Adaptive behavior in the fragile X syndrome: Profile and development

Wiegers, A. M.; Curfs, L.M.G.; Vermeer, E. L. M. H.

doi:10.1002/ajmg.1320470215

Cited by 20 publications

(12 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Eight fra(X) males had SQ scores greater than 70, whereas only one MR male and none of the AD males had SQ scores greater than 70. young fra(X) males decline as a function of age [Lachiewicz et al, 1987;Hagerman et al, 1989; Curfs et al, 1989a;Fisch et al, 19911. Recently, Dykens et al [1993] reported declines in VABS retest scores in 5 of 7 fra(X) males aged 10 to 17 years, although Wiegers et al (1993) show no agerelated decline in adaptive behavior as measured by the Social and Adaptive Scale for Mentally Handicapped (SRZ) [Kraijer and Kema, 19811. Therefore, we examined both IQ and SQ scores as a function of age.…”

Section: Resultsmentioning

confidence: 99%

Is fragile X syndrome a pervasive developmental disability? Cognitive ability and adaptive behavior in males with the full mutation

Fisch

Holden²,

Simensen

et al. 1994

Am. J. Med. Genet.

View full text Add to dashboard Cite

In addition to mental retardation (MR), fragile X [fra(X)] syndrome has been associated with various psychopathologies, although it appears that the link is secondary to MR. It has been proposed that individuals with the full mutation be classified as a subcategory of pervasive developmental disorders (PDD). If fra(X) males are to be categorized as PDD, how do they compare with other types of developmental disabilities? We examined 27 fra(X) males aged 3-14 years, from 4 sites in North America. Measures of cognitive abilities were obtained from the Stanford-Binet Fourth Edition (SBFE), while levels of adaptive behavior were evaluated using the Vineland Adaptive Behavior Scales (VABS). Control subjects were sex-, age-, and IQ matched children and adolescents ascertained from the Developmental Evaluation Clinic (DEC) at Kings County Hospital. At the DEC, control subjects were diagnosed as either MR (n = 43) or autistic disorder (AD; n = 22). To compare subjects' adaptive behavior (SQ) with their cognitive abilities (IQ), a ratio of [(SQ/IQ) x 100] was computed. Results graphed as cumulative distribution functions (cdf) revealed that the cdf for AD males, who by definition are socially impaired, was positioned to the left of the cdf for MR controls, as expected. Mean ratio for AD males (70) was lower than for MR males (84). On the other hand, the cdf for fra(X) males was positioned far to the right of either AD or MR controls (mean ratio = 125). Statistical tests showed that SQ of fra(X) males was significantly higher than controls.(ABSTRACT TRUNCATED AT 250 WORDS)

show abstract

Section: Resultsmentioning

confidence: 99%

Is fragile X syndrome a pervasive developmental disability? Cognitive ability and adaptive behavior in males with the full mutation

Fisch

Holden²,

Simensen

et al. 1994

Am. J. Med. Genet.

View full text Add to dashboard Cite

show abstract

“…Results suggest that people with fragile X syndrome have strengths in self-help and daily living skills, with weaknesses in socialization and communication skills. 1,[8][9][10][11] Reports also suggest that there may be a change in adaptive behavior over time, although the direction of this change differs across studies and depends on whether age-equivalent scores or standard scores are examined. Some reports indicate that there is growth in adaptive behavior skills at ∼10 years of age, and others suggest a decline in these abilities as people grow older.…”

mentioning

confidence: 99%

Longitudinal Profiles of Adaptive Behavior in Fragile X Syndrome

Klaiman

Quintin

et al. 2014

Pediatrics

View full text Add to dashboard Cite

WHAT'S KNOWN ON THIS SUBJECT: To date, studies of adaptive behavior in fragile X syndrome have focused on particular age points, either longitudinally or cross-sectionally across a broad age spectrum. Studies have shown variable patterns in adaptive behavior among people with fragile X syndrome. WHAT THIS STUDY ADDS:This study fills a critical gap in knowledge about the profile of adaptive behavior across childhood, adolescence, and young adulthood in fragile X syndrome. This study is the first to incorporate longitudinal data from an age-matched typically developing group. abstract OBJECTIVE: To examine longitudinally the adaptive behavior patterns in fragile X syndrome.METHOD: Caregivers of 275 children and adolescents with fragile X syndrome and 225 typically developing children and adolescents (2-18 years) were interviewed with the Vineland Adaptive Behavior Scales every 2 to 4 years as part of a prospective longitudinal study. RESULTS:Standard scores of adaptive behavior in people with fragile X syndrome are marked by a significant decline over time in all domains for males and in communication for females. Socialization skills are a relative strength as compared with the other domains for males with fragile X syndrome. Females with fragile X syndrome did not show a discernible pattern of developmental strengths and weaknesses.CONCLUSIONS: This is the first large-scale longitudinal study to show that the acquisition of adaptive behavior slows as individuals with fragile X syndrome age. It is imperative to ensure that assessments of adaptive behavior skills are part of intervention programs focusing on childhood and adolescence in this condition. Pediatrics 2014;134:315-324 AUTHORS:

show abstract

“…They summarized that the declines were most remarkable in the verbal area with the use of language decreasing over time. Wiegers et al [] also found IQ decline in 39 males with FXS ages 4–26 years. However, on a Dutch adaptive behaviors scale, the self‐help skills improved with age, although the social skills did not; the latter were the lowest area of functioning [Wiegers et al, ].…”

Section: Cognition and Aging In Fxsmentioning

confidence: 96%

Fragile X syndrome: An aging perspective

Schneider

Ligsay

Hagerman

2013

Dev Disabil Res Revs

View full text Add to dashboard Cite

Cognitive and behavioral correlates of molecular variations related to the FMR1 gene have been studied rather extensively, but research about the long-term outcome in individuals with fragile X spectrum disorders remains sparse. In this review, we present an overview of aging research and recent findings in regard to cellular and clinical manifestations of aging in fragile X syndrome, and the FMR1 premutation.

show abstract

Adaptive behavior in the fragile X syndrome: Profile and development

Cited by 20 publications

References 5 publications

Is fragile X syndrome a pervasive developmental disability? Cognitive ability and adaptive behavior in males with the full mutation

Is fragile X syndrome a pervasive developmental disability? Cognitive ability and adaptive behavior in males with the full mutation

Longitudinal Profiles of Adaptive Behavior in Fragile X Syndrome

Fragile X syndrome: An aging perspective

Contact Info

Product

Resources

About