Journal of Intellectual Disability Research
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Novel insights into maladaptive behaviours in Prader–Willi syndrome: serendipitous findings from an open trial of vagus nerve stimulation Abstract Background We report striking and unanticipated improvements in maladaptive behaviours in Prader–Willi syndrome (PWS) during a trial of vagus nerve stimulation (VNS) initially designed to investigate effects on the overeating behaviour. PWS is a genetically determined neurodevelopmental disorder associated with mild–moderate intellectual disability (ID) and social and behavioural difficulties, alongside a characteristic and severe hyperphagia. Methods Three individuals with PWS underwent surgery to implant the VNS device. VNS was switched on 3 months post‐implantation, with an initial 0.25 mA output current incrementally increased to a maximum of 1.5 mA as tolerated by each individual. Participants were followed up monthly. Results Vagal nerve stimulation in these individuals with PWS, within the stimulation parameters used here, was safe and acceptable. However, changes in eating behaviour were equivocal. Intriguingly, unanticipated, although consistent, beneficial effects were reported by two participants and their carers in maladaptive behaviour, temperament and social functioning. These improvements and associated effects on food‐seeking behaviour, but not weight, indicate that VNS may have potential as a novel treatment for such behaviours. Conclusions We propose that these changes are mediated through afferent and efferent vagal projections and their effects on specific neural networks and functioning of the autonomic nervous system and provide new insights into the mechanisms that underpin what are serious and common problems affecting people with IDs more generally.
Journal of Intellectual Disability Research - Tập 60 Số 2 - Trang 149-155 - 2016
Clumsiness in autism and Asperger syndrome: a further report Clumsiness has been proposed as a diagnostic feature of Asperger syndrome (AS), a type of pervasive developmental disorder recently introduced in the ICD‐10 and DSM‐IV. However, the extent to which this symptom is specific to AS is not clear. To investigate this issue, we compared a sample of AS children with age‐ and sex‐matched groups of children with autistic disorder and pervasive developmental disorder not otherwise specified (PDDNOS). Twelve subjects with AS (ICD‐10/DSM‐IV; 11 males; average age 11.4 years; mean full‐scale IQ 104.9) were compared with 12 subjects with autistic disorder (DSM‐III‐R; 11 males; average age 10.3 years; mean full‐scale IQ 78.4) and 12 subjects with PDDNOS (DSM‐III‐R; 10 males; average age 10.1 years; mean full‐scale IQ 78.2). The BruininksOseretsky test, a standardized test of motor coordination, was administered blind by the same investigator to all the three groups. While coordination deficits were found in all three groups, children with AS were found to be less impaired than those with autistic disorder and PDDNOS. However, no significant relationship was found between coordination scores and diagnosis after adjusting for the level of intelligence. These findings suggest that some patients with AS may be less clumsy than those with autistic disorder and that this difference may be the result of their higher level of intelligence. Studies based on larger samples using multiple measures of coordination are needed to further clarify the role of clumsiness in the classification of pervasive developmental disorders.
Journal of Intellectual Disability Research - Tập 42 Số 1 - Trang 43-48 - 1998
Reports of life skills training for students with intellectual disabilities in and out of school Abstract Background Life skills can be critical to the success of individuals with intellectual disabilities (ID) in terms of postschool outcomes. Yet, research suggests a decreasing emphasis on the acquisition of life skills in school for students with ID, raising the question if students then receive training in these areas after graduation.Method This study represented a secondary analysis of the National Longitudinal Transition Study‐2 data to understand the reported receipt of life skills instruction in school and out of school for individuals with mild and moderate/severe ID. Frequency distributions, significant tests and a t ‐test were used to understand receipt of life skills in and out of school for both individuals with mild and moderate/severe ID.Results The results suggest low‐reported receipt of life skills instruction/training in school and postschool for individuals with mild ID, few students with moderate/severe ID report receiving life skills training out of school, and receipt of life skills instruction in school is not related to receipt of life skills training/therapy after school by either individuals with mild ID or moderate/severe ID.Conclusions Given the current educational policy situation (i.e. a predisposition towards inclusive general education placements for students with disabilities and participation in the accountability system for all students), educators who believe in the value of a life skills curriculum will need to be creative in its implementation and look towards transition plan and activities to provide students with the needed training. Regardless, teachers will need to rectify providing students with the academic skills they need to be successful on a general large‐scale assessment with providing them with the life skills (e.g. independent living, daily living, financial) to be successful after school in employment and independent living.
Journal of Intellectual Disability Research - Tập 54 Số 12 - Trang 1093-1103 - 2010
Correlates of maternal behaviours in mothers of children with fragile X syndrome Abstract Background The behaviours of 24 mothers of children with fragile X syndrome (FXS) with their affected children were examined during planned observations in their homes. The goal of this study was to describe concurrent maternal interactive behaviour and the factors that influence the type and frequency of these behaviours within this group.Methods The frequency of maintaining , directive and scaffolding behaviours and the extent to which the mothers displayed warm sensitivity and restrictions were examined within a 60‐min naturalistic observation and a 10‐min toy play observation. Rating scales and parent questionnaires were used to assess selected maternal mental health factors, including depression, anxiety, stress and social support. The cognitive status of mothers and developmental and behavioural abilities of children were also examined.Results The women in this study used primarily maintaining behaviours in interactions with their children. Maintaining behaviours and warm sensitivity were consistent across structured and unstructured settings, while directive, scaffolding and restricting were not correlated across the two settings. Child receptive language skills were related to higher rates of maintaining and scaffolding behaviours. The women reported clinically significant levels of stress, depression and anxiety at a prevalence rate higher than that of the general public. Child behaviour problems contributed to maternal stress, and mothers with higher stress engaged in fewer interactions with their children.Conclusions The relations between maternal stress, child problem behaviour and number of interactive behaviours exhibited by the mothers in this study provide information that can inform interventions and provide direction for future research exploring environmental influences on the development of children with fragile X syndrome.
Journal of Intellectual Disability Research - Tập 51 Số 6 - Trang 447-462 - 2007
A survey of sleep problems in autism, Asperger's disorder and typically developing children Abstract Background Sleep problems are common in typically developing (TD) children and in children with autism, however, less is known about the sleep of children with Asperger's disorder (AD). The aim of this study was to compare sleep patterns of children with autism and AD to a TD group of children.Methods Sixty‐six parents of TD children, 53 parents of children with autism, and 52 parents of children with AD completed a survey on their child's sleep patterns, the nature and severity of any sleep problems and success of any treatment attempted.Results The results showed high prevalence of sleep problems with significantly more problems reported in the autism and AD groups (TD = 50%, autism = 73%, AD = 73%), with no significant differences between groups on severity or type of sleep problem. Children with AD were significantly more likely to be sluggish and disoriented after waking and had a higher Behavioral Evaluation of Disorders of Sleep (BEDS) total score compared to the other two groups. The autism and AD groups reported significantly better treatment success for medication compared to the TD group. The autism group reported significantly better success for behavioural treatment compared to the AD group.Conclusions In conclusion, children with AD may have more symptoms of sleep disturbance, and different types of sleep problems than children with autism. As this is the first study to compare autism and AD and to survey treatment outcomes, further research is needed to validate these findings.
Journal of Intellectual Disability Research - Tập 49 Số 4 - Trang 260-268 - 2005
Profiles and cognitive predictors of motor functions among early school‐age children with mild intellectual disabilities Abstract Background The purpose of the study was to describe sensorimotor profile in children with mild intellectual disability (ID), and to examine the association between cognitive and motor function.Methods A total of 233 children with mild ID aged 7 to 8 years were evaluated with measures of cognitive, motor and sensory integrative functioning.Results Children with mild ID performed significantly less well on all test measures. 44.2% of children scored in the impaired range on seven out of 22 sensorimotor measures. They had weaker fine motor skills than gross motor skills. Sensory integrative functions were only mildly impaired. Total IQ substantially predicted overall performance on each motor test. Specifically, verbal comprehension and processing speed indexes were significant predictors of gross and fine motor function.Conclusions Sensorimotor dysfunctions were found to be very frequent in children with mild ID. Early identification of sensorimtor impairments is essential to prompt early intervention and facilitate better integration into regular school settings.
Journal of Intellectual Disability Research - Tập 52 Số 12 - Trang 1048-1060 - 2008
Moving from the quality of life concept to a theory Abstract Background The Special Interest Quality of Life Group has updated its set of statements defining the quality of life (QOL) construct to reflect emerging areas of agreement and the framework for understanding better the QOL construct.Method This article examines the major areas currently under discussion involving the objective–subjective dichotomy, needs, and core domains.Results It is concluded that while the new statements constitute a significant advance, further progress requires testable theory. In order to facilitate such future research, a conceptual model is proposed that distinguishes causal and indicator variables within the framework of a homeostatic management system.Conclusion Several lines of empirical investigation are suggested to test this and similar theoretical models with a view to taking our conceptualization of QOL to the next level.
Journal of Intellectual Disability Research - Tập 49 Số 10 - Trang 699-706 - 2005
Differences in the clinical presentation of Trisomy 21 with and without autism Abstract Background Autism occurs 10 times more often in children with Down syndrome than in the general population, but diagnosing co‐occurring autism in Down syndrome with severe intellectual disability is challenging. The objective of this case–control study was to identify characteristics differentiating children with trisomy 21 with and without autism and to determine the extent to which severe cognitive impairment affects the measures of autism symptomatology.Method Twenty children with trisomy 21 and autism (cases) were compared with children with trisomy 21 without autism (controls) matched on chronologic age, race and gender. Communication, cognitive and adaptive behaviour skills were assessed with standardized instruments. Medical history was reviewed and medical records were examined for early head growth. Scores on the diagnostic algorithm of the Autism Diagnostic Interview – Revised (ADI‐R) were compared after adjusting for cognitive ability as measured by the Stanford‐Binet (Fifth Edition) non‐verbal change‐sensitive score.Results Cases performed significantly more poorly on all assessments. Mean case–control differences for matched pairs were all significant at P < 0.0001 for receptive and expressive language skills, cognitive skills and adaptive skills. Seven cases had a history of seizures compared with one control (P = 0.01). After adjusting for cognitive ability, the mean scores on the Reciprocal Social Interaction, Communication, and Restricted, Repetitive and Stereotyped Behaviours domains of the ADI‐R diagnostic algorithm remained significantly higher in cases compared with controls (P < 0.0001). All participants had decreased head size consistent with Down syndrome, with no case–control differences.Conclusion Children with trisomy 21 and autism have significantly more impaired brain function than children with trisomy 21 without autism. However, the deficits in the core domains of social reciprocity and communication, and the restricted and repetitive interests are not entirely explained by the more severe cognitive impairment. This autism phenotype in children with trisomy 21 which includes an increased risk for seizures may indicate a widespread loss of functional connectivity in the brain.
Journal of Intellectual Disability Research - Tập 53 Số 2 - Trang 143-151 - 2009
Natural history of thyroid function in adults with Down syndrome – 10‐year follow‐up study Abstract Background The natural history of thyroid function in adults with Down syndrome (DS) is unknown.Method This study investigated annual thyroid function tests in 200 adults with DS over a 10‐year period.Results Transient and persistent thyroid dysfunction was common. The 5‐ and 10‐year incidence of definite hypothyroidism was 0.9%−1.64% and 13.6%, respectively. Subclinical hypothyroidism was not found to be an early sign for definite hypothyroidism.Conclusions Routine screening for adults with DS who are euthyroid can be reduced to every 5 years rather than the present policy of every 1–2 years.
Journal of Intellectual Disability Research - Tập 51 Số 4 - Trang 312-317 - 2007
Neurological changes and emotional functioning in adults with Down Syndrome Abstract This study was designed to examine emotional changes in adults with Down Syndrome (DS) over time and whether changes in these psychological variables were associated with brain atrophy on MRI scan and the presence of pathological reflexes on the neurological examination. Participants were 26 adults with DS and their caregivers. Caregivers completed a measure of emotional functioning about individuals with DS at two different time points (1 year apart). Levels of cognitive functioning were measured and neurological and MRI examinations were performed on all subjects at initial testing. Significant group effect separated those with and without pathological findings on MRI and neurological exam across three different scales: depression, indifference, and pragmatic language functioning. Problems of poor pragmatic language functioning appeared later in the course of suspected Alzheimer's disease (AD), as demonstrated by a significant group effect at time 2, but not at initial testing. In these subjects, the primary emotional change was a decline in social discourse (e.g. conversational style, literal understanding, verbal expression in social contexts). These emotional levels were stable over time, regardless of degree of cognitive decline. Specific emotional changes occur during the course of AD which were associated with abnormal findings from MRI and from neurological examination. These results, along with abnormalities in brain imaging and the presence of pathological reflexes, suggested that frontal lobe dysfunction is likely to be an early manifestation of Alzheimer's Disease in Down Syndrome.
Journal of Intellectual Disability Research - Tập 45 Số 5 - Trang 450-456 - 2001
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