The present study was designed to assess object identification ("what") and location ("where") skills among girls with fragile X or Turner syndrome and girls with neither disorder. Participants completed standardized subtests of visual perception and tasks of visuospatial "what" and "where" memory. Girls with fragile X had average performance on most object identification tasks, yet 53% failed to accurately recreate the gestalt of a design during the "where" memory task. Fewer than 7% of girls in the Turner or comparison group made this error. Girls with Turner syndrome had lower scores and longer response times on object perception tasks and had poorer recall of location for internal features of the design on the "where" memory task, relative to girls in the comparison or fragile X group. When limiting analyses to IQ-matched samples, correlations between math and visual perception tasks emerged, but only for girls with fragile X. These results reflect important differences between two cognitive phenotypes and have implications for the role of visuospatial processing in early math performance.
Social skills impairment in children with Turner or fragile X syndrome has been documented using parental reports. Anxiety, shyness, and difficulty understanding social cues have been reported for females with Turner syndrome; whereas social withdrawal, avoidance of social interactions, and anxiety are often reported for females with fragile X syndrome. Social interaction anxiety in these two populations may be a framework for understanding the difficulty these children experience in social situations. In the present study, 29 females with Turner syndrome and 21 females with fragile X syndrome ages 6-22 years were compared to females in a comparison group, on a videotaped role-play interaction. Behavioral indices examined included eye-contact maintenance, duration of speech, and body discomfort as observed during the brief interaction. Three of eight such behavioral measures of social skills differentiated the participant groups from each other. Specifically, participants with fragile X required more time to initiate interactions than did participants in either of the remaining groups; and females with Turner syndrome made fewer facial movements than did females in the fragile X or comparison group. Self-report and parental ratings did not suggest higher levels of anxiety in females with Turner or fragile X syndrome, but did reflect higher levels of social difficulty. The authors discuss these findings in terms of understanding the nature of social dysfunction in females with Turner or fragile X syndrome.
Fragile X and Turner syndromes are associated with risk of atypical social function. We examined language use, including normal and atypical speech, during initial social interactions among participants engaged in a brief social role play with an unfamiliar adult. There were 27 participants with Turner syndrome, 20 with fragile X syndrome and 28 in an age-matched comparison group. Females with fragile X did not exhibit more abnormal language, but exhibited less of what is typical during initial interactions. Overall rates of dysfluencies did not differ, although females with fragile X made more phrase repetitions. Females with Turner syndrome had no language use abnormalities. Our findings suggest that language use may influence social function in females with fragile X syndrome and that such language characteristics may be observed in the context of brief encounters with an unfamiliar adult.
The effect of a warning regarding detection of simulated cognitive and motor deficits on the Wechsler Memory Scale-Revised (WMS-R; Wechsler, 1987) and Grooved Pegboard (Klove, 1963; Matthews & Klove, 1964) was examined. Undergraduates (N = 87) were randomly assigned to one of three conditions: simulators without warning, simulators with warning, and controls. It was predicted that warning participants that simulation efforts will be detected would reduce malingering behavior and this hypothesis was generally supported. Analyses indicated simulators with warning frequently approximated control group performances and were significantly better than simulators without warning on some measures. Stepwise discriminant function analysis accurately classified 57% of all participants. Results provide support for effectiveness of warning in reducing malingering behavior on selected measures and are discussed in terms of the use of warning as an adjunct to existing procedures, role of the neuropsychologist, and informed consent for assessment in personal injury litigation.
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