Abstract
Children with fragile X syndrome (FXS) exhibit deficits in a variety of cognitive processes within the executive function domain. As working memory (WM) is known to support a wide range of cognitive, learning and adaptive functions, WM computer-based training programs have the potential to benefit people with FXS and other forms of intellectual and developmental disability (IDD). However, research on the effectiveness of WM training has been mixed. The current study is a follow-up “deep dive” into the data collected during a randomized controlled trial of Cogmed (Stockholm, Sweden) WM training in children with FXS. Analyses characterized the training data, identified training quality metrics, and identified subgroups of participants with similar training patterns. Child, parent, home environment and training quality metrics were explored in relation to the clinical outcomes during the WM training intervention. Baseline cognitive level and training behavior metrics were linked to gains in WM performance-based assessments and also to reductions in inattention and other behaviors related to executive functioning during the intervention. The results also support a recommendation that future cognitive intervention trials with individuals with IDD such as FXS include additional screening of participants to determine not only baseline feasibility, but also capacity for training progress over a short period prior to inclusion and randomization. This practice may also better identify individuals with IDD who are more likely to benefit from cognitive training in clinical and educational settings.
Highlights
Fragile X syndrome (FXS) is a genetic condition associated with the full mutation of the fragile X mental retardation 1 (FMR1) gene
The present analysis afforded an opportunity to examine details of the cognitive training process and individual differences that are typically omitted from standard clinical trial reports
Remains an open question, the high-resolution training data we report allowed for identification of more- vs. less-responsive participants and further highlight possibilities to integrate cognitive training paradigms in treatment research for this population
Summary
Fragile X syndrome (FXS) is a genetic condition associated with the full mutation of the fragile X mental retardation 1 (FMR1) gene. Males tend to be more severely affected, with over 90% of males but only 30–50% of females with the full mutation having IQ scores in the intellectually disabled range (IQ < 70; [2]) Extensive research using both neuropsychological testing and functional magnetic resonance imaging (fMRI) studies has demonstrated the significant deficits in executive function (EF) associated with the condition. These deficits include problems with working memory (WM), inhibitory control, cognitive flexibility/perseveration and selective and divided attention [3,4,5,6]. While there has been extensive preclinical research and human clinical trials focused on potential disease-modifying pharmacological treatment, primarily focused on improving behavior, mood and anxiety, there has been limited research targeting cognitive function in FXS
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