ESPE Abstracts (2016) 86 P-P1-18

Adult Individuals with Classic Congenital Adrenal Hyperplasia Exhibit Deficits in Executive Functions

Leif Karlsson, Marius Zimmermann, Lena Wallensteen, Michela Barbaro, Anna Nordenström, Tatja Hirvikoski & Svetlana Lajic


Karolinska Institutet, Stockholm, Sweden


Background: Individuals with classic congenital adrenal hyperplasia (CAH) are treated postnatally with glucocorticoids. Earlier research with animals and other disorders with excess GC exposure implicate that GCs can influence memory. Deficits in working memory can be seen already during childhood in children with classic CAH.

Objective: We tested the hypothesis that adult individuals with classic CAH show impaired cognitive functions.

Methods: We evaluated cognitive functions in 100 adult subjects (42 with CAH, mean age 24 years; 58 population controls, mean age 20.5 years). They were assessed with standardized neuropsychological tests: WAIS-IV Matrices (estimation of fluid intelligence), Vocabulary (estimation of verbal intelligence), Digit Span (short term memory/verbal working memory), and Coding. Learning and long-term memory were assessed with the list learning test from WMS-III, and visual-spatial working memory was assessed with the Span Board test. The Stroop test was used to assess Processing speed and Inhibition.

Results: Individuals with CAH showed impaired verbal working memory (P=0.01) and visual-spatial working memory (P=0.01) as well as slower speed of processing (all P<0.05) compared to population controls. The differences had large effect sizes with Cohen’s d=0.8–2.5. There were no differences in verbal intelligence, logical reasoning, coding, or learning and long-term memory between CAH and control subjects.

Conclusion: Having classic CAH seem to be associated with a poorer performance in executive functions. This may be a result of postnatal GC treatment, salt-losing crisis or hypoglycemic episodes. Early detection of deficits is warranted given the importance of working memory for academic performance. Appropriate support should be offered if needed.

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