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Neurocognitive outcomes of individuals with a sex chromosome trisomy: XXX, XYY, or XXY: a systematic review - LEGGETT - 2010 - Developmental Medicine & Child Neurology - Wiley Online Library

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Neurocognitive outcomes of individuals with a sex chromosome trisomy: XXX, XYY, or XXY: a systematic review

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  • Re-use of this article is permitted in accordance with the Creative Commons Deed, Attribution 2.5, which does not permit commercial exploitation.

Professor Dorothy Bishop at the Department of Experimental Psychology, University of Oxford, South Parks Road, Oxford OX1 3UD, UK. E-mail: [email protected], [email protected]

Abstract

Aim  To review systematically the neurodevelopmental characteristics of individuals with sex chromosome trisomies (SCTs).

Method  A bibliographic search identified English-language articles on SCTs. The focus was on studies unbiased by clinical referral, with power of at least 0.69 to detect an effect size of 1.0.

Results  We identified 35 articles on five neonatally identified samples that had adequate power for our review. An additional 11 studies were included where cases had been identified for reasons other than neurodevelopmental concerns. Individuals with an additional X chromosome had mean IQs that were within broadly normal limits but lower than the respective comparison groups, with verbal IQ most affected. Cognitive outcomes were poorest for females with XXX. Males with XYY had normal-range IQs, but all three SCT groups (XXX, XXY, and XYY) had marked difficulties in speech and language, motor skills, and educational achievement. Nevertheless, most adults with SCTs lived independently. Less evidence was available for brain structure and for attention, social, and psychiatric outcomes. Within each group there was much variation.

Interpretation  Individuals with SCTs are at risk of cognitive and behavioural difficulties. However, the evidence base is slender, and further research is needed to ascertain the nature, severity, and causes of these difficulties in unselected samples.

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DOI

10.1111/j.1469-8749.2009.03545.x

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© The Authors. Journal compilation © Mac Keith Press 2010

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Publication History

  • Issue online: 15 January 2010
  • Version of record online: 5 January 2010
  • PUBLICATION DATA Accepted for publication 8th September 2009. Published online 5th January 2010.

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