PMID- 25684214
OWN - NLM
STAT- MEDLINE
DCOM- 20151223
LR  - 20150326
IS  - 1601-183X (Electronic)
IS  - 1601-183X (Linking)
VI  - 14
IP  - 2
DP  - 2015 Feb
TI  - Executive dysfunction and the relation with behavioral problems in children with 
      47,XXY and 47,XXX.
PG  - 200-8
LID - 10.1111/gbb.12203 [doi]
AB  - Neuroimaging studies have shown that having an extra X chromosome is associated 
      with abnormal structure and function of brain areas in the frontal lobe, which is 
      crucially involved in executive functioning. However, there is little of 
      knowledge of the type and severity of executive dysfunction, and the impact on 
      emotional and behavioral problems. The present study aims to provide in this. In 
      total, 40 children (23 boys with 47,XXY and 17 girls with 47,XXX) with an extra X 
      chromosome and 100 non-clinical controls (47 boys and 53 girls) participated in 
      the study. The participants were 9-18 years old. Processing speed and executive 
      functioning were assessed using the Amsterdam Neuropsychological Testbattery 
      (ANT) and the Dysexecutive Questionnaire (DEX). Problems in emotional and 
      behavioral functioning were assessed with the Childhood Behavior Checklist 
      (CBCL). Children with an extra X chromosome showed deficits in inhibition, mental 
      flexibility, sustained attention and visual working memory. Parental report 
      showed high levels of everyday manifestations of executive dysfunction. More 
      severe inhibition difficulties were associated with higher levels of thought 
      problems, aggression and rule breaking behavior. Boys and girls with an extra X 
      chromosome could not be differentiated based on severity of executive 
      dysfunction, however, girls had lower information processing speed than boys. 
      These findings suggest that executive dysfunction may be part of the phenotype of 
      children with an extra X chromosome, impacting the ability to function adequately 
      in everyday life. Furthermore, children with impairments in inhibition may have 
      more problems in regulating their thinking, emotions and behavior.
CI  - (c) 2015 John Wiley & Sons Ltd and International Behavioural and Neural Genetics 
      Society.
FAU - van Rijn, S
AU  - van Rijn S
AD  - Clinical Child and Adolescent Studies, Leiden University, Leiden, The 
      Netherlands; Leiden Institute for Brain and Cognition, Leiden, The Netherlands.
FAU - Swaab, H
AU  - Swaab H
LA  - eng
PT  - Journal Article
PL  - England
TA  - Genes Brain Behav
JT  - Genes, brain, and behavior
JID - 101129617
RN  - Triple X syndrome
SB  - IM
MH  - *Aggression
MH  - Brain/physiopathology
MH  - Child
MH  - Chromosomes, Human, X/genetics
MH  - Cognition Disorders/*genetics
MH  - *Executive Function/physiology
MH  - Female
MH  - Humans
MH  - Klinefelter Syndrome/*genetics
MH  - Male
MH  - Neuropsychological Tests
MH  - *Problem Behavior/psychology
MH  - Sex Chromosome Aberrations
MH  - Sex Chromosome Disorders of Sex Development/*genetics
MH  - Trisomy/*genetics
OTO - NOTNLM
OT  - Behavioral problems
OT  - Klinefelter syndrome
OT  - Trisomy X
OT  - cognition
OT  - executive functions
OT  - sex chromosome disorders
EDAT- 2015/02/17 06:00
MHDA- 2015/12/24 06:00
CRDT- 2015/02/17 06:00
PHST- 2014/09/16 00:00 [received]
PHST- 2015/01/16 00:00 [revised]
PHST- 2015/01/20 00:00 [accepted]
PHST- 2015/02/17 06:00 [entrez]
PHST- 2015/02/17 06:00 [pubmed]
PHST- 2015/12/24 06:00 [medline]
AID - 10.1111/gbb.12203 [doi]
PST - ppublish
SO  - Genes Brain Behav. 2015 Feb;14(2):200-8. doi: 10.1111/gbb.12203.