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PubMed2003

Repair behavior in children with intellectual impairments: evidence for metalinguistic competence.

This paper tested the prediction that children with cognitive impairments who can use language intentionally will be able to carry out the metalinguistic operations involved in speech monitoring and repair. The specific linguistic characteristics of responses to requests for clarification given by 4 children with lower than normal IQ, ages 3 years 5 months to 6 years 10 months, were investigated. The analysis focused on children's ability to locate the specific errors that provoked neutral requests for clarification and produce repair. Three children could locate their errors and partly succeed in providing repair. It is suggested that ability to perform metaprocedures such as are implicated in repair behavior may be preserved in children with intellectual disabilities and that this ability does not implicate conscious awareness, nor does it depend on mature linguistic competence.

Child↗
PubMed2004

Assessment of phonemic awareness and word reading skills of people with complex communication needs.

A series of phonemic awareness (PA) and single-word reading tasks, which did not require spoken responses, was developed for administration to people with complex communication needs. The aims of the study were to (a) determine the construct validity of the PA tasks and (b) investigate the relationship between PA and single-word reading in adults with complex communication needs. Forty adults with physical and/or intellectual disability were administered these tasks and a standardized measure of receptive spoken vocabulary. In assessing construct validity, data from all participants, including those who used speech, were included in a factor analysis, which indicated that the PA tasks loaded onto a single factor. This factor was interpreted to be PA. The relationship between PA and single-word reading in adults with complex communication needs was determined using correlational and multiple regression analyses of data from 34 of the original participants who did not have functional speech skills. These analyses indicated that receptive spoken vocabulary accounted for a significant amount of variance on most tasks. Additional significant variance in performance on the single-word reading tasks was accounted for by performance on the PA tasks, in particular, Nonword Blending and Phoneme Analysis. These results indicate that the tasks developed provide a valid means of assessing PA and single-word reading skills. In addition, the results indicate that adults with complex communication needs demonstrate the same positive association between PA and reading as has been found in other groups of individuals with and without disability.

Adult↗
PubMed2000

Anaesthesia and pain management in cerebral palsy.

Cerebral palsy is the result of an injury to the developing brain during the antenatal, perinatal or postnatal period. Clinical manifestations relate to the area affected. Some of the conditions associated with cerebral palsy require surgical intervention. Problems during the peri-operative period may include hypothermia, nausea and vomiting and muscle spasm. Peri-operative seizure control, respiratory function and gastro-oesophageal reflux also require consideration. Intellectual disability is common and, in those affected, may range from mild to severe. These children should be handled with sensitivity as communication disorders and sensory deficits may mask mild or normal intellect. They should be accompanied by their carers at induction and in the recovery room as they usually know how best to communicate with them. Postoperative pain management and the prevention of muscle spasm is important and some of the drugs used in the management of spasm such as baclofen and botulinum toxin are discussed. Epidural analgesia is particularly valuable when major orthopaedic procedures are performed.

Analgesia↗
PubMed1998

Affective psychosis and Prader-Willi syndrome.

Previous studies have demonstrated that maladaptive behaviours are common amongst adults with Prader-Willi syndrome (PWS). Case reports have also previously demonstrated that psychosis can occur amongst adults with PWS. The present study was undertaken in order to gain a better understanding of the psychopathology of the psychosis of PWS. Twenty-three out of 25 adults identified with PWS living in Northamptonshire, UK, agreed to participate. Comprehensive psychiatric assessments (using the PPS-LD), and measures of adaptive and maladaptive behaviours (using the AAMR-ABS) were completed. Comparisons were made for the prevalence of psychiatric disorders against those from a previous epidemiological study of adults with intellectual disability of other aetiologies from a neighbouring county. The PWS group was found to have higher rates of affective disorders (a point prevalence of 17.4%), in which psychotic symptoms were common, but similar rates of schizophrenia/delusional disorders (4.3%) compared with the comparison group. Behaviour disorders were also common. Surprisingly, none of the PWS group was found to have generalized anxiety or phobic disorders. The diagnostic criteria for the episodes including psychotic symptoms are explored. The high rates of affective disorders is of clinical (i.e. treatment) importance as well as being of academic interest with regard to the genetics of psychiatric disorders.

Adult↗
PubMed1998

The latent variable structure of the Compulsive Behaviour Checklist in people with Prader-Willi syndrome.

The presence and severity of compulsive behaviours may be evaluated via the Compulsive Behaviour Checklist (CBC) and this instrument has been successfully employed in people with intellectual disability. However, the applicability of the overall CBC scoring system, which entails tallying the number of behavioural categories represented (i.e. five) as well as the number of individual behaviours endorsed (i.e. 25), is not known in the population with Prader-Willi syndrome (PWS). The present investigation examined the latent variable structure of the CBC in people with PWS in order to identify possible population-specific scoring and interpretation considerations. The 25 behaviour-specific items of the CBC were analysed for 75 people with PWS (44 females and 31 males) aged between 4 and 41 years (mean +/- SD = 11.4+/-9.4) via factor analysis with principal component extraction and equamax rotation. The most suitable solution was determined on the basis of multiple empirical criteria: (1) the scree test; (2) eigenvalues >1.00; (3) salient loadings >0.30; (4) the clarity of item assignment to a single latent dimension; (5) the internal consistency of the latent dimension(s) (coefficient alpha > or = 0.70); and (6) item-total correlations between 0.20 and 0.79. In addition, solutions were examined with respect to psychological theory and previous research. A 'general factor' (i.e. single latent dimension) solution which adhered to all a priori criteria was indicated. Twenty-four out of 25 items achieved salient loadings ranging from 0.46 to 0.80 on the general factor. The single item which failed to achieve salience, 'deviant grooming-skin picking', exhibited both substantial unique variance (0.997) and moderate reliability (r = 0.59, P<0.001). The internal consistency of the general factor was strong (alpha = 0.93) and all salient items were suitably correlated with the unit-weighted total score (r(item-total) = 0.41-0.77). The traditional CBC scoring system, which includes tallying the number of categories represented, would not be relevant in this PWS sample. In addition, the recommended tallying of the number of individual behaviours endorsed does not reflect the empirically indicated notion of compulsive behaviour in this special population. These findings indicate that the 24 salient items should be scored as a unit-weighted composite and that the score on the substantially unique item (skin picking) should be considered a separate measure when evaluating compulsive behaviours via the CBC in people with PWS.

Adolescent↗
PubMed1998

Distinctiveness and correlates of maladaptive behaviour in children and adolescents with Smith-Magenis syndrome.

This two-part study examines the distinctiveness and correlates of maladaptive behaviour in 35 children and adolescents with Smith-Magenis syndrome, a developmental disorder caused by an interstitial deletion of chromosome 17 (p11.2). Study I compares Child Behavior Checklist scores in 35 children with Smith-Magenis syndrome to age- and gender-matched subjects with Prader-Willi syndrome and mixed intellectual disability. Subjects with Smith-Magenis syndrome had significantly higher levels of maladaptive behaviour than the other groups. Although some problems were shared across groups, 12 behaviours differentiated the three groups with 100% accuracy. Study 2 assessed the frequency and correlates of self-injurious and stereotypical behaviours, including unusual features such as nail-yanking, inserting objects into bodily orifices, self-hugging and a 'lick-and flip' behaviour. Nail-yanking and bodily insertions were less common than other types of self-injury, and self-hugs and the 'lick-and flip' stereotypies were seen in about half the sample. Although age and degree of delay were correlated with problem behaviours, sleep disturbance emerged as the strongest predictor of maladaptive behaviour. The implications are discussed for clinical diagnostic ambiguities between the Smith-Magenis and Prader-Willi syndromes, and for intervention.

Adolescent↗
PubMed1999

Mortality in Down's syndrome in relation to congenital malformations.

Down's syndrome (DS) is the most common form of intellectual disability. The syndrome is characterized by congenital malformations, especially of the heart and gastrointestinal tract, which can result in high mortality rates in the affected population. Many improvements have been made in the medical treatment of this syndrome during the past few decades and the survival of individuals with DS has increased in the industrial world. The aim of the present study was to investigate mortality in relation to congenital malformations. Medical records from all liveborn children with DS delivered between 1973 and 1980 in northern Sweden were studied, and malformations and causes of death were recorded. Out of the 219 children included in the study, a congenital heart defect was reported in 47.5% of subjects, 42.1% of whom had complete atrioventricular septal defect. Gastrointestinal tract malformations were present in 7.3% of subjects, and was frequently associated with a cardiac malformation and a very high mortality rate. Other major and minor congenital anomalies were present in 5.5% and 5.5% of subjects, respectively. In the 14.5-year follow-up of 213 children, the rate of survival was 75.6%. Mortality rates within one and 10 years after birth were 14.6% and 23.5%, respectively. Mortality within 10 years differed significantly between children with (44.1%) and without (4.5%) a congenital heart defect. A very high mortality rate was observed among children with a congenital heart defect, especially when it was combined with a gastrointestinal malformation.

Adolescent↗
PubMed1999

Fragile-X syndrome, Down's syndrome and autism: awareness and knowledge amongst special educators.

Fragile-X syndrome is the commonest cause of inherited intellectual disability. There is good evidence for a behavioural phenotype. This has implications for school staff using standard educational techniques. Similarly, autism is known to create particular educational requirements. The present study examined the awareness and knowledge of fragile-X syndrome, Down's syndrome and autism amongst staff in special and mainstream education. One hundred and two special school staff and 40 mainstream school staff completed questionnaires. Most staff offered a variety of features 'typical' of Down's syndrome and autism. In contrast, staff knew less about fragile-X syndrome. Specific knowledge about the learning styles of these children was very poor, but was associated with having taught an affected child. Mainstream and special school staff offered similar levels of knowledge for all three conditions. Staff did not demonstrate a sufficiently specialized knowledge of fragile-X syndrome to ensure that the special educational needs of these children were being met fully.

Adult↗
PubMed1999

Diagnosis of fragile-X syndrome: the experiences of parents.

In order to assess some aspects of the quality of care for families seeking the cause of their child(ren)s intellectual disability, a postal questionnaire was sent to parents of children with fragile-X syndrome, who were members of the UK Fragile-X Society. Although the interval taken to get a diagnosis ('lagtime') has fallen over time, other aspects of care could still be improved. Most families feel that having a diagnosis is an advantage, but many still find the diagnostic process distressing and feel unsupported. Not all families are referred for genetic counselling, and even those who are do not always understand or retain the information given. Most families feel that having a diagnosis is a benefit rather than a disadvantage.

Child↗
PubMed2000

Sequence of cognitive decline in dementia in adults with Down's syndrome.

Adults with Down's syndrome (DS) are known to be at risk of dementia of the Alzheimer type (DAT), but because of their lifelong intellectual deficits, it is difficult to determine the earliest signs and characteristics of age-associated decline and dementia. In a longitudinal study in which all participants were healthy at the time of their entry into the study, the present authors compared the amount of decline on the subtests of the WISC-R to determine the sequence of cognitive decline associated with varying stages of dementia. Twenty-two individuals with varying degrees of cognitive decline were compared to 44 adults with DS who have remained healthy. All participants functioned in the mild or moderate range of intellectual disability at initial testing. On each subtest of the WISC-R, the amount of change experienced by the healthy participants over the study period was compared to the amount of change found for each of the groups with decline. Out of the individuals who showed declines, 10 adults with DS were classified as having 'questionable' decline based on the presence of memory impairment, and five and seven adults with DS were classified as in the 'early stage' and 'middle stage' of DAT, respectively, based on the presence of memory impairment, score on the Dementia Scale for Down Syndrome and a physician's diagnosis. It was found that participants who were identified as 'questionable', in addition to the memory loss that determined their classification, also showed significant declines on the Block Design and Coding subtests. The five adults in the early stage of dementia showed declines on these subtests, and in addition, on the Object Assembly, Picture Completion, Arithmetic and Comprehension subtests. The seven adults in the middle stage of dementia showed declines on these subtests, plus declines on Information, Vocabulary and Digit Span subtests. The Picture Arrangement and Similarities subtests were not useful in distinguishing between the groups because of baseline floor effects for a substantial proportion of participants. The present longitudinal study showed a sequence of cognitive decline associated with DAT, beginning with a possible 'pre-clinical' stage, and progressing through the early and middle stages. This approach begins to define the sequence of declining cognitive capacities that contributes to the observed functional deterioration caused by Alzheimer's disease and that is likely to reflect the involvement of cortical areas as the disease progresses.

Adult↗
PubMed2001

Treating food refusal in a child with Williams syndrome using the parent as therapist in the home setting.

The present authors examined the effectiveness of a behavioural intervention which included escape extinction and differential reinforcement of each bite eaten to treat non-organic food refusal in a child with Williams syndrome. The intervention was implemented by the child's mother in the home during normal meal schedules. The child was not allowed to leave the meal situation for a predetermined time period and was praised by the mother for each bite consumed. The intervention was evaluated using a multiple baseline design across meals (i.e. breakfast and lunch). The results demonstrate an increase in food consumed and decreases in other inappropriate behaviours. The mother continued to implement the treatment successfully during follow-up assessments up to 3 months after the intervention. This is a minimally intrusive intervention in comparison to typical treatments for non-organic food refusal in children with intellectual disabilities.

Behavior Therapy↗
PubMed2001

Working memory and everyday cognition in adults with Down's syndrome.

A number of previous studies have suggested that young people with Down's syndrome (DS) have a specific deficit of the phonological loop component of the working memory. However, there have also been studies which have proposed a specific deficit of the central executive component of working memory and suggested similarities of working memory functioning with patients with Alzheimer's disease. Fifteen middle-aged people with DS were matched for their individual scores of non-verbal intelligence to 15 individuals with mixed aetiology of intellectual disability. A versatile range of tasks was used in order to evaluate the functioning of working memory components. In addition, several everyday cognition skills were assessed. The subjects with DS performed significantly more poorly in all tasks assessing the phonological loop. Performance in other working memory tasks and compound variables representing different working memory components was equal in the groups. In addition, both groups had equal everyday cognition skills. The functioning of the phonological loop seems to be clearly deficient in people with DS. Interestingly, the deficit does not seem to affect the vocabulary or other everyday cognition skills in individuals with DS. No signs of specific deficit of the central executive component of working memory were found.

Activities of Daily Living↗
PubMed2001

Alzheimer dementia in Down's syndrome: the relevance of cognitive ability.

More years of education have been found to be associated with a lower rate of Alzheimer disease (AD) in individuals without intellectual disability. It has been proposed that education reflects greater 'synaptic reserve' and that greater synaptic reserve may defer the development of AD. The present study compared individuals with Down's syndrome (DS) who were found to have symptoms of dementia with those who remained symptom-free to determine if the two groups differed in their level of education, employment, recreational activities, years in an institution or overall level of cognitive functioning. Thirty-five adults with DS aged between 29 and 67 years were assessed. The participants were recruited from a community health facility and included individuals with a wide range of ability levels. Neuropsychological testing, caregiver report and the Dementia Scale for Down Syndrome (Gedye 1995) were used to identify decline in participants over periods of 6 months to 3 years. After the effect of age was statistically removed, multiple regression analyses revealed that level of cognitive functioning was significantly associated with decline such that a higher level of cognitive functioning predicted less decline. None of the environmental variables (i.e. educational level, years in an institution and employment) were directly associated with decline; however, a post hoc regression using level of cognitive functioning as the outcome variable revealed that level of cognitive functioning itself was associated with these environmental variables. A higher level of cognitive functioning was associated with fewer cases of dementia in individuals with DS, and level of cognitive functioning appears to be associated with environmental factors such as level of education, years in an institution and employment. The present findings suggest that environmental interventions aimed at improving level of cognitive functioning may also be useful in deferring the onset of dementia.

Adult↗
PubMed2001

Causal explanations, concern and optimism regarding self-injurious behaviour displayed by individuals with Cornelia de Lange syndrome: the parents' perspective.

Research into behaviours associated with specific syndromes, such as Cornelia de Lange syndrome (CdLS), has neglected to explore the parents' perspective, particularly the potential impact of the notion of behavioural phenotypes on parents' causal explanations. Given the research focus on self-injurious behaviour (SIB) in CdLS, the present study examined parental concern across four topographies of challenging behaviour, causal explanations for these behaviours and optimism for change. As part of a larger study, a questionnaire survey of 86 parents of children and adults with CdLS was conducted. Quantitative data on parental concern and optimism with regard to behaviour problems were collected. Causal explanations for behaviour problems were examined by subjecting open-ended responses to a content analysis. Parents were as concerned about physical aggression and disruptive behaviours as they were about SIB. The majority of parents had deconstructed how CdLS might be associated with SIB in terms of other factors associated with CdLS, such as degree of intellectual disability. Parents did not believe that CdLS influenced SIB more than other challenging behaviours and their beliefs did not effect optimism regarding future change in the behaviour. Despite the focus of research on SIB in CdLS, parents of children and adults with CdLS are also concerned about other challenging behaviours. There was no evidence that a deterministic perspective had been adopted by parents and causal explanations were unrelated to optimism for future change.

Adolescent↗
PubMed2001

Psychopathology and young people with Down's syndrome: childhood predictors and adult outcome of disorder.

There is a scarcity of follow-up studies into adult life of psychiatric disorder in young people with intellectual disability. The key aims of the present study were: (1) to determine the outcome of psychopathology present in childhood in individuals with Down's syndrome (DS); and (2) to look at childhood predictors of adult psychiatric disorder. Fifty-two young people with DS were identified from a sample of 193 subjects examined in childhood and adolescence for psychiatric and behaviour disorder. These young adults were interviewed for the presence of psychiatric disorder. No significant relationship was found between childhood mental disorder and psychiatric disorder in adult life for those individuals with DS. Early childhood factors of psychiatric disorder, challenging behaviour and family environment, except social background, did not predict adult psychopathology in young people with DS. Childhood disorder in individuals with DS has a good early prognosis with little evidence of continuity of the disorder into adult life.

Adolescent↗
PubMed2002

Changes in explicit memory associated with early dementia in adults with Down's syndrome.

BACKGROUND: A modified version of the Selective Reminding Test (SRT) (Buschke 1973) was used to examine the changes in memory that occur with early-stage dementia of the Alzheimer's type (DAT) in adults with intellectual disability (ID) and Down's syndrome (DS), and to compare these changes to those occurring with 'normal' ageing. METHOD: Hierarchical linear modelling analyses showed steep declines in the performance of participants who had met the criteria for the onset of DAT. Non-demented participants also showed declines in performance which were related to their age. However, the absolute magnitude of these declines was consistent with a 'normal' ageing pattern and not with undetected dementia. RESULTS: In analysing the specific memory components that are compromised, the present authors found that participants with early-stage DAT showed severely diminished long-term storage and retrieval processing abilities compared to their non-demented peers. Notably, these declines preceded other symptoms of dementia, in most cases by more than a full year and sometimes by as much as 3 years. CONCLUSIONS: Thus, the present results clearly confirm that memory processes are affected during early dementia in adults with DS, and that the SRT has promise as a clinical tool.

Adult↗
PubMed2002

Behavioural knowledge, causal beliefs and self-efficacy as predictors of special educators' emotional reactions to challenging behaviours.

Theoretical models and emerging empirical data suggest that the emotional reactions of staff to challenging behaviours may affect their responses to challenging behaviours and their psychological well-being. However, there have been few studies focusing on factors related to staff emotional reactions. Seventy staff working in educational environments with children with intellectual disability and/or autism completed a self-report questionnaire that measured demographic factors, behavioural causal beliefs, behavioural knowledge, perceived self-efficacy, and emotional reactions to challenging behaviours. Regression analyses revealed that behavioural causal beliefs were a positive predictor, and self-efficacy and behavioural knowledge were negative predictors of negative emotional reactions to challenging behaviours. Staff with formal qualifications also reported more negative emotional reactions. No other demographic factors emerged as significant predictors. The results suggest that behavioural causal beliefs, low self-efficacy and low behavioural knowledge may make staff vulnerable to experiencing negative emotional reactions to challenging behaviours. Researchers and clinicians need to address these issues in staff who work with people with challenging behaviours.

Adolescent↗