Background This study aimed to test the effect of a new training programme on emotional competencies, named EMO-T, and to show the value of an integrative developmental approach. This approach postulates that the emotion regulation disturbances commonly observed in neurodevelopmental disorders are the consequence of potential disruptions in the prerequisite emotion skills. This integrative approach is particularly suitable in the case of complex and multidimensional disorders such as Prader–Willi syndrome (PWS), a rare genetic disease. Methods We examined the emotion expression, recognition, comprehension, and regulation skills in 25 PWS children aged 5–10 and 50 typically developing children (TD) aged 3–10. After a pre-test session, half of the PWS children participated in the EMO-T programme with their regular therapist for 6 weeks, while the other half continued their usual rehabilitation programme. Two post-test sessions were conducted, one at the end of the programme and one 3 months later. Results At pre-test, PWS children displayed a deficit in the four emotional competencies (EC). PWS children who participated in the EMO-T programme showed a significant and sustainable post-test improvement regarding voluntary expression and emotion recognition abilities, such that the level reached was no longer different from the baseline level of TD children. They also tended to improve in their emotion regulation, although they received no specific training in this skill. Discussion These results support that emotion regulation abilities require prerequisite emotion skills, which should be more fully considered in current training programmes. Because emotion regulation disorders strongly impact all areas of life, an integrative developmental approach appears crucial especially in the case of neurodevelopmental disorders. Further studies should be conducted to explore this perspective.
Résumé Le Syndrome de Prader-Willi (SPW) est une maladie génétique rare impliquant des troubles hormonaux et neurodéveloppementaux. Les personnes qui en sont atteintes présentent également des difficultés émotionnelles et sociales. Les quelques études réalisées concernant le développement émotionnel dans cette population mettent en évidence un déficit dans l’identification et la compréhension des émotions, mais ces résultats sont trop lacunaires pour proposer une prise en charge adaptée des difficultés émotionnelles manifestées par ces enfants. L'objectif de cette étude interventionnelle est donc de mieux caractériser les difficultés émotionnelles des enfants avec le SPW en élaborant et testant l’effet d’un programme centré sur l’entraînement aux compétences émotionnelles proposé par l’un de leurs thérapeutes habituels, ce qui n’a jamais été réalisé auprès de cette population alors que la demande est importante. Vingt-deux enfants avec SPW âgés de 5 à 10 ans répartis sur le territoire national ont été inclus soit dans un groupe expérimental (qui bénéficie du programme), soit dans un groupe contrôle. Trois évaluations ont été effectuées à domicile : pré-test et post-tests immédiat et différé. Les résultats révèlent que le programme d’intervention proposé aux enfants du groupe expérimental leur a été bénéfique et leur a permis d’améliorer un certain nombre de compétences émotionnelles, notamment en production, en reconnaissance et en régulation. Au regard de ces résultats, les limites et intérêts de cette étude ainsi que les possibilités d’évolution du programme d’intervention sont discutés.
Prader-Willi syndrome (PWS) is a rare genetic disease that causes hormonal and neurodevelopmental disorders. People with PWS have social and emotional difficulties. The few available studies have shown deficits in emotion recognition or comprehension, but these results are not sufficiently precise to propose adapted assessment and interventions based on emotions. The aims of our research were to analyze the emotional deficits presented by children with PWS by developing and testing an intervention program on emotions conducted by the children’s regular therapists, which has never been done before in this population, whereas there are significant needs. Twenty-two 5 to 10-year-old children with PWS were included in experimental vs. control groups; children in the experimental group benefited from the intervention program established by their therapist. The three assessments were performed at home: pre-test and immediate and delayed post-tests. Results show that the intervention program was beneficial for children of the experimental group and enabled them to improve some emotion abilities such as emotional expression production, emotion recognition, and emotion regulation. These results will be discussed in the light of possibilities of enhancement of intervention programs.
Pour explorer si la loi de 2005 a pu contribuer a un meilleur acces a la scolarisation des enfants avec handicap psychique severe, nous comparons la scolarisation de tous les enfants de Haute-Garonne accueillis en hopital de jour de psychiatrie avant la loi (2005) et cinq ans apres (2010). Le pourcentage d’enfants non scolarises reste stable (6,5 %) ; le nombre d’enfants scolarises en milieu scolaire ordinaire augmente : en 2010, pres des trois quarts des enfants beneficient d’une scolarisation individuelle en classe ordinaire, souvent avec auxiliaire de vie scolaire ; le temps de scolarisation a l’ecole de l’hopital diminue, au profit d’une scolarisation partagee avec l’ecole ordinaire ; la coordination s’ameliore entre partenaires du soin et de l’enseignement.
Abstract Background People with Prader-Willi Syndrome (PWS) experience great difficulties in social adaptation that could be explained by disturbances in emotional competencies. However, current knowledge about the emotional functioning of people with PWS is incomplete. In particular, despite being the foundation of social adaptation, their emotional expression abilities have never been investigated. In addition, motor and cognitive difficulties - characteristic of PWS - could further impair these abilities. Method To explore the expression abilities of children with PWS, twenty-five children with PWS aged 5 to 10 years were assessed for 1) their emotional facial reactions to a funny video-clip and 2) their ability to produce on demand the facial and bodily expressions of joy, anger, fear and sadness. Their productions were compared to those of two groups of children with typical development, matched to PWS children by chronological age and by developmental age. The analyses focused on the proportion of expressive patterns relating to the target emotion and to untargeted emotions in the children’s productions. Results The results showed that the facial and bodily emotional expressions of children with PWS were particularly difficult to interpret, involving a pronounced mixture of different emotional patterns. In addition, it was observed that the emotions produced on demand by PWS children were particularly poor and equivocal. Conclusions As far as we know, this study is the first to highlight the existence of particularities in the expression of emotions in PWS children. These results shed new light on emotional dysfunction in PWS and consequently on the adaptive abilities of those affected in daily life.
Obesity is an epidemic problem not limited to Western society, but also in emerging industrial nations with large populations, especially in Asia. The connection between the gut and the brain is probably one of the most promising therapeutic targets for the treatment of obesity and metabolic syndrome. This book brings together reviews on the current understanding of how the gut and brain communicate in the regulation of metabolism. Individual chapters explore novel aspects of this interaction. A comprehensive update on the roles of smell and taste, the gut microbiome, and novel gut-derived neuropeptides in regulating metabolism via the brain is offered. Furthermore, the regulation of insulin sensitivity in the brain is discussed in detail. This overview of current findings is meant to spark new ideas and/or approaches, thus leading to much-needed new medical treatments. Physicians involved in the treatment of metabolic disease and scientists performing research in the fields of nutrition and obesity will find this book to be a valuable addition to their bookshelves.
Pour explorer si la loi de 2005 a pu contribuer à un meilleur accès à la scolarisation des enfants avec handicap psychique sévère, nous comparons la scolarisation de tous les enfants de Haute-Garonne accueillis en hôpital de jour de psychiatrie avant la loi (2005) et cinq ans après (2010). Le pourcentage d’enfants non scolarisés reste stable (6,5 %) ; le nombre d’enfants scolarisés en milieu scolaire ordinaire augmente : en 2010, près des trois quarts des enfants bénéficient d’une scolarisation individuelle en classe ordinaire, souvent avec auxiliaire de vie scolaire ; le temps de scolarisation à l’école de l’hôpital diminue, au profit d’une scolarisation partagée avec l’école ordinaire ; la coordination s’améliore entre partenaires du soin et de l’enseignement.
Patients with Prader-Willi syndrome (PWS) display poor feeding and social skills as infants and fewer hypothalamic oxytocin (OXT)-producing neurons were documented in adults. Animal data demonstrated that early treatment with OXT restores sucking after birth. Our aim is to reproduce these data in infants with PWS.We conducted a phase 2 escalating dose study of a short course (7 days) of intranasal OXT administration. We enrolled 18 infants with PWS under 6 months old (6 infants in each step) who received 4 IU of OXT either every other day, daily, or twice daily. We investigated the tolerance and the effects on feeding and social skills and changes in circulating ghrelin and brain connectivity by functional MRI.No adverse events were reported. No dose effect was observed. Sucking assessed by the Neonatal Oral-Motor Scale was abnormal in all infants at baseline and normalized in 88% after treatment. The scores of Neonatal Oral-Motor Scale and videofluoroscopy of swallowing significantly decreased from 16 to 9 (P < .001) and from 18 to 12.5 (P < .001), respectively. Significant improvements in Clinical Global Impression scale scores, social withdrawal behavior, and mother-infant interactions were observed. We documented a significant increase in acylated ghrelin and connectivity of the right superior orbitofrontal network that correlated with changes in sucking and behavior.OXT is well tolerated in infants with PWS and improves feeding and social skills. These results open perspectives for early treatment in neurodevelopment diseases with feeding problems.
