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Background: Interventions for social difficulties have not been investigated in the neurofibromatosis type 1 (NF1) population despite observations of elevated rates of social difficulties. In this pilot study, the effectiveness of a 14-week telehealth PEERS® intervention with nineteen adolescents with NF1 (Mage=13.79 years, SD = 1.32) with social skills difficulties was examined. Measures of social outcomes were completed at three timepoints (before, immediately after, and at 14-week follow-up). Results: Caregiver-reported social-emotional skills, social impairment, caregiver-reported number of adolescent get-togethers, and teen social knowledge showed significant improvement following the intervention. Conclusions: The PEERS® intervention is promising to support the social and friendship skills of adolescents with NF1 who have social difficulties. [ABSTRACT FROM AUTHOR]

A systematic review was conducted to determine the scope and nature of the literature for adolescents with neurofibromatosis type 1 (NF1) by (1) characterizing the NF1 cognitive and psychosocial literature focusing on adolescence; (2) examining the risk of bias within the available literature; and (3) describing the extent to which the literature provides demographic information. Forty articles included adolescents in their samples, though most did not focus on adolescence specifically. This review underscores gaps in our understanding of cognitive and behavioral functioning and in reporting relevant demographic information at this critical developmental timepoint. [ABSTRACT FROM AUTHOR]

Children with neurofibromatosis type 1 (NF1) often have attention difficulties with emerging evidence that these difficulties can be seen even in early childhood. This study aimed to explore the relative utility of two versions of a commonly used computerized attention measure for young children with NF1 and to explore relations with parent-reported attention in young children with NF1. Two independent samples of young children with NF1 participated. One sample (Study 1; N = 22; Mage = 4.95 (SD = 0.66)) completed the Conner's Kiddie Continuous Performance Test (K-CPT). A second and separate sample (Study 2; N = 19; Mage = 5.46 (SD = 0.74)) completed the K-CPT second edition (K-CPT 2). Relations of the K-CPT and K-CPT 2 with concurrent parent-reported attention (Kiddie Disruptive Behavior Disorder Schedule; Conners parent report questionnaires) were explored. The K-CPT sample's scores significantly differed from the normative median on Commissions, Hit Rate Standard Error, Variability, Detectability, Perseverations, and Hit Rate Inter Stimulus Interval. No relations with parent-report were identified. The K-CPT 2 sample's scores were significantly worse than normative data on every score except Hit Rate Block Change. Multiple scores on the K-CPT 2 were significantly related to parent-report of inattention and hyperactivity with some evidence of construct validity for the distinction between inattention and hyperactivity. The K-CPT 2 may be more useful for the assessment of attention problems in young children with NF1 as more challenges were observed and performance was more closely related to parent-reported attention difficulties than its predecessor the K-CPT. [ABSTRACT FROM AUTHOR]

Objective: Social functioning patterns vary across measures in children with neurofibromatosis type 1 (NF1; Glad et al., 2021) with broad psychosocial screening measures having shown no impairment (Klein-Tasman et al., 2014; Martin et al., 2012; Sangster et al., 2011) while a more specific social functioning measure indicated poorer social skills (Barton & North, 2004; Huijbregts & de Sonneville, 2011; Loitfelder et al., 2015). The current aims were to characterize caregiver-reported social skills using three different measures and determine which measure appears to best capture social difficulties for young children with NF1. Participants and Methods: Fifty children with NF1 (31 males; M=3.96, SD=1.05) and 20 unaffected siblings (11 males; M =4.34, SD =0.88) in early childhood (ages 3-6) were rated by a caregiver on one social functioning measure (the Social Skills scale on the Social Skills Rating System (SSRS)) and two broader functioning measures that include assessment of social functioning (the Social Skills scale on the Behavior Assessment System for Children-Second Edition (BASC-2), Social Interaction and Communication domain on the Scales of Independent Behavior-Revised (SIB-R)). Results: For children with NF1, the SSRS mean standard score was significantly lower than the BASC-2 and SIB-R (f=-5.11, p<.001; f=-4.63, p<.001) while there was no significant difference between the BASC-2 and SIB-R. No significant differences emerged between measures for unaffected siblings. No significant group differences in mean standard score were found for the SSRS, BASC-2 or SIB-R. Fisher's exact tests revealed the NF1 group had significantly more frequent difficulties than unaffected siblings on the BASC-2 (p=.017) but not on the SSRS or SIB-R. For both groups, Cochran's Q tests determined a significant difference in the proportion of identified social difficulties across measures (NF1: X2(2)=16.33, p<.001; Siblings: X2(2)=9.25, p=.01). Follow up McNemar's tests demonstrated significantly more difficulties reported on the SSRS compared to the BASC-2 for both groups (NF1: p<.001; Siblings: p=.016). Significantly more frequent difficulties were also reported on the SSRS compared to the SIB-R for the NF1 group (p=.002) but not for the unaffected siblings group. No difference in the frequency of difficulties was evident between the BASC-2 and SIB-R for either group. Conclusions: Social skills difficulties appear to be best captured using the SSRS in young children, particularly for children with NF1 as this measure resulted in the lowest mean score and the greatest frequency of difficulties observed within the NF1 group. However, it is notable that group differences in comparison to unaffected siblings were not observed in mean score or frequency of difficulties, such that these young children with NF1 are not showing marked social challenges but rather, social difficulties may be mild when present at this age. Nevertheless, using a measure that specifically targets social functioning, rather than a measure where social functioning is merely a component of a broad measure, appears beneficial to capturing social difficulty. Using measures that best capture social difficulties will contribute to early identification and assessment of intervention effectiveness. Further work with additional age ranges and longitudinal trajectory is needed. [ABSTRACT FROM AUTHOR]

The COVID-19 pandemic sparked a worldwide transition to providing online services overnight, highlighting the urgent need for empirically supported telehealth interventions. The current study examined the effects of PEERS® for Adolescents Telehealth, an adaptation from the original social skills intervention developed for in-person provision, among 22 autistic adolescents and their caregivers. To evaluate the intervention, caregivers completed questionnaires assessing core autistic features and frequency of get-togethers. Adolescents completed questionnaires measuring social knowledge and frequency of get-togethers. Improvements in social skills knowledge, increased get-togethers, and decreased core autistic symptoms were evident. Preliminary results suggest PEERS® for Adolescents Telehealth improves social competence, as found for the in-person version. Further research exploring the equivalence of telehealth to in-person social skills intervention is recommended. [ABSTRACT FROM AUTHOR]

Aim: We examined key features of two outcome measures for social dysfunction and autism spectrum disorder traits, the Social Responsiveness Scale, Second Edition (SRS-2) and the Social Skills Improvement System - Rating Scales (SSIS-RS), in children with neurofibromatosis type 1 (NF1). The aim of the study was to provide objective evidence as to which behavioural endpoint should be used in clinical trials.Method: Cross-sectional behavioural and demographic data were pooled from four paediatric NF1 tertiary referral centres in Australia and the United States (N=122; 65 males, 57 females; mean age [SD] 9y 2mo [3y], range 3-15y).Results: Distributions of SRS-2 and SSIS-RS scores were unimodal and both yielded deficits, with a higher proportion of severely impaired scores on the SRS-2 (16.4%) compared to the SSIS-RS (8.2%). Pearson's product-moment correlations revealed that both questionnaires were highly related to each other (r=-0.72, p<0.001) and to measures of adaptive social functioning (both p<0.001). Both questionnaires were significantly related to attention-deficit/hyperactivity disorder symptoms, but only very weakly associated with intelligence.Interpretation: The SRS-2 and SSIS-RS capture social dysfunction associated with NF1, suggesting both may be suitable choices for assessing social outcomes in this population in a clinical trial. However, careful thought needs to be given to the nature of the intervention when selecting either as a primary endpoint.What This Paper Adds: The Social Responsiveness Scale, Second Edition yielded a large deficit relative to population norms. The Social Skills Improvement System - Rating Scales yielded a moderate deficit relative to population norms. Both scales were highly correlated, suggesting that they are measuring a unitary construct. [ABSTRACT FROM AUTHOR]