Cognitive features that distinguish preschool-age children with neurofibromatosis type 1 from their peers: a matched
Jennifer Lorenzo1, Belinda Barton, Shelley S Arnold
1Institute for Neuroscience and Muscle Research, The Children's Hospital at Westmead, New South Wales, Australia; Discipline of Pediatrics and Child Health, Faculty of Medicine, University of Sydney, New South Wales, Australia.
Insights
Children with neurofibromatosis type 1 (NF1) show poorer cognitive skills, including general intelligence, language, and visual perception, by age 40 months. Early identification and intervention are recommended to support their development.
Area of Science:
- Neurodevelopmental disorders
- Pediatric neurology
- Cognitive psychology
Background:
- Neurofibromatosis type 1 (NF1) is a genetic disorder associated with cognitive deficits.
- Understanding the specific cognitive profile in young children with NF1 is crucial for early intervention.
Purpose of the Study:
- To assess the cognitive functioning of 40-month-old children diagnosed with NF1.
- To compare cognitive abilities between children with NF1 and a matched control group.
Main Methods:
- A case-control study involving 43 children with NF1 and 43 controls, matched for age, sex, and maternal education.
- Cognitive assessment using the Wechsler Preschool and Primary Scale of Intelligence, Third Edition, and specific tests for language, visual perception, and executive functioning.
- Parental questionnaires on child behavior were also utilized.
Main Results:
- Children with NF1 exhibited significantly lower general intelligence compared to controls.
- NF1 group showed deficits in language, visual perception, response inhibition, and preliteracy skills.
- Specific subtests of the Wechsler Preschool and Primary Scale of Intelligence, Third Edition, predicted group membership; no behavioral differences were reported by parents.
Conclusions:
- Young children with NF1 demonstrate significant impairments in intellectual functioning, expressive language, and visual perception, even after controlling for demographic factors.
- These cognitive differences are detectable in the preschool years and can impact early academic performance.
- Targeted interventions focusing on these cognitive areas are essential to optimize developmental outcomes for children with NF1.
Objective:
To examine the cognitive functioning of 40-month-old children with neurofibromatosis type 1 (NF1).
Study Design:
In this case-control study, 43 children with NF1 and 43 comparison children (matched by age, sex, and maternal years of education) were assessed using the Wechsler Preschool and Primary Scale of Intelligence, Third Edition. Language, visual perception, preliteracy, and executive functioning were also examined. Parents completed questionnaires about their child's behavior. Group differences were examined using the paired-samples t test or the related Wilcoxon signed rank test. Conditional logistic regression was conducted to identify which cognitive variables predicted group membership (ie, NF1 or control).
Results:
The NF1 group had significantly poorer general intelligence than matched comparisons. Preschool-age children with NF1 had significantly poorer language, visual perception, response inhibition, and preliteracy skills than comparison children. The Wechsler Preschool and Primary Scale of Intelligence, Third Edition, Object Assembly and Information subtests were significant predictors of group membership. Parent ratings indicated no group differences in behavior.
Conclusions:
After accounting for potentially confounding variables of age, sex, and maternal years of education, young children with NF1 have significantly poorer intellectual functioning, expressive language, and visual perception. These cognitive features that distinguish young children with NF1 from healthy peers can be detected in the preschool age group and are likely to impact on learning and performance during early school years. These areas should be targeted for intervention to maximize the developmental outcomes of young children with NF1.
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