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People 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. Emotion 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. An 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. Many 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.

Background A small percentage of people with autism spectrum disorders (ASD) have alterations in chromosome 15q11.2-q3, the critical region for Prader-Willi syndrome (PWS). Data are limited, however, on the rates and characteristics of ASD in PWS. Previous estimates of ASD in PWS (25 to 41%) are questionable as they are based solely on autism screeners given to parents. Inaccurate diagnoses of ASD in PWS can mislead intervention and future research. Methods One hundred forty-six children and youth with PWS aged 4 to 21 years ( M  = 11) were assessed with the Autism Diagnostic Observation Schedule-2 (ADOS-2). An expert clinical team-made best-estimate ASD diagnoses based on ADOS-2 videotapes, calibrated severity scores, and children’s developmental histories and indices of current functioning. Children were also administered the Kaufman Brief Intelligence Test-2, and parents completed the Repetitive Behavior Scale-Revised and Vineland Adaptive Behavior Scales. Scores were compared across children with PWS + ASD versus PWS only. The performance of an ASD screener, the Social Communication Questionnaire (SCQ) and the ADOS-2 were evaluated in relation to best-estimate diagnoses. Results Best-estimate diagnoses of ASD were made in 18 children, or 12.3% of the sample, and the majority of them had the maternal uniparental disomy (mUPD) PWS genetic subtype. Compared to the PWS-only group, children with PWS + ASD had lower verbal and composite IQ’s and adaptive daily living and socialization skills, as well as elevated stereotypies and restricted interests. Regardless of ASD status, compulsivity and insistence on sameness in routines or events were seen in 76–100% of children and were robustly correlated with lower adaptive functioning. The SCQ yielded a 29–49% chance that screen-positive cases will indeed have ASD. The ADOS-2 had higher sensitivity, specificity and predictive values. Communication problems were seen in children who were ADOS-2 positive but deemed not to have ASD by the clinical team. Conclusions Autism screeners should not be the sole index of probable ASD in PWS; children need to be directly observed and evaluated. Compulsivity and insistence on sameness are salient in PWS and likely impede adaptive functioning. Most children with PWS only evidenced sub-threshold problems in social interactions that could signal risks for other psychopathologies.

Death had an important and pervasive presence in the middle ages. It was a theme in medieval public life, finding expression both in literature and art. The beliefs and procedures accompanying death were both complex and fascinating. Christopher Daniell's appproach to this subject is unusual 1n bringing together knowledge accumulated from historical, archaeological and literary sources. The book includes the very latest research, both of the author and of others working in this area. The result is a comprehensive and vivid picture of the entire phenomenon of medieval death and burial.

Using a combination of original sources and sharp analysis, this book is sheds new light on a crucial period in England's development. From Norman Conquest to Magna Carta is a wide-ranging history of England from 1066 to 1215 ideal for students and researchers throughout the field of medieval history. Starting with the build-up to the Battle of Hastings and ending with the Magna Carta, Christopher Daniell traces the profound change England underwent over the period, from religion and the life of the court through to arts and architecture. Central discussion topics include: how the Papacy became powerful enough to proclaim Crusades and to challenge kings how new monastic orders revitalized Christianity in England and spread European learning throughout the country how new Norman conquerors built cathedrals, monastries and castles, which changed the English landscape forever how by 1215 the king's administration had become more sophisticated and centralized how the acceptance of the Magna Carta by King John in 1215 would revolutionize the world in centuries to come. This volume will make essential reading for all students and researchers of medieval history.

Using a combination of original sources and sharp analysis, this book is sheds new light on a crucial period in England's development. From Norman Conquest to Magna Cartais a wide-ranging history of England from 1066 to 1215 ideal for students and researchers throughout the field of medieval history. Starting with the build-up to the Battle of Hastings and ending with the Magna Carta, Christopher Daniell traces the profound change England underwent over the period, from religion and the life of the court through to arts and architecture. Central discussion topics include: how the Papacy became powerful enough to proclaim Crusades and to challenge kings how new monastic orders revitalized Christianity in England and spread European learning throughout the country how new Norman conquerors built cathedrals, monastries and castles, which changed the English landscape forever how by 1215 the king's administration had become more sophisticated and centralized how the acceptance of the Magna Carta by King John in 1215 would revolutionize the world in centuries to come. This volume will make essential reading for all students and researchers of medieval history.

Background: The genetic mechanisms associated with Prader-Willi Syndrome (PWS) are often used to study biological models of Autism Spectrum Disorder (ASD). Data are limited, however, on the rates and characteristics of ASD in PWS, particularly so for adults. Previous estimates (25% to 41%) are predominantly based on autism screeners given to parents and may not be accurate. Additionally, less is known about adulthood than children in this population. Methods: 60 adults aged 17 to 55 (M = 26 years) recruited for a national longitudinal study on behavior and development in PWS were assessed with the Autism Diagnostic Observation Schedule-2 (ADOS-2). An expert clinical team made best-estimate ASD diagnoses based on ADOS-2 videotapes, developmental histories, and indices of current functioning. Adults were also administered the Kaufman Brief Intelligence Test-2 (KBIT-2); parents completed the Child Behavior Checklist (CBCL) and Vineland Adaptive Behavior Scales-Second Edition. Scores were compared across adults with PWS+ASD versus PWS only. Results: Diagnoses were made in 7 adults (11.7%), the majority of whom were the mUPD PWS genetic subtype. Adults with PWS+ASD did not differ from those with PWS alone in cognitive or adaptive functioning. Health adversities and lack of social supports were found throughout the entire sample. Conclusions: Autism screeners should not be the sole index of probable ASD in PWS as rates through single measures alone may be overinflated. Diagnosis within genetic syndromes requires direct observation and consideration of developmental context. A review of adults with PWS both with and without comorbid ASD indicates the need for social activity, employment, and supports