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PubMed2002

Involvement with peers: comparisons between young children with and without Down's syndrome.

BACKGROUND: It has been well established that heterogeneous groups of young children with mild intellectual disability are at considerable risk of becoming socially isolated from their peers in school, home and community settings. METHOD: Matched groups of young children with and without Down's syndrome (DS) were compared in terms of the children's involvement with peers, maternal arranging and monitoring of peer play, and maternal beliefs about inclusion. RESULTS: Despite aetiology-specific expectations for children with DS, no differences were found for a variety of measures of peer involvement focusing on the frequency of contacts and the characteristics of children's peer social networks. Maternal arranging of activities with peers was similarly related to peer involvement for both groups of children. Higher ratings of the benefits of inclusion were obtained from mothers of children with DS, but these maternal beliefs were unrelated to maternal arranging or peer involvement. CONCLUSIONS: Parental adaptations to the aetiology-specific behavioural patterns of children and the general influence of children's experiences within a developmental framework are discussed in the context of interpreting aetiology-specific findings.

Child↗

PubMed2002

Cued recall in early-stage dementia in adults with Down's syndrome.

BACKGROUND: Memory declines were evaluated with an adaptation of the Cued Recall Test (CRT) in 19 adults with Down's syndrome (DS) with mild or moderate intellectual disability (ID) who were at an early-stage of dementia of the Alzheimer type (DAT), and their performance was compared to peer groups of 75 adults with DS and 66 adults with ID without DS who were not suspected of functional declines. METHOD: The CRT consisted of a training period in which 12 items were presented, four at a time, with each item accompanied by a unique category cue. The testing phase consisted of three trials which generated two measures, a free recall score (FRS; spontaneous recall of the list of 12 items) and a total score (TS; FRS plus items recalled when the category cue was provided). RESULTS: It was found that a cut-off value of < or = 23 on the TS resulted in a sensitivity of 94.7% and a specificity of 93.9% with a positive predictive value of 81.9% when those individuals with DAT were compared to the participants with ID without DS. Eight of these individuals with DAT had relatively poor performance on the CRT compared to their healthy peers at a baseline when they were not suspected of functional declines, suggesting that memory declines can occur several years prior to the identification of DAT. In addition, 17 participants with DS without a diagnosis of DAT met the criterion for the cut-off score. Longitudinal data and converging measures indicated that there was the possibility that 15 of these individuals are in a 'pre-clinical' stage of decline. CONCLUSION: The usefulness of the CRT as a screening test for early memory deficits for this population needs to be confirmed by following these participants for an extended period of time and by studying an independent sample.

Aged↗

PubMed2002

Challenging behaviour and insecure attachment.

BACKGROUND: The present authors investigated a database about people with severe intellectual disability (ID) to explore whether secure/insecure attachment plays a role in challenging behaviour. METHODS: The study took the form of a survey of staff and carers involved with a cohort of 54 school-leavers with severe ID. RESULTS: Thirty-four per cent of students were rated by placement staff as 'over-investing in one or a few relationships which become a source of jealousy'. Students without such problems were significantly less likely to show challenging behaviours, while those with them were significantly more likely to be living outside the family home. CONCLUSIONS: These preliminary results justify more detailed and longitudinal research into the relationship between attachment status and challenging behaviour.

Adult↗

PubMed2002

Maladaptive behaviour in children and adolescents with Down's syndrome.

BACKGROUND: Although children with Down's syndrome (DS) are at lower risk for psychopathology than others with intellectual disability, they do show more problems than typically developing children. However, age-related trends in these problems remain unclear. METHODS: The present authors examined age-related changes in the maladaptive behaviours of 211 children and adolescents with DS aged between 4 and 19 years (mean = 9.74 years). Most participants (n = 180) were recruited from families residing in the greater Los Angeles area, California, USA, while a minority (n = 31) were patients from a clinic specializing in the psychiatric management of people with DS. The participants were divided into four age groups: (1) 4-6 years, (2) 7-9 years, (3) 10-13 years and (4) 14-19 years. RESULTS: Externalizing behaviours were lower across both the community and clinic samples, while internalizing behaviours were significantly higher in older adolescents aged between 14 and 19 years. Increases were found in withdrawal, seen in 63% of community-based adolescents, and 75% of clinic adolescents. CONCLUSIONS: Older adolescents with DS may show decreased externalizing symptoms and subtle increases in withdrawal. Possible relationships are discussed between these shifts and increased risks of later-onset depression and Alzheimer's disease in adults with DS.

Adolescent↗

PubMed2002

Behaviour problems in adult women with Rett syndrome.

BACKGROUND: Despite considerable interest in the genetic, physical and neurological aspects of Rett syndrome (RS), there have been few studies of associated behavioural and emotional features. Furthermore, few case studies or surveys have included adult women with RS. The main aim of the present study was to compare behaviour problems in a sample of women with RS against data from normative samples. METHODS: The primary carers of 50 women with RS completed the community version of the Aberrant Behavior Checklist. RESULTS: Women with RS were rated as having lower levels of irritability, hyperactivity and inappropriate speech behaviours than normative samples of adults with intellectual disability. CONCLUSIONS: A number of factors may affect the presentation of behaviour problems in women with RS (e.g. cognitive impairments or physical disabilities). Therefore, more research is needed in order to generate information about the behavioural phenotype of RS. The implications of the present data for future research are also discussed.

Adult↗

PubMed2003

Magnetic resonance imaging, Down's syndrome and Alzheimer's disease: research and clinical implications.

BACKGROUND: The diagnosis of Alzheimer's disease (AD) remains at times difficult to make using available neuropsychological measures. Neuro-imaging is a relatively new form of detecting the changes associated with dementia. The present study investigated the role of magnetic resonance imaging (MRI) in diagnosing AD in adults with Down's syndrome (DS). METHODS: Subjects with DS and Alzheimer-type dementia were matched to non-demented controls with DS. Magnetic resonance imaging findings (i.e. volumetric and two-dimensional scans) were compared between the two groups in order to show a relationship between the changes of AD and structural MRI abnormalities. RESULTS: Specific structural abnormalities which are seen in non-intellectually disabled subjects with dementia are also found in individuals with both DS and AD. However, such findings cannot be used to diagnose clinical AD with good accuracy in adults with DS. A number of practical issues of patient compliance and over-sedation are demonstrated by the findings. CONCLUSIONS: Magnetic resonance imaging has an important but limited role to play in the management of AD in the population with DS. If intravenous sedation is used, medical support is essential to prevent a serious mishap.

Adult↗

PubMed2003

The Prudhoe Cognitive Function Test, a scale to assess cognitive function in adults with Down's syndrome: inter-rater and test-retest reliability.

BACKGROUND: The Prudhoe Cognitive Function Test (PCFT) was designed to measure cognitive function in subjects with all degrees of intellectual disability (ID) and to be administered by non-specialist raters. The aim of the present study was to measure the test-retest and inter-rater reliability of the PCFT. METHODS: Three raters, one a specialist and two non-specialists, administered the PCFT to 14 subjects with Down's syndrome on two occasions separated by 4 weeks. RESULTS: The intra-class correlation coefficients (ICCs) between the rater pairs (n=3) were 0.99, 0.99 and 0.98 (P<0.01). Floor effects were recorded for two items. The ICC for test-retest reliability was 0.99 (P<0.01). CONCLUSIONS: The PCFT is a highly reliable instrument when used by either specialist or non-specialist raters. It has excellent temporal stability.

Adult↗

PubMed2003

Preliminary evaluation of a scale to assess cognitive function in adults with Down's syndrome: the Prudhoe Cognitive Function Test.

BACKGROUND: In the clinical diagnosis of dementia in Down's syndrome (DS), it may be difficult to distinguish between cognitive deterioration and the various degrees of pre-existing intellectual disability (ID). Serial measurements of both cognitive function and behaviour are required. The aim of the present study was to evaluate the performance of non-demented adults with DS on a subject-directed instrument, the Prudhoe Cognitive Function Test (PCFT), preliminary to its serial use in a prospective study. METHODS: From 1985 to 1986, 85 non-demented hospitalized adults with DS were interviewed using the PCFT. The Adaptive Behavior Scale (ABS) was administered to the carers. The subjects' levels of ID (graded from mild through moderate, severe and profound to untestable) were based on their scores on the Stanford-Binet Intelligence Scale, as reported in the medical records, and the relationship between level of disability and performance on the PCFT and ABS, and their respective domains, was examined. RESULTS: Both scales produced a wide range of scores and the correlation between them was highly significant. Both scales correlated highly significantly with the degree of ID, but more subjects with high levels (i.e. profound to untestable) of disability obtained very low or zero scores on the PCFT and its domains than on the ABS. CONCLUSIONS: The PCFT provides a reliable quantitative measure of cognitive function in subjects with DS, and could be a useful adjunct to the diagnosis of dementia in prospective studies. However, the almost uniformly low scores obtained by those with high levels of ID suggests that its power to detect cognitive decline will be limited to those who are less disabled, while the ABS may be more useful than the PCFT in detecting deterioration in people with profound ID.

Adult↗

PubMedDate not supplied

Pre-school children with and without developmental delay: behaviour problems and parenting stress over time.

BACKGROUND: Children with intellectual disability are at heightened risk for behaviour problems and diagnosed mental disorder. METHODS: The present authors studied the early manifestation and continuity of problem behaviours in 205 pre-school children with and without developmental delays. RESULTS: Behaviour problems were quite stable over the year from age 36-48 months. Children with developmental delays were rated higher on behaviour problems than their non-delayed peers, and were three times as likely to score in the clinical range. Mothers and fathers showed high agreement in their rating of child problems, especially in the delayed group. Parenting stress was also higher in the delayed group, but was related to the extent of behaviour problems rather than to the child's developmental delay. CONCLUSIONS: Over time, a transactional model fit the relationship between parenting stress and behaviour problems: high parenting stress contributed to a worsening in child behaviour problems over time, and high child behaviour problems contributed to a worsening in parenting stress. Findings for mothers and fathers were quite similar.

Adult↗

PubMedDate not supplied

Supporting families over the life course: mapping temporality.

BACKGROUND: The present paper addresses a rather neglected dimension of family caregiving, its temporality. Many accounts of caregiving assume a state of stasis, and therefore, overlook factors which shape the evolving experience of family caregiving over the life course. METHODS: The paper begins by offering some reflections on theoretical and methodological issues identified by life-course researchers. RESULTS: Based both on theoretical propositions and a growing body of empirical evidence, this paper offers a heuristic for thinking about caregiving stages applied to families supporting people with intellectual disability. CONCLUSIONS: This heuristic is used to suggest further avenues of research and development.

Caregivers↗

PubMed2003

The Human Genome Project--threat or promise?

This paper reflects on some of the arguments against screening from the perspective of a relative of individuals with Fragile X syndrome. It proposes to think about intellectual disability (ID) as including a wide range of limitations beyond that of only the mental handicap. It argues that these limitations impose conditions upon both people with disabilities and their parents, as well as upon their siblings, that in various ways amount to suffering. The claim that people with disabilities are enriching the lives of their relatives is rejected. Furthermore, it is argued that those who ascribe a high moral status to people with disabilities tend to neglect that society does not make much of an effort to offer the necessary support to materialize this status. The claim that screening negatively affects the moral status of persons with ID is rebutted on grounds of the freedom of choice.

Disability Evaluation↗

PubMed2002

Consequences of oral rehabilitation on dyskinesia in adults with Down's syndrome: a clinical report.

The aim of this article is to demonstrate that the presence of orofacial dyskinesia is often owing to underlying facial dysmorphology in persons with Down's syndrome. A series of cases is presented where orofacial dyskinesia was successfully treated by therapy establishing occlusal stability. The diagnosis of dyskinesia owing to dysmorphology should be precluded before any link with the degree of intellectual disability or neurological deficit is presumed. A multidisciplinary approach may be necessary to diagnose and treat these patients.

Adolescent↗

PubMed1999

Population-based study of infants born at less than 28 weeks' gestation in New South Wales, Australia, in 1992-3. New South Wales Neonatal Intensive Care Unit Study Group.

The aims of the study were to use the population base of New South Wales (NSW) to study all births from 20 to 27 weeks' gestation in 1992-3 and to compare two data sources for perinatal deaths. The prospective population-based statewide audit (NICUS) of infants admitted to tertiary neonatal intensive care units (NICUs) in NSW was used to collect data on infants less than 28 weeks' gestation registered in 1992-3. This audit also surveyed the 160 obstetric hospitals in NSW to ascertain information on stillbirths and early neonatal deaths in the study period. The NSW midwives data collection (MDC) was the other source of information on stillbirths and labour ward deaths. Data were analysed using SAS. In 1992-3 in NSW 1170 infants were born at 20-27 weeks' gestation. There were 556 stillbirths and 614 live births, of whom 180 (29.3%) died in the labour ward and 434 (70.7%) were admitted to a tertiary NICU. Sixty-six per cent of stillbirths were identified by both data collections, 16.5% by the MDC only and 17% by NICUS only. There was a high major congenital anomaly rate (18.5%) among the stillbirths. Two-thirds of the infants admitted to NICUs survived to 1 year. Information was available on at least one follow-up parameter for 89% (255/288) of the survivors to 12 months (corrected age). Of the 244 infants who had a neurological assessment by a paediatrician, 17% were diagnosed to have cerebral palsy. Eleven per cent of the 239 who had a formal Griffiths developmental assessment had a major intellectual disability. Five (2% of 255) of the 1-year-olds were blind, and 12 (4.7% of 255) had bilateral hearing aids. Seventy-one per cent of the infants examined at 1 year did not have a major disability. For accurate perinatal death data, collection from more than one source is recommended. Infants born at 20-27 weeks' gestation contribute 40% of all stillbirths in NSW, most of the costs of neonatal intensive care as well as the costs of long-term morbidity. In Australia in the early 1990s, the survival of infants born at less than 28 weeks' gestation was best from 26 weeks gestational age onwards. Long-term morbidity did not change from that of earlier cohorts. The most common major disability was cerebral palsy.

Cerebral Palsy↗

PubMed2000

Using genetic epidemiology to study Rett syndrome: the design of a case-control study.

Rett syndrome is a neurological disorder that is seen almost exclusively in females. Although generally considered to have a genetic basis, the underlying mechanism remains obscure. One favoured hypothesis is that the syndrome is an X-linked dominant disorder, lethal or non-expressed in males. Genealogical research has also suggested that the mode of transmission in Rett syndrome may involve a premutation which over several generations is converted to a full mutation. Geographical clustering has been reported, and it has also been proposed that Rett syndrome is a clinically variable condition and that other neurological disorders may be occurring more commonly in families with Rett syndrome. Other studies have found an apparent increase in intellectual disability and seizures in the extended families of girls with Rett syndrome. The science of genetic epidemiology can be used to identify familial aggregation, which is the clustering of a disorder within a family. We have used a case-control study design to investigate both fetal wastage and familial aggregation of other disorders in families of girls with Rett syndrome. The Australian Rett Syndrome Database provided the source of cases, and control probands were girls of a similar age with normal development. This paper describes the methodology for a case-control study of this rare condition using pedigree data and discusses issues in the collection and evaluation of such data. The use of a control population is an important feature. Both the strengths and the shortcomings of our design are identified, and recommendations are made for future research.

Australia↗

PubMed2002

Children with disruptive behaviours I: service utilization.

OBJECTIVE: To describe the demographic characteristics and patterns of service utilization in the Hunter region (NSW, Australia) of families caring for a child manifesting disruptive behaviour. METHODOLOGY: Families were eligible to participate in the survey if they had at least one child known to have one of the DSM-IV disruptive behaviour disorders, autistic spectrum disorders, behaviour problems associated with rarer forms of brain disease, brain injury or mild intellectual disability, or identified by school personnel as having significant behaviour problems. Families were recruited to the survey via schools, early education centres and clinical services. Parents completed a mail survey about demographic details and service utilization. RESULTS: In total, 1412 families responded to the survey, a participation rate of approximately 65%. The demographic characteristics of respondent families were similar to those of the regional population. A high proportion (85%) of children from respondent families were receiving treatment. Nearly half those children attending clinical services were attending two or more services. Approximately one-third each of the sample was attending public clinics, private clinics or a combination of both. Children were more likely to be treated by a paediatrician (64%) than a child psychiatrist (39%) or a psychologist (23%). Compared with a normative Australian sample, parents of children manifesting disruptive behaviour reported a high level of family stress. CONCLUSIONS: Families of children manifesting disruptive behaviour are a highly stressed group who often access several clinical services. Paediatricians make a significant contribution to the management of children with disruptive behaviours and need to be considered in service planning.

Adolescent↗

PubMed2002

Children with disruptive behaviours II: clinical and community service needs.

OBJECTIVE: To assist in health service planning by determining the perceived clinical and community service needs of families resident in the Hunter region who care for a child manifesting disruptive behaviour. METHODOLOGY: Families were eligible to participate in the survey if they had at least one child known to have one of the DSM-IV disruptive behaviour disorders, autistic spectrum disorders, behaviour problems associated with rarer forms of brain disease, brain injury or mild intellectual disability or were identified by school personnel as having significant behaviour problems. Families were recruited to the survey via schools, early education centres and clinical services. Parents completed a questionnaire, mailed to them by educational and/or clinical services. Parents were asked to prioritize options for improving or expanding clinical services and for reducing their treatment costs. RESULTS: A total of 1412 families responded to the survey. The highest-ranked clinical service options involved the expansion of mainstream community treatment services for children with disruptive behaviour problems and their families, with a particular emphasis on counselling services. Respite care and in-patient services were given relatively low priority. Subgroup analyses showed that disadvantaged and stressed families gave higher rankings to out of home options, such as respite care, in-patient care and subsidised holiday camps, than the aggregate sample. CONCLUSIONS: Enhancement of community based counselling services would meet the needs of the greatest number of participating families. Resource-intense residential services are required by a small but important group of families who experience disadvantage and high levels of stress.

Adolescent↗

PubMed2002

Congenital hypothyroidism in Western Australia 1981-1998.

OBJECTIVE: Newborn screening for congenital hypothyroidism began in Western Australia in 1981. We describe the epidemiology, including trends over time. METHODOLOGY: Population-based registers and databases were used to identify cases of congenital hypothyroidism in 1981-1998 (n = 126), a random sample of controls (n = 1260), and descriptive data on cases and controls. The prevalence of congenital hypothyroidism per 10 000 live births was calculated, and a case-control analysis conducted. RESULTS: The prevalence of congenital hypothyroidism was 1.74 per 10 000 in the period 1981-1987, compared with 3.54 per 10 000 between 1988 and 1998. Infants identified with congenital hypothyroidism were significantly more likely to have other birth defects (particularly heart defects), be female, have a birthweight heavier than 4500 g, and be born either preterm or after 41 weeks gestation. No cases had cerebral palsy or intellectual disability. CONCLUSIONS: Population-based registers and linked databases can provide very useful information for evaluating screening programmes, and extending our knowledge of the epidemiology of congenital hypothyroidism.

Abnormalities, Multiple↗

PubMed2001

Ataxia telangiectasia with vascular abnormalities in the brain parenchyma: report of an autopsy case and literature review.

A 25-year-old man was admitted to the Department of Neurology, Gunma University Hospital, in June 1997. He had an intellectual disability and had suffered from repeated infection since childhood. Cerebellar ataxia had developed at 19 years of age and he had been clinically diagnosed with ataxia telangiectasia (AT) comprising cerebellar ataxia and oculocutaneous telangiectasia at 24 years of age. He died from pneumonia and renal failure at 26 years of age. Neuropathological examination revealed Purkinje cell loss and atrophy of the dentate nuclei in the cerebellum, anterior horn-cell atrophy and demyelination of the gracile fasciculi in the spinal cord, and the existence of nucleocytomegalic cells in the anterior pituitary gland. These neuropathological findings correlated with previously reported cases of AT. In addition, spongy degeneration was found, predominantly around the blood vessels in the cerebral cortex. Diffuse spongy degeneration and multiple foci of coagulative necrosis with calcification were noted in the white matter. Abnormal vasculature was noted in both degenerative and necrotic areas in the cerebral cortex and in the white matter. The vessels at the center of the areas of spongy degeneration in the cerebral cortex had irregularly arranged and enlarged smooth-muscle-cell nuclei and a distorted, narrow lumen. The vessels present in the white matter were hyalinized. To our knowledge, these vascular abnormalities in the brain parenchyma have not been reported previously.

Adult↗