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Background: Research suggests that adults with autism tend to have poor outcomes. Outcomes have mostly been defined using summary outcome ratings, with less focus on specific outcomes such as employment, living situation, social satisfaction, loneliness, and friendships. This study aimed to explore social and community outcomes, including employment, education, living arrangements, friendships, and social satisfaction, for autistic adults with and without intellectual disability. Method: Eighty‐four adults with autism (mean age 34.2 years, SD = 4.5; 67% with co‐occurring intellectual disability), recruited as children and adolescents, participated in the current study. Adult social and community inclusion outcomes were explored through interview and questionnaire, both parent/carer‐report and self‐report. Results: Participants predominantly lived with family or in supported accommodation, did not pursue higher education, and mostly participated in day activity programmes. Most had limited friendships as reported by parents/carers; however, self‐report data (n = 28) indicated that adults were largely satisfied with their social relationships. Overall outcome was poor for 57%, and good/very good for 34%. Adults with intellectual disability generally had poorer outcomes. Conclusion: Autistic adults encountered numerous difficulties in leading an independent life. Adults with co‐occurring intellectual disability were most likely to experience difficulties; however, outcomes ranged from poor to very good for adults without intellectual disability. Discrepancies in parent/carer‐report and self‐reported experiences of friendships highlight the need to ensure individual experiences are captured in addition to parent/carer‐report. Appropriate resources and programmes are crucial for adults with autism to support them to have the choice to live independently. [ABSTRACT FROM AUTHOR]

Background: Sleep problems are common in many neurodevelopmental disorders, but little is known about how sleep is related to behavioural symptoms in Angelman syndrome (AS) or other genetic disorders. Hyperactive behaviour, sleep problems and epilepsy seem to be more common in AS than in other genetic conditions associated with severe intellectual disability. We hypothesised that both more sleep problems and earlier onset of epileptic seizures would predict more symptoms of hyperactivity. Hence, the aim of the project was to explore the association between hyperactive behaviour, sleep problems and age of epilepsy onset in individuals with AS. Method: All known parents/guardians (n = 115) of individuals with AS in Norway were invited to participate in this descriptive correlational study. Fifty‐six individuals (49%) responded, and 42 people (25 male and 17 female; mean age 18.5 years, range 2–57 years) with genetically verified AS were included. Scores for 'hyperactivity' and 'sleep problems' were derived from questionnaire data. Information on epilepsy was obtained from medical records. Results: 'Hyperactivity' was positively correlated with 'total sleep problems' (r = 0.46, P = 0.002) and negatively correlated with 'age of epilepsy onset' (r = −0.47, P = 0.01). 'Age of epilepsy onset' was not correlated with 'total sleep problems'. An overall multiple regression model with 'hyperactivity' as the dependent variable and 'age of epilepsy onset' and 'total sleep problems' as covariates was significant (R2 = 0.39, F = 8.16, P = 0.002). Hence, hyperactivity in AS could be predicted from both age of epilepsy onset and current sleep problems. Conclusions: Sleep problems may increase hyperactivity symptoms in individuals with AS. The association between hyperactivity and sleep problems in AS indicates that both should be investigated together as part of routine clinical assessment and intervention for either area of difficulty. Younger age of epilepsy onset was associated with more hyperactivity in AS, which may be related to encephalopathic effects of seizures and epilepsy. [ABSTRACT FROM AUTHOR]

Psychopathology is prevalent in Williams (WS), fragile X (FXS) and Prader–Willi (PWS) syndromes. However, little is known about the potential correlates of psychopathology in these groups. A questionnaire study was completed by 111 caregivers of individuals with WS (n = 35); FXS (n = 50) and PWS (n = 26). Mean age was 26 years (range 12–57 years); 74 (67%) were male. Multiple regression analyses indicated that higher rates of health problems and sensory impairments predicted higher psychopathology in WS (p <.0001). In PWS, poorer adaptive ability predicted higher overall psychiatric disturbance (p =.001), generalised anxiety (p =.006) and hyperactivity (p =.003). There were no significant predictors in FXS. This study highlights dissociations in the potential risk markers of psychopathology between genetic syndromes. Implications for intervention are discussed. [ABSTRACT FROM AUTHOR]

Background The Strengths and Difficulties Questionnaire (SDQ) is widely used to measure emotional and behavioural problems in typically developing young people, although there is some evidence that it may also be suitable for children with intellectual disability (ID). The Developmental Behaviour Checklist – Parent version (DBC-P) is a measure of emotional and behavioural problems that was specifically designed for children and adolescents with an ID. The DBC-P cut-off has high agreement with clinical diagnosis. The aim of this study was to estimate the relationship between DBC-P and SDQ scores in a sample of children with ID. Method Parents of 83 young people with ID aged 4–17 years completed the parent versions of the SDQ and the DBC-P. We evaluated the concurrent validity of the SDQ and DBC-P total scores, and the agreement between the DBC-P cut-off and the SDQ cut-offs for ‘borderline’ and ‘abnormal’ behaviour. Results The SDQ total difficulties score correlated well with the DBC-P total behaviour problem score. Agreement between the SDQ borderline cut-off and the DBC-P cut-off for abnormality was high (83%), but was lower for the SDQ abnormal cut-off (75%). Positive agreement between the DBC-P and the SDQ borderline cut-off was also high, with the SDQ borderline cut-off identifying 86% of those who met the DBC-P criterion. Negative agreement was weaker, with the SDQ borderline cut-off identifying only 79% of the participants who did not meet the DBC-P cut-off. Conclusion The SDQ borderline cut-off has some validity as a measure of overall levels of behavioural and emotional problems in young people with ID, and may be useful in epidemiological studies that include participants with and without ID. However, where it is important to focus on behavioural profiles in children with ID, a specialised ID instrument with established psychometric properties, such as the DBC-P, may provide more reliable and valid information. [ABSTRACT FROM AUTHOR]

This study describes the profile of repetitive behaviour in individuals with Williams syndrome, utilising cross-syndrome comparisons with people with Prader-Willi and Down syndromes. The Repetitive Behaviour Questionnaire was administered to caregivers of adults with Williams (n = 96), Prader-Willi (n = 103) and Down (n = 78) syndromes. There were few group differences, although participants with Williams syndrome were more likely to show body stereotypies. Individuals with Williams syndrome also showed more hoarding and less tidying behaviours than those with Down syndrome. IQ and adaptive ability were negatively associated with repetitive questioning in people with Williams syndrome. The profile of repetitive behaviour amongst individuals with Williams syndrome was similar to the comparison syndromes. The cognitive mechanisms underlying these behaviours in genetic syndromes warrant further investigation. [ABSTRACT FROM AUTHOR]

Individuals with specific genetic syndromes associated with intellectual disability (ID), such as Williams syndrome (WS), are at increased risk for developing anxiety disorders. A systematic literature review identified sixteen WS papers that could generate pooled prevalence estimates of anxiety disorders for WS. A meta-analysis compared these estimates with prevalence estimates for the heterogeneous ID population and the general population. Estimated rates of anxiety disorders in WS were high. WS individuals were four times more likely to experience anxiety than individuals with ID, and the risk was also heightened compared to the general population. The results provide further evidence of an unusual profile of high anxiety in WS. [ABSTRACT FROM AUTHOR]

Background Service responses to behaviour phenotypes include care by expert clinicians, syndrome-specific clinics, disability-specific mental health services and generic mental health services. While these services contribute to care, they are often of limited accessibility. Methods We describe a population-wide public health intervention aimed at increasing the accessibility of services to the target population. Stepping Stones Triple P (SSTP) is a public health intervention of known efficacy in reducing behaviour problems when delivered to parents of children aged 0-12 with mixed developmental disabilities. Results The strategy we discuss involves enhancing SSTP with modules for specific causes of developmental disabilities including Down, Fetal Alcohol, Fragile X, Prader-Willi and Williams syndromes. Conclusions We propose that enhancing SSTP with syndrome specific modules will increase the accessibility of support to families who have a child with a specific behaviour phenotype. We suggest that future research should confirm the public health impact of the modified SSTP programme using the RE-AIM framework. [ABSTRACT FROM AUTHOR]

Background Recent studies have indicated an increased risk of autism, behavioural and emotional problems and attention-deficit/hyperactivity disorder in individuals with Down syndrome. Method In a large-scale survey-based study, we examined the rates of these problems and their relationship to age and gender, in a sample of 674 individuals (4-18 years) with Down syndrome. The relationship with IQ level was also explored in a subsample ( n = 175). The Strengths and Difficulties Questionnaire and the Social Communication Questionnaire were used to assess behavioural and emotional problems and autism traits. Results On the Strengths and Difficulties Questionnaire, peer problems were the most frequently reported difficulty (48% > cut-off), followed by hyperactivity/inattention (34% > cut-off). On the Social Communication Questionnaire, 37% scored at or above cut-off (≥15) for autism spectrum disorder; 17% were at or above the suggested cut-off (≥22) for autism. Little association between age and behavioural or emotional problems or with severity of autistic symptomatology was found. However, peer problems were more common in adolescents than in junior school children ( P < 0.001); Hyperactivity/inattention was less prevalent among adolescents ( P < 0.001). Conclusions High rates of autistic features, emotional and behavioural problems are documented. These problems are related to age, gender and degree of intellectual disability. [ABSTRACT FROM AUTHOR]

Background Recent research suggests that around 16% to 18% of children with Down syndrome (DS) also meet diagnostic criteria for autism spectrum disorder (ASD). However, there are indications that profiles of autism symptoms in this group may vary from those typically described in children with ASD. Method Rates of autism symptoms and emotional and behavioural problems among children with DS who screened positive for ASD on the Social Communication Questionnaire (SCQ) (n = 183) were compared with a group of children with clinical diagnoses of ASD (n = 189) attending specialist schools in the UK. Groups were matched for age and approximate language level (use of phrase speech). Results Profiles of autistic symptoms in the two groups were generally similar, but children with DS meeting ASD cut-off on the SCQ tended to show fewer problems in reciprocal social interaction than those in the ASD group. They also showed slightly lower rates of emotional and peer-related problems. The results mostly confirm findings from a previous study in which the original validation sample for the SCQ was used as a comparison group. Conclusion Findings suggest that children with DS who meet screening criteria for ASD show similar profiles of communication and repetitive behaviours to those typically described in autism. However, they tend to have relatively milder social difficulties. It is important that clinicians are aware of this difference if children with DS and ASD are to be correctly diagnosed and eligible for specialist intervention and education services. [ABSTRACT FROM AUTHOR]

Background Formal IQ tests are an important part of the diagnostic and needs-based assessment process for children with neurodevelopmental disorders. However, resources for such assessments are not always available. It has been suggested that parental estimates of their child's developmental age could serve as a proxy IQ when formal measures are unavailable. Method Parental estimates of their child's developmental age were converted to a developmental quotient (DQ) in 197 children with Autism Spectrum Disorder (ASD) aged 4-9 years, and 108 children with ADHD and intellectual disability (ADHD + ID) aged 7-15 years. Formal IQ assessments were then conducted. Parents completed the Social Communication Questionnaire ((SCQ), a measure of autism symptomatology) and a demographic questionnaire. Results In the ASD sample, 58% of parent estimates were within 15 points (i.e. one standard deviation) of the child's measured IQ score. Lower measured IQ and lower SCQ total score predicted higher parental accuracy. In the ADHD + ID sample, 74% of parental estimates were within 15 points of measured IQ. In this group, higher child IQ predicted greater parental accuracy. Parents in the ADHD + ID group were more likely to overestimate children's ability level than parents in the ASD group. Conclusions In this study, the majority of parents of children with ADHD and ID were able to estimate their child's intellectual ability level with some accuracy. Parents of children with ASD were less accurate, but this may be because these parents were focussing more on children's level of adaptive functioning, which is known to be typically lower than cognitive ability in ASD. [ABSTRACT FROM AUTHOR]

The current study focuses on mothers and fathers of children with three rare genetic syndromes that are relatively unexplored in terms of family experience: Angelman syndrome, Cornelia de Lange syndrome and Cri du Chat syndrome. Parents of children with Angelman syndrome ( n = 15), Cornelia de Lange syndrome ( n = 16) and Cri du Chat syndrome ( n = 18), and a matched comparison group of parents of children with autism and intellectual disabilities ( n = 20) completed questionnaires on both psychological distress (stress, anxiety, depression) and positive psychological functioning. Parents of children with Angelman syndrome consistently reported the highest levels of psychological distress, and parents of children with Cornelia de Lange syndrome the lowest, with parents of children with Cri du Chat syndrome and autism scoring between these two. Positive psychological functioning was similar across the four aetiology groups. Parents of children with rare genetic syndromes are at risk for high levels of stress and mental health problems. Methodological issues and the practical applications of these results are discussed. [ABSTRACT FROM AUTHOR]

There are few well validated brief measures that can be used to assess the general progress of young children with autism spectrum disorders (ASD) over time. In the present study, the Autism Treatment Evaluation Checklist (ATEC) was used as part of a comprehensive assessment battery to monitor the progress of 22 school-aged children with ASD who had previously taken part in intensive home- or school-based intervention programmes in their pre-school years. Parents completed the ATEC when the children were on average 5.5 years and then again 5-6 years later (mean age 10.4 years). Standardised measures were also used to assess cognitive, language and adaptive behaviour skills and severity of autism symptoms over the same period. The ATEC had high internal consistency at both time points. ATEC total and sub-scale scores remained relatively stable over time and were highly and significantly correlated with cognitive, language and adaptive behaviour skills and severity of autism symptoms at both assessment points. Initial ATEC total scores predicted 64% of the variance in scores at the subsequent follow-up. However, there was also considerable variation in the patterns of scores shown by individual children over time. This study provides some preliminary evidence of the ATEC's potential value for monitoring progress of children with ASD over time. Its advantages and limitations are discussed in the context of the need systematically to monitor the progress of children with ASD over time or in response to intervention. [ABSTRACT FROM AUTHOR]

Background An emerging literature on behavioural phenotypes has highlighted apparent associations between autism spectrum disorders (ASDs) or ASD-related phenomenology and a number of different genetically determined syndromes. Method A systematic review of the current literature regarding the association with ASD and ASD characteristics was conducted in the following syndrome groups: Fragile X, Rett, Tuberous Sclerosis Complex, Down, Angelman, CHARGE and Phenylketonuria. Specific consideration was given to the role of intellectual disability in assessing the association between ASD and these syndrome groups. Results The review highlights that while formal diagnostic assessments may indicate an association between ASD and specific syndrome groups, detailed investigation has revealed subtle but qualitative differences in the presentation of ASD-like phenomenology in particular syndrome groups. The degree of ID of the individual clearly has a role to play with regard to the development and presentation of ASD-like characteristics, and caution should be taken when assessing ASD symptomatology in genetically determined syndromes associated with severe ID. However, degree of ID cannot solely account for the heightened prevalence of ASD characteristics in some specific syndrome groups. Conclusions There is a need for caution in interpreting the significance of superficial similarities between ASD and the behavioural phenotypes of certain genetically determined syndromes. However, recognition of ASD-like characteristics (even where a true diagnosis of ASD may not be relevant) in individuals with genetic syndromes is crucial in ensuring that individuals receive appropriate behavioural management and educational placement. Further research in this field requires fine-grained investigation of behavioural phenomenology within individual syndrome groups. [ABSTRACT FROM AUTHOR]

Recent reviews highlight limitations in the evidence base for early interventions for children with autism. We conducted a systematic review of controlled studies of early intensive behavioral interventions (EIBI) for young children with autism. Eleven studies met inclusion criteria (including two randomized controlled trials). At group level, EIBI resulted in improved outcomes (primarily measured by IQ) compared to comparison groups. At an individual level, however, there was considerable variability in outcome, with some evidence that initial IQ (but not age) was related to progress. This review provides evidence for the effectiveness of EIBI for some, but not all, preschool children with autism. [ABSTRACT FROM AUTHOR]

This study examined the stability of scores on the ADI-R from pre-school to elementary school age in children with autism spectrum disorders (ASD). Participants were 35 children who, at T1, all had a clinical diagnosis of ASD. On initial assessment (mean age 3.5 years; SD 0.6 years), all met ADI-R algorithm criteria for autism. ADI-R assessments were repeated at follow up (FU; mean age 10.5 years; SD 0.8 years). Changes in ADI-R total, domain and ADI-R algorithm item scores were assessed. Twenty-eight children continued to score above the ADI-R cut-off for autism at FU, although significant decreases in ADI-R domain and item scores were also found. In conclusion while classification of children according to ADI-R criteria generally remained stable between pre-school and elementary school age, many children demonstrated significant improvements in symptom severity. [ABSTRACT FROM AUTHOR]

Background Although there has been considerable research into the genotype and phenotype of Williams syndrome, there have been relatively few studies of long-term prognosis. As a preliminary to a more detailed investigation of adults with Williams syndrome, a parental questionnaire was distributed to members of the UK Williams Syndrome Foundation, focusing on areas of skill and difficulties in adult life. Method A postal questionnaire was distributed to all members of the UK Williams Syndrome Foundation who had a son or daughter aged 18 years or over. The questionnaire covered issues related to the physical and mental health of the adults with Williams syndrome, together with questions about educational and employment status, self-help and independence skills. Results Out of a total of 290 questionnaires distributed, 239 were returned (82% response rate). The data highlighted the continuing high rates of physical problems in this group and apparent increases in rates of mental health problems with age. Parents also expressed their concerns about the lack of adequate support and care. Educational and employment attainments were generally low and self-help skills were relatively poor. Discussion The findings highlight the need for far better understanding of conditions such as Williams syndrome among professionals working in adult services and the need for more adequate support from educational, health and social services. [ABSTRACT FROM AUTHOR]

Karpf J, Turk J, Howlin P. Cognitive, language, and adaptive behavior profiles in individuals with a diagnosis of Cohen syndrome. Although the physical characteristics of Cohen syndrome have been studied in considerable detail, data on other aspects of development are relatively limited. We report findings on cognitive, linguistic, and adaptive profiles in a group of 45 individuals clinically diagnosed with Cohen syndrome when aged between 4 and 49 years. The profile of skills observed was consistent with other recent findings. Thus, independence levels generally were poor, but socialization skills as assessed by the Vineland were relatively less impaired. This particular area of strength probably underlies the ‘sociable’ temperament typically associated with Cohen syndrome. However, the range of cognitive ability was wider than reported in most previous research, raising the issue of whether mental retardation should be considered as a necessary component of the phenotype. The implications for genetic testing are discussed. [ABSTRACT FROM AUTHOR]

A pilot study was conducted to evaluate the effects of training teachers of children with autistic spectrum disorders (ASDs) in the use of the Picture Exchange Communication System (PECS). Thirty-four children with ASDs (29 boys and 5 girls) were selected from eight specialist schools. Teaching staff attended a 2 day PECS workshop and received six half-day visits from PECS consultants. Data on the children's use of PECS, spontaneous communication, and adaptive behaviour were collected before the study and at set times following the workshop. Significant, rapid increases were recorded in the level of PECS attained by the children, in their PECS vocabulary, and in their frequency of PECS use over time. Improvements in children's general level of communication were slower to occur. The majority of participants showed improvements in their ability to use PECS. The results are discussed in relation to the methodological and practical difficulties that arose during the project. [ABSTRACT FROM AUTHOR]

Background: In recent years there has been an upsurge of interest in the study of distinctive patterns of behavioural and psychological characteristics associated with specific, biologically determined, intellectually disabling conditions. This study investigates whether such a profile can be identified in adults with Williams syndrome.Method: Parents and other care-givers were interviewed about the social, emotional and behavioural characteristics of 70 adults with Williams syndrome, aged 19 years to 39 years 9 months.Results: The adults were reported to have high rates of behavioural and emotional difficulties, particularly in terms of poor social relationships, over-friendliness and social disinhibition, preoccupations and obsessions, and high levels of anxiety and distractibility.Conclusions: The findings provide preliminary support for the existence of a specific pattern of behavioural and personality characteristics and associated difficulties in adults with Williams syndrome, which persist from childhood and often require intervention from mental health professionals. Implications for clinical practice are considered. [ABSTRACT FROM AUTHOR]

A study of the adjustment difficulties and needs of 70 adults with Williams syndrome found that the majority continued to live at home and remained heavily dependent on their families for their self-care. Twenty-nine families (41.4%) had had no contact with a social worker in the preceding 2 years, and 34 out of the 48 families whose children still lived at home (70.8%) had no access to respite care. Advice regarding benefits, and appropriate living and occupational arrangements for the adults was also patchy. Despite progressive medical problems, and high rates of behavioural and emotional difficulties, only 20 adults (29%) were receiving regular health checks, while 21 (30%) had had some contact with a mental health service in the preceding 2 years. In the majority of cases, families continued to shoulder the main burden of care for their sons and daughters with Williams syndrome well into adulthood, with little support from statutory and voluntary agencies. The implications of these findings are considered with regard to the principles of community care. [ABSTRACT FROM AUTHOR]

Many different treatments have been claimed to have a dramatic impact on children with autism. This paper reviews what is known about the outcome in adult life and examines the limitations and advantages of a variety of intervention approaches. It concludes that there is little evidence of any “cure” for autism, but appropriately structured programmes for education and management in the early years can play a significant role in enhancing functioning in later life. [ABSTRACT FROM AUTHOR]

Many different treatments have been claimed to have a dramatic impact on children with autism. This paper reviews what is known about the outcome in adult life and examines the limitations and advantages of a variety of intervention approaches. It concludes that there is little evidence of any 'cure' for autism, but appropriately structured programmes for education and management in the early years can play a significant role in enhancing functioning in later life. [ABSTRACT FROM AUTHOR]

Assesses the long-term effects of abuse on a group of children who had attended a specialist autistic school. Documentation of physical and emotional mistreatment of pupils; Differential changes in the patterns of children's behavior as reported by parents; Impact of the school regime on children's development.

Although it is now recognized that children may exhibit widespread symptoms of stress following exposure to abuse or neglect, the impact of traumatic experiences on children with pervasive developmental disorders has received little attention. The present paper describes a strategy devised to assess the long-term effects of abuse on a group of children who had attended a specialist autistic school where physical and emotional mistreatment of pupils was well documented. Because most of the children had very limited communication skills, the evidence relied heavily on retrospective parental reports. Despite the problems inherent in using such data it was possible to derive reliable measures that could be used to test hypotheses about predicted patterns of behavioral change, based on work with other children who have been abused. In most cases, too, parental reports could be corroborated by information from other sources. Consistent patterns in both the nature and timing of behavioral disturbances were found, which seemed to relate specifically to the period of abuse at the school. [ABSTRACT FROM AUTHOR]

Until recently, intervention research for young children with autism has been strongly criticized because of the paucity of well controlled comparative studies and particularly the lack of randomized control trials (RCT’s). Over the last decade, however, there has been a steady improvement of the quality of research in this area. RCT’s, although still relatively rare, have increased in number, and have been used to examine the effectiveness of a range of different treatment approaches, including parent management style; early, intensive behavioural interventions; social communication, joint attention and play programmes, and augmentative communication systems. Well controlled trials, involving closely matched groups of children have also added to our knowledge of treatment effects. The present paper will focus on a series of recent studies, utilizing both randomized and matched case-control comparison designs. Overall, group results indicate moderate, but encouraging improvements for children involved in each of these interventions. However, at an individual child level, outcome is much more variable, with some children in each programme showing considerable change, others only minor improvements and some showing no improvement, or even deterioration over time. Identification of the children who respond most to specific types of intervention remains a major challenge for research in this field in the future. [ABSTRACT FROM AUTHOR]

Background: Little is known about sleep architecture in Angelman (AS), Cri du Chat (CdC) and Cornelia de Lange (CdLS) syndromes although sleep problems are commonly reported by parents of children with AS and CdC syndromes. Given the broad similarity of these syndromes we compared sleep architecture and parental reports of sleep problems across the syndromes. Method: Actigraph data were collected on children with AS, CdC or CdLS syndromes aged between 4 and 19 years (AS, mean age 6.67 years; CdC, mean age 9.13 years and CdLS, mean age 9.75 years) over 4 to 8 days (mean 6.4). Two children with AS were currently taking melatonin for sleep problems. A questionnaire regarding sleep (Simonds & Parraga, 1982) was completed by caregivers. Results: The CdLS group slept for longer than the AS and CdC groups (CdLS median: 8.31 hours, range: 7.1–9.3; AS median: 7.13 hours, range: 5.04–9.37; CdC median: 6.98 hours, range: 2.35–8.29) and showed less activity during the night than the AS and CdC groups (CdLS median: 18.79, range: 8.78–29.5; AS median: 38.15, range: 17.99–179.53; CdC median: 27.54, range: 15.63–358.11). Across the groups, 75% of parents reported that their child had a sleep problem and those children whose parents reported that they had sleep problems were awake for longer during the night than those without a sleep problem and this difference approached significance (U = 3.00, N = 12, p = 0.052). Parents of children with CdLS were less likely to report a sleeping problem than parents in the other two groups (CdLS: 33.3%, AS: 100%, CdC: 83.3%). Conclusion: Children with CdLS appear to demonstrate fewer sleep problems than children with AS and CdC and show consistent patterns of sleep within the group whilst the AS and CdC groups are noticeably more varied. Of clinical interest is the finding that two children with CdC were sleeping for a mean of 2.35 and 3.58 hours per night. [ABSTRACT FROM AUTHOR]

Background: Individuals with Williams syndrome (WS) are reported to experience a range of physical and mental health problems. However, much of this information is derived from children and young adults. Little is known about these issues in older adults. The aims of this study were to (1) examine these issues in adults with WS of a wide age range and (2) examine the long term pattern of these problems. Method: Two groups of adults with WS and their parents/carers were interviewed to examine physical and mental health problems. Study 1 comprised 92 adults with WS (19 years 11 month–55 years 4 months). Study 2 consisted of 47 adults with WS (25 years 7 months–49 years 7 months) who had taken part in a previous study of adults with WS 10 years previously. Results: The studies indicated that high numbers of adults with WS (75%–81%) had physical health problems that impacted upon their everyday life. These included heart, bowel and blood pressure problems (18%–33%). Eating, sleeping and toileting problems were reported in 7%–16% of the sample. Mental health problems were common (25%–33%). There were increases over time in the number of individuals with health problems relating to the heart, bowel and blood pressure and a small decrease in the numbers with joint problems, hernias and dental problems. Overall, there was a decrease in the number of participants with emotional/behavioural problems. Specifically, rates of problems such as anxiety and compulsions decreased, but rates of depression and overactivity increased. Conclusions: Adults with WS have a range of physical and mental problems. While the overall number of individuals with physical health problems remained high over time, the rates of different types of problems changed. Emotional/behavioural problems generally decreased with age. [ABSTRACT FROM AUTHOR]

The question of whether Asperger syndrome and high-functioning autism should be considered as the same or different conditions has been a source of debate and controversy over recent years. In the present study, 34 adults with autism who had shown early delays in language were compared with 42 individuals who were reported to have had no such delays, either in their use of words or phrases. All participants were at least 18 years of age, had a nonverbal IQ of 70 or above and met ADI-R criteria for age of onset, communication and social impairments, and stereotyped behaviors. Those in the language delay group were diagnosed as having high-functioning autism. The remainder were designated as having Asperger syndrome. The groups were matched for age, nonverbal IQ and gender. No significant differences were found between the groups either in their total ADI-R algorithm scores, or in their algorithm scores on individual domains. Social outcome ratings and ADI-R scores based on current functioning also failed to differentiate between the groups. Scores on tests of language comprehension and expression were also similar, but in both groups language abilities were well below chronological age level. The implications of these results with respect to the differences between Asperger syndrome and high-functioning autism are discussed. The poor performance on language tests also challenges the assumption that early language development in Asperger syndrome is essentially normal. [ABSTRACT FROM AUTHOR]