Executive function profiles of males and females with an additional X chromosome.
| In addition to the abstract below Lennie Wilson of KS&A made a comment: "Here is an abstract of the NIMH team’s work comparing the girls with an extra X to the boys with an extra X. It’s also the first published work from Giedd and Lenroot addressing neuropsychological function for the girls" |
| 1. the girls do indeed have executive functioning deficits! Some families might greet this information with sadness, but this provides evidence that the girls have problems. It will help to get the girls vital services. 2. Notice that the IQ scores are very similar between the boys and girls, even though the boys are older. This is in contrast to the Robinson, Bender & Linden prospective studies out of Denver, which if memory serves me right, the girls had lower mean IQ scores than the boys. 3. Both the girls and the boys showed executive functioning deficits when compared to children matched for socioeconomic status. This is an important finding because with brain and behavioral functioning, there is evidence that dysfunctions go up as the SES goes down. 4. Notice that the girls had impaired performance in both verbal and nonverbal domains. This will be a relief to mom’s who have struggled mightily when being told that their daughter doesn’t fit the profile of trisomy X… there must be something else going on…. 5. Notice the average girl age is 10. 6. I hope I hear a collective sigh when you get this email, it confirms what we have known; demand services for the girls! |
Autors: Lee, N., Wallace, G., Weddle, C., Liverpool, M., Clasen, L., Blumenthal, J., et al. (2009). Background: Research has suggested that the neuropsychological phenotypes of Klinefelter’s (XXY) and Trisomy X (XXX) syndromes are characterized by executive function (EF) weaknesses. However, EF has not been examined extensively in a large sample of young people with XXY and XXX utilizing both laboratory and parent-report measures. Here we aimed to describe the EF skills of a large sample of children and young adults with XXY (n = 59; mean age: 14 ± 5 years; mean IQ: 98.65 ± 15.63) and XXX (n = 37; mean age: 10 ± 5 years; mean IQ: 94.78 ± 15.04) relative to two groups of gender and age-matched typically developing control participants; one matched on socioeconomic status (SES) and one matched on IQ. Both groups were anticipated to present with EF weaknesses, particularly relative to SES-matched controls. Based on prior research, it was expected that males with XXY would have more pronounced deficits on EF tasks with greater verbal demands, while females with XXX would have impaired performance across verbal and nonverbal domains. |
| Method: Participants completed EF tasks (Verbal Fluency, Trailmaking, Stockings of Cambridge [SOC], Spatial Working Memory [SWM]) as part of a neuroimaging and neuropsychological study of sex chromosome variation. Parents completed the Behavior Rating Inventory of Executive Function (BRIEF). |
| Results: SES-matched controls outperformed both the XXY and XXX groups on all EF laboratory tests. When compared to IQ-matched controls, the XXY group demonstrated significant weaknesses on Verbal Fluency and Trailmaking, while the XXX group demonstrated significant weakness on SOC and SWM. Both groups showed EF deficits relative to published norms on the BRIEF. Conclusion: These results indicate that both groups present with EF weaknesses; however, the pattern of these weaknesses differs. According to parent report, both groups also present with EF difficulties in everyday life. These findings will be discussed in relation to extant neuropsychological and neuroimaging studies of these groups. |