Decreased Gross Motor, Working Memory, and Adaptive Behavior in Children with Congenital Adrenal Hyperplasia
Nozomi L Yamashita1, Edwin A Deras1, Sharon H O Apos Neil2,3
1Center for Endocrinology, Diabetes and Metabolism, Children's Hospital Los Angeles, Los Angeles, California, USA.
Insights
Congenital adrenal hyperplasia (CAH) in young children is linked to developmental delays. Infants show motor skill deficits, while older children experience issues with memory and adaptive behaviors, indicating early disease-related impacts.
Area of Science:
- Pediatric Endocrinology
- Neurodevelopmental Disorders
- Genetics
Background:
- Classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is associated with cognitive and brain changes in youth and adults.
- Early developmental skills in pediatric CAH patients are not well understood.
- This study investigates developmental trajectories in young children with CAH.
Purpose of the Study:
- To assess cognitive and motor development in infants, toddlers, and children with classical CAH.
- To identify age-dependent developmental deficits in pediatric CAH patients.
- To understand the early emergence of developmental challenges in CAH.
Main Methods:
- Cross-sectional study of 13 infants/toddlers (0.4-3.5 yrs) and 21 children (3.5-11 yrs) with classical CAH.
- Utilized Bayley-4 for infants/toddlers; WISC-V, NIH Toolbox, WRAML-2, CVLT-C for children.
- Parents completed Vineland-3 for adaptive behavior and motor skills assessment.
Main Results:
- Infants and toddlers with CAH showed significantly lower gross motor skills compared to normative means (p=0.03).
- Children with CAH exhibited deficits in auditory working memory (p=0.01) and nonverbal learning (p=0.02).
- Parents reported lower adaptive behavior (p=0.004) and overall motor skills (p=0.006) in their children with CAH.
Conclusions:
- Pediatric CAH patients display distinct, age-dependent developmental deficits.
- Early gross motor deficits are observed in infants/toddlers with CAH.
- Children with CAH show impaired auditory working memory and adaptive behaviors, suggesting early disease-related impacts.
Introduction:
Youth and adults with classical congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) are at risk for cognitive deficits and brain structural changes. Although factors inherent to CAH have been implicated in cognitive dysfunction, little is known about early developmental skills in these patients. Thus, we aimed to investigate development in younger children with CAH.
Methods:
Cross-sectional study of 13 infants and toddlers (0.4-3.5 yrs) and 21 children (3.5-11 yrs) with classical CAH due to 21OHD. Infants and toddlers completed cognitive and motor skills screening (Bayley-4). Children also completed age-appropriate assessment of cognition (WISC-V Digit Span and Spatial Span subtests, NIH Toolbox-Version 2, WRAML-2 Design Memory subtest, CVLT-C) and motor skills (NIH Toolbox-Version 2). Their parents completed standardized reports of adaptive behavior and motor skills (Vineland-3). T tests compared the CAH group (mean percentile, mean ± SD) with normative means.
Results:
Infants and toddlers with CAH exhibited lower gross motor skills (27th percentile, 8.2 ± 2.4, p = 0.03) compared to the normative mean. Children with CAH exhibited decreased auditory working memory skills (WISC-V, 27th percentile, 8.2 ± 1.8, p = 0.01), and lower nonverbal learning skills (WRAML-2, 21st percentile, 7.6 ± 3.4, p = 0.02). Their parents reported lower adaptive behavior (Vineland-3, 30th percentile, 92.2 ± 9.1, p = 0.004) and overall motor skills (26th percentile, 90.3 ± 11.4, p = 0.006) than the normative mean.
Conclusions:
Pediatric patients with CAH due to 21OHD exhibit distinct, age-dependent developmental deficits, with infants and toddlers displaying lower gross motor performance, and children exhibiting decreased auditory working memory and adaptive behavior skills. These findings suggest that early signs of developmental deficits in CAH may emerge at a younger age than previously recognized, emphasizing the role of clinical factors inherent to the disease.
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