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Cognitive function in children with classic congenital adrenal hyperplasia

Sherifa Ahmed Hamed1, Kotb Abbass Metwalley2, Hekma Saad Farghaly3

  • 1Department of Neurology and Psychiatry, Faculty of Medicine, Assiut University, Assiut, Egypt.

European Journal of Pediatrics
|August 9, 2018
PubMed

Insights

Children with salt wasting congenital adrenal hyperplasia (CAH) may experience lower intelligence quotient (IQ) and cognitive deficits. These deficits are potentially linked to glucocorticoid dosage, androgen levels, and hyponatremic episodes.

Area of Science:

  • Pediatric Endocrinology
  • Neuropsychology
  • Genetics

Background:

  • Congenital adrenal hyperplasia (CAH) comprises inherited disorders affecting cortisol biosynthesis.
  • Existing research on cognitive function in CAH patients is limited and yields conflicting results.

Purpose of the Study:

  • To investigate general intelligence and specific cognitive functions in children with the salt-wasting (SW) form of CAH.
  • To explore the relationship between cognitive function and demographic, clinical, and laboratory variables in these patients.

Main Methods:

  • Assessment of 36 children with classic 21-hydroxylase deficiency SW-type CAH using the Wechsler Intelligence Scale for Children (WISC-III) and Stanford Binet Subsets Test (SBST4).
  • Comparison of cognitive scores between CAH patients and controls.
  • Correlation analysis between full-scale IQ (FSIQ) and clinical/laboratory variables.

Main Results:

  • CAH patients exhibited lower mean full-scale IQ (FS IQ) and performance IQ scores compared to controls.
  • Significant deficits were observed in comprehension, pattern analysis, quantitation, bead memory, and memory for sentences.
  • Lower IQ correlated with poorly controlled medical treatment, higher glucocorticoid dose, elevated 17-OHP and testosterone levels, and a greater number of hyponatremic episodes.

Conclusions:

  • Children with SW-CAH may present with reduced IQ and cognitive impairments.
  • Potential contributing factors include glucocorticoid dosage, androgen excess, and a history of hyponatremic episodes.

Keywords:
CognitionCongenital adrenal hyperplasiaGlucocorticoidsMemoryTestosterone

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