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  1. Article: Adolescent adaptive behavior profiles in Williams-Beuren syndrome, Down syndrome, and autism spectrum disorder.

    Del Cole, Carolina Grego / Caetano, Sheila Cavalcante / Ribeiro, Wagner / Kümmer, Arthur Melo E E / Jackowski, Andrea Parolin

    Child and adolescent psychiatry and mental health

    2017  Volume 11, Page(s) 40

    Abstract: Background: Adaptive behavior can be impaired in different neurodevelopmental disorders and may be influenced by confounding factors, such as intelligence quotient (IQ) and socioeconomic classification. Our main objective was to verify whether adaptive ... ...

    Abstract Background: Adaptive behavior can be impaired in different neurodevelopmental disorders and may be influenced by confounding factors, such as intelligence quotient (IQ) and socioeconomic classification. Our main objective was to verify whether adaptive behavior profiles differ in three conditions-Williams Beuren syndrome (WBS), Down syndrome (DS), and autism spectrum disorder (ASD), as compared with healthy controls (HC) and with each other. Although the literature points towards each disorder having a characteristic profile, no study has compared profiles to establish the specificity of each one. A secondary objective was to explore potential interactions between the conditions and socioeconomic status, and whether this had any effect on adaptive behavior profiles.
    Methods: One hundred and five adolescents were included in the study. All adolescents underwent the following evaluations: the Vineland Adaptive Behavior Scale (VABS), the Wechsler Intelligence Scale for Children (WISC), and the Brazilian Economic Classification Criteria.
    Results: Our results demonstrated that the WBS group performed better than the DS group in the communication domain, β = -15.08, t(3.45), p = .005, and better than the ASD group in the socialization domain, β = 8.92, t(-2.08), p = .013. The DS group also performed better than the ASD group in socialization, β = 16.98, t(-2.32), p = .024. IQ was an important confounding factor, and socioeconomic status had an important effect on the adaptive behavior of all groups.
    Conclusions: There is a heterogeneity regarding adaptive behavior profiles in WBS, DS, and ASD. These data are important to better design specific strategies related to the health and social care of each particular group.
    Language English
    Publishing date 2017-07-24
    Publishing country England
    Document type Journal Article
    ZDB-ID 2379599-2
    ISSN 1753-2000
    ISSN 1753-2000
    DOI 10.1186/s13034-017-0177-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Brain abnormalities in Williams syndrome: a review of structural and functional magnetic resonance imaging findings.

    Jackowski, Andrea Parolin / Rando, Kenneth / Maria de Araújo, Célia / Del Cole, Carolina Grego / Silva, Ivaldo / Tavares de Lacerda, Acioly Luiz

    European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society

    2009  Volume 13, Issue 4, Page(s) 305–316

    Abstract: Williams syndrome (WS) is rare genetic form of mental retardation caused by a microdeletion on chromosome 7q11.23 that causes cognitive impairment and a variety of physical abnormalities. MRI studies of WS have demonstrated a series of brain ... ...

    Abstract Williams syndrome (WS) is rare genetic form of mental retardation caused by a microdeletion on chromosome 7q11.23 that causes cognitive impairment and a variety of physical abnormalities. MRI studies of WS have demonstrated a series of brain abnormalities, including decreased brain size, with a relatively greater decrease in the volume of the cerebral white matter volume as compared to the cerebral gray matter. Moreover there is evidence that the posterior cerebrum is more affected in that persons with WS have a greater ratio of frontal to posterior regional volume. These findings are further supported by automated analyses that have shown reduced gray matter density in the superior parietal lobe areas. Functional MRI studies have demonstrated hypofunction immediately adjacent to, and anterior to, the intraparietal sulcus, a region in which structural brain differences had been identified. These anatomical and functional differences are consistent with the neuropsychological profile of WS - in particular, with evidence of dorsal stream visual processing deficits. To date, however, studies have always been performed in comparison to intellectually average controls. It is not clear, therefore, if findings are specific to the WS population or whether they represent a morphological disturbance characteristic of mental retardation, irrespective of genetic etiology. In this article, we reviewed recent advances underlying the structural and functional neural substrate of WS in Medical Literature Analysis and Retrieval System Online (MEDLINE; 1997-2007).
    MeSH term(s) Brain/abnormalities ; Brain/blood supply ; Brain/pathology ; Humans ; Image Processing, Computer-Assisted/methods ; Magnetic Resonance Imaging/methods ; Oxygen/blood ; Williams Syndrome/diagnosis ; Williams Syndrome/physiopathology
    Chemical Substances Oxygen (S88TT14065)
    Language English
    Publishing date 2009-07
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1397146-3
    ISSN 1532-2130 ; 1090-3798
    ISSN (online) 1532-2130
    ISSN 1090-3798
    DOI 10.1016/j.ejpn.2008.07.002
    Database MEDical Literature Analysis and Retrieval System OnLINE

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