NDLI: The course of cognitive-behavioral development in children with the FMR1 mutation, Williams–Beuren syndrome, and neurofibromatosis type 1: The effect of $gender^{†}$

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The course of cognitive-behavioral development in children with the FMR1 mutation, Williams–Beuren syndrome, and neurofibromatosis type 1: The effect of $gender^{†}$

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The course of cognitive-behavioral development in children with the FMR1 mutation, Williams–Beuren syndrome, and neurofibromatosis type 1: The effect of $gender^{†}$

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Fisch, Gene S. Carpenter, Nancy Howard-Peebles, Patricia N. Holden, Jeanette J. A. Tarleton, Jack Simensen, Richard
Description	Country affiliation: United States Author Affiliation: Fisch GS ( NYU College of Dentistry New York, New York 10003, USA. gene.fisch@nyu.edu)
Abstract	The course of cognitive-behavioral development in children with intellectual disabilities produced by genetic disorders has only recently begun to be examined systematically. Unfortunately, these studies are few in number. Previously, we examined cognitive-behavioral development in children with the fragile X (FMR1) mutation and found longitudinal decreases in both IQ and adaptive behavior (DQ) scores in most males and females with the full mutation. In this study, we examine longitudinal changes in IQ and DQ in children with neurofibromatosis type 1 (NF1) and Williams–Beuren Syndrome (WBS) by examining differences in composite IQ and DQ scores between the first test (T1) and retest (T2), and compare their developmental trajectory to children with the FMR1 mutation. Sixty-five children with the FMR1 mutation, or NF1, or WBS, ages 4–16 years, were retested two years after initial testing with the Stanford-Binet 4th Edition (SBFE) and the Vineland Adaptive Behavior Scale (VABS). In addition to significant longitudinal declines in IQ and DQ noted previously in children with the FMR1 mutation, we found significant decreases in IQ in males compared to females in the remainder of our sample. We also observed statistically significant decreases in DQ scores among children the FMR1 mutation, as noted previously, but not among children with NF1 or WBS. Moreover, significant declines were found only among males with the FMR1 mutation. Unlike declines in IQ scores, decreases in DQ were not significantly different between males and females. © 2010 Wiley-Liss, Inc.
File Format	HTM / HTML
ISSN	15524825
Issue Number	6
Volume Number	152A
e-ISSN	15524833
Journal	American Journal of Medical Genetics Part A
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2010-06-01
Publisher Place	United States
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Human Discipline Genetics Child Development Cognition Fragile X Syndrome Psychology Neurofibromatosis 1 Williams Syndrome Adolescent Child Child, Preschool Female Fragile X Mental Retardation Protein Genetics Humans Male Mutation Sex Factors Comparative Study Journal Article
Content Type	Text
Resource Type	Article
Subject	Genetics Genetics (clinical)

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