Adaptive behavior in the fra(X) syndrome: A longitudinal study in eight patients

Curfs, Paul M. G.; Schreppers-Tijdink, G.; Wiegers, A. M.; Velzen, Wim van

doi:10.1002/ajmg.1320340409

Cited by 9 publications

(4 citation statements)

References 2 publications

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“…Some studies report the event as a "plateau" of ageequivalent scores [Dykens et al, 1993[Dykens et al, , 1996 or as scores of differing dimensions of adaptive behavior [Wiegers et al, 1993]. Others portray a negatively accelerating function of composite scores that reach a minimum during adolescence [Curfs et al, 1989;Fisch et al, 1994Fisch et al, , 1996b. We examined three domains of adaptive behavior and found that communication domain scores were the most severely impacted and they plateau earlier than either socialization domain or daily living skills scores, as reported previously in cross-sectional and retrospective longitudinal studies [Dykens et al, 1993[Dykens et al, , 1996Wiegers et al, 1993].…”

Section: Discussionsupporting

confidence: 61%

“…Like studies of cognitive deficits, early crosssectional investigations of adaptive behavior in fra(X) males found negative correlations between chronological age and adaptive behavior composite scores [Curfs et al, 1989;Fisch et al, 1994] or noted that ageequivalent scores of adaptive behavior leveled off as chronological age increased [Dykens et al, 1993]. Therefore, cognitive ability and adaptive behavior reach an asymptote as young fra(X) males age.…”

Section: Introductionmentioning

confidence: 98%

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Longitudinal assessment of adaptive and maladaptive behaviors in fragile X males: Growth, development, and profiles

Fisch

Carpenter²,

Holden

et al. 1999

Am. J. Med. Genet.

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As young fully mutated fragile X [fra(X)] males age, cognitive levels (IQ scores) and adaptive behavior levels (DQ scores) decline. Given the variable behavioral profiles reported previously, we wondered whether changes in specific attributes of adaptive behavior are related to declines in composite adaptive behavior levels. We also examined maladaptive behavior to determine if changes are related to age. Therefore, we evaluated three areas of adaptive behavior, as well as maladaptive behavior, in 28 fully mutated fra(X) males, ages 4-14 years. To develop a profile of adaptive behavior, we analyzed nine subscale scores from the Vine-land Adaptive Behavior Scale (VABS). To assess maladaptive behavior, we graded part I of the VABS Maladaptive Behavior Scale. Subjects were sorted into three age cohorts, according to their initial test age: younger than 6 years; 6 to 9 years; older than 9 years. Results indicate that, in all age groups, the communications domain is the most severely impacted compared with either the socialization domain or daily living skills and that, in all age groups, the socialization domain is a relative strength compared with either the communications domain or daily living skills. The youngest cohort manifested significant increases in age-equivalent community living skills. Significant differences in age-equivalent scores between cohorts were observed in written language and play skills. Maladaptive behavior scores were available from cross-sectional data only. Twenty males (74%) showed significantly higher maladaptive scores than expected from other children their age. Our data analysis also revealed a moderate and significant negative correlation between maladaptive behavior levels and age (r = -0.54; P < 0.01). Curiously, adaptive and maladaptive behaviors did not correlate with each other.

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Section: Discussionsupporting

confidence: 61%

Section: Introductionmentioning

confidence: 98%

Longitudinal assessment of adaptive and maladaptive behaviors in fragile X males: Growth, development, and profiles

Fisch

Carpenter²,

Holden

et al. 1999

Am. J. Med. Genet.

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“…Longitudinal decreases in IQ have been observed previously in male fra(X) children and adolescents. Declines in SQ have been observed primarily in adolescent fra(X) males [Curfs et al, 1989b;Dykens et al, 19931, although Wiegers et al [1993] show no relationship between age and adaptive score. It may be that the SRZ used by Wiegers et al draws upon a different set of behaviors than the VABS.…”

Section: Discussionmentioning

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.

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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)

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“…Work identifying endophenotypes in FXS has demonstrated behavioral deficits across the domains of spatiotemporal processing, numerical or arithmetic processing, to some extent executive function, and specific visual biological motion processing abnormalities (Bregman et al , 1987; Curfs et al , 1989a, 1989b, 1989c; Dykens et al , 1987; Mazzocco et al , 1993; Van der Molen et al , 2010). In the spatiotemporal domain, there have been reports of specific deficits in spatial and temporal memory performance in FXS, even in cases where FSIQ is controlled as a covariate and verbal performance is equal to or surpasses that of controls ( cf.…”

Section: Specific Examplesmentioning

confidence: 99%

Comprehensive neurocognitive endophenotyping strategies for mouse models of genetic disorders

Hunsaker

2012

Progress in Neurobiology

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There is a need for refinement of the current behavioral phenotyping methods for mouse models of genetic disorders. The current approach is to perform a behavioral screen using standardized tasks to define a broad phenotype of the model. This phenotype is then compared to what is known concerning the disorder being modeled. The weakness inherent in this approach is twofold: First, the tasks that make up these standard behavioral screens do not model specific behaviors associated with a given genetic mutation but rather phenotypes affected in various genetic disorders; secondly, these behavioral tasks are insufficiently sensitive to identify subtle phenotypes. An alternate phenotyping strategy is to determine the core behavioral phenotypes of the genetic disorder being studied and develop behavioral tasks to evaluate specific hypotheses concerning the behavioral consequences of the genetic mutation. This approach emphasizes direct comparisons between the mouse and human that facilitate the development of neurobehavioral biomarkers or quantitative outcome measures for studies of genetic disorders across species.

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Adaptive behavior in the fra(X) syndrome: A longitudinal study in eight patients

Cited by 9 publications

References 2 publications

Longitudinal assessment of adaptive and maladaptive behaviors in fragile X males: Growth, development, and profiles

Longitudinal assessment of adaptive and maladaptive behaviors in fragile X males: Growth, development, and profiles

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

Comprehensive neurocognitive endophenotyping strategies for mouse models of genetic disorders

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