El Síndrome Prader-Willi: Características cognitivas e implicaciones educativas

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Martinez Pérez, Lourdes
Muñoz Ruata, Jorge
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Se estudian las características cognitivas del Síndrome Prader-Willi (SPW). Se han evaluado 10 sujetos con el WISC-R y el ITPA. Se han dividido los sujetos en tres grupos mediante la técnica K-means, realizando posteriormente un análisis de Kruskal-Wallis para la caracterización de los tres grupos. Las características de estos grupos se acomodan a las descritas en la literatura para los tres grupos genéticos principales del SPW. El primer grupo presenta características similares a las descritas en los disómicos: buenas habilidades verbales, tanto expresivas como comprensivas, y rasgos dispráxicos. El segundo grupo se acomodan a las características de las delecciones cortas, con un perfil homogéneo de deficiencia mental ligera y predominio de las habilidades práxicas; sus habilidades lingüísticas comprensivas son superiores a las expresivas. El tercer grupo se acomoda a las características de la delección larga, con un nivel homogéneo dentro de deficiencia mental media y deterioro de las habilidades tanto expresivas como comprensivas. Se comentan algunos principios y estrategias para la intervención educativa. [ABSTRACT]This study analyses the cognitive characteristics of Prader-Willi Syndrome (SPW). Ten SPW patients with WISC-R and ITPA were evaluated. Subjects were divided into three groups using the K-means method and a Kruskal-Wallis analysis was later carried out, in order to characterize the three groups. The characteristics of these three groups are in accordance with those described in the literature for the three main genetic groups of SPW. The first group shows similar characteristics for those described in people with disomy: good verbal abilities, both expressive and comprehensive, as well as dispraxic features. The second group coincides with the characteristics of short deletions, with a homogeneous profile of slight mental retardation and a predominance of praxic abilities; their comprehensive linguistic abilities are higher than their expressive abilities. The third group matches the characteristics of long deletion, with a homogeneous level of average mental retardation and deterioration of expressive and comprehensive abilities. Some principles and strategies are discussed for the purpose of educational intervention.
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