Engaging and Adaptive Assessment for Attention in 16p11.2 Deletion Syndrome

Background:

16p11.2 deletion syndrome is associated with a wide range of neurodevelopmental conditions including Attention Deficit and Hyperactivity Disorder (ADHD) and Autism. Despite the high prevalence of ADHD in this population, estimated to be at least 19%, their cognitive control deficits have not been directly measured. Traditional measures of selective attention are often limited by floor effects and lack of participant engagement.  Consequently, we hypothesize that a digital platform assessment, with engaging game-like function and adaptive algorithms, would be a more powerful measure of selective attention deficits.

Objectives:

In this study, we aim to determine if children with 16p11.2 deletion show deficits in visual motor processing and sustained attention using the Project: EVO^TM (EVO) cognitive assessment.  We further investigate sustained and selective attention using standard cognitive measures to investigate whether a difference in delivery platform changes our ability to detect deficits in this clinical population.

Methods:

We recruited children with and without 16p11.2 genetic copy number variations for this cognitive assessment study. Twenty children had a 16p11.2 deletion (mean age 10.1 years +/- 3.9, 6 females) and they were compared with 91 children without known 16p11.2 copy number variation, 16 of which were siblings of the affected individuals (mean age 10.7 years +/-2.2, 41 females). We recruited carrier children and their non-carrier siblings at the Simons VIP annual meetings (Orlando, 2013 and Tyson’s Corner, 2015).  Neurotypical controls and one family with 16p11.2 deletion were recruited from the UCSF Sensory Neurodevelopment & Autism Program and collaborations with Akili interactive. Children were administered two traditional measures of selective attention, the Flanker Task and the Visual Search Task and the EVO assessment. EVO consists of three assessment components: visuomotor tracking (participants must steer the iPad through winding paths), perceptual discrimination (responding to relevant targets while ignoring distractors), and multitasking (preforming tracking and discrimination, simultaneously). In addition, we assessed basic motor speed with an iPad tapping assessment.

Results:

Using an ANCOVA analysis (controlling for age), we found no significant difference between response time or response time variability in children with or without 16p11.2 deletions on standard, non-adaptive, assessment tools: the Flanker Task and the Visual Search Task. Using the EVO platform, children with 16p11.2 deletions displayed significantly lower visuomotor tracking level, slower response time, and higher response time variability relative to their siblings and unrelated children without 16p11.2 deletion. A group effect was not present for multitasking abilities. In a subset of children who were able to complete these tasks, we found no statistical difference in motoric tapping speed between groups, suggesting that observed performance disparities using EVO for response time and response time variability are not simply a result of impaired motoric control or familiarity with iPad use.

Conclusions:

These findings suggest that children with 16p11.2 deletion have measurable performance deficits in selective attention and visuomotor tracking compared to related and un-related children without this genetic mutation. Furthermore, an engaging, adaptive assessment tool may be a more powerful assessment strategy than traditional measures.