ESPE Abstracts

Objective: The balance between hypocortisolism and hyperandrogenism in patients with classic congenital adrenal hyperplasia (CAH) treated with lifelong glucocorticoid (GC) is challenging. Glucocorticoid receptors are widely expressed in the brain; therefore it has been hypothesized that alterations in the exposure to glucocorticoids may affect cognitive ability in individuals with CAH. Only few studies have addressed this issue in children and results are scanty and conflicting. Aim of our study is to extensively evaluate cognitive function in an homogenous population of children with 21-OHD.

Methods: 24 children (M/F=15/9) with 21-OHD, aged 11±3.1 years, performed a cognitive evaluation through Wechsler Intelligence Scale-IV (WISC-IV), to obtain information about total QI, Verbal Comprehension Index (VCI), Perceptual Reasoning Index (PRI), Working Memory Index (WMI), and Processing Speed Index (PSI). Additionally, an evaluation through NEPSY-II to also explore “attention and executive function” and “memory and learning”.

Results: The average QI of 21-OHD patients (102±13.4) was in the normal range (90-110). At the same time, results of VCI, PRI, and PSI (109.9±13.6, 107.2±12.3, 95±13.4 respectively) were also normal. Interestingly, children with 21-OHD scored lower than normal range in WMI (88±15.9). Moreover the NESPY-II subtest narrative memory, revealed that males scored lower than females (6.8±4.1 vs 12.0±1.0, P=0.03) although results were still within the normal range.

Conclusion: Preliminary results of our study document that children with 21-OHD have normal QI. However, a mild impairment was found in working memory indexes suggesting that 21-OHD might be associated with cognitive alteration mainly involving memory function. Furthermore a greater impairment in narrative memory was found in males in comparison to females, thus raising issues about possible gender differences related to androgen exposure. Further studies on larger samples are needed in order to confirm these results and unravel pathogenesis of brain involvement in children with 21OHD CAH.