Social behavior in RASopathies and idiopathic autism / Allison M.H. FOY in Journal of Neurodevelopmental Disorders, 14 (2022)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 14 (2022) Titre : Social behavior in RASopathies and idiopathic autism Type de document : texte imprimé Auteurs : Allison M.H. FOY, Auteur ; Rebekah L. HUDOCK, Auteur ; Ryan SHANLEY, Auteur ; Elizabeth I. PIERPONT, Auteur Langues : Anglais (eng) Mots-clés : Autism Spectrum Disorder/complications/psychology  Autistic Disorder/complications  Child  Child, Preschool  Cross-Sectional Studies  Failure to Thrive/complications/genetics  Humans  Social Behavior  Autism  Cardiofaciocutaneous syndrome  Costello syndrome  Neurofibromatosis type 1  Noonan syndrome  Prosocial  RASopathy  Social competence  Social function  Social skills Index. décimale : PER Périodiques Résumé : BACKGROUND: RASopathies are genetic syndromes that result from pathogenic variants in the RAS-MAPK cellular signaling pathway. These syndromes, which include neurofibromatosis type 1, Noonan syndrome, cardiofaciocutaneous syndrome, and Costello syndrome, are associated with a complex array of medical and behavioral health complications. Despite a heightened risk for social challenges and autism spectrum disorder (ASD), few studies have compared different aspects of social behavior across these conditions. It is also unknown whether the underlying neuropsychological characteristics that contribute to social competence and socially empathetic ("prosocial") behaviors differ in children with RASopathies as compared to children with nonsyndromic (i.e., idiopathic) ASD. METHODS: In this cross-sectional, survey-based investigation, caregivers of preschool and school-aged children with RASopathies (n = 202) or with idiopathic ASD (n = 109) provided demographic, medical, and developmental information about their child, including psychiatric comorbidities. For children who were able to communicate verbally, caregivers also completed standardized rating scales to assess social competence and empathetic behavior as well as symptoms of hyperactivity/inattention and emotional problems. RESULTS: As compared to children with idiopathic ASD, children with RASopathies were rated as demonstrating more resilience in the domain of empathy relative to their overall social competence. Similarities and differences emerged in the psychological factors that predicted social behavior in these two groups. Stronger communication skills and fewer hyperactive-impulsive behaviors were associated with increased empathy and social competence for both groups. Greater emotional challenges were associated with lower social competence for children with RASopathies and stronger empathy for children with idiopathic ASD. Among children with RASopathy and a co-occurring ASD diagnosis, socially empathetic behaviors were observed more often as compared to children with idiopathic ASD. CONCLUSIONS: Findings suggest that the development of social behavior among children with RASopathies involves a distinct pattern of strengths and weaknesses as compared to a behaviorally defined disorder (idiopathic ASD). Identification of areas of resilience as well as behavioral and social challenges will support more targeted intervention. En ligne : https://dx.doi.org/10.1186/s11689-021-09414-w Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=574 [article] Social behavior in RASopathies and idiopathic autism [texte imprimé] / Allison M.H. FOY, Auteur ; Rebekah L. HUDOCK, Auteur ; Ryan SHANLEY, Auteur ; Elizabeth I. PIERPONT, Auteur. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 14 (2022) Mots-clés : Autism Spectrum Disorder/complications/psychology  Autistic Disorder/complications  Child  Child, Preschool  Cross-Sectional Studies  Failure to Thrive/complications/genetics  Humans  Social Behavior  Autism  Cardiofaciocutaneous syndrome  Costello syndrome  Neurofibromatosis type 1  Noonan syndrome  Prosocial  RASopathy  Social competence  Social function  Social skills Index. décimale : PER Périodiques Résumé : BACKGROUND: RASopathies are genetic syndromes that result from pathogenic variants in the RAS-MAPK cellular signaling pathway. These syndromes, which include neurofibromatosis type 1, Noonan syndrome, cardiofaciocutaneous syndrome, and Costello syndrome, are associated with a complex array of medical and behavioral health complications. Despite a heightened risk for social challenges and autism spectrum disorder (ASD), few studies have compared different aspects of social behavior across these conditions. It is also unknown whether the underlying neuropsychological characteristics that contribute to social competence and socially empathetic ("prosocial") behaviors differ in children with RASopathies as compared to children with nonsyndromic (i.e., idiopathic) ASD. METHODS: In this cross-sectional, survey-based investigation, caregivers of preschool and school-aged children with RASopathies (n = 202) or with idiopathic ASD (n = 109) provided demographic, medical, and developmental information about their child, including psychiatric comorbidities. For children who were able to communicate verbally, caregivers also completed standardized rating scales to assess social competence and empathetic behavior as well as symptoms of hyperactivity/inattention and emotional problems. RESULTS: As compared to children with idiopathic ASD, children with RASopathies were rated as demonstrating more resilience in the domain of empathy relative to their overall social competence. Similarities and differences emerged in the psychological factors that predicted social behavior in these two groups. Stronger communication skills and fewer hyperactive-impulsive behaviors were associated with increased empathy and social competence for both groups. Greater emotional challenges were associated with lower social competence for children with RASopathies and stronger empathy for children with idiopathic ASD. Among children with RASopathy and a co-occurring ASD diagnosis, socially empathetic behaviors were observed more often as compared to children with idiopathic ASD. CONCLUSIONS: Findings suggest that the development of social behavior among children with RASopathies involves a distinct pattern of strengths and weaknesses as compared to a behaviorally defined disorder (idiopathic ASD). Identification of areas of resilience as well as behavioral and social challenges will support more targeted intervention. En ligne : https://dx.doi.org/10.1186/s11689-021-09414-w Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=574

Social skills in children with RASopathies: a comparison of Noonan syndrome and neurofibromatosis type 1 / Elizabeth I. PIERPONT in Journal of Neurodevelopmental Disorders, 10-1 (December 2018)  

Public ISBD [article]  inJournal of Neurodevelopmental Disorders > 10-1 (December 2018) . - 21 p. Titre : Social skills in children with RASopathies: a comparison of Noonan syndrome and neurofibromatosis type 1 Type de document : texte imprimé Auteurs : Elizabeth I. PIERPONT, Auteur ; Rebekah L. HUDOCK, Auteur ; Allison M. FOY, Auteur ; Margaret SEMRUD-CLIKEMAN, Auteur ; Mary Ella PIERPONT, Auteur ; Susan A. BERRY, Auteur ; Ryan SHANLEY, Auteur ; Nathan RUBIN, Auteur ; Katherine SOMMER, Auteur ; Christopher L. MOERTEL, Auteur Année de publication : 2018 Article en page(s) : 21 p. Langues : Anglais (eng) Mots-clés : Language  Nf1  Neurofibromatosis type 1  Neuropsychological  Noonan syndrome  RASopathies  Social Index. décimale : PER Périodiques Résumé : BACKGROUND: Gene mutations within the RAS-MAPK signaling cascade result in Noonan syndrome (NS), neurofibromatosis type 1 (NF1), and related disorders. Recent research has documented an increased risk for social difficulties and features of autism spectrum disorder (ASD) among children with these conditions. Despite this emerging evidence, the neuropsychological characteristics associated with social skills deficits are not well understood, particularly for children with NS. METHODS: Parents of children with NS (n = 39), NF1 (n = 39), and unaffected siblings (n = 32) between the ages of 8 and 16 years were administered well-validated caregiver questionnaires assessing their child's social skills, language abilities, attention-deficit hyperactivity disorder (ADHD) symptoms and anxiety. RESULTS: With respect to overall social skills, average ratings of children in both clinical groups were similar, and indicated weaker social skills compared to unaffected siblings. Although ratings of social skills were outside of normal limits for more than four in ten children within the clinical groups, most of the deficits were mild/moderate. Fifteen percent of the children with NS and 5% of the children with NF1 were rated as having severe social skills impairment (< - 2SD). Independent of diagnosis, having fewer ADHD symptoms or better social-pragmatic language skills was predictive of stronger social skills. CONCLUSIONS: Amidst efforts to support social skill development among children and adolescents with RASopathies, neuropsychological correlates such as social language competence, attention, and behavioral self-regulation could be important targets of intervention. En ligne : http://dx.doi.org/10.1186/s11689-018-9239-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=386 [article] Social skills in children with RASopathies: a comparison of Noonan syndrome and neurofibromatosis type 1 [texte imprimé] / Elizabeth I. PIERPONT, Auteur ; Rebekah L. HUDOCK, Auteur ; Allison M. FOY, Auteur ; Margaret SEMRUD-CLIKEMAN, Auteur ; Mary Ella PIERPONT, Auteur ; Susan A. BERRY, Auteur ; Ryan SHANLEY, Auteur ; Nathan RUBIN, Auteur ; Katherine SOMMER, Auteur ; Christopher L. MOERTEL, Auteur . - 2018 . - 21 p. Langues : Anglais (eng) in Journal of Neurodevelopmental Disorders > 10-1 (December 2018) . - 21 p. Mots-clés : Language  Nf1  Neurofibromatosis type 1  Neuropsychological  Noonan syndrome  RASopathies  Social Index. décimale : PER Périodiques Résumé : BACKGROUND: Gene mutations within the RAS-MAPK signaling cascade result in Noonan syndrome (NS), neurofibromatosis type 1 (NF1), and related disorders. Recent research has documented an increased risk for social difficulties and features of autism spectrum disorder (ASD) among children with these conditions. Despite this emerging evidence, the neuropsychological characteristics associated with social skills deficits are not well understood, particularly for children with NS. METHODS: Parents of children with NS (n = 39), NF1 (n = 39), and unaffected siblings (n = 32) between the ages of 8 and 16 years were administered well-validated caregiver questionnaires assessing their child's social skills, language abilities, attention-deficit hyperactivity disorder (ADHD) symptoms and anxiety. RESULTS: With respect to overall social skills, average ratings of children in both clinical groups were similar, and indicated weaker social skills compared to unaffected siblings. Although ratings of social skills were outside of normal limits for more than four in ten children within the clinical groups, most of the deficits were mild/moderate. Fifteen percent of the children with NS and 5% of the children with NF1 were rated as having severe social skills impairment (< - 2SD). Independent of diagnosis, having fewer ADHD symptoms or better social-pragmatic language skills was predictive of stronger social skills. CONCLUSIONS: Amidst efforts to support social skill development among children and adolescents with RASopathies, neuropsychological correlates such as social language competence, attention, and behavioral self-regulation could be important targets of intervention. En ligne : http://dx.doi.org/10.1186/s11689-018-9239-8 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=386

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