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A longitudinal study of the developmental and behavioral characteristics of 26 boys (ages 12-36 months) with fragile X syndrome, found there was an increase in their developmental skills over time, although there was a great deal of variability. Motor skills appeared least delayed, whereas communication skills appeared most delayed. (Contains references.) (Author/CR)

A study of 50 boys with fragile X syndrome, their parents, and teachers, found early intervention started on average at 21.6 months and there was a steady increase in the amount of early intervention across age periods. Although parents reported satisfaction with services, most would have preferred more services. (Contains references.) (Author/CR)

Students with visual impairment (VI) lack access to the same models and reinforcers as students with sight. Consequentially, behaviors that children with sight acquire through observation must be explicitly taught to children with VI. In addition, children with VI have difficulty maintaining such behaviors. Therefore, interventions that promote self-management of students' own behavior are desirable. In this study, three adolescents used self-talk to increase chewing with a closed mouth during mealtimes. We used explicit instruction to teach students the target behavior and to use a small vibrating device as a reminder to self-talk during mealtimes. Prompts were faded as participants increased chewing with a closed mouth. Following mealtimes, performance feedback and reinforcement were provided. All students increased percentage of intervals chewing with a closed mouth immediately at the start of intervention and maintained this behavior for up to 1 month post-intervention. Implications for practice and directions for future research are discussed.

The association made between the meaning, spelling, and pronunciation of a word has been shown to help children remember the meanings of words. The present study addressed whether the presence of a target word in Braille during instruction facilitated vocabulary learning more efficiently than an auditory-only instructional condition. The authors used an adapted alternating treatments single-case experimental design with three students with visual impairments who read Braille, collecting data on definition recall and spelling during each session. Data on definition recall were used to determine mastery. The results of this study are not consistent with previous findings with students who read print. Visual analyses of the data indicated that participants reached mastery in both conditions, but all three reached mastery on definition recall in fewer sessions in the auditory-only condition. Spellings of words were learned in the flashcard condition only, and possible implications of this are discussed. The difference in the unit of recognition and working memory load between reading Braille and reading print is discussed as one possible explanation.

Students with visual impairment (VI) were taught to request using the Picture Exchange Communication System (PECS) and tangible symbols. Participants were four males with additional disabilities, 5 to 11 years old, who had little to no functional vision. A functional relation between PECS Phase 1 and requesting was established using a multiple baseline single subject design. All students learned to request independently within 7 to 14 sessions in Phase 1. Students maintained requesting above criterion for 1 to 5 months. All students generalized requesting to a new communication partner (CP); however, only two students reached criterion during generalization sessions. Two students learned to seek out a CP 5 feet away to request; however, no functional relation was established for Phase 2. More research is needed to establish PECS as an evidence-based practice for children with VI.

Children with fragile X syndrome (FXS) face high risk for anxiety disorders, yet no studies have explored FXS as a high-risk sample for investigating early manifestations of anxiety outcomes. Negative affect is one of the most salient predictors of problem behaviors and has been associated with both anxiety and autistic outcomes in clinical and non-clinical pediatric samples. In light of the high comorbidity between autism and anxiety within FXS, the present study investigates the relationship between longitudinal trajectories of negative affect (between 8 and 71 months) and severity of anxiety and autistic outcomes in young males with FXS (n = 25). Multilevel models indicated associations between elevated anxiety and higher fear and sadness, lower soothability, and steeper longitudinal increases in approach. Autistic outcomes were unrelated to negative affect. These findings suggest early negative affect differentially predicts anxiety, not autistic symptoms, within FXS. Future research is warranted to determine the specificity of the relationship between negative affect and anxiety, as well as to explore potential moderators. Characterizing the relationship between early negative affect and anxiety within FXS may inform etiology and treatment considerations specific to children with FXS, as well as lend insight into precursors of anxiety disorders in other clinical groups and community samples.

Introduction: This study evaluated whether children with visual impairments who receive repeated reading instruction exhibit an increase in their oral reading rate and comprehension and a decrease in oral reading error rates. Methods: A single-subject, changing-criterion design replicated across three participants was used to demonstrate the association between a repeated reading intervention and the oral reading rate, comprehension, and error rate. Results: Visual analysis of the data indicates that there was a functional relation between repeated reading and oral reading rate for two participants, and a functional relation between repeated reading and comprehension for all participants. There was not a functional relation between repeated oral reading and error rate. Discussion: Based on the results of this study, repeated reading appears to be an effective practice for some students with visual impairments. Implications for practitioners: Teachers and parents can engage children in repeated reading activities in an effort to develop automaticity in reading and to improve oral reading rates. (Contains 2 tables and 3 figures.)

Aberrant attention is a core feature of fragile X syndrome (FXS), however, little is known regarding the developmental trajectory and underlying physiological processes of attention deficits in FXS. Atypical visual attention is an early emerging and robust indicator of autism in idiopathic (non-FXS) autism. Using a biobehavioral approach with gaze direction and heart activity, we examined visual attention in infants with FXS at 9, 12, and 18 months of age with a cross-sectional comparison to 12-month-old typically developing infants. Analyses revealed lower HR variability, shallower HR decelerations, and prolonged look durations in 12-month old infants with FXS compared to typical controls. Look duration and increased latency to disengage attention were correlated with severity of autistic behavior but not mental age.

The findings from a sample of 22 young children with visual impairments and no additional disabilities suggest that potential readers of braille or dual media had better syllable-segmentation, sound-isolation, and sound-segmentation skills than potential readers of print. Potential readers of print seemed to have slightly better letter-identification and letter-sound identification skills than potential readers of braille or dual media. (Contains 2 tables.)

To describe the early phenotype of girls with full mutation fragile X, we used 54 observations of 15 girls between the ages of 6 months and 9 years to examine developmental trajectories as measured by the Battelle Development Inventory. In this sample, autistic behavior was associated with poorer developmental outcomes, primarily due to interactions of age with autistic behavior, even though autistic behavior, measured continuously, was relatively mild. Although this small sample, ascertained primarily through male relatives with fragile X syndrome, limits generalizability, considerable variability in developmental outcome in young girls was documented. In addition, findings support previous research suggesting that even mild autistic behaviors in girls can be associated with developmental outcomes.

Social avoidance is a core phenotypic characteristic of fragile X syndrome (FXS) that has critical cognitive and social consequences. However, no study has examined modulation of multiple social avoidant behaviors in children with FXS. In the current study, we introduce the "Social Approach Scale" (SAS), an observation scale that includes physical movement, facial expression, and eye contact approach behaviors collected across multiple time points. Our findings suggested that social approach behaviors in children with FXS were affected by age, gender, setting, and time spent with an examiner. Selected social approach behaviors were related to autistic behavior. Increased eye contact over the course of a research assessment, in particular, was found to be a strong predictor of lower autistic behavior.

Adaptive behavior was measured over time in 70 children, ages 1 to 12 years, with fragile X syndrome. With a mean of 4.4 assessments per child, adaptive behavior skills increased steadily and gradually over time. Children with less autistic behavior and higher percentages of the fragile X mental retardation gene protein showed better performance on all areas of adaptive behavior. (Contains references.) (Author/DB)

A study obtained data on 406 children from birth to age 3 with visual impairments at the point of entry into specialized early intervention programs in nine states. The majority were legally blind and had additional disabilities. About a six-month mean lag was found between diagnosis and referral for services. (Contains references.) (Author/CR)

This study examined whether the variability in development in fragile X syndrome is related to FMR protein deficiency in 53 young males with various degrees of the syndrome. FMR protein expression accounted for a small but significant amount of variance in level, but not rate, of development. Findings support the hypothesized relationship but suggest other factors also play a role. (Contains references.) (Author/DB)

This study compared the developmental status, functional abilities, and temperaments of 31 young (36 to 95 months) boys with fragile X syndrome (FXS) who did not have autism, matched on chronological age, gender, and race, with 31 boys with autism but no FXS. Overall, the boys with autism exhibited a more variable profile of development than did children with FXS. (Contains extensive references.) (Author/DB)

A study of 57 boys with fragile X syndrome (ages 24-133 months) assessed the extent to which autism and autistic features were evident using the Childhood Autism Rating Scale (CARS). Fourteen subjects scored above the cut-off for autism, with 12 of the 14 in the mildly or moderately autistic range. (Author/CR)

Findings from a prospective longitudinal study of 46 boys with fragile X syndrome (ages 24-72 months) found that, although children varied widely, overall development was significantly delayed to approximately half of the rate of typical children. At every age tested, motor and adaptive development was higher than communication and cognitive development. (Author/CR)

Used growth curve analysis to describe the development of 186 preschoolers with vision impairments. Found that mental retardation and developmental delay were associated with lower developmental ages and slower growth rates. Visual function of 20/800 or worse was associated with lower developmental ages and slower growth rates in personal-social and motor domains on the Battelle Developmental Inventory. (Author/KB)