Publications by authors named "L Plissart"

At what age do children with Williams syndrome (WS) achieve major developmental milestones? Is their early development harmonious or are some of the typical discrepancies described in older children already noticeable? To address these questions we analysed information gathered over a five year period on 14 children with WS. Each child was evaluated at least twice between the ages of 5 and 48 months, using the Bayley Scales of Infant Development and during more informal observation sessions. Parents and professionals were also interviewed.

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In this study we evaluated the temperament characteristics of a group of 13 subjects with Williams-Beuren syndrome (WBS) and compared the results to the findings in a control group of 13 individuals with the same degree of mental retardation of different etiology. On the different subscales of the Dutch adaptation of the Parent Temperament Questionnaire no statistically significant differences between the WBS and the control group were noted. An easier temperament was noted in the control group, and we also found greater intensity, less persistence and lower treshold in WBS subjects.

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In order to evaluate the medical, psychological and behavioral aspects of Williams-Beuren syndrome in adulthood, data were collected on 11 patients aged 17 to 66 years. The medical data did not confirm previous reports of significant morbidity. All adults were found to have a moderate or severe degree of mental handicap.

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The Prader-Willi syndrome is characterized by four cardinal symptoms i.e. hypotonia, hypogonadism, mental retardation and extreme obesity.

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