice and non-voice processing in Prader-Willi syndrome
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(2020) 15:22
RESEARCH
Open Access
A study of voice and non-voice processing in Prader-Willi syndrome Kuzma Strenilkov1,2,3*, Jimmy Debladis1,2, Juliette Salles1,2,4, Marion Valette4, Carine Mantoulan4, Denise Thuilleaux5, Virginie Laurier5, Catherine Molinas4, Pascal Barone1,2† and Maïthé Tauber4,6†
Abstract Background: Prader-Willi syndrome (PWS) is a rare and complex neurodevelopmental disorder of genetic origin. It manifests itself in endocrine and cognitive problems, including highly pronounced hyperphagia and severe obesity. In many cases, impaired acquisition of social and communication skills leads to autism spectrum features, and individuals with this syndrome are occasionally diagnosed with autism spectrum disorder (ASD) using specific scales. Given that communicational skills are largely based on vocal communication, it is important to study human voice processing in PWS. We were able to examine a large number of participants with PWS (N = 61) recruited from France’s national reference center for PWS and other hospitals. We tested their voice and nonvoice recognition abilities, as well as their ability to distinguish between voices and nonvoices in a free choice task. We applied the hierarchical drift diffusion model (HDDM) with Bayesian estimation to compare decision-making in participants with PWS and controls. Results: We found that PWS participants were impaired on both voice and nonvoice processing, but displayed a compensatory ability to perceive voices. Participants with uniparental disomy had poorer voice and nonvoice perception than participants with a deletion on chromosome 15. The HDDM allowed us to demonstrate that participants with PWS need to accumulate more information in order to make a decision, are slower at decisionmaking, and are predisposed to voice perception, albeit to a lesser extent than controls. Conclusions: The categorization of voices and nonvoices is generally preserved in participants with PWS, though this may not be the case for the lowest IQ. Keywords: Prader-Willi syndrome, Voice processing, Social interactions, Autism spectrum disorder
Background Prader-Willi syndrome (PWS) is a rare genetic disease that was first described in 1956. It is caused by the absence or inactivation of paternal genes in the 15q11.2-q13 region of chromosome 15. The absence of gene expression is due to one of the following genetic subtypes: q11–13 de novo deletion on chromosome 15 of paternal origin (DEL; 60% incidence); chromosome 15 maternal uniparental disomy (UPD; 35%) [1]. Nowadays, diagnosis is made during the first months of life, and the prevalence of each genetic * Correspondence: [email protected] † Pascal Barone and Maïthé Tauber contributed equally to this work. 1 Brain & Cognition Research Center (CerCo), University of Toulouse Paul Sabatier, Toulouse, France 2 Brain & Cognition Research Center (CerCo), CNRS, Toulouse, France Full list of author information is available at the end of the article
subtype currently stands at 50% for DEL and 50% for nonDEL. The main diagnos
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