Cognitive features that distinguish preschool-age children with neurofibromatosis type 1 from their peers: a matched case-control study

Abstract

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.

Keywords: BASC-II; BRIEF-P; Behavior Assessment System for Children, Second Edition; Behavior Rating Inventory of Executive Function, Preschool Version; Developmental Neuropsychological Assessment; EOWPVT; Expressive One-Word Picture Vocabulary Test; NEPSY; NF1; Neurofibromatosis type 1; SES; Socioeconomic status; WPPSI-III; Wechsler Preschool and Primary Scale of Intelligence, Third Edition.