Stress in Families of Young Children with Down Syndrome,Williams Syndrome,and Smith-Magenis Syndrome

This study examined whether stress levels differ in families of young children with three different genetic etiologies of mental retardation, and whether child characteristics associated with those genetic etiologies may help explain these differences. Participants were sixty families of young children with Down syndrome, Williams syndrome, and Smith-Magenis syndrome. All children were between the ages of 3 and 10 years. Parents completed Achenbach's Child Behavior Checklist, the Questionnaire on Resources and Stress-Friedrich edition, and a demographic questionnaire. Families of children with Down syndrome experienced significantly less Pessimism than the other two etiology groups and significantly less Parent and Family Problems than families of children with Smith-Magenis syndrome. The strongest predictor of Parent and Family Problems was maladaptive behavior in Smith-Magenis syndrome, younger age in Down syndrome, and both maladaptive behavior and younger age in Williams syndrome. Maladaptive behavior predicted Pessimism in families of children with Smith-Magenis syndrome, but none of the variables examined significantly predicted Pessimism in the other two syndromes. The importance of behavioral phenotype research is discussed for practitioners working with young children with mental retardation and their families.     

弱智儿童亲社会行为研究综述

亲社会行为是弱智儿童适应行为中的一个重要方面,对弱智儿童教育十分重要。弱智儿童的亲社会行为与正常儿童、自闭症儿童相比表现不同,其中唐氏综合征儿童又是弱智儿童的一个特殊类型,影响弱智儿童亲社会行为的因素主要有年龄、性别、病因、智力等。     

Compulsive-like behavior in individuals with Down syndrome: its relation to mental age level, adaptive and maladaptive behavior

This study examined the nature of repetitive, ritualistic, and compulsive-like behaviors in 50 typically developing children and 50 individuals with Down syndrome (DS), matched on mental age (MA; M = 59.72 months). Parents reported on their children's compulsive-like behaviors-including ritualistic habits-and perfectionistic behaviors, as well as their children's adaptive and maladaptive behaviors. Results indicated that children with DS show similar MA-related changes in compulsive-like behaviors compared to the MA-matched comparison group. Younger children (both typical and DS) exhibited significantly more compulsive-like behaviors than older children. In general, children with and without DS did not differ from each other in terms of the number of compulsive-like behaviors they engaged in, although participants with DS engaged in more frequent, more intense repetitive behaviors. Compulsive-like behaviors were differentially related to adaptive and maladaptive behaviors across the MA and mental retardation groups. The results extend the "similar sequence" model of development to the construct of compulsive-like behaviors, and also suggest that some repetitive behaviors may be among the behavioral phenotype of individuals with DS.     

A Mental Age-Matched Comparison Study of Delay of Gratification in Children with Down Syndrome

The performance of 25 children with Down syndrome on delay of gratification tasks was compared with that of a mental age-matched group of typically developing children. Delay tasks included both other- and self-imposed tasks. Children with Down syndrome were significantly less able to delay gratification than the comparison group on two of the three tasks. Receptive language was associated with delay on the self-imposed task for the typically developing group but not for children with Down syndrome. It is hypothesised that there may be a lag in the development of self-regulation that is greater than the lag between chronological and mental age for children with Down syndrome, with expressive language playing a role in this lag. The practice of using mental age as the method for matching groups of children with Down syndrome with typically developing children is called into question by the results of this study.     

Goal-Directed Behavior in Children with Down Syndrome: The Role of Joint Play Situations

Children's goal-directed behaviors were examined in independent play sessions before and after a joint-play interaction with their mothers for a group of children with Down syndrome (n = 22) and a control group of mental and language age matched typically developing children (n = 24). While both groups showed comparable amounts of time spent in independent goal-directed play during the pre session and similar play responses with their mothers during the joint play session, only the control children significantly increased their goal-directed behavior from the pre to the post session. Maternal behaviors that provided information about how to use the toy predicted increases from the pre to post sessions in independent goal-directed play but only for the control children. These findings suggest that children with Down syndrome may have more difficulty transferring the goal-directed play behaviors they can use with support from their mothers to an independent play situation. Findings are discussed in relation to early education program design for children with Down syndrome.     

Less Stress,More Rewarding: Parenting Children With Down Syndrome

Objective. We argue that, compared to other children with disabilities, parents of children with Down syndrome may experience less stress and more rewards. Design. After reviewing changes in studies examining parenting children with disabilities, we note how specific genetic disorders predispose children to different, etiology-related behaviors, which in turn predispose their parents to particular reactions. We then survey studies of both stress and rewardingness in parents of children with Down syndrome versus children with other disabilities. Results. Parents of children with Down syndrome report less stress and more child-related rewards than parents of children with other disabilities; indeed, parents of children with Down syndrome may feel equally rewarded compared to parents of same-aged typical children. Conclusions. By comparing feelings of parents of children with Down syndrome versus children with other disabilities, we begin to understand which child behaviors bring about which parental reactions. Such information provides both theoretical and practical benefits to professionals interested in parenting.     

Mental retardation general intelligence and modularity

This article presents a case for distinguishing between mental retardation as a general deficit of thinking and mental retardation that might result from the global effects of a specific deficit in a cognitive module. Using Anderson's (1992a) theory of the minimal cognitive architecture of intelligence and developmental, I show how this distinction can explain the pattern of intellectual strengths and weaknesses in Savant syndrome, Williams syndrome, Down syndrome, and autism. In addition, I discuss the developmental versus difference view and the distinction between organic and cultural familial mental retardation in the light of this theory. I conclude that not only is there no inherent incompatibility between the constructs of general intelligence and modularity of mind but that both are essential to understanding the different patterns of abilities and developmental profiles found in individuals with low IQ.     

Development in Children With Down Syndrome: facts,findings, the future

This broad overview of progress in our understanding of Down syndrome and of the problems it presents for children affected by it was prompted by the occasion of the 20th Anniversary of the Down Syndrome Research Program at the Fred and Eleanor Schonell Special Education Research Centre, The University of Queensland. This internationally‐acclaimed program has focused on many aspects of development in Down syndrome and on how Down syndrome impacts on the lives of children and their families. Some of the many contributions the Schonell team have made to our knowledge of Down syndrome are covered in other papers in this special issue and will also be mentioned below. In evaluating the role of this work, however, it is important to put it into context. This paper therefore provides a brief‐‐and necessarily selective‐‐ account of some past and present findings from research into Down syndrome and makes some tentative suggestions as to what may be important avenues of investigation for the next generation of researchers.     

Goal-Directed Behavior in Children with Down Syndrome: The Role of Joint Play Situations

Children's goal-directed behaviors were examined in independent play sessions before and after a joint-play interaction with their mothers for a group of children with Down syndrome (n = 22) and a control group of mental and language age matched typically developing children (n = 24). While both groups showed comparable amounts of time spent in independent goal-directed play during the pre session and similar play responses with their mothers during the joint play session, only the control children significantly increased their goal-directed behavior from the pre to the post session. Maternal behaviors that provided information about how to use the toy predicted increases from the pre to post sessions in independent goal-directed play but only for the control children. These findings suggest that children with Down syndrome may have more difficulty transferring the goal-directed play behaviors they can use with support from their mothers to an independent play situation. Findings are discussed in relation to early education program design for children with Down syndrome.     

Self‐awareness in Young Adults with Down Syndrome: II. Self‐understanding

This study explored the self‐understanding of 77 young people with Down syndrome aged from 17 to 24 years, with verbal mental ages ranging from less than 2 years 6 months to 12 years 4 months. The aim was to determine whether the self‐understanding of these young people followed a typical developmental pathway, whether they were making social comparisons to others, and if these comparisons were downward, upward or lateral. The data came from interviews with the young people and with their parents. Results confirmed the typical developmental progression in so far as relative comparisons to others were only made by those with significantly higher mental ages. These young people made more downward than upward comparisons, whereas parents largely referred to lateral comparisons. This again is similar to findings with people in general who tend to maintain their self‐esteem by making downward comparisons. Aspirations were age appropriate, albeit with relatively low levels of cognitive understanding. Reference groups predominantly consisted of families and other young people with intellectual disabilities.     

Family Influences on Adaptive Development in Young Children with Down Syndrome

In this study we investigated the extent to which the family environment predicted differences in trajectories of adaptive development in young children with Down syndrome. The sample was comprised of 54 children with Down syndrome and their families who were studied from infancy through the age of 5 years as part of a longitudinal study of children with disabilities. Hierarchical linear modeling (HLM) was used to estimate the parameters of hierarchical growth models in domains of adaptive development. Results indicated that growth in communication, daily living skills, and socialization domains were predicted by measures of the family environment (i.e., family cohesion and mother-child interaction) above and beyond that predicted by maternal education. Further, Bayley MDI measures during infancy did not predict changes in adaptive development in any of the domains. The results are discussed in terms of implications for service provision and for expanding theoretical frameworks to include the development of children with disabilities.     

Language and Williams Syndrome: How Intact Is "Intact"?

It has been claimed that Williams syndrome (WS), a rare neurodevelopmental disorder, is characterized by serious cognitive deficits alongside intact language. The syndrome is often used as a prime example of the modularity of an innate faculty for morphosyntactic rules. We challenge this claim and hypothesize that morphosyntax, although surprisingly good given WS level of mental retardation, is by no means intact. We make an initial test of this hypothesis through an analysis of the receptive language of a group of English-speaking WS individuals on a standardized morphosyntactic test. We then present an experimental study of expressive language that examines grammatical gender assignment in French-speaking WS patients. Despite a Verbal Mental Age selected to be higher than the chronological age of the young control group, these people with WS continue even in adulthood to show clear-cut deficits in their production of an aspect of morphosyntax that normal children acquire effortlessly very early. The results of the 2 studies, one focusing on receptive language and the other on expressive language, challenge the notion that comprehension and use of morphosyntactic rules in WS individuals are intact. The within-domain dissociations regarding the use of grammatical gender assignment across several sentence elements and their difficulties in understanding embedded sentences—two quintessentially linguistic skills—suggest that we must rethink the notion of spared, modular, language capacities in Williams syndrome. We conclude that WS language follows a different path to normal acquisition and may turn out to be more like second language learning.     

Developmental Attainment of Infants and Young Children with Down Syndrome

INFANTS and young children with Down syndrome who were living at home and attending exemplary early intervention programs were assessed by trained examiners in the five domains of the Battelle Developmental Inventory. Data was compared to the 50th percentile attainment and found that infants and young children with Down syndrome are more similar to other children in Personal Social and Adaptive Domains and less similar in Communication and Cognitive Domains. These differences begin to show more dramatically as the child reaches the age of 36 months. The older the child the greater the measured differences. Documenting and understanding this uneven developmental path is significant in program planning.     

Behaviour Problems in the Siblings of Children With Down Syndrome: associations with family responsibilities and parental stress

Forty‐five families with a child with Down syndrome and 88 comparison families provided information about their children's behaviour problems and their involvement in household tasks. In addition, parental stress was measured using the Parenting domain of the Parenting Stress Index (Abidin, 1990). There were no differences between the siblings of a child with Down syndrome and comparison children on mothers’ or fathers’ reports of problem behaviour. Siblings of a child with Down syndrome also did not differ in their contribution to family tasks, however, for the brothers of a child with Down syndrome there were significant negative correlations between household tasks and behaviour problems on fathers’ report. Parents of a child with Down syndrome reported more stress than comparison parents and stress was related to reports of problem behaviour for some parent groups.     

Self‐awareness in Young Adults with Down Syndrome: I. Awareness of Down syndrome and disability

The limited literature on awareness of differences and stigma in people with intellectual disabilities is largely sociological, emphasises pathology, and has rarely used a developmental perspective with representative samples. Interviews, photographs and standardised tests were used to investigate such awareness with 77 young adults with Down syndrome and their parents. Awareness and social categorisation were significantly associated with verbal mental age, and closely approximated the typical social‐cognitive developmental sequence. No associations were found between awareness and chronological age, parent telling, gender, and mainstream experience. Only those with verbal mental ages from around 8 years were making relative social comparisons and beginning to form complex social categories of Down syndrome/disability. Around 13% were rated as showing a negative emotional reaction to Down syndrome/disability, and most of these were male. A similar percentage, mostly female and with higher verbal mental ages, discussed concerns and limitations. Even so, they all had high self‐esteem and awareness of Down syndrome and disability did not appear to be a major issue. A number of coping mechanisms to maintain a positive sense of self were suggested. It is argued that both sociological and developmental models are required to inform parent and professional attempts to facilitate self‐awareness.     

Using Total Communication With Young Children With Down Syndrome: A Literature Review and Case Study

This paper presents a literature review regarding language abilities of children with Down syndrome and presents a case study concerning the effectiveness of using Total Communication with a young child with Down syndrome. The prevalence of expressive language delays in children with Down syndrome highlights the need to develop early interventions to promote language development. The existing literature on the usefulness of Total Communication as an intervention method with children and adults with special needs is documented and a rationale for the use of Total Communication with children with Down syndrome is presented. In the case study, a single subject simultaneous treatment design was used which involved introducing 20 words during free play (10 oral and 10 Total Communication), which were matched on phonetic complexity and reinforcement value. Results indicated that comprehension was not differentially affected by the type of communication approach used. However, expressively the child was able to use manual signs many months before any understandable words were produced. The use of manual signs did not inhibit use of speech. The results of this case study suggest that the early use of Total Communication can be an effective transitional mode of communication for at least some children with Down syndrome. Further research is needed to explore individual differences between children with Down syndrome and to identify factors that predict those children who benefit most from the use of Total Communication.     

Using Total Communication With Young Children With Down Syndrome: A Literature Review and Case Study

This paper presents a literature review regarding language abilities of children with Down syndrome and presents a case study concerning the effectiveness of using Total Communication with a young child with Down syndrome. The prevalence of expressive language delays in children with Down syndrome highlights the need to develop early interventions to promote language development. The existing literature on the usefulness of Total Communication as an intervention method with children and adults with special needs is documented and a rationale for the use of Total Communication with children with Down syndrome is presented. In the case study, a single subject simultaneous treatment design was used which involved introducing 20 words during free play (10 oral and 10 Total Communication), which were matched on phonetic complexity and reinforcement value. Results indicated that comprehension was not differentially affected by the type of communication approach used. However, expressively the child was able to use manual signs many months before any understandable words were produced. The use of manual signs did not inhibit use of speech. The results of this case study suggest that the early use of Total Communication can be an effective transitional mode of communication for at least some children with Down syndrome. Further research is needed to explore individual differences between children with Down syndrome and to identify factors that predict those children who benefit most from the use of Total Communication.     

Adaptive Functioning in Williams Syndrome: A Systematic Review

Literature on the level of adaptive functioning and relative strengths and weaknesses in functioning of individuals with Williams syndrome (WS) was reviewed. The electronic databases PsycINFO, PubMed, Expanded Academic, Web of Science, Scopus and ProQuest were searched electronically for relevant articles and dissertations using the search terms ‘Williams syndrome’ or ‘Williams-Beuren syndrome’ combined with ‘adaptive function*’, ‘adaptive behavio*’, ‘independ*’ and ‘autonomy’. Selection criteria included English language articles, theses and book chapters, participants with a diagnosis of Williams syndrome and inclusion of a standardised assessment of adaptive functioning. Twenty-two published articles and two PhD. dissertations (one subsequently published as a poster presentation) met the selection criteria for inclusion in the review. Fifteen investigated adaptive functioning in children and adolescents aged up to 19 years of age, five investigated adaptive functioning in adults and four included participants across a wider age range and included both children and adults. Along with identifying methodological issues, the review addressed the following areas: overall level of adaptive functioning in WS, domain strengths and weaknesses, evidence of heterogeneity, relationship to intellectual ability, changes with chronological age, relationship with maladaptive behaviour, gender differences and the potential influence of environmental factors which may be related to adaptive functioning in WS.     

彝族地区智力落后儿童生活质量现状研究

调研目的：了解彝族地区智力落后儿童社会适应行为、社会支持与生活质量的现状。方法：采用问卷调查法,在云南省、四川省彝族地区的两所特殊学校,整群抽取126名智力落后儿童。结果：彝族智力落后儿童的社会适应行为性别差异显著,女生显著高于男生（p〈0．01）;不同居住地的彝族智力落后儿童的社会适应行为差异显著（p〈0．05）;父母不同文化程度的智力落后儿童的社会适应水平差异显著（p〈0．05）;不同支持水平的彝族智力落后儿童的生活质量差异显著（p〈0．01）;支持利用、社会责任进入彝族智力落后儿童的生活质量模型（p〈0．01）。     

A pilot study of early childhood mental health consultation for children with behavioral problems in preschool

This study reports the findings of a pilot demonstration project called Together for Kids, which used a mental health consultation model to address the needs of young children with challenging behaviors who are identified in preschool classrooms. The study was conducted in four preschool programs and one Head Start program serving children ages 3–5, including both private-pay families and those using public subsidies. Rates of significant behavior problems as assessed by preschool teachers using a standardized scale were high, with 34% of all children enrolled in preschool classrooms in these sites over a 3-year period identified at-risk of externalizing or internalizing problems. Classroom teachers, as well as individual children and families identified as at-risk, were provided services, including, classroom observation and teacher training, individual child assessment and therapy, family assessment and support, and referrals for other family needs. Analysis of outcomes for 47 children and families with externalizing behavior problems who received individualized consultation, compared to 89 control children, and analysis of outcomes of a matched group of 19 intervention and 19 control children, revealed that the intervention was associated with significant improvements in classroom aggressive and maladaptive behavior, and growth in adaptive behavior. Improvements in child behavior were associated with total hours of individual child services provided, and with improvements in child developmental skills. Significant reductions in the rate of children suspended or terminated from child care programs were also found. Implications for further development of models of early childhood mental health consultation are discussed.