title: Exceptional lexical skills but executive language deficits in school starters and young adults with Turners syndrome: implications for X chromosome effects on brain function.
abstract: TS school starters had enhanced receptive and expressive language on standardised assessment (CELF-P) and enhanced rhyme judgements, spoonerisms, and lexical decision, indicating enhanced phonological skills and word representations. There was marginal but consistent advantage across lexico-semantic tasks. On executive tasks, speeded naming of numbers was impaired but not pictures. Young TS adults had enhanced naming and receptive vocabulary, indicating enhanced semantic skills. There were consistent deficits in executive language: phonemic oral fluency, rhyme fluency, speeded naming of pictures, numbers and colours; sentence completion requiring supression of prepotent responses. Haploinsufficiency of X-chromosome drives mechanisms that affect the anatomical and neurochemical development of the brain, resulting in enhanced temporal lobe aspects of language. These strengths co-exist with impaired development of frontal lobe executive language systems. This means not only that these elements of language can decouple in development but that their very independence is driven by mechanisms linked to the X-chromosome.
authors.affiliation: Developmental Neuropsychology Unit, Department of Psychology, University of Essex, Wivenhoe Park, Colchester CO7 9JU, UK. tempc@essex.ac.uk
authors.forename: Christine M
authors.initials: CM
authors.lastname: Temple
authors.forename: Elizabeth E
authors.initials: EE
authors.lastname: Shephard
mesh_terms: D000367:Age Factors; D002675:Child, Preschool; D041321:Chromosomes, Human, X; D003430:Cross-Sectional Studies; D004192:Discrimination, Psychological / Q000502:physiology; D056344:Executive Function / Q000502:physiology*; D005260:Female; D005625:Frontal Lobe / Q000502:physiology*; D057895:Haploinsufficiency / Q000502:physiology; D006801:Humans; D007804:Language Development; D007805:Language Development Disorders / Q000235:genetics* / Q000503:physiopathology; D012660:Semantics; D014424:Turner Syndrome / Q000235:genetics* / Q000503:physiopathology; D014706:Verbal Learning / Q000502:physiology; D014825:Vocabulary; D055815:Young Adult