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Abstract.Introduction: Huntington disease (HD) is a progressive neurodegenerative condition characterised by motor, cognitive and behavioural dysfunction, and has an autosomal dominant mode of inheritance. As there is currently no treatment to delay progression of the disease, pharmacological intervention is aimed at symptomatic relief. Methods: We set out to assess the current evidence on the pharmacological treatment of motor and non-motor symptoms in HD by carrying out a systematic literature review across five large scientific databases. Results: The search generated 23 original studies meeting our search criteria. Studies on the following drug classes were obtained: dopamine (DA) depleting agents, neuroleptics, anti-glutamatergic agents, acetylcholinesterase inhibitors, GABA agonists, cannabinoids, antidepressants and potential neuroprotective agents. Tetrabenazine (TBZ), a DA depleting agent, was the only pharmacotherapy shown to have a clinically meaningful, statistically significant effect on chorea. The majority of the reviewed studies focussed on the treatment of motor symptoms of HD. Discussion: Overall, the evidence base for the pharmacological management of HD is poor. There is a clear need for future high quality randomised controlled trials on the symptomatic treatment of HD, particularly on the pharmacotherapy of non-motor symptoms of HD.

Background: A subset of individuals with autism spectrum disorder (ASD) resemble descriptions of extreme/ 'pathological' demand avoidance, displaying obsessive avoidance of everyday demands and requests, strategic or 'socially manipulative' behaviour and sudden changes in mood. Investigating challenging presentations using dimensional description may prove preferable to identifying subgroups. However, there remains an imperative to explore which behavioural traits appear most problematic to inform quantitative investigation. This study provides an in-depth exploration of parent perspectives on maladaptive behaviour in children reported to have an autism spectrum diagnosis and features of extreme/'pathological' demand avoidance. Method: Parents completed a tailored semistructured interview about their child's behaviour, focusing on difficulties relevant to descriptions of extreme/'pathological' demand avoidance. The 26 interviews rated as scoring above threshold for 'substantial' features of extreme/'pathological' demand avoidance on relevant indicators were analysed qualitatively using a general inductive approach. Results: New themes that emerged from these data included attempts by the child to control situations and others' activities. Avoidance behaviours in this sample could be described as 'strategic' rather than 'manipulative'. A range of factors, including a negative emotional response to demands, but also phobias, novelty, and uncertainty, were perceived to play a role in triggering extreme behaviour. Conclusions: These descriptions highlight the importance of systematically measuring noncompliance, attempts to control situations and others' activities, and extreme mood variability in individuals with ASD. These dimensions represent important targets for intervention, given their considerable impact on daily life. Key Practitioner Message• Children with ASD who resemble descriptions of extreme/'pathological' demand avoidance obsessively avoid complying with everyday demands, engage in apparently 'manipulative' behaviour and exhibit sudden changes in mood. We collected interview data from parents of children with ASD (parent-reported) and these features about their child's maladaptive behaviour.• Subtle differences emerged compared to previous accounts of this profile. These included additional problems relating to the child's frequent attempts to control situations and others' activities (e.g. by insisting that others abide by their rules or meet their demands). In our sample, avoidance behaviours could be described as 'strategic' rather than 'manipulative'. Parents perceived that a range of factors played a role in triggering extreme behaviour, including a negative emotional response to perceived pressure (in line with previous accounts), as well as sensory sensitivities, phobias and anxiety about the unknown.• These findings add to the literature on extreme noncompliance, mood variability and attempts to control situations and others' activities in ASD, and they motivate examination of similarities and...

BackgroundThe practice of evidence-based medicine and critical appraisal are essential for the modern doctor. Early engagement of medical students in research methodology is considered as a rising need for most medical school curricula; however, few peer-reviewed initiatives have been reported so far. We developed a Medical Education Research Group (eMERG) as part of a novel undergraduate surgical masterclass, which aimed to train undergraduate students on basic research methodology, as well as to motivate them to pursue a clinical and academic career in surgical specialties.Methods: eMERG consists of an international structured network of senior academics, consultant-level clinicians, senior and junior trainees who support undergraduate trainees. Students are selected from a competitive pool of applicants. Several small prospective studies in skills-based education, as well as systematic reviews on similar topics, have run under the umbrella of this framework, in the form of scholarship awards. Structured feedback questionnaires were distributed to evaluate the experience of the first three years.Results12 students have participated in this pilot initiative. 11 manuscripts have been submitted for publication and 8 were accepted following peer-review in MEDLINE-indexed journals. Delegates perceived this experience as an excellent training opportunity which improved their research productivity. Delegates also stated engagement in research developed interest in the relevant surgical speciality, impacting their career aspirations.ConclusionseMERG is one of the first reported European educational research networks for undergraduates. Research outcomes and students' perceptions conclude that eMERG enhances engagement with research methodology and motivation towards a career in surgery.