Systematic Review and Meta-analysis of Executive Functions in Preschool and School-Age Children With Neurofibromatosis Type 1

Abstract

Objectives:Neurofibromatosis type 1 (NF1) is a genetic disorder in which the most frequent complication in children is learning disabilities. Over the past decade, growing arguments support the idea that executive dysfunction is a core deficit in children with NF1. However, some data remain inconsistent. The aim of this study was to determine the magnitude of impairment for each executive function (EF) and clarify the impact of methodological choices and participant’s characteristics on EFs.Methods:In this meta-analysis, 19 studies met the selection criteria and were included with data from a total of 805 children with NF1 and 667 controls. Based on the Diamond’s model (2013), EF measures were coded separately according to the following EF components: working memory, inhibitory control, cognitive flexibility, planning/problem solving. The review protocol was registered with PROSPERO (International prospective register of systematic reviews; CRD42017068808).Results:A significant executive dysfunction in children with NF1 is demonstrated. Subgroup analysis showed that the impairment varied as a function of the specific component of executive functioning. The effect size for working memory and planning/problem solving was moderate whereas it was small for inhibitory control and cognitive flexibility. Executive dysfunction seems to be greater with increasing age whereas assessment tool type, intellectual performance, attention deficit hyperactivity disorder and control group composition did not seem to affect EF results.Conclusions:EF deficits are a core feature in children with NF1 and an early identification of executive dysfunctions is essential to limit their impact on the quality of life. (JINS, 2018,24, 977–994)

Can the Cognitive Phenotype in Neurofibromatosis Type 1 (NF1) Be Explained by Neuroimaging? A Review

“…-Language disabilities in about 50% of the cases, especially in phonological processes (2). -Attention deficit according to the diagnostic criteria for Attention Deficit and Hyperactivity Disorder (ADHD) in 30 to 40% of the cases (3, 4), and in general, executive function deficits (5). -Social cognition deficit, sometimes with the Autism Spectrum Disorder (ASD) criteria (6).…”

Cognitive Issues Experienced by Individuals Living with Neurofibromatosis

“…Despite this, executive dysfunction has been noted on performance measures as well. Beaussart et al [50] conducted a meta-analysis of executive functioning in children with NF1. They included 19 studies in their analysis, with a total of 805 children with NF1 and 667 control subjects.…”

“…Several studies have documented verbal and visual working memory weaknesses [10,14,49]. Beaussart et al [50] found a moderate to large effect size for working memory problems. The effect size for verbal working memory was larger than nonverbal working memory.…”

“…The effect size for verbal working memory was larger than nonverbal working memory. They note that differences in effect size may be due to the psychometric properties of the working memory tasks [50].…”

Associations between cognitive performance and sigma power during sleep in children with attention-deficit/hyperactivity disorder, healthy children, and healthy adults

“…Attention-deficit/hyperactivity disorder (ADHD) is a neurodevelopmental disorder characterized by the core symptoms of inattention, hyperactivity, and impulsivity [19] affecting 2.6–4.5 percent of children worldwide [20]. Children with ADHD display deficits in several cognitive domains ranging from simple alertness performance to intellectual capacities [21–24]. Besides this, ADHD is accompanied by subjective sleep complaints [25–28].…”