A Behavioural Assessment of Social Anxiety and Social Motivation in Fragile X, Cornelia de Lange and Rubinstein-Taybi Syndromes.

Unique socio-behavioural phenotypes are reported for individuals with different neurodevelopmental disorders. Here, the effects of adult familiarity and nature of interaction on social anxiety and social motivation were investigated in individuals with fragile X (FXS; n = 20), Cornelia de Lange (CdLS; n = 20) and Rubinstein-Taybi (RTS; n = 20) syndromes, compared to individuals with Down syndrome (DS; n = 20). The Social Anxiety and Motivation Rating Scale was employed whilst participants completed four social tasks, each administered separately by a familiar adult, and also by an unfamiliar adult. Compared to participants with DS, those with FXS and RTS exhibited high levels of social anxiety but similar levels of social motivation. Participants with CdLS showed heightened social anxiety and reduced social motivation only during interactions with an unfamiliar adult when active participation was voluntary.

An experimental study of executive function and social impairment in Cornelia de Lange syndrome.

BACKGROUND: Extreme shyness and social anxiety is reported to be characteristic of adolescents and adults with Cornelia de Lange syndrome (CdLS); however, the nature of these characteristics is not well documented. In this study, we develop and apply an experimental assessment of social anxiety in a group of adolescents and adults with CdLS to determine the nature of the social difficulties and whether they are related to impairments in executive functioning. METHODS: A familiar and unfamiliar examiner separately engaged in socially demanding tasks comprising three experimental conditions with a group of individuals with CdLS (n = 25; % male = 44; mean age = 22.16; SD = 8.81) and a comparable group of individuals with Down syndrome (DS; n = 20; % male = 35; mean age = 24.35; SD = 5.97). Behaviours indicative of social anxiety were coded. The Behavior Rating Inventory of Executive Function-Preschool version, an informant measure of executive function, was completed by participants' caregivers. RESULTS: Significantly less verbalisation was observed in the CdLS group than the DS group in conditions requiring the initiation of speech. In the CdLS group, impairments in verbalisation were not associated with a greater degree of intellectual disability but were significantly correlated with impairments in both planning and working memory. This association was not evident in the DS group. CONCLUSIONS: Adolescents and adults with CdLS have a specific difficulty with the initiation of speech when social demands are placed upon them. This impairment in verbalisation may be underpinned by specific cognitive deficits, although further research is needed to investigate this fully.

Executive functioning in Cornelia de Lange syndrome: domain asynchrony and age-related performance.

BACKGROUND: The aim of this study was to examine executive functioning in adolescents and adults with Cornelia de Lange syndrome (CdLS) to identify a syndrome and age-related profile of cognitive impairment. METHODS: Participants were 24 individuals with CdLS aged 13-42 years (M = 22; SD = 8.98), and a comparable contrast group of 21 individuals with Down syndrome (DS) aged 15-33 years (M = 24; SD = 5.82). Measures were selected to test verbal and visual fluency, inhibition, perseverance/flexibility, and working memory and comprised both questionnaire and performance tests. RESULTS: Individuals with CdLS showed significantly greater impairment on tasks requiring flexibility and inhibition (rule switch) and on forwards span capacity. These impairments were also reported in the parent/carer-rated questionnaire measures. Backwards Digit Span was significantly negatively correlated with chronological age in CdLS, indicating increased deficits with age. This was not identified in individuals with DS. CONCLUSIONS: The relative deficits in executive functioning task performance are important in understanding the behavioural phenotype of CdLS. Prospective longitudinal follow-up is required to examine further the changes in executive functioning with age and if these map onto observed changes in behaviour in CdLS. Links with recent research indicating heightened responses to oxidative stress in CdLS may also be important.

Text or talk? Social anxiety, loneliness, and divergent preferences for cell phone use.

This paper investigates whether social anxiety and loneliness lead to contrasting beliefs and preferences among cell phone users towards texting and talking on their cell phones. Three hypotheses are examined: (1) that social anxiety and loneliness are differentially associated with generalized preferences either for texting or for talking on the cell phone, (2) that these preferences are linked to contrasting beliefs concerning the social functionality of the short message service (SMS), and (3) that these divergent beliefs mediate the effects of social anxiety and loneliness on cell phone users' generalized preferences for texting or talking. Results from an Internet questionnaire (N=158) showed that, whilst lonely participants preferred making voice calls and rated texting as a less intimate method of contact, anxious participants preferred to text, and rated it a superior medium for expressive and intimate contact. These divergent beliefs accounted for 36% and 16% of the variance in preference for texting and voice calls, respectively, and significantly attenuated the influence of loneliness and social anxiety when they were added to the regression equations for these measures. Results are discussed in terms of the hyperpersonal possibilities of mobile communications technologies.

Living with severe mental illness--what families and friends must know: evaluation of a one-day psychoeducation workshop.

One-day "family survival" psychoeducation workshops are a promising, convenient method of disseminating basic information to families with a relative who is diagnosed with a serious mental illness such as schizophrenia, major depression, or other affective disorders. At five separate psychoeducation workshops, 83 participating families completed the self-report North-Sachar Family Life Questionnaire and open-ended "problem lists" of issues facing the families both before and after the workshops. Outcomes consistently demonstrated positive change pre- to post-workshop. Issues reported by workshop participants included desire for education about illness, identification of resources, coping with the illness, and family relationships. The workshop model demonstrated consistent achievement of the outcomes measured, meeting short-term goals. Although models such as the family responsive approach reported in this article are not designed to create long-term gains for the family, they appear to benefit families and may help connect families with more intensive services to facilitate long-term change.

Certification and program regulations for inpatient services to youth with addiction: a state-level analysis.

Recent evidence has indicated that the prevalence of addiction in youth is far higher than previously expected. High demand for services to this population require attention to programs providing interventions, in particular to the regulations and structures that assure youth receive quality services. Thus, the current research examines state-level certification and program requirements for inpatient youth facilities. Current data (from August 2002 through July 2003) on state-level regulations was collected from a variety of sources. Wherever possible, confirmation was sought from multiple sources. Multiple authors interpreted information. Youth with addictions are currently being treated across a wide variety of governmental agencies. Level of detail and clarity of information varied widely, as did distinctness of youth requirements from adult ones. Adequate regulatory attention was evident in a minority of states, while a handful of states did little or nothing to distinguish youth from adult services. While it is undoubtedly the case that providers within each state offer outstanding services, there is often little or no available protection for these youth through governmental regulations. Attention is needed in almost all states to providing additional regulations and program requirements that assure youth consistency in service quality. Recommendations are made for standards of certification requirements.