Brain and Language

1982

DOI: 10.1016/0093-934x(82)90005-0

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Verbal deficits in children with 47, XXY and 47, XXX karyotypes: A descriptive and experimental study

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Discussion1

Clinical Findings and Phenotype In A Toddler With 48xxyy Sy1

Development Behavior and Psychology1

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Cited by 69 publications

(36 citation statements)

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“…25,116 The cell cycle slows down in cases with an extra X chromosome 117 and in other cells with an extra chromosome in humans 117,118 as well as in yeast 119 and in seedlings. 120 DNA replication is apparently the rate-limiting step in the cell cycle.…”

Section: Discussionmentioning

confidence: 99%

Triple X syndrome: a review of the literature

Schrander-Stumpel

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2009

Eur J Hum Genet

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The developmental and clinical aspects in the literature on triple X syndrome are reviewed. Prenatal diagnosis depends on karyotyping. The incidence is 1 of 1000 females. At birth, 47,XXX girls have a lower mean birth weight and a smaller head circumference. Triple X diagnosis was not suspected at birth. The maternal age seems to be increased. Toddlers with triple X syndrome show delayed language development. The youngest girls show accelerated growth until puberty. EEG abnormalities seem to be rather common. Many girls show motor-coordination problems and auditory-processing disorders are not rare. Scoliosis is probably more common in adolescent cases. The IQ levels are 20 points below that of controls, and verbal IQ is lowest. The girls struggle with low self-esteem and they need psychological, behavioural and educational support. They perform best in stable families. After leaving school they seem to feel better. In adults, premature ovarian failure seems to be more prevalent than in controls. MRIs of the brain seem to show decreased brain volumes. The 47,XXX women most often find jobs that reflect their performance abilities. Psychotic illness seems to be more prevalent in triple X adult women than in controls. Psychotic disorders respond well to psychotropic drugs. Triple X adults suffer more frequently from cyclothymic and labile personality traits.Research on triple X syndrome may yield more insight into brain and behaviour relations, developmental psychopathology, auditory-processing disorders, EEG disorders, personality and psychotic disorders, etc.

“…25,116 The cell cycle slows down in cases with an extra X chromosome 117 and in other cells with an extra chromosome in humans 117,118 as well as in yeast 119 and in seedlings. 120 DNA replication is apparently the rate-limiting step in the cell cycle.…”

Section: Discussionmentioning

confidence: 99%

Triple X syndrome: a review of the literature

Schrander-Stumpel

²

,

³

2009

Eur J Hum Genet

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The developmental and clinical aspects in the literature on triple X syndrome are reviewed. Prenatal diagnosis depends on karyotyping. The incidence is 1 of 1000 females. At birth, 47,XXX girls have a lower mean birth weight and a smaller head circumference. Triple X diagnosis was not suspected at birth. The maternal age seems to be increased. Toddlers with triple X syndrome show delayed language development. The youngest girls show accelerated growth until puberty. EEG abnormalities seem to be rather common. Many girls show motor-coordination problems and auditory-processing disorders are not rare. Scoliosis is probably more common in adolescent cases. The IQ levels are 20 points below that of controls, and verbal IQ is lowest. The girls struggle with low self-esteem and they need psychological, behavioural and educational support. They perform best in stable families. After leaving school they seem to feel better. In adults, premature ovarian failure seems to be more prevalent than in controls. MRIs of the brain seem to show decreased brain volumes. The 47,XXX women most often find jobs that reflect their performance abilities. Psychotic illness seems to be more prevalent in triple X adult women than in controls. Psychotic disorders respond well to psychotropic drugs. Triple X adults suffer more frequently from cyclothymic and labile personality traits.Research on triple X syndrome may yield more insight into brain and behaviour relations, developmental psychopathology, auditory-processing disorders, EEG disorders, personality and psychotic disorders, etc.

“…Additional support for the influence of testosterone in the axonal loss of isthmus of the corpus callosum comes from animal studies (e.g., Fitch, Berrebi, Cowell, Schrott, & Denenberg, 1990), as well as from studies on individuals with Klinefelter's syndrome, a condition that has been related to low levels of testosterone and increased prevalence of left-handedness (e.g., Netley & Rovet, 1982).…”

mentioning

confidence: 97%

Digit ratio (2D:4D) in individuals with intellectual disability: Investigating the role of testosterone in the establishment of cerebral lateralisation

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et al. 2008

Laterality: Asymmetries of Body, Brain and Cognition

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It has been proposed that the ratio of the second to fourth digits (2D:4D) may be a proxy of prenatal androgen exposure, such that low 2D:4D ratio is associated with high prenatal androgen exposure. The aim of the present study was to measure the 2D:4D ratio in 100 right- and non-right-handed individuals with intellectual disability of unknown idiopathic origin and compare them to a control group of 85 typically developing individuals. We also sought to determine whether sexually dimorphic traits, such as 2D:4D ratio, tend to be more pronounced on the right hand of these groups than on the left. Our results indicated that males had lower 2D:4D ratios than females in both groups, and individuals with intellectual disability had higher ratios only in their right hand compared to typically developing individuals. Further, right-handed individuals had lower ratios in both hands compared to non-right-handed individuals. Our results are discussed in relation to the "Geschwind-Behan-Galaburda theory of cerebral lateralisation" and Witelson's callosal hypothesis that differential levels of prenatal testosterone exposure will cause atypical cerebral laterality in individuals with intellectual disability, and the suggestion that this atypicality will become evident in the 2D:4D ratio (Manning, Scutt, Wilson, & Lewis-Jones, 1998).

“…Many of the phenotypic traits are also observed in patients with 48,XXXX and 49,XXXXY. Netley and Rovet [1982] reported that verbal IQ was particularly depressed in those with a distinctive dermatoglypic anomaly and atypical patterns of cerebral organization.…”

Section: Clinical Findings and Phenotype In A Toddler With 48xxyy Symentioning

confidence: 98%

Clinical findings and phenotype in a toddler with 48,XXYY syndrome

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2003

American J of Med Genetics Pt A

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