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Neuropsychological profiles of adults with Klinefelter syndrome

Boone, Kyle Brauer and Swerdloff, Ronald S. and Miller, Bruce L. and Geschwind, Daniel H. and Razani, Jill and Lee, Alison and Gonzalo, Irene Gaw and Haddal, Anna and Rankin, Katherine and Lu, Po and Paul, Lynn (2001) Neuropsychological profiles of adults with Klinefelter syndrome. Journal of the International Neuropsychological Society, 7 (4). pp. 446-456. ISSN 1355-6177.

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Children and adolescents with Klinefelter syndrome (XXY) have been reported to show deficits in language processing including VIQ < PIQ and a learning disability in reading and spelling. However, whether this is characteristic of adults with Klinefelter syndrome has not been established. Thirty-five men with Klinefelter syndrome, aged 16 to 61, and 22 controls were evaluated with a comprehensive neuropsychological battery. The Klinefelter patients scored significantly below controls in language skills, verbal processing speed, verbal and nonverbal executive abilities, and motor dexterity. Within the Klinefelter sample, three cognitive subgroups were identified: VIQ 7 or more points below PIQ (n = 10), VIQ within 6 points of PIQ (n = 12), and PIQ 7 or more points below VIQ (n = 12). The deficits detected in language, verbal processing speed, and verbal executive skills were found to be isolated to the VIQ < PIQ subgroup, while the abnormalities in motor dexterity and nonverbal executive skills were confined to the PIQ < VIQ subgroup. Older age was significantly correlated with increases in VIQ relative to PIQ in the patient group, which suggests the intriguing possibility that the PIQ < VIQ subgroup primarily emerges in young adulthood, perhaps in response to the reported hormonal abnormalities detected in Klinefelter syndrome patients during puberty.

Item Type:Article
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Geschwind, Daniel H.0000-0003-2896-3450
Paul, Lynn0000-0002-3128-8313
Additional Information:© 2001 The International Neuropsychological Society. Published online by Cambridge University Press: 16 May 2001.
Subject Keywords:Klinefelter syndrome; 47,XXY; Sex chromosome abnormalities; Neuropsychological scores
Issue or Number:4
Record Number:CaltechAUTHORS:20210301-123913869
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Official Citation:BOONE, K., SWERDLOFF, R., MILLER, B., GESCHWIND, D., RAZANI, J., LEE, A., . . . PAUL, L. (2001). Neuropsychological profiles of adults with Klinefelter syndrome. Journal of the International Neuropsychological Society, 7(4), 446-456. doi:10.1017/S1355617701744013
Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:108249
Deposited By: Tony Diaz
Deposited On:01 Mar 2021 21:35
Last Modified:01 Mar 2021 21:35

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