Résumé
This review aims to present an overview of current research findings on the possible relationship between phonologicalawareness and visual-spatial skills among individuals with dyslexia. Narrative review of the relevant articles wereobtained through computerized searches of databases such as PubMed, PubMed Central (PMC) and Google Scholarwhich included articles from SAGE, Taylor & Francis and Massachusetts Institute of Technology (MIT) Press from theyear 2000 to 2014. The key words were explored, both exclusively and in combination with each other, so as to provide abetter understanding of the relationship between them among individuals with dyslexia. Although it is evident that thereis a phonological deficit in individuals with dyslexia, however, it is inconclusive with regards to the visual-spatial deficitand strength. There is a consensus on the nature of phonological awareness skill deficits but not on the visual spatialabilities in dyslexia. In fact, the relationship between phonological awareness and visual spatial abilities in dyslexia isdependent on the area of visual ability measured.
Résumé
The Williams-Beuren syndrome (SWB), also known as Williams syndrome, is a contiguous gene deletion of the region 7q.11.23. The main clinical characteristics are typical faces, supravalvular aortic stenosis, failure to thrive, short stature, transient neonatal hypercalcemia, delayed language, friendly personality, hyperacusis and intellectual disability. The diagnosis of SWB is confirmed by the detection of micro deletion by different techniques of molecular cytogenetics, FISH, MLPA or polymorphic markers. This study assessed the verbal intelligence quotient (IQ) and performance and visuo-spatial skills in children and adults with WBS. The composed group was of 31 WBS patients (19 M and 12 F), whose ages ranged from 9 to 26 years (M 14.45 y). All patients had the diagnosis confirmed molecularly. The tests used were the WISC-III, WAIS-III and Rey-Osterrieth Complex Figure Test. The results indicated a total IQ ranged from 51 to 86 (M 63): 22 with mild intellectual disability, 4 with moderate intellectual disability, 4 borderlines and 1 below the normal media. All patients had marked visual-spatial deficits. The results suggest nonverbal reasoning, visuo-spatial perception, spatial representation, working memory, motor planning and executive functions are very affected in this group.
El síndrome de Williams-Beuren (SWB), también conocido como síndrome de Williams, es un síndrome de deleción de genes contiguos de la región 7q.11.23. Se caracteriza por dimorfismo facial típico asociado a anomalías cardiovasculares, personalidad amigable, hiperacusia y deficiencia intelectual. El diagnóstico del SWB es confirmado por la detección de microdeleción a partir de las diferentes técnicas de citogenética molecular: FISH, marcadores polimórficos o MLPA. Este estudio evaluó el cociente intelectual verbal y manipulativo, así como las habilidades visuoespaciales en niños y adultos con SWB. El grupo estuvo formado por 31 pacientes con SWB (19 de sexo masculino y 12 de sexo femenino), cuyas edades variaron entre 9 y 26 años (media 14.45 años). Todos los pacientes tenían el diagnóstico confirmado molecularmente. Los test utilizados fueron las escalas WISC-III, WAIS-III y el Test Figuras Complejas Rey-Osterrieth. Los resultados indicaron un cociente intelectual que osciló de 51 a 86 (media 63), distribuido así: 22 con deficiencia intelectual leve, 4 con deficiencia intelectual moderada, 4 limítrofes, 1 en la media inferior. Todos los pacientes presentaron déficit visuoespacial. Los resultados sugieren que el razonamiento no verbal, la percepción visuoespacial, la representación espacial, la memoria de trabajo, la planificación motora y las funciones ejecutivas están muy comprometidos en el grupo estudiado.