Profiles and trajectories of impaired social cognition in people with Prader-Willi syndrome

Abstract

IntroductionPeople with Prader-Willi syndrome (PWS) have a distinctive behavioral phenotype that includes intellectual disability, compulsivity, inattention, inflexibility and insistence on sameness. Inflexibility and inattention are at odds with the cognitive flexibility and attention to social cues needed to accurately perceive the social world, and implicate problems in social cognition. This study assessed two social cognition domains in people with PWS; emotion recognition and social perception. We identified changes in social cognition over an approximate two-year time period (M = 2.23 years), relative strengths and weakness in social cognition, and correlates and predictors of social cognition.MethodsEmotion recognition and social perception were examined at two time points in 94 individuals with PWS aged 5 to 62 years (M = 13.81, SD = 10.69). Tasks administered included: standardized IQ testing; parent-completed measures of inattention and inflexibility; standard emotion recognition photos (fear, sadness, anger, happy); and videotaped social perception vignettes depicting negative events with either sincere/benign or insincere/hostile interactions between peers.ResultsAn atypical trajectory of negative emotion recognition emerged, marked by similar levels of poor performances across age, and confusion between sad and anger that is typically resolved in early childhood. Recognition of sad and fear were positively correlated with IQ. Participants made gains over time detecting social cues, but not in forming correct conclusions about the intentions of others. Accurately judging sincere intentions remained a significant weakness over time. Relative to sincere intentions, participant’s performed significantly better in detecting negative social cues, and correctly judging trickery, deceit and lying. Age, IQ, inattention, and recognition of happy and sad accounted for 29% of variance in social perception.ConclusionMany people with PWS have deficits in recognizing sad, anger and fear, and accurately perceiving the sincere intentions of other people. The impact of these deficits on social behavior and relationships need to be better understood.

Neurobehavioral dimensions of Prader Willi Syndrome: Relationships between sleep disturbance and psychotic experiences

“…Although our cognitive assessment was only completed by a subset of the larger sample, ndings reveal differential impairment in social cognitive domains, particularly tasks involving processing of faces, implicating cortico-limbic dysfunction (Gur et al, 2017). This pattern of ndings is highly consistent with other recent work in individuals with PWS, involving in-lab assessment (Dykens et al 2019;Debladis et al, 2019). Another recent study offers a translational perspective on psychiatric manifestations of PWS, through the RDoC matrix (Salles et al, 2020): de cits in social processes are highlighted, with eventrelated potential (ERP) evidence that PWS patients have altered processing of faces (Key et al, 2013).…”

“…With regard to neurocognition, global cognitive function is typically in the impaired range (average Full Scale IQ of ~ 60, but highly variable, ranging from 40-100 across studies) (Dykens et al, 1992;Whittington & Holland, 2017). De cits in emotion recognition and social perception (i.e., accurately judging the intentions of others) are particularly prominent in individuals with PWS (Dimitropoulos et al, 2019;Dykens et al, 2019). There is also some evidence that cognition may differ as a function of genetic subtype: Torrado et al found that less than 10 percent of subjects with paternal deletion had Full-Scale IQ (FSIQ) 70 or greater, whereas over 60% of subjects without the deletion subtype had FSIQ of 70 or higher (Torrado et al, 2007).…”

A Comprehensive Review of Genetically Engineered Mouse Models for Prader-Willi Syndrome Research

“…They are frequently affected by respiratory malfunction symptoms, sleep disorders, hypogonadism, mild mental deficiency, and disruptions of their endocrine axis. Individuals display behavioral abnormalities including temper tantrums, obsessive compulsion and skin picking [ 4 , 9 , 10 , 11 ]. PWS is a complex disease, and symptoms vary considerably between patients, depending on the size of the chromosomal deletion [ 2 , 12 , 13 ].…”

Validation of dynamic virtual faces for facial affect recognition

“…There is consistent evidence that patients with different neuropsychiatric conditions such as autism [ 9 , 10 ], schizophrenia [ 11 , 12 ], major depressive disorder [ 13 ], bipolar disorder [ 14 , 15 ], Alzheimer’s dementia [ 16 ], or Prader-Willi syndrome [ 17 ] have difficulty in accurately recognising the emotions expressed by others. This deficit can generate a misinterpretation of social situations and, therefore, a significant deficit in social functioning and quality of life [ 18 ].…”