Longitudinal analysis of developmental delays in children with neurofibromatosis type 1
Lauren E Wessel1, Feng Gao, David H Gutmann
11Department of Neurology, Washington University School of Medicine, St. Louis, MO, USA.
Insights
Children with neurofibromatosis type 1 (NF1) experience developmental delays that worsen with age. Gross motor delays are persistent, highlighting the need for early screening and intervention in NF1.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Genetics
Background:
- Neurofibromatosis type 1 (NF1) is associated with diverse developmental delays.
- The natural history and progression of these deficits throughout childhood are not well-documented.
Purpose of the Study:
- To define the natural history of developmental delays in children with NF1.
- To identify specific areas of delay and their progression across different age groups.
Main Methods:
- Cross-sectional study assessing 124 children (0-8 years) with NF1 using the Parents' Evaluation of Developmental Status (PEDs).
- Longitudinal assessment of 43 subjects to track developmental changes over time.
Main Results:
- School-age children (6-8 years) showed significantly more developmental delays than infants (0-2 years) and preschool-age children (3-5 years).
- Delays were more frequent in older children across math, reading, gross motor, fine motor, and self-help domains.
- Longitudinal data indicated frequent migration between delayed and non-delayed status, except for gross motor development, which remained consistently delayed.
Conclusions:
- Developmental delays in children with NF1 tend to increase with age, particularly in gross motor function.
- Early and consistent developmental screening, with a focus on gross motor skills, is crucial for children with NF1.
- Timely intervention strategies should be implemented to address the progressive nature of these delays.
Abstract:
Children with neurofibromatosis type 1 exhibit a variety of developmental delays. However, there is little information about the progression of these deficits over the course of development. Using the Parents' Evaluation of Developmental Status measurement tool, we assessed 124 infants (0-2 years of age), preschool-age children (3-5 years of age), and school-age children (6-8 years of age) with neurofibromatosis type 1 to define the natural history of delays. School-age children exhibited significantly more areas of delay than infants or preschool-age children. Delays in math, reading, gross motor, fine motor, and self-help development were observed more frequently in older than younger children. Finally, analysis of 43 subjects for whom longitudinal assessments were available revealed that children often migrated between delayed and nondelayed groups in all areas except gross motor development. Based on these findings, we advocate early developmental screening and intervention for this at-risk pediatric population, especially in the area of gross motor function.
