Pilot study of the effectiveness of a telehealth group for improving peer relationships for adolescents with neurofibromatosis type 1.

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.

Psychometric Properties of Attention Measures in Young Children with Neurofibromatosis Type 1: Preliminary Findings.

Children with neurofibromatosis type 1 (NF1) often demonstrate difficulties with attention and executive functioning that can be evident starting at a young age. There has been little research about which measures of attention are most suitable for use with young children with NF1. This pilot study explored several computerized measures of attention, a digits forward task, and parent report measures of attention to compare their reliability, validity, and the degree to which they capture attention difficulty in this population. Participants with NF1 ages 4 to 6 years were seen for one (n=2) or two (n=18) time points. Statistical analyses for evaluating evidence for test-retest reliability, convergent and discriminant validity, practice effects, and identification of difficulties were conducted. Each measure demonstrated relative strengths and weaknesses, and there may not be a "one size fits all" measure for use with young children with NF1. However, the Behavior Rating Inventory of Executive Function Preschool/Second Edition, Conners Early Childhood Inattention/Hyperactivity Scale, and the Conners Kiddie Continuous Performance Test Second Edition generally had the highest reliability and most evidence of validity. More specific recommendations are provided for the appropriate measure to use in clinical and research batteries.

Brief Report: A Pilot Study Examining the Effects of PEERS® for Adolescents Telehealth for Autistic Adolescents.

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.

Patterns of performance of children with neurofibromatosis type 1 on the K-CPT and K-CPT 2.

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.

Longitudinal Investigation of Early Motor Development in Neurofibromatosis Type 1.

OBJECTIVE: Research indicates that children with neurofibromatosis type 1 (NF1) have weaknesses in fine and gross motor development in early childhood; however, little is known about the stability and developmental trajectory of motor functioning. We investigated (1) whether motor difficulties are evident and stable in the preschool period in children with NF1 and (2) whether there are particular patterns of motor development in this population. METHODS: Participants with NF1 and a control group of unaffected siblings were enrolled at ages 3-8 years and were assessed yearly. Motor functioning was assessed longitudinally using the Scales of Independent Behavior-Revised Motor Scale and the Differential Ability Scales-II Copying subtest. Wilcoxon sign tests were used to compare motor functioning at 3 or 4 years to 5 or 6 years old for children with NF1 seen during both time periods (N = 27). Linear mixed model growth curve analyses were used to compare trajectories for both children with NF1 (N = 62) and unaffected siblings (N = 37). RESULTS: Children with NF1 made relative gains in raw scores, but not standard scores, across measures. Growth curve analyses revealed a significant effect of NF1 status on gross motor, fine motor, and copying scores, as well as an age by NF1 status effect on fine and gross motor scores. CONCLUSIONS: Motor difficulties are evident early in life in children with NF1. Though children with NF1 clearly acquire motor skills over time, they continue to fall behind unaffected siblings, with the gap potentially widening over time. Further implications are discussed.

Parent-Reported Social Skills in Children with Neurofibromatosis Type 1: Longitudinal Patterns and Relations with Attention and Cognitive Functioning.

OBJECTIVE: Social skills difficulties are commonly reported by parents and teachers of school age (SA) children with neurofibromatosis type 1 (NF1). Investigations of social skills of young children with NF1 are scarce. This study aimed to characterize the emergence of social skills challenges beginning in early childhood, examine social skills longitudinally into SA, and explore interrelations with attention-deficit hyperactivity disorder (ADHD) symptomatology and cognitive functioning among children with NF1 cross-sectionally and longitudinally. METHOD: Three samples of children with NF1 and their parents participated: (1) early childhood (n = 50; ages 3-6; mean [M] = 3.96, SD = 1.05), (2) SA (n = 40; ages 9-13; [M] = 10.90, SD = 1.59), and (3) both early childhood and SA (n = 25). Parent-reported social skills (Social Skills Rating System and Social Skills Improvement System), ADHD symptomatology (Conners Parent Rating Scales - Revised and Conners - Third Edition), and parent-reported cognitive abilities (Differential Ability Scales - Second Edition) were evaluated. RESULTS: Parental ratings of social skills were relatively stable throughout childhood. Ratings of social skills at the end of early childhood significantly predicted school-age social skills. Parental ratings of ADHD symptomatology showed significant negative relations with social skills. Early childhood inattentive symptoms predicted school-age social skills ratings. Cognitive functioning was not significantly related to social skills. CONCLUSION: Parent-reported social skills difficulties are evident during early childhood. This work adds to the literature by describing the frequency and stability of social skills challenges in early childhood and in the school-age period in NF1. Research about interventions to support social skills when difficulties are present is needed.

Adaptive Behavior and Executive Functioning in Children with Neurofibromatosis Type 1 Using a Mixed Design.

OBJECTIVE: Children with neurofibromatosis type 1 (NF1) demonstrate poorer adaptive functioning compared with same-aged peers; however, there is limited research about the longitudinal pattern of adaptive behavior. The aim of this investigation was to examine parent-reported adaptive behavior of children with NF1 longitudinally beginning in early childhood and to examine relations with executive functioning. METHOD: Children with NF1 were assessed during early childhood (n = 59; aged 3-7; mean = 4.8, SD = 1.42) or school age (n = 39; aged 9-13; mean = 10.85, SD = 1.58), and a subset was seen at both time points (n = 26). The Scales of Independent Behavior-Revised was used to assess adaptive functioning, and the Behavior Rating Inventory of Executive Function-Preschool Version/Behavior Rating Inventory of Executive Function was used to evaluate everyday executive functioning. RESULTS: Adaptive behavior in early childhood was significantly correlated with adaptive behavior at school age (with the exception of social interaction and communication skills) and was significantly poorer at school age. The frequency of difficulties increased over time for overall adaptive behavior and motor skills. Executive functioning was related to adaptive behavior cross-sectionally within early childhood and at school age and showed longitudinal predictive value over time. CONCLUSION: This research contributes to the limited NF1 adaptive behavior literature by characterizing the longitudinal pattern of adaptive behavior and relations with executive abilities.

Social skills and autism spectrum disorder symptoms in children with neurofibromatosis type 1: evidence for clinical trial outcomes.

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.