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An extra X or Y chromosome: contrasting the cognitive and motor phenotypes in childhood in boys with 47,XYY syndrome or 47,XXY Klinefelter syndrome.
- Source :
-
Developmental disabilities research reviews [Dev Disabil Res Rev] 2009; Vol. 15 (4), pp. 309-17. - Publication Year :
- 2009
-
Abstract
- Objective: The goal of this study was to contrast the cognitive phenotypes in boys with 47,XYY (XYY) karyotype and boys with 47,XXY karyotype [Klinefelter syndrome, (KS)], who share an extra copy of the X-Y pseudoautosomal region but differ in their dosage of strictly sex-linked genes.<br />Methods: Neuropsychological evaluation of general cognitive ability, language, memory, attention, visual-spatial abilities, visual-motor skills, and motor function.<br />Results: Study cohort: 21 boys with 47,XYY and 93 boys with 47,XXY (KS), age 4-17 years, and 36 age-matched control boys. Both the XYY and KS groups performed less well, on average, than the controls on tests of general cognitive ability, achievement, language, verbal memory, some aspects of attention, and executive function, and motor function. The boys with XYY on average had more severe and pervasive language impairment, at both simple and complex levels, and the boys with KS on average had greater motor impairment in gross motor function and coordination, especially in running speed and agility.<br />Conclusions: The results from these large XYY and KS cohorts have important neurocognitive and educational implications. From the neurocognitive standpoint, the presenting findings afford an opportunity to gain insights into brain development in boys with XYY and those with KS. From the educational standpoint, it is critical that boys with XYY or KS receive appropriate educational interventions that target their specific learning challenges. These findings also provide important information for counseling clinicians and families about these disorders.
- Subjects :
- Achievement
Adolescent
Child
Child, Preschool
Cognition Disorders diagnosis
Humans
Karyotyping
Klinefelter Syndrome blood
Male
Motor Skills Disorders diagnosis
Neuropsychological Tests
Testosterone blood
Testosterone genetics
Chromosomes, Human, X genetics
Chromosomes, Human, Y genetics
Cognition Disorders epidemiology
Klinefelter Syndrome epidemiology
Klinefelter Syndrome genetics
Motor Skills Disorders epidemiology
Phenotype
Subjects
Details
- Language :
- English
- ISSN :
- 1940-5529
- Volume :
- 15
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Developmental disabilities research reviews
- Publication Type :
- Academic Journal
- Accession number :
- 20014371
- Full Text :
- https://doi.org/10.1002/ddrr.85