ABSTRACT
This study investigated the influence of maternal and child variables on the maternal responsivity of 55 mothers with young children with fragile X syndrome. Data included video observations of mother-child interactions in four different contexts, standardized assessments with the children, and standardized questionnaires for the mothers. The video observations were coded for child communication acts; maternal responsivity was coded at two levels: a more general measure and a behavior-by-behavior measure. Results indicated that child developmental level and language ability strongly influenced behavior-by-behavior responsivity, while maternal IQ was the strongest predictor of both general and behavior-by-behavior responsivity, after controlling for child developmental level.
Subject(s)
Fragile X Syndrome/psychology , Maternal Behavior , Mother-Child Relations , Mothers/psychology , Adult , Child Development/physiology , Child, Preschool , Female , Humans , Infant , Intelligence/physiology , Male , Predictive Value of Tests , Psychiatric Status Rating Scales , Qualitative Research , Surveys and Questionnaires , Video RecordingABSTRACT
Recent evidence suggests that there are age-related neurocognitive implications for fragile X premutation carriers, including deficits in executive function, and that such deficits are more common in male than female premutation carriers. The purpose of the current study is to examine one aspect of executive function, language dysfluencies, in a group of 193 women with the premutation, and to contrast them with a comparison group (mothers of children with autism spectrum disorders). Our results demonstrate a linguistic profile in the female premutation carriers characterized by dysfluencies associated with deficits in organization and planning, with a clear impact of age. The comparison group, matched on both age and education level, did not demonstrate the age effect. Our results suggest dysfluencies could be an early indicator of cognitive aging in some female premutation carriers, and could be used to target early intervention.
Subject(s)
Aging/genetics , Fragile X Mental Retardation Protein/genetics , Mutation , Speech Disorders/genetics , Adult , Age Factors , Aged , Executive Function/physiology , Female , Heterozygote , Humans , Language , Middle AgedABSTRACT
ABSTRACT This study compared the receptive and expressive language profiles of verbally expressive children and adolescents with Down Syndrome (DS) and those with Fragile X syndrome (FXS) and examined the extent to which these profiles reliably differentiate the diagnostic groups. A total of twenty-four verbal participants with DS (mean age: 12 years), twenty-two verbal participants with FXS (mean age: 12 years), and twenty-seven participants with typical development (TD; mean age = 4 years) completed standardized measures of receptive and expressive vocabulary and grammar, as well as a conversational language sample. Study results indicate that there are distinct DS and FXS language profiles, which are characterized by differences in grammatical ability. The diagnostic groups were not differentiated based on vocabulary performance. This study supports the existence of unique language profiles associated with DS and FXS.
Subject(s)
Down Syndrome/psychology , Fragile X Syndrome/psychology , Language Tests , Adolescent , Case-Control Studies , Child , Down Syndrome/diagnosis , Female , Fragile X Syndrome/diagnosis , Humans , Intelligence Tests , Language , MaleABSTRACT
PURPOSE: The current study investigated finiteness marking (e.g., he walk s, he walk ed) in boys with fragile X syndrome (FXS); the boys were grouped based on receptive vocabulary (i.e., borderline, impaired). METHOD: Twenty-one boys with the full mutation of fragile X, between the ages of 8 and 16 years participated. The boys completed probes from the Test of Early Grammatical Impairment (TEGI; Rice & Wexler, 2001), a language sample, a nonverbal IQ test (Leiter-R; Roid & Miller, 1997), a receptive vocabulary test (the Pearson Picture Vocabulary Test-Fourth Edition [PPVT-IV]; Dunn & Dunn, 2007), and a measure of autistic symptoms (the Childhood Autism Rating Scale [CARS]; Schopler, Reichler, & Renner, 2002). RESULTS: There were group differences for finiteness responses on the 3rd person singular probe; the group with impaired vocabulary omitted markers with greater frequency compared to the borderline vocabulary group. There were not significant differences on the past tense probe, with both groups performing lower than expectations based on receptive vocabulary ability. Nonverbal IQ was not correlated with the measures of finiteness marking. CONCLUSION: Boys with FXS demonstrate delays in finiteness marking, in particular, on past tense verbs. Boys with FXS show a unique profile, unlike children with SLI, in which their use of tense markers may exceed expectations benchmarked to clause length.
