Literature DB >> 9192263

Intelligence and psychosocial adjustment in velocardiofacial syndrome: a study of 37 children and adolescents with VCFS.

A Swillen1, K Devriendt, E Legius, B Eyskens, M Dumoulin, M Gewillig, J P Fryns.   

Abstract

We report data on a group of 37 VCFS patients with specific reference to their intelligence, behaviour, and social competence. Fifty five percent of the children had a borderline to normal IQ. Mental retardation (defined as IQ < 70 or > -2 SD below the mean) was found in 45%. In the majority, the mental retardation was mild (38%) and only two patients had moderate mental retardation. Severe mental retardation seems to be rare in VCFS. The present study shows also that the incidence of mental retardation is much higher in the familial than the de novo group. Intelligence is not correlated with the presence or absence of a heart defect. Significantly higher verbal IQs than performance IQs (probably related to deficits in visuospatial-perceptual functioning) were found. Problems in social-emotional functioning and attention were also found. Further longitudinal studies are necessary to provide an accurate prognosis and appropriate intervention for VCFS children.

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Year:  1997        PMID: 9192263      PMCID: PMC1050966          DOI: 10.1136/jmg.34.6.453

Source DB:  PubMed          Journal:  J Med Genet        ISSN: 0022-2593            Impact factor:   6.318


  14 in total

1.  Neuropsychological aspects of Turner's syndrome.

Authors:  D P Waber
Journal:  Dev Med Child Neurol       Date:  1979-02       Impact factor: 5.449

2.  Velo-cardio-facial syndrome: language and psychological profiles.

Authors:  K J Golding-Kushner; G Weller; R J Shprintzen
Journal:  J Craniofac Genet Dev Biol       Date:  1985

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Authors:  R J Shprintzen; R B Goldberg; D Young; L Wolford
Journal:  Pediatrics       Date:  1981-02       Impact factor: 7.124

4.  The neuropsychological phenotype in Turner syndrome.

Authors:  B F Pennington; R K Heaton; P Karzmark; M G Pendleton; R Lehman; D W Shucard
Journal:  Cortex       Date:  1985-09       Impact factor: 4.027

Review 5.  Velocardiofacial (Shprintzen) syndrome: an important syndrome for the dysmorphologist to recognise.

Authors:  A H Lipson; D Yuille; M Angel; P G Thompson; J G Vandervoord; E J Beckenham
Journal:  J Med Genet       Date:  1991-09       Impact factor: 6.318

6.  Variable phenotypes in velocardiofacial syndrome with chromosomal deletion.

Authors:  B Motzkin; R Marion; R Goldberg; R Shprintzen; P Saenger
Journal:  J Pediatr       Date:  1993-09       Impact factor: 4.406

Review 7.  Velo-cardio-facial syndrome: a review of 120 patients.

Authors:  R Goldberg; B Motzkin; R Marion; P J Scambler; R J Shprintzen
Journal:  Am J Med Genet       Date:  1993-02-01

8.  Intelligence, behaviour and psychosocial development in Turner syndrome. A cross-sectional study of 50 pre-adolescent and adolescent girls (4-20 years).

Authors:  A Swillen; J P Fryns; A Kleczkowska; G Massa; M Vanderschueren-Lodeweyckx; H Van den Berghe
Journal:  Genet Couns       Date:  1993

9.  Velo-cardio-facial syndrome associated with chromosome 22 deletions encompassing the DiGeorge locus.

Authors:  P J Scambler; D Kelly; E Lindsay; R Williamson; R Goldberg; R Shprintzen; D I Wilson; J A Goodship; I E Cross; J Burn
Journal:  Lancet       Date:  1992-05-09       Impact factor: 79.321

10.  A new syndrome involving cleft palate, cardiac anomalies, typical facies, and learning disabilities: velo-cardio-facial syndrome.

Authors:  R J Shprintzen; R B Goldberg; M L Lewin; E J Sidoti; M D Berkman; R V Argamaso; D Young
Journal:  Cleft Palate J       Date:  1978-01
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  102 in total

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3.  22q11.2 deletion syndrome: are motor deficits more than expected for IQ level?

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5.  Working Memory Impairments in Chromosome 22q11.2 Deletion Syndrome: The Roles of Anxiety and Stress Physiology.

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7.  Biological effects of COMT haplotypes and psychosis risk in 22q11.2 deletion syndrome.

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Journal:  Biol Psychiatry       Date:  2013-08-28       Impact factor: 13.382

8.  White matter abnormalities in 22q11.2 deletion syndrome: preliminary associations with the Nogo-66 receptor gene and symptoms of psychosis.

Authors:  Matthew D Perlstein; Moeed R Chohan; Ioana L Coman; Kevin M Antshel; Wanda P Fremont; Matthew H Gnirke; Zora Kikinis; Frank A Middleton; Petya D Radoeva; Martha E Shenton; Wendy R Kates
Journal:  Schizophr Res       Date:  2013-12-08       Impact factor: 4.939

Review 9.  Converging levels of analysis on a genomic hotspot for psychosis: insights from 22q11.2 deletion syndrome.

Authors:  Matthew J Schreiner; Maria T Lazaro; Maria Jalbrzikowski; Carrie E Bearden
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Review 10.  Schizophrenia and genetics: new insights.

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Journal:  Curr Psychiatry Rep       Date:  2002-08       Impact factor: 5.285

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