Assessment of Intellectual and Visuo-Spatial Abilities in Children and Adults with Williams Syndrome
Carregando...
Citações na Scopus
5
Tipo de produção
article
Data de publicação
2013
Editora
PONTIFICA UNIV JAVERIANA, FAC PSYCH
Indexadores
Título da Revista
ISSN da Revista
Título do Volume
Autores
NUNES, M. M.
AMARAL, V. S.
AMARAL, V. A. S.
OH, H. K.
ALBANO, L. M. J.
Autor de Grupo de pesquisa
Editores
Coordenadores
Organizadores
Citação
UNIVERSITAS PSYCHOLOGICA, v.12, n.2, p.581-589, 2013
Resumo
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.
Palavras-chave
Williams Syndrome, intelligence, assessment, cognition, visual-spatial abilities
Referências
- Atkinson J, 2001, DEV MED CHILD NEUROL, V43, P330, DOI 10.1017/S0012162201000615
- Bellugi U, 1999, TRENDS NEUROSCI, V22, P197, DOI 10.1016/S0166-2236(99)01397-1
- Bellugi U, 2000, J COGNITIVE NEUROSCI, V12, P7, DOI 10.1162/089892900561959
- BELLUGI U, 1992, MINN SYM CHILD PSYCH, V24, P201
- BIHRLE AM, 1989, BRAIN COGNITION, V11, P37, DOI 10.1016/0278-2626(89)90003-1
- Farran EK, 2003, DEV NEUROPSYCHOL, V23, P173, DOI 10.1207/S15326942DN231&2_8
- Fernando K, 2003, NEW ZEAL J PSYCHOL, V32, P33
- Gaser C, 2006, NEUROIMAGE, V33, P46, DOI 10.1016/j.neuroimage.2006.06.018
- Jarrold C, 1998, J CHILD PSYCHOL PSYC, V39, P511
- Jarrold C, 1999, NEUROPSYCHOLOGIA, V37, P637, DOI 10.1016/S0028-3932(98)00128-6
- Jones W., 1995, Society for Neuroscience Abstracts, V21, P1926
- Jordan H, 2002, PSYCHOL SCI, V13, P162, DOI 10.1111/1467-9280.00429
- Klein-Tasman BP, 2003, DEV NEUROPSYCHOL, V23, P269, DOI 10.1207/S15326942DN231&2_12
- La Femina F, 2009, CLIN NEUROPSYCHOL, V23, P691, DOI 10.1080/13854040802572426
- Landau B, 2006, COGNITION, V100, P483, DOI 10.1016/j.cognition.2005.06.005
- Lenhoff HM, 1997, SCI AM, V277, P68
- Lu PH, 2003, CLIN NEUROPSYCHOL, V17, P426, DOI 10.1076/clin.17.3.426.18083
- Marriage J., 1995, GENETIC COUNSELING, V6, P131
- Martens MA, 2008, J CHILD PSYCHOL PSYC, V49, P576, DOI 10.1111/j.1469-7610.2008.01887.x
- Mervis CB, 2000, BRAIN COGNITION, V44, P604, DOI 10.1006/brcg.2000.1232
- Meyer-Lindenberg A, 2006, NAT REV NEUROSCI, V7, P380, DOI 10.1038/nrn1906
- Meyer-Lindenberg A, 2004, NEURON, V43, P623, DOI 10.1016/j.neuron.2004.08.014
- Mimura M, 2010, J NEURODEV DISORD, V2, P93, DOI 10.1007/s11689-009-9041-8
- Montgolfier-Aubron I., 2000, ARCH PEDIAT ADOL MED, V7, P1085
- Oliveira M S., 2004, AVAL PSICOL, V3, P33
- Oliveira M. S., 1999, FIGURAS COMPLEXAS RE
- Rossi N. F., 2007, REV SOC BRAS FONOAUD, V12, P1
- Sampaio Adriana, 2008, Brain Cogn, V66, P290, DOI 10.1016/j.bandc.2007.09.005
- Tassabehji M, 2003, HUM MOL GENET, V12, P229
- Udwin O, 1996, DEV MED CHILD NEUROL, V38, P1020
- UDWIN O, 1991, J CLIN EXP NEUROPSYC, V13, P232, DOI 10.1080/01688639108401040
- Vicari S, 2003, DEV MED CHILD NEUROL, V45, P269, DOI 10.1017/S0012162203000513
- WANG PP, 1994, J CLIN EXP NEUROPSYC, V16, P317, DOI 10.1080/01688639408402641
- WANG PP, 1993, AM J DIS CHILD, V147, P1246
- Wechsler D., 1997, WAIS 3 ESCALA INTELI
- Wechsler D., 2002, WISC 3 ESCALA INTELI
- Withers S, 1996, ARCH DIS CHILD, V75, P89