Acquiescence in interviews with people who have mental retardation

Evidence for acquiescence (yea-saying) in interviews with people who have mental retardation is reviewed and the different ways it has been assessed are discussed. We argue that acquiescence is caused by many factors, each of which is detected differentially by these methods. Evidence on the likely causes of acquiescence is reviewed, and we suggest that although researchers often stress a desire to please or increased submissiveness as the must important factor, acquiescence should also be seen as a response to questions that are too complex, either grammatically or in the type of judgments they request. Strategies to reduce acquiescence in interviews are reviewed and measures that can be taken to increase the inclusiveness of interviews and self-report scales in this population suggested.

Genetic Aspects of Familial Thyroid Cancer

Familial thyroid cancer is rare, accounting for

Task-switching deficits and repetitive behaviour in genetic neurodevelopmental disorders: Data from children with Prader-Willi syndrome chromosome 15 q11-q13 deletion and boys with Fragile X syndrome

Prader-Willi syndrome (PWS) and Fragile X syndrome (FraX) are associated with distinctive cognitive and behavioural profiles. We examined whether repetitive behaviours in the two syndromes were associated with deficits in specific executive functions. PWS, FraX, and typically developing (TD) children were assessed for executive functioning using the Test of Everyday Attention for Children and an adapted Simon spatial interference task. Relative to the TD children, children with PWS and FraX showed greater costs of attention switching on the Simon task, but after controlling for intellectual ability, these switching deficits were only significant in the PWS group. Children with PWS and FraX also showed significantly increased preference for routine and differing profiles of other specific types of repetitive behaviours. A measure of switch cost from the Simon task was positively correlated to scores on preference for routine questionnaire items and was strongly associated with scores on other items relating to a preference for predictability. It is proposed that a deficit in attention switching is a component of the endophenotypes of both PWS and FraX and is associated with specific behaviours. This proposal is discussed in the context of neurocognitive pathways between genes and behaviour.

Rejecting the label: A social constructionist analysis

Research has shown that people often do not claim labels associated with mental retardation or learning difficulties. We discussed the interpretation that this rejection is an example of a denial process, the purpose of which is to protect self-esteem. Alternative explanations for this lack of identification were offered, based on an understanding of the socially constructed nature of diagnostic labels and on the distinction between diagnostic labels and social categories. Some of the problems in using the label as a descriptive or explanatory resource are illustrated using quotes from a study in which people who have been labeled discussed the label.

Mosaic monosomy 14: clinical features and recognizable facies

A 1-year-old child with clinical features of monosomy 14 is reported. She has dysmorphic facial features including ocular colobomata, dolichocephaly and microcephaly, retinal pigmentation, severe seizures, fair curly hair and tapering fingers. There was severe mental retardation. This is the first reported case of severe mosaic monosomy 14, with up to 30% mosaicism. A recognizable facial gestalt is present in children with 14q deletions or partial monosomy 14, as well as susceptibility to infection, feeding difficulties, seizures and retinal pigmentation. (C) 2004 Lippincott Williams Wilkins.

Sibling correlation of deficit syndrome in the Irish study of high-density schizophrenia families

The deficit syndrome is a subtype of schizophrenia characterized by primary and enduring negative features of psychopathology. It appears to reflect a distinct subtype within the syndrome of schizophrenia. Little is known about the familial or genetic aspects of the deficit syndrome. The purpose of this study was to determine whether deficit versus nondeficit subtypes are correlated in sibling pairs affected with schizophrenia.

Prevalence, phenomenology, aetiology and predictors of challenging behaviour in Smith-Magenis syndrome

Background

The prevalence, phenomenology aetiology and correlates of four forms of challenging behaviour in 32 children and adults with Smith-Magenis syndrome (SMS) were investigated.

Methods

Cognitive assessments, questionnaires and semi-structured interviews were used to gather data on intellectual disability, verbal and physical aggression, destructive behaviour and self-injury and on characteristics known to be associated with aggression.

Results

Aggression in SMS was more prevalent (87%), but not more severe than aggression in contrast groups. Aggressive behaviour was more frequently associated with environmental contingencies (e.g. attention, escape and access to tangibles) than self-injury and destructive behaviours. Severity of challenging behaviours was associated with high impulsivity.

Conclusion

Aggression is seen in the majority of people with SMS. Results suggest that behavioural disinhibition and operant social reinforcement are associated with the manifestation of aggression.

Associations between repetitive questioning, resistance to change, temper outbursts and anxiety in Prader-Willi and Fragile-X syndromes

The behavioural phenotypes of Prader-Willi (PWS) and Fragile-X (FraX) syndromes both comprise repetitive behaviours with differences between the profiles. In this study we investigated the context and antecedents to the repetitive behaviours and the association with other behavioural phenotypic characteristics in order to generate testable hypotheses regarding the cause of the behaviours.

The parents or carers of 46 children with PWS (mean age 14.1 years; 20 girls), and 33 boys with FraX (mean age 13.11 years) were interviewed about their children's repetitive behaviour in a semi-structured format.

Children showed negative emotional behaviour (PWS: 87.0%; FraX: 79.4%) and repetitive questions (PWS: 78.3%; FraX: 73.5%) following changes in routine or expectations. Significantly more temper outbursts were reported to follow changes in children with PWS (89.1%) compared with boys with FraX (41.2%) (chi(2) = 20.93; P <0.001). Anxiety that was frequently associated with repetitive and self-injurious behaviours in boys with FraX, followed changes in significantly more boys with FraX (76.5%) compared with children with PWS (6.5%) (chi(2) = 43.19, P <0.001).

On the basis of these reports and existing literature, we hypothesise that decreases in predictability are aversive to children with PWS and FraX. We also hypothesise that these children have a propensity to show a syndrome-related pattern of behaviour (temper outbursts in PWS and displays of anxiety in FraX) when an event in the environment has this aversive property. We hypothesise that questions may be reinforcing to children in their own right by increasing the predictability of the environment. We outline how a specific cognitive deficit in the endophenotypes associated with both PWS and FraX could be investigated as a potential explanation for the hypothesised aversive properties of decreased predictability.

The relationship between the recognition of facial expressions and self-reported anger in people with intellectual disabilities

Background This study aims to examine the relationship between how individuals with intellectual disabilities report their own levels of anger, and the ability of those individuals to recognize emotions. It was hypothesized that increased expression of anger would be linked to lower ability to recognize facial emotional expressions and increased tendency to interpret facial expressions in a hostile or negative manner. It was also hypothesized increased levels of anger may lead to the altered perception of a particular emotion.

Method A cross-sectional survey design was used. Thirty participants completed a test of facial emotion recognition (FER), and a self-report anger inventory (Benson & Ivins 1992) as part of a structured interview.

Results Individuals with higher self-reported anger did not show significantly reduced performance in FER, or interpret facial expressions in a more hostile manner compared with individuals with less self-reported anger. However, they were less accurate in recognizing neutral facial emotions.

Conclusions It is tentatively suggested that individuals with high levels of anger may be likely to perceive emotional content in a neutral facial expression because of their high levels of emotional arousal.

Genetic optimism:Framing genes and mental illness in the news

Over the past two decades the pace and specificity of discoveries associating genetics with mental illness has accelerated, which is reflected in an increase in news coverage about the genetics of mental disorder. The news media is a major source of public understanding of genetics and a strong influence on public discourse. This paper examines the news coverage of genetics and mental illness (i.e., bipolar illness and schizophrenia) over a 25 year period, emphasizing the peak period of 1987-1994. Using a sample of 110 news stories from 5 major American newspapers and 3 news magazines, we identify the frame of "genetic optimism" which dominated the reporting of genetics and mental illness beginning in the mid-1980s. The structure of the frame is comprised of 3 elements: a gene for the disorder exists; it will be found; and it will be good. New discoveries of genes were announced with great fanfare, but the most promising claims could not be replicated or were retracted in short order. Despite these disconfirmations, genetic optimism persisted in subsequent news stories. While the scientific accuracy of the gene stories is high, the genetic optimism frame distorts some of the findings, misrepresents and reifies the impact of genes on mental disorder, and leaves no space for critics or an examination of potential negative impacts. The stances of reporters, scientists and editors may all in different ways contribute to the perpetuation of genetic optimism. Genetic optimism presents an overly sanguine picture of the state of genetics; as we enter the genetic age it is important to balance the extraneous "hype and hope" contained in news stories of genetics and mental illness.

Shaping the Future of Mental Health Policy and Legislation in Northern Ireland: The Impact of Service User and Professional Social Work Discourses

This paper explores the complex interrelationship between service user and professional social work discourses and provides a critical commentary on their respective contributions to the recent review of mental health policy and legislation in Northern Ireland. The analysis indicates that dominant trends in mental health care, as mediated through service structures and institutional identities, have tended to prioritize the more coercive aspects of the social work role and reinforce existing power inequalities with service users. It is argued that such developments underline the need for a ‘refocusing’ debate in mental health social work to consider how a more appropriate balance can be achieved between its participatory/empowering and regulatory/coercive functions. Whilst highlighting both congruence and dissonance between respective discourses, the paper concludes that opportunities exist within the current change process for service users and social workers to build closer alliances in working together to reconstruct practice, safeguard human rights and develop innovative alternatives to a traditional bio-medical model of treatment.

A Longitudinal Study of the Effects of Childhood Trauma on Symptoms and Functioning of People with Severe Mental Health Problems

This study examines the relationship between childhood trauma and the psychiatric symptoms and psychosocial functioning of adults with severe mental health problems. Participants (n = 31) were recruited from the caseloads of community mental health services in Northern Ireland and assessed at baseline, 9 months, and 18 months. More than half had a history of childhood trauma (n = 17). There were no differences between the no childhood trauma (n = 14) and childhood trauma groups on psychiatric symptoms, but a significant relationship was found between trauma history and all aspects of social functioning. Those with no history of trauma showed improved psychosocial functioning over time, whereas those with a history of trauma deteriorated. These findings have implications for current service provision.

BELFAST nonagenarians; Nature or Nurture? Immunological, cardiovascular and genetic factors

Nonagenarians are the fastest growing sector of populations across Western European and the developed world. They are some of the oldest members of our societies and survivors of their generation and may help us understand how to age not only longer, but better.The Belfast Longevity Group enlisted the help of 500 community-living, mobile, mentally competent, 'elite' nonagenarians, as part of an ongoing study of ageing. We assessed some immunological, cardiovascular, nutritional and genetic factors and some aspects of their interaction in this group of 'oldest old'.Here we present some of the evidence related to genetic and nutritional factors which seem to be important for good quality ageing in nonagenarians from the Belfast Elderly Longitudinal Free-living Ageing STudy (BELFAST).