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.

Causal Models of Clinically Significant Behaviors in Angelman, Cornelia de Lange, Prader-Willi and Smith-Magenis Syndromes

The operant learning theory account of behaviors of clinical significance in people with intellectual disability (ID) has dominated the field for nearly 50 years. However, in the last two decades, there has been a substantial increase in published research that describes the behavioral phenotypes of genetic disorders and shows that behaviors such as self-injury and aggression are more common in some syndromes than might be expected given group characteristics. These cross-syndrome differences in prevalence warrant explanation, not least because this observation challenges an exclusively operant learning theory account. To explore this possible conflict between theoretical account and empirical observation, we describe the genetic cause and physical, social, cognitive and behavioral phenotypes of four disorders associated with ID (Angleman, Cornelia de Lange, Prader-Willi and Smith-Magenis syndromes) and focus on the behaviors of clinical significance in each syndrome. For each syndrome we then describe a model of the interactions between physical characteristics, cognitive and motivational endophenotypes and environmental factors (including operant reinforcement) to account for the resultant behavioral phenotype. In each syndrome it is possible to identify pathways from gene to physical phenotype to cognitive or motivational endophenotype to behavior to environment and back to behavior. We identify the implications of these models for responsive and early intervention and the challenges for research in this area. We identify a pressing need for meaningful dialog between different disciplines to construct better informed models that can incorporate all relevant and robust empirical evidence.

Self-generated expressions of residual complaints following brain injury

Following brain injury there is often a prolonged period of deteriorating psychological condition, despite neurological stability or improvement. This is presumably consequent to the remission of anosognosia and the realisation of permanently worsened status. This change is hypothesised to be directed partially by the socially mediated processes which play a role in generating self-awareness and which here direct the reconstruction of the self as a permanently injured person. However, before we can understand this process of redevelopment, we need an unbiassed technique to monitor self-awareness. Semi-structured interviews were conducted with 30 individuals with long-standing brain injuries to capture their spontaneous complaints and their level of insight into the implications of their difficulties. The focus was on what the participants said in their own words, and the extent to which self-knowledge of difficulties was spontaneously salient to the participants. Their responses were subjected to content analysis. Most participants were able to say that they had brain injuries and physical difficulties, many mentioned memory and attentional problems and a few made references to a variety of emotional disturbances. Content analysis of data from unbiassed interviews can reveal the extent to which people with brain injuries know about their difficulties. Social constructionist accounts of self-awareness and recovery are supported.

Aggression after traumatic brain injury: Analysing socially desirable responses and the nature of aggressive traits.

Primary objective: To compare patients with traumatic brain injury (TBI) with controls on sub-types of aggression and explore the role of social desirability.
Design: Quasi-experimental, matched-participants design.
Methods and procedures: Sixty-nine participants were included in the study. The sample comprised a TBI group (n = 24), a spinal cord injury (SCI) group (n = 21) and an uninjured (UI) group of matched healthy volunteers (n = 24). Participants were given self-report measures of aggression, social desirability and impulsivity. Sixty-one independent ‘other-raters’ were nominated, who rated participant pre-morbid and post-morbid aggression.
Main outcomes and results: Using standardized norms, 25–39% of participants with TBI were classified as high average–very high on anger and 35–38% as high average–very high on verbal aggression. Other-raters rated participants with TBI as significantly higher on verbal aggression than SCI and UI participants. There were no differences between the groups on physical aggression. The TBI group also had higher levels of impulsivity than SCI and UI groups. Social desirability was a highly significant predictor of self-reported aggression for the entire sample.
Conclusions: Impulsive verbal aggression and anger are the principal aggressive traits after brain injury. Physical aggression may present in extreme cases after TBI, but appears less prominent overall in this population. Social desirability, previously overlooked in research examining TBI aggression, emerged as an influential variable that should be considered in future TBI research.

The causal attributions of nursing students towards adolescent survivors of brain injury

Background: The hidden nature of brain injury means that it is often difficult for people to understand the sometimes challenging behaviors that individuals exhibit. The misattribution of these behaviors may lead to a lack of consideration and public censure if the individual is seen as simply misbehaving.

Objective: The aim of this study was to explore the impact of visual cues indicating the presence or absence of brain injury on prejudice, desire for social interaction, and causal attributions of nursing and computing science students.

Method: An independent-groups design was employed in this research, which recruited 190 first-year nursing students and 194 first-year computing science students from a major university in Belfast, UK. A short passage describing an adolescent’s behavior after a brain injury, together with one of three images portraying a young adolescent with a scar, a head dressing, or neither of these, was given to participants. They were then asked to answer questions relating to prejudice, social interaction, locus of control, and causal attributions. The attributional statements suggested that the character’s behavior could be the result of brain injury or adolescence.

Results: Analysis of variance demonstrated a statistically significant difference between the student groups, where nursing students (M = 45.17, SD = 4.69) desired more social interaction with the fictional adolescent than their computer science peers (M = 38.64, SD = 7.69). Further, analysis of variance showed a main effect of image on the attributional statement that described adolescence as a suitable explanation for the character’s lack of self-confidence.

Discussion: Attributions of brain injury were influenced by the presence of a visible but potentially specious indicator of injury. This suggests that survivors of brain injury who do not display any outward indicator may receive less care and face expectations to behave in a manner consistent with the norms of society. If their injury does not allow them to meet with these expectations, they may face public censure and discrimination.

“It’s like a problem that doesn’t exist”: The emotional well-being of mothers caring for a child with brain injury.

Background: Although research has shown that significant burden and adverse psychological impact are associated with caring for a child with brain injury, limited knowledge exists concerning the qualitative experience and impact of this burden.
Objective: To provide an account of the experiences of mothers who care for a childhood survivor of brain injury.
Research design: Postal survey.
Methods and procedures: A self-report questionnaire was sent to a consecutive sample of mothers (n=86) of children (aged 8-28) with acquired brain injury, registered with a UK children’s brain injury charity. Five essay style questions enabled mothers to reflect on and describe at length their caring experiences, with particular emphasis placed on the perceived impact on emotional well-being.
Main outcomes and results: Thematic analysis identified five key themes: Perpetually Anxious, The Guilty Carer, The Labour of Caring, A Self-Conscious Apologist and Perpetually Grieving. Collectively, these themes highlight two core processes shaping mothers’ caring experiences and concomitant mental well-being. Firstly, the collective and enduring nature of caregiver burden over time. Second, the crucial role played by socio-cultural values in perpetuating caregiver burden.
Conclusions: Societal norms, particularly those relating to the nature and outcome of brain injury and motherhood, serve to marginalise mothers and increase feelings of isolation. Findings suggest the value of peer support programs as an effective means of providing appropriate social support.

Towards a Mobile Assistive Technology for Monitoring and Assessing Cognitive Fatigue in Individuals with Acquired Brain Injury

Those living with an acquired brain injury often have issues with fatigue due to factors resulting from the injury. Cognitive impairments such as lack of memory, concentration and planning have a great impact on an individual’s ability to carry out general everyday tasks, which subsequently has the effect of inducing cognitive fatigue. Moreover, there is difficulty in assessing cognitive fatigue, as there are no real biological markers that can be measured. Rather, it is a very subjective effect that can only be diagnosed by the individual. Consequently, the traditional way of assessing cognitive fatigue is to use a self-assessment questionnaire that is able to determine contributing factors. State of the art methods to evaluate cognitive! fa tigue employ cognitive tests in order to analyse performance on predefined tasks. However, one primary issue with such tests is that they are typically carried out in a clinical environment, therefore do not have the ability to be utilized in situ within everyday life. This paper presents a smartphone application for the evaluation of fatigue, which can be used daily to track cognitive performance in order to assess the influence of fatigue.