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

Autor/inn/en	Ross, Judith L.; Zeger, Martha P. D.; Kushner, Harvey; Zinn, Andrew R.; Roeltgen, David P.
Titel	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
Quelle	In: Developmental Disabilities Research Reviews, 15 (2009) 4, S. 309-317 PDF als Volltext Verfügbarkeit
Sprache	englisch
Dokumenttyp	gedruckt; online; Zeitschriftenaufsatz
ISSN	1940-5510
Schlagwörter	Forschungsbericht; Genetic Disorders; Males; Sex; Genetics; Children; Adolescents; Cognitive Ability; Verbal Ability; Memory; Attention; Spatial Ability; Psychomotor Skills; Academic Achievement; Language Impairments; Control Groups; Body Height; Handedness; Pennsylvania; Texas; Continuous Performance Test; Beery Developmental Test of Visual Motor Integration; Bruininks Oseretsky Test of Motor Proficiency; California Verbal Learning Test for Children; Wide Range Achievement Test + Suchen Sie Ihr Suchwort? Male; Männliches Geschlecht; Geschlecht; Geschlechtsverkehr; Humangenetik; Child; Kind; Kinder; Adolescent; Adolescence; Adoleszenz; Jugend; Jugendalter; Jugendlicher; Denkfähigkeit; Mündliche Leistung; Gedächtnis; Aufmerksamkeit; Räumliches Vorstellungsvermögen; Psychomotorische Aktivität; Schulleistung; Speech disorder; Speech disorders; Speech disabilities; Speech disability; Speech handicap; Speech handicaps; Speech impairment; Speech impairments; Language handicaps; Sprachbehinderung; Körpergröße; Linkshändigkeit
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. Methods: Neuropsychological evaluation of general cognitive ability, language, memory, attention, visual-spatial abilities, visual-motor skills, and motor function. 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. 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. (Contains 5 tables.) (As Provided).
Anmerkungen	Wiley-Blackwell. 111 River Street, Hoboken, NJ 07030-5774. Tel: 800-825-7550; Tel: 201-748-6645; Fax: 201-748-6021; e-mail: subinfo@wiley.com; Web site: http://www3.interscience.wiley.com/browse/?type=JOURNAL
Begutachtung	Peer reviewed
Erfasst von	ERIC (Education Resources Information Center), Washington, DC
Update	2017/4/10