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Biomedical subjects

S L Einfeld

Publications and source records attributed to S L Einfeld.

At least 19 recordsLinked to original sources

The development of a new measure for the assessment of psychopathology in adults with intellectual disability.

BACKGROUND: People with intellectual disability (ID) and untreated psychiatric disorder lead unnecessarily difficult and unhappy lives. The prevalence of mental illness in children and adults with ID is greater than that found in the general population. A carer-completed checklist of psychopathology that could be used with both children and adults would help identify those individuals with ID most likely to have a mental health problem, help ensure that they receive the limited services that are usually available and also assist the process of clinical assessment, diagnosis and management. METHOD: This research aimed to develop a reliable and valid carer-completed checklist of psychopathology for adults with ID by redeveloping an existing measure for children with ID, the Developmental Behaviour Checklist (DBC-P). The new checklist, The Developmental Behaviour Checklist for Adults (DBC-A) was devised by changing, deleting and adding to DBC-P items. Reliability studies were conducted with paid and family carers, and DBC-A scores were compared with the results from two other measures of psychopathology. RESULTS: One DBC-P item was deleted, seven items changed and 12 items added. The psychometric properties of this new checklist, the DBC for Adults with ID (DBC-A), were investigated and found to be satisfactory. Intraclass correlations for test-retest and inter-rater reliability ranged from 0.72 to 0.85, and concurrent validity with two measures of emotional and behavioural disturbance was satisfactory. CONCLUSIONS: The carer-completed DBC-A provides a broad and comprehensive survey of the emotional and behavioural problems of adults with ID. It has satisfactory psychometric properties and therefore can be used with confidence in clinical, research and service settings, and its development allows continuous assessment of psychopathology across the lifespan for all people with ID.

Adolescent↗

Assessment of change with the Developmental Behaviour Checklist.

BACKGROUND: The Developmental Behaviour Checklist (DBC) is a 96-item instrument designed for assessing behavioural and emotional problems among young people with intellectual disability. METHODS: The present study investigated the validity of the DBC as a measure of change. Changes in individual's DBC scores were correlated with changes in expert clinicians' ratings of the same subjects. RESULTS: A high correlation was found between expert clinician ratings of change in behaviour and change in the total behaviour problem score of the DBC. CONCLUSIONS: The DBC is able to measure changes in behaviour and emotions which are recognized by an experienced clinician who is following the progress of a child with ID.

Adolescent↗

From efficacy to effectiveness: managing organisational change to improve health services for young people with deliberate self harm behaviour.

Repeat Deliberate Self Harm is a recognised risk factor for completed suicide and therefore reduction by effective health service response represents a valid contribution to suicide prevention. However, only a small fraction of people with deliberate self harm presentations to general health settings actually reach specialist mental health follow-up appointments. Therefore, even if responses at that point are known to be effective they do not make a significant contribution to reducing repeat self-harm overall. We describe health system organisational change strategies to improve health service engagement for the target group, and present data demonstrating the effectiveness of these strategies.

Adolescent↗

Longitudinal course of behavioral and emotional problems in Williams syndrome.

A follow-up study of behavior and emotional problems in a cohort of young people with Williams syndrome 5 years after first assessment is described. Using a between-/within-subjects factorial layout, we compared scores on the Developmental Behaviour Checklist between young people with Williams syndrome and a large epidemiological control sample of young people with mental retardation due to other causes from Time 1 (1990/1991) to Time 2 (1995/1996). Results showed substantial persistence of the overall level of behavior and emotional problems. However, there were changes in certain types of behavior. Participants with Williams syndrome had significantly higher overall behavioral and emotional problems, communication disturbance, and anxiety over the 5-year period. Further, 10 or 13 checklist items maintained significantly higher levels among the Williams syndrome sample.

Adolescent↗

Epilepsy and associated psychopathology in young people with intellectual disability.

OBJECTIVE: This study aimed to investigate the role of epilepsy in psychopathology among an epidemiological sample of young people with intellectual disability (ID). The study also compared behavioural and emotional problems in young people on medication to control their epilepsy with those not on medication, and young people with epilepsy who were having current seizure activity with those with no current seizure activity. METHODOLOGY: An epidemiological cohort of 392 participants, representative of the general Australian population of young people with ID, were used in the study. One hundred and fifteen individuals from this cohort were identified as suffering from epilepsy. The Developmental Behaviour Checklist was used to measure symptoms of behavioural and emotional disturbance. RESULTS: Results confirmed that young people with ID and epilepsy did not differ from controls without epilepsy on measures of psychopathology. Furthermore, those with epilepsy on medication had no difference in levels of problem behaviours than epileptics not on medication. CONCLUSIONS: The results suggest that epilepsy has little or no influence on problem behaviours for young people with ID. Our attempt to understand the pathogenesis of behaviour problems in persons with ID may be better directed towards understanding genetic mechanisms than epilepsy pathologies.

Adolescent↗

Behavior and emotional disturbance in Prader-Willi syndrome.

To determine if persons with the Prader-Willi syndrome (PWS) have increased psychopathology when compared with matched controls, and whether there is a specific behavior phenotype in PWS, the behavior of 46 persons with PWS was compared with that of control individuals derived from a community sample (N = 454) of persons with mental retardation (MR). Behaviors were studied using the Developmental Behaviour Checklist, an instrument of established validity in the evaluation of behavioral disturbance in individuals with MR. PWS subjects were found to be more behaviorally disturbed than controls overall, and especially in antisocial behavior. In addition, some individual behaviors were more common in PWS subjects than controls. When these behaviors are considered together with findings from other studies using acceptably rigorous methods, a consensus behavior phenotype for PWS can be formulated. This will provide a valid foundation for studies of the mechanism of genetic pathogenesis of behavior in PWS.

Adolescent↗

Observations on the use of the ICD-10 guide for mental retardation.

In acknowledgement of the challenges posed by the diagnosis of mental disorders in people with intellectual disability (ID), the World Health Organization (WHO) has recently published a multi-axial guide to assist clinicians in applying the ICD-10 to this group of people. The WHO has invited users to review of the ICD-10 Guide for Mental Retardation. In order to review its usefulness in clinical practice, the present authors used the Guide together with the ICD-10 in the psychiatric assessment of 106 young people with ID, and emotional and behavioural problems of varying severity. Strengths and weaknesses in the utility of the Guide were noted. As a result of the clinical audit process, the present authors identified a number of inconsistencies, significant gaps and assertions unsupported by the available literature in the Guide. They also noted aspects of the ICD-10 itself which are problematic when applied to people with ID. Despite these limitations, the Guide is a valuable first attempt to establish a standardized structure for multi-axial diagnosis in this population. A number of suggestions for major changes and refinements to future editions are made, and a strategy for development of research to establish validity and reliability is proposed.

Adolescent↗

Behavioral and emotional disturbance in individuals with Williams syndrome.

Behavioral and emotional disturbance was assessed in 70 children and adolescents with Williams syndrome. They were compared with an epidemiological control population, which was statistically controlled for age, gender, and level of mental retardation. Those with Williams syndrome were more likely to be diagnosed as suffering psychiatric disorder. The disorder was characterized by anxiety, hyperactivity, preoccupations, and inappropriate interpersonal relating. Significantly increased rates of other individual symptoms were also found, including sleep disturbance and hyperacusis. These results, considered with earlier findings, suggest that there is a valid behavior phenotype of Williams syndrome. This is frequently associated with sufficient impairment to consider inclusion of the behavior phenotype in future official taxonomies of mental disorders.

Analysis of Variance↗

Intellectual handicap in contemporary psychiatry.

OBJECTIVE: To apprise readers of the 'state of play' in the psychiatry of intellectual handicap. METHOD: A review was conducted of relevant journals and conference abstracts covering a range of scientific aspects of intellectual disability. Those developments considered to be most significant were summarised. In addition, consideration is given to issues relevant to the politics of disability affecting psychiatric practice in Australia. RESULTS: There have been considerable developments in our understanding of the pathogenesis of intellectual handicap in pharmacotherapy and the behavioural therapies, in the taxonomy of psychopathology, in epidemiology, in delineation of behaviour phenotypes, and in assessment and measurement of psychopathology. CONCLUSION: Intellectual handicap and psychiatry have had a chequered relationship in Australia, but the two fields are now clearly moving again to a closer and more productive collaboration. Recent scientific advances have provided a stimulating environment for this increased activity and interest.

Australia↗

Population prevalence of psychopathology in children and adolescents with intellectual disability: I. Rationale and methods.

Our knowledge of the epidemiology of psychopathology in children and adolescents with intellectual disability (ID) is hampered by a number of factors. These include the relative scarcity of studies of children rather than adults, the study of non-epidemiological samples such as those in institutions or those attending psychiatric clinics, a lack of standardized methods of assessment of psychopathology, studies with numbers too small to provide adequate confidence intervals around identified prevalence rates, insufficient detail concerning symptoms or syndromes as well as disorder, and a lack of consistent data concerning the effects of basic demographic variables such as age, sex and IQ. Despite these limitations, it is clear that psychopathology is several times more prevalent in children and adolescents with ID than in those without this disability. This paper reviews findings from previous studies and describes the methodology of a new study using the Developmental Behaviour Checklist.

Adolescent↗

Population prevalence of psychopathology in children and adolescents with intellectual disability: II. Epidemiological findings.

This paper reports findings from an epidemiologically derived population in a multicentre study in NSW, Australia. The design of this study is described in the accompanying paper. Those with mild intellectual disability (ID) were likely to have been underascertained, but identification and participation rates for those with more severe ID were high. The study found that in the regions surveyed 40.7% of those with ID and aged between 4 and 18 could be classified as having severe emotional and behaviour disorder or as being psychiatrically disordered. The profoundly intellectually handicapped had lower levels of disturbance overall compared with those with mild, moderate and severe ID. The level of ID affected scores on a number of behavioural dimensions, with disruptive and antisocial behaviours more prominent in the mild ID group, and 'self-absorbed' and 'autistic' behaviours more prominent in those with severe ID. Age and sex did not affect prevalence, a finding that is in contrast to that found in general child psychopathology. The study found that fewer than 10% of children with intellectual disability and major psychiatric disorder had received specialist assistance.

Adolescent↗

The use of factor analysis for ascertaining patterns of psychopathology in children with intellectual disability.

The Developmental Behaviour Checklist (DBC) was developed to assess psychopathology in children with intellectual disabilities. A cut-off point between "non-cases' and "cases' was determined by comparison of the total scores with psychiatric clinical assessment. This paper describes a method aimed at determining the types of psychiatric problems in those regarded as "cases'. Factor analysis with varimax rotation carried out on a sample of 1093 subjects extracted six factors. Standardized factor scores were calculated for each subject in a community sample (n = 450), and the only or the dominant positive score was determined for each of the "cases'. Over 80% of "cases' could be allotted to one condition. Only a small number had none, or three or more conditions. The same procedure was replicated on a validation sample (n = 448) with even more satisfactory results. Differences in the prevalence of the six conditions by sex, age and level of mental retardation were ascertained. The validity and clinical relevance of this method are discussed.

Adolescent↗

The Developmental Behavior Checklist: the development and validation of an instrument to assess behavioral and emotional disturbance in children and adolescents with mental retardation.

Describes the development and validation of the Developmental Behavior Checklist (DBC), a standardized instrument completed by lay informants to assess behavioral and emotional disturbance in children and adolescents with mental retardation (MR). Items describing common behavioral and emotional problems in this population were generated by extracting descriptions from 664 case files of children and adolescents with behavior disorders seen at a specialist developmental assessment service over 12 years. These items were reduced to a set of 96 items administered to a sample of 1,093 children and adolescents with mental retardation and then submitted to a principal components analysis. Six interpretable and partly validated subscales were obtained which explained 36% of the total variance and had satisfactory internal consistency. Interrater and test-retest agreement were satisfactory for both total scale score and for scores on each of the subscales. Good evidence of concurrent validity was provided by substantial positive correlations between total scores on the DBC completed by lay informants and the ratings of experienced psychiatrists based upon interviews and scores on two standardized instruments that must be completed by health professionals. The discriminative validity of the total score as assessed by area under the ROC curve was excellent (92%). Standardized norms for the DBC are derived from an epidemiological study of behavior problems in children and adolescents with mental retardation undertaken in two Australian States. Norms are available for the mild, moderate, severe, and profound MR groups and for the MR population as a whole.

Adolescent↗

Issues in the taxonomy of psychopathology in mental retardation.

Several factors appear to impede the development of a valid taxonomy of psychopathology in children and adolescents with mental retardation. These include (a) the lack of a widely accepted definition of psychopathology in mental retardation, (b) disagreement on the nature of the relationship between mental retardation and psychopathology, and (c) insufficient evidence for the reliability and validity of current DSM or ICD systems in this population. In this article, we offer a definition of psychopathology in children with mental retardation; review concepts of the relationship between psychopathology and mental retardation; argue that in moving toward a valid taxonomy factors to be considered should include data from multivariate studies, findings related to behavior phenotypes, and diagnostic considerations with stereotypic behavior and self-injury, organic brain syndromes and pervasive developmental disorders. Finally, we outline a research strategy that may serve as a useful framework for developing a valid taxonomy of psychopathology in this population.

Adolescent↗

Behavioural and emotional disturbance in fragile X syndrome.

A range of behaviours has been postulated as specific manifestations of the Fragile X (fra(X)) syndrome. This study examines a broad range of behaviours in subjects with fra(X) syndrome and in control individuals. It was designed to take account of methodological factors which may account for differing findings in previous studies. Subjects were 48 children, adolescents, and young adults with fra(X) syndrome. They were compared with a large community epidemiological sample (N = 454) of persons with mental retardation (M.R.), matched for age, sex, and IQ. Behavioural problems were studied using the Developmental Behaviour Checklist, an instrument of established reliability and validity in the evaluation of behavioural and emotional disturbance in children and adolescents with mental retardation. Fragile X subjects had more shyness and avoidance of eye contact and less antisocial behaviour. Further methodological issues pertaining to studies of behaviour phenotype in general are discussed.

Adolescent↗

Clinical assessment of psychiatric symptoms in mentally retarded individuals.

Mentally retarded Australians with emotional and behavioural disorders are seriously under-served by psychiatrists, despite such individuals being as prevalent as those with schizophrenia. These disorders have serious consequences for the individual, family and carers, and the community as a whole. In the psychiatric assessment of mentally retarded persons, a key principle is that while intellectual handicap increases vulnerability to psychiatric illness, it does not of itself cause psychopathology. A biopsychosocial framework provides a model whereby those factors which are contributing to psychopathology can be systematically elucidated. Biological factors include brain pathology associated with particular mental retardation syndromes, drug side-effects and epilepsy. Psychological factors include temperament, a history of abuse, and current life stresses. Social factors include distortions in family roles and hostel or workshop conflicts. Although clinical assessment can be challenging, and confident syndrome diagnosis is not always possible, a systematic approach to the analysis of symptoms will provide a guide to useful interventions.

Humans↗

Sudden death in childhood in a case of the G syndrome.

We report on the unexpected death at almost 8 years of a boy with the G syndrome who had successfully survived many prior life-threatening complications of the condition. The patient had a characteristic facial appearance, hypertelorism, second-degree hypospadias, stridor and cough on feeding with aspiration of barium, and uncoordinated esophageal swallowing mechanism, but no obvious laryngotracheobranchial defect on endoscopy (or coroner's autopsy). He had a prior cardiac arrest and had outgrown need for a tracheostomy. Aspiration is presumed to be the cause of death.

Abnormalities, Multiple↗