Feasibility and acceptability of an online response inhibition cognitive training program for youth with Williams syndrome.

Williams syndrome (WS) is a genetic neurodevelopmental disorder often accompanied by inhibitory difficulties. Online cognitive training programs show promise for improving cognitive functions. No such interventions have been developed for individuals with WS, but to explore the practicality of large-scale online cognitive training for this population, we must first investigate whether families of those with WS find these programs feasible and acceptable. Twenty individuals aged 10-17 years with WS, along with parents, participated in a pilot online cognitive training program supervised in real time using videoconference software. We evaluated the feasibility and acceptability of this response inhibition training using three parent questionnaires. Descriptive data are reported for the measures of feasibility and acceptability. Overall, the online procedures received a positive reaction from families. Parents were likely to recommend the study to others. They indicated training was ethical and acceptable despite feeling neutral about effectiveness. The frequency and duration of sessions were acceptable to families (two 20-to-30-min sessions per week; 10 sessions total). Families provided feedback and offered suggestions for improvement, such as more flexibility in scheduling and decreasing time spent in review of procedures.

A randomized waitlist-controlled pilot trial of voice over Internet protocol-delivered behavior therapy for youth with chronic tic disorders.

INTRODUCTION: Comprehensive Behavioral Intervention for Tics (CBIT) has been shown to be efficacious for chronic tic disorders (CTDs), but utilization is limited by a lack of treatment providers and perceived financial and time burden of commuting to treatment. A promising alternative to in-person delivery is voice over Internet protocol (VoIP), allowing for remote, real-time treatment delivery to patients' homes. However, little is known about the effectiveness of VoIP for CTDs. Therefore, the present study examined the preliminary efficacy, feasibility, and acceptability of VoIP-delivered CBIT (CBIT-VoIP). METHODS: Twenty youth (8-16 years) with CTDs participated in a randomized, waitlist-controlled pilot trial of CBIT-VoIP. The main outcome was pre- to post-treatment change in clinician-rated tic severity (Yale Global Tic Severity Scale). The secondary outcome was clinical responder rate (Clinical Global Impressions - Improvement Scale), assessed using ratings of 'very much improved' or 'much improved' indicating positive treatment response. RESULTS: Intention-to-treat analyses with the last observation carried forward were performed. At post-treatment (10-weeks), significantly greater reductions in clinician-rated, (F(1,18) = 3.05, p < 0.05, partial η(2 )= 0.15), and parent-reported tic severity, (F(1,18) = 6.37, p < 0.05, partial η(2 )= 0.26) were found in CBIT-VoIP relative to waitlist. One-third (n = 4) of those in CBIT-VoIP were considered treatment responders. Treatment satisfaction and therapeutic alliance were high. DISCUSSION: CBIT can be delivered via VoIP with high patient satisfaction, using accessible, low-cost equipment. CBIT-VoIP was generally feasible to implement, with some audio and visual challenges. Modifications to enhance treatment delivery are suggested.

Parent and teacher perspectives about problem behavior in children with Williams syndrome.

Problem behavior of 52 children with Williams syndrome ages 6 to 17 years old was examined based on both parent and teacher report. Generally good inter-rater agreement was found. Common areas of problem behavior based both on parent and teacher report included attention problems, anxiety difficulties, repetitive behaviors (e.g., obsessions, compulsions, picking nose or skin), and social problems, reflecting a robust behavioral phenotype in Williams syndrome present across contexts. Some rater differences were observed; most notably, parents reported more attention and mood difficulties than did teachers, while teachers reported more oppositionality and aggression than did parents. Relations to intellectual functioning, age, and gender were examined. The implications of the findings for understanding the behavioral phenotype associated with Williams syndrome are discussed.

Relations between fine motor skill and parental report of attention in young children with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders presenting in approximately 1 in 3,500 live births. NF1 is a highly variable condition with a large number of complications. A common complication is neuropsychological problems, including developmental delays and learning difficulties that affect as many as 60% of patients. Research has suggested that school-aged children with NF1 often have poorer fine motor skills and are at greater risk for attention difficulties than the general population. Thirty-eight children with NF1 and 23 unaffected children between the ages of 4 and 6 years, who are enrolled in a study of early development in NF1, were included in the present study. Varying levels of fine motor functioning were examined (simple to complex fine motor tasks). For children with NF1, significant difficulties were demonstrated on lab-based mid-level and complex fine motor tasks, even after controlling for nonverbal reasoning abilities, but not on simple fine motor tasks. Parental report also indicated difficulties in everyday adaptive fine motor functioning. No significant correlations were found between complex fine motor ability and attention difficulties. This study provides much needed descriptive data on the early emergence of fine motor difficulties and attention difficulties in young children with NF1.

Language in young children with neurofibromatosis-1: relations to functional communication, attention, and social functioning.

In this study, the language abilities of 30 children with Neurofibromatosis Type 1 (NF1) aged 4-6 years were examined using a standardized measure of language. Relations of language to multiple parental report measures of functional communication, social skills, and attention problems were investigated. Difficulties in core language skills were observed, and more than 1/3 of the children struggled on at least one language index. Language abilities were significantly related to parental report of functional communication, social interaction and communication, and social skills, such that language difficulties may be a risk factor for communication and social interaction challenges and communication-related adaptive behavior in children with NF1. Though receptive language abilities were an area of particular difficulty for many children with NF1, they were not significantly related to parental ratings of social functioning and functional communication. Few significant relations were found between language and parent-reported attention problems, although some trends were noted. Hence attention difficulties in children with NF1 may contribute to, but do not appear to fully account for, language difficulties. In sum, there is an increased risk of language difficulties for young children with NF1, and lab-measured language difficulties appear to relate to everyday communication and social interaction functioning.

Adaptive behavior in young children with neurofibromatosis type 1.

Neurofibromatosis-1 is the most common single gene disorder affecting 1 in 3000. In children, it is associated not only with physical features but also with attention and learning problems. Research has identified a downward shift in intellectual functioning as well, but to date, there are no published studies about the everyday adaptive behavior of children with NF1. In this study, parental reports of adaptive behavior of 61 children with NF1 ages 3 through 8 were compared to an unaffected contrast group (n = 55) that comprised siblings and community members. Significant group differences in adaptive skills were evident and were largely related to group differences in intellectual functioning. In a subsample of children with average-range intellectual functioning, group differences in parent-reported motor skills were apparent even after controlling statistically for group differences in intellectual functioning. The implications of the findings for the care of children with NF1 are discussed.