Background: Although Huntington’s disease (HD) can cause a wide range of psychological difficulties, no review has ever been carried out on the range of psychological interventions adopted with this population. Objective: To scope the literature on psychological interventions for psychological difficulties in people affected by HD. Methods: A systematic scoping review was performed across MEDLINE, PsycINFO, CINAHL, Academic Search Ultimate, and Cochrane Library up to 1 March 2020. Results: From an initial return of 1579 citations, a total of nine papers were considered eligible for review. These included a qualitative investigation, three case studies, two case series, two uncontrolled pretest-posttest designs, and only one randomised control trial (RCT). Despite the wide range of psychological difficulties which can be experienced by people affected by the HD gene expansion, the adopted interventions only accounted for five main psychological outcomes (anxiety, apathy, depression, irritability, and coping). Further discussion and suggestions for future research are provided for each outcome. Conclusion: The current literature on psychological interventions in people affected by HD is extremely limited both in terms of methods and addressed clinical outcomes. Consequently, no conclusions can be offered yet as to which psychological therapy may help this population. As further more comprehensive research is urgently needed for this group, the ultimate aim of the present review is to act as a call to arms for HD researchers worldwide to help shed light on the most effective way to translate psychological theory into practice for the benefit of people affected by HD.

Sheffield Teaching Hospitals NHS Foundation Trust, UKMotor neuron disease (MND), also known as amyotrophic lateral sclerosis, is a neurodegenerative disorder that causes progressive muscle paralysis and typically leads to death within 3 years. As no cure is currently available, symptomatic management is the mainstay of treatment. An important part of this is optimizing nutritional intake with evidence that this may positively affect survival and quality of life. Health care professionals (HCPs) play a pivotal role in nutritional management of people with MND (pwMND) but, to date, their views on the psychological barriers faced by pwMND have not been explored. Such an exploration may identify ways in which the delivery of nutritional care for pwMND can be optimized. Methods. Five qualitative focus groups were carried out across the United Kingdom in June 2018 with 51 participants, including 47 HCPs involved with MND care and four service user representatives. Data were analysed through thematic analysis.Results. Four overarching themes were identified: psychological adjustment and patient engagement; nutrition and the need for control; knowledge of nutrition and the complexity of MND; and the psychosocial nature of eating.Conclusions. The findings suggest that the nutritional management of pwMND should be mindful of factors such as the impact of distress at the time of diagnosis, the availability of clear information on nutrition and MND, as well as the importance of illness perceptions and coping strategies. Moreover, tailored psychological interventions should be considered to mitigate the impact on MND on the experience of eating.

Objectives This study explored the perspectives of people affected by Huntington's disease (HD) on their own communicative abilities. Methods Qualitative semi-structured interviews were carried out with eight people with early HD. The data were analysed through thematic analysis. Results Four themes were constructed from the data, characterised by the following core topics: How HD directs and mediates communication; Regaining control to improve communication; Emotional outflows into communication and the struggle for separation; Sheltering as a way to boost confidence in communication. Discussion Separating patients' identity as individuals from that of a person with a disease can help increase communicative control. Consistent with the general theory and model of self-regulation, patients should be allowed a wider range of choices to regain control over communication. Achieving better emotion regulation is of paramount importance for communication, and factors such as medication regimes, relationships and existing coping strategies should be strengthened. Consistent with previous research, feelings of safety and the idea of a safe place ('sheltering') represent an effective coping mechanism. Practical implications include the refinement of communication and relationships among clinicians, caregivers, and patients with HD by considering a wider range of medical, psychological and socio-environmental factors.

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To scope the literature concerning irritability in Huntington's disease to determine whether or not irritability is a valid and meaningful construct within this population. Method: A scoping literature review was conducted based on findings from a search of five databases (Academic Search Ultimate, PsycINFO, CINAHL, Scopus and Web of Science) in November 2018. From an initial return of 453 papers, 40 were found suitable for review. Results: Review of the 40 studies highlighted several aspects of irritability in people with HD which influence its validity as an independent construct in context of the disease. While various measures are used to assess irritability, a gold standard has yet to be identified and consequently irritability is assessed inconsistently across the literature. In addition, the results suggest that irritability may not reflect pathological disease processes in HD, but rather comprises a multidimensional construct which appears to be strongly associated with other psychological difficulties such as depression and anxiety. Conclusions: The current concept of irritability in people with HD continues to lack a general consensus in the clinical literature, in terms of both operationalisation and assessment. Consequently, further research is warranted in order to determine the extent to which irritability is a valid construct within the context of HD, including its associated behavioural, cognitive and affective dimensions.

Purpose To explore the heterogeneity of the literature on psychological interventions for psychological difficulties in people with Parkinson’s disease (PD). Methods A scoping review was performed across five major databases (MEDLINE Complete, PsycINFO, CINAHL, Academic Search Ultimate, and Cochrane Library) up to June 2020. Results From an initial return of 4911 citations, 56 studies were included, of which 21 were RCTs. A relatively wide range of therapeutic models have been adopted with people with PD, from common therapies such as cognitive behavioural therapy (CBT) and mindfulness, to less frequent approaches, for example, acceptance and commitment therapy (ACT) and psychodrama. The clinical implications of the findings are discussed, and suggestions are provided for future research on intervention studies and key psychological outcomes. Conclusions CBT appears to be effective in treating depression and sleep disorders in people with PD, while psychoeducation programmes alone should be avoided. The use of CBT to improve anxiety, quality of life, and impulse control, as well mindfulness‐based interventions, should be undertaken with some caution because of insufficient research and inconsistent results. As we enter the new decade, more high‐quality evidence is required for psychological interventions in people with PD in general and to corroborate preliminary positive findings on the adoption of less frequent approaches such as ACT. Practitioner points Parkinson’s disease is a progressive neurodegenerative condition associated with several psychological difficulties which be targeted by psychological interventions. Currently, cognitive behavioural therapy (CBT) can be recommended to treat depression and sleep disorders in people with Parkinson’s, while psychoeducation alone should be avoided. Caution is advised regarding the use of CBT and mindfulness‐based interventions to improve anxiety, quality of life, and impulse control. Further evidence is required for less common approaches, such as acceptance and commitment therapy, psychodrama, and EMDR.

Interest in the role of both emotion regulation and recognition in our understanding of mental health has been steadily increasing, especially in people with chronic illness who also have psychological difficulties. One illness which belongs to this category is Huntington's disease. Huntington's disease (HD) is a chronic neurodegenerative disorder that can cause a number of cognitive and psychological difficulties, including emotion recognition deficits, even before the onset of the symptoms required to make a formal diagnosis. Despite the lack of definite evidence, recent studies have suggested that deficits of emotion regulation and recognition may be expected to play a pivotal role in the early cognitive manifestations of HD. In this study, we hypothesised that the ability to regulate emotions can be impaired in people with presymptomatic HD, and that such impairment may be associated with a deficit of emotion recognition. To test this, an online survey was carried out with 117 English and Italian-speaking people with presymptomatic HD, compared to 217 controls matched for age and education. The results suggest that, in presymptomatic participants, emotion regulation and emotion recognition are generally not significantly impaired, and no significant relationships between performances on the two constructs were observed. However, a specific impairment in emotional awareness (a subscale on the Difficulties in Emotion Regulation Scale, DERS) was observed, which appears to be enhanced by the co-occurrence of depressive symptoms, even at a subclinical level. Consequently, it is suggested that difficulties in emotional awareness may represent a precursor of more general emotion recognition impairments, which only become apparent as the disease reaches a more symptomatic level. Clinical implications of the findings are discussed and directions for future research are proposed.

Dr Nicolò Zarotti is an academic psychologist interested in neuropsychology and clinical and health psychology, with a particular focus on motor neurodegenerative conditions such as Huntington's disease, Parkinson's disease, and motor neuron disease/amyotrophic lateral sclerosis. Some of his other interests include theoretical and practical research around emotion recognition and regulation, as well as perceptions of control. Dr EmilyMayberry works in the NHS as a clinical psychologist in the Sheffield Motor Neuron Disease Care Centre. She also holds a Clinical Research Academy Fellowship at the School for Health and Related Research (ScHARR) and is an Honorary Research Associate at the Sheffield Institute for Translational Neuroscience (SITraN). She is particularly interested in improving psychological assessment and support for people with motor neuron disease and for those who support them. She has previous experience in other neuropsychology specialties and working in adult mental health and adult autism settings. Dr Noora Ovaska-Stafford is a clinical psychologist working in neurological rehabilitation with a specialist interest in psychological resilience in neurodegenerative diseases. Dr Fiona Eccles is an academic and clinical psychologist whose research focuses on the psychological well-being of individuals with neurological conditions, with a particular interest in neurodegenerative conditions such as Huntington's disease, Parkinson's disease and motor neurone disease.