title: Deficits in voice and multisensory processing in patients with Prader-Willi syndrome.
abstract: Prader-Willi syndrome (PWS) is a rare neurodevelopmental and genetic disorder that is characterized by various expression of endocrine, cognitive and behavioral problems, among which a true obsession for food and a deficit of satiety that leads to hyperphagia and severe obesity. Neuropsychological studies have reported that PWS display altered social interactions with a specific weakness in interpreting social information and in responding to them, a symptom closed to that observed in autism spectrum disorders (ASD). Based on the hypothesis that atypical multisensory integration such as face and voice interactions would contribute in PWS to social impairment we investigate the abilities of PWS to process communication signals including the human voice. Patients with PWS recruited from the national reference center for PWS performed a simple detection task of stimuli presented in an uni-o or bimodal condition, as well as a voice discrimination task. Compared to control typically developing (TD) individuals, PWS present a specific deficit in discriminating human voices from environmental sounds. Further, PWS present a much lower multisensory benefits with an absence of violation of the race model indicating that multisensory information do not converge and interact prior to the initiation of the behavioral response. All the deficits observed in PWS were stronger for the subgroup of patients suffering from Uniparental Disomy, a population known to be more sensitive to ASD. Altogether, our study suggests that the deficits in social behavior observed in PWS derive at least partly from an impairment in deciphering the social information carried by voice signals, face signals, and the combination of both. In addition, our work is in agreement with the brain imaging studies revealing an alteration in PWS of the "social brain network" including the STS region involved in processing human voices.
authors.affiliation: Université Toulouse, CerCo, Université Paul Sabatier, Toulouse, France; CNRS, UMR 5549, Faculté de Médecine de Purpan, Toulouse, France; Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France; Service de psychiatrie et psychologie médicale, Hôpital de psychiatrie, Toulouse, France.
authors.forename: Juliette
authors.initials: J
authors.lastname: Salles
authors.affiliation: Université Toulouse, CerCo, Université Paul Sabatier, Toulouse, France; CNRS, UMR 5549, Faculté de Médecine de Purpan, Toulouse, France.
authors.forename: Kuzma
authors.initials: K
authors.lastname: Strelnikov
authors.affiliation: Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.
authors.forename: Mantoulan
authors.initials: M
authors.lastname: Carine
authors.affiliation: Hôpital Marin d'Hendaye, France.
authors.forename: Thuilleaux
authors.initials: T
authors.lastname: Denise
authors.affiliation: Hôpital Marin d'Hendaye, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Laurier
authors.affiliation: Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France.
authors.forename: Catherine
authors.initials: C
authors.lastname: Molinas
authors.affiliation: Centre de Référence du Syndrome de Prader-Willi, Hôpital des Enfants, Toulouse, France; INSERM, Centre de Physiopathologie de Toulouse-Purpan UMR 1043 3, Toulouse, France.
authors.forename: Maïthé
authors.initials: M
authors.lastname: Tauber
authors.affiliation: Université Toulouse, CerCo, Université Paul Sabatier, Toulouse, France; CNRS, UMR 5549, Faculté de Médecine de Purpan, Toulouse, France. Electronic address: Pascal.barone@cerco.ups-tlse.fr.
authors.forename: Pascal
authors.initials: P
authors.lastname: Barone
mesh_terms: D000328:Adult; D003072:Cognition Disorders / Q000209:etiology*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007398:Interpersonal Relations; D008297:Male; D011218:Prader-Willi Syndrome / Q000150:complications* / Q000235:genetics / Q000523:psychology; D011930:Reaction Time / Q000502:physiology; D012678:Sensation Disorders / Q000209:etiology*; D017603:Signal Detection, Psychological / Q000502:physiology; D014832:Voice Disorders / Q000209:etiology*; D055815:Young Adult
keywords: Autism spectrum disorders; Multisensory integration; Prader Willi syndrome; Voice discrimination