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Congenital adrenal hyperplasia often leads to impaired executive function
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Adolescents and adults with congenital adrenal hyperplasia demonstrated normal psychometric intelligence but had impaired executive functions compared with controls, according to researchers in Sweden.
“Postnatally, patients with CAH are treated with lifelong glucocorticoid replacement therapy and, for the classical form of CAH, treatment with fludrocortisone is also necessary,” Svetlana Lajic, MD, PhD, of the department of women’s and children’s health at Karolinska University Hospital, Stockholm, and colleagues wrote. “During different periods throughout life, it may be difficult to achieve perfect dosing of glucocorticoid replacement therapy, leading to over- or under-treatment and, in both cases, a plausible negative effect on cognition over time. The additive negative effect of salt-wasting crises and hypoglycemia may also contribute to the cognitive outcome seen among patients with CAH.”
The researchers performed an observational study of 55 patients with congenital adrenal hyperplasia (CAH), comparing them with 58 healthy controls. All participants were aged 16 to 33 years, and nine patients with CAH had received prenatal dexamethasone treatment. Lajic and colleagues administered standardized neuropsychological tests and executive functioning questionnaires to both groups.
The two groups did not show significant differences in learning and memory, and patients with CAH showed normal general intelligence, the researchers reported. However, patients with CAH performed worse in tests of verbal working memory (P = .024), inhibition (P = .002) and visual-spatial working memory (P = .005 and P = .003) compared with controls. Male patients with CAH had lower scores for fluid intelligence and nonverbal logical reasoning than male controls (P = .033).
Patients with salt-wasting and simple virilizing CAH performed equally, Lajic and colleagues wrote, although those with a null genotype showed a poorer performance compared with a non-null genotype, as well as a worse performance in fluid intelligence and nonverbal logical reasoning (P = .042). Women who were prenatally treated had poorer scores on “most cognitive measures” than those who were not, the researchers reported.
“Our results show that patients with CAH have normal general intelligence and learning and memory performance,” Lajic and colleagues wrote. “However, we observed poorer performance in tests of executive functions than controls, ie, both the verbal and visuospatial working memory and inhibition were affected. ... It is important to assess all patients with CAH to detect cognitive impairment at an early stage in order to be able to give individual support as these deficits may affect learning and school outcomes.” – by Andy Polhamus
Disclosures: The authors report no relevant financial disclosures.
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