Experiences of young people growing up in a family with Huntington's disease: A meta-ethnography of qualitative research.

Huntington's disease is a genetic neurodegenerative condition with wide physical and psychological impacts. Children of a parent with the condition have a 50% chance of carrying the gene expansion and developing the condition themselves. This systematic review and meta-ethnography presents a synthesis of the qualitative research on the experiences of young people growing up in a family with Huntington's disease. The MEDLINE, PsycINFO, and CINAHL databases were systematically searched, and 13 papers met the inclusion criteria. Through the process of meta-ethnography, four themes were identified highlighting aspects of childhood that were stolen and fought for: thief of relationships, thief of self, thief of transparency, and search for reclamation. Within the themes, the complex challenges young people faced when growing up in a HD family were explored such as the impact of adverse childhood experiences and the possible effects of HD on attachment and social relationships. Clinical implications are considered, and recommendations are made for future research.

Guided self-help for anxiety among Huntington's disease gene expansion carriers (GUIDE-HD) compared to treatment as usual: a randomised controlled feasibility trial.

BACKGROUND: Huntington's disease (HD) is an adult-onset genetic neurodegenerative condition associated with cognitive decline, motor impairments, and emotional difficulties. Anxiety affects up to 71% of HD gene expansion carriers (i.e., those with the version of the gene that causes HD) and can negatively impact quality of life, worsen other HD symptoms, and increase suicide risk. Therefore, helping people with their anxiety should be a clinical priority. A significant evidence base now exists for low-cost talking therapies for anxiety, such as guided self-help, and with people with other neurodegenerative conditions (e.g., Parkinson's disease). However, this type of intervention has not been specifically assessed with HD gene expansion carriers. METHODS: This protocol describes an exploratory randomised controlled feasibility study of a psychological intervention for anxiety for HD gene expansion carriers. The 10 session guided self-help intervention ('GUIDE-HD') is based on a blend of second and third wave cognitive behavioural models of anxiety (cognitive behaviour therapy [CBT] and acceptance and commitment therapy [ACT]) and is adapted to meet the specific needs of an HD population. This study will compare guided self-help with treatment as usual (TAU), with 15 HD gene expansion carriers randomly allocated to each group. Participants will be recruited across the UK. Quantitative data will be collected pre-intervention, immediately post-intervention, 3-month post-intervention and 6-month post-intervention. Qualitative data will be collected at one month post-intervention from participants, including HD carers. The data will be analysed to assess whether the current intervention and study design are feasible to progress to a larger randomised controlled trial. Feasibility has been defined in terms of recruitment rate, retention rate to both trial arms, intervention adherence, and acceptability of the intervention and measurement tools. DISCUSSION: Given the lack of evidenced interventions to date to support the wellbeing of people with the expanded Huntington's gene, this study will assess the feasibility of progressing this particular intervention to a full trial. To try and increase the acceptability of the intervention, a number of stakeholders, including those affected by HD and in caring roles, have been fundamental to the creation of the intervention (e.g., therapy manual, planned therapy process) to date. TRIAL REGISTRATION: Trial ID: ISRCTN47330596 . Date registered: 28/09/2022. Protocol version and date: Version 2, 09/06/22. Trial sponsor organisation and contact: Leicestershire Partnership NHS Trust (Dave Clarke). Role of sponsor: Overall responsibility for the conduct and governance of the trial. Role of funder: Review of initial research proposal.

Mental health symptoms among those affected by Huntington's disease: A cross-sectional study.

BACKGROUND: Although cognitive and motor symptoms of Huntington's disease (HD) are associated with disease progression, the underlying causes of psychological symptoms are not as clearly understood. Recent evidence suggests that some mental health difficulties experienced by people with HD are shared by noncarriers within HD families. Accordingly, there is a need to evaluate potential systemic contributors to HD mental distress, to support meaningful interventions for psychological symptoms in people with HD and affected families. METHOD: We used short-form Problem Behaviors Assessment mental health symptom data from the international Enroll-HD data set to characterize mental health symptoms across eight HD groups: Stages 1-5, premanifest and genotype-negative individuals, and family controls (n = 8567) using chi-square analysis with post hoc comparisons. RESULTS: We identified that people with later-stage HD (Stages 2-5) had significantly higher apathy, obsessive-compulsiveness, and (from Stage 3) disorientation than the remaining groups at a medium effect size, and that these findings largely held across three measure administrations over time. CONCLUSIONS: These findings highlight the critical symptoms in manifest HD from Stage 2 onward, but also demonstrate that crucial symptoms such as depression, anxiety, and irritability are present across HD-affected groups (including noncarriers of the gene expansion). The outcomes highlight a need for specific clinical management of later-stage HD psychological symptoms, and for systemic support across affected families.

Using a Clinical Formulation to Understand Psychological Distress in People Affected by Huntington's Disease: A Descriptive, Evidence-Based Model.

Huntington's disease (HD) is an inherited, life-limiting neurodegenerative condition. People with HD experience changes in cognitive, motor and emotional functioning, and can also, mainly at later stages, exhibit behaviours that professionals and carers might find distressing such as hitting others, throwing objects, swearing or making inappropriate comments. While clinical formulation (an individualised approach used by mental health professionals to describe an individual's difficulties) is a helpful tool to conceptualise patients' wellbeing, a specific formulation framework has not yet been developed for HD. However, evidence has shown that formulation can help guide clinical interventions and increase consistency of approach across multi-disciplinary teams, refine risk management, and improve staff or carers' empathic skills and understanding of complex presentations. As a consequence, this paper proposes a new clinical formulation model for understanding distress among people with HD, based on a biopsychosocial framework. More specifically, this includes key elements centring on an individual's past experience and personal narratives, as well as anticipatory cognitions and emotions about the future. In-depth discussions regarding the components of the model and their importance in HD formulations are included, and a fictional yet representative case example is presented to illustrate their application within the context of personalised care.

More than Just a Brain Disorder: A Five-Point Manifesto for Psychological Care for People with Huntington's Disease.

Huntington's disease (HD) is a rare and complex condition where affected individuals, family members, caregivers, and clinicians face a number of both long-term and fluctuating challenges. The predominant biomedical framework adopted in HD to date has traditionally viewed it as a brain disorder first and foremost. As a consequence, one of the most challenging aspects of the condition-psychological difficulties and their care-is often not given the emphasis it deserves in everyday clinical practice. Here, we propose a manifesto outlining five points to address the quality, effectiveness, availability, and accessibility of psychological care in HD. These include (1) Listening to People with HD, (2) Reformulating Difficulties Psychologically, (3) Exploring New Interventions, (4) Increasing Psychological Provision, and (5) Learning from Other Conditions. As the search for a cure continues, we hope that this manifesto will create a new impetus towards refining the current approach to psychological difficulties in HD and ultimately improve the quality of life of the tens of thousands of families affected by HD worldwide.

Adjusting to living with Parkinson's disease; a meta-ethnography of qualitative research.

PURPOSE: Parkinson's disease (PD) is a condition which causes significant difficulties in physical, cognitive and psychological domains. It is a progressive condition which people have to live with for a long time; consequently, there is a need to understand what contributes to individual adjustment. This review aimed to answer the question "how do individuals adjust to PD?" METHOD: A systematic search of three databases (MEDLINE, CINAHL and PsycINFO) was carried out of papers documenting the adjustment process when living with PD and the findings were synthesised using a meta-ethnographic approach. RESULTS: After exclusion based on eligibility criteria, 21 articles were included and were assessed for quality prior to analysing the data. Three main themes are proposed relating to the process of adjustment: "maintaining a coherent sense of self", "feeling in control" and "holding a positive mindset". Although many of the studies described challenges of living with PD, the results are dominated by the determination of individuals to self-manage their condition and maintain positive wellbeing. CONCLUSION: The results highlight the need to empower patients to self-manage their illness, mitigating the effects of Parkinson's disease and supporting future wellbeing.IMPLICATIONS FOR REHABILITATIONIndividual identity disruption impacts on the self-value and sense of self coherence in individuals living with Parkinson's disease.Healthcare professionals should appreciate the complexity of the adjustment process which is related to the ability to maintain a coherent sense of self, to feel in control and to hold a positive mindset.Healthcare professionals should ensure information and knowledge related to self-management is tailored to an individual's understanding and experience of the disease.

The experience of a sample of individuals in the United Kingdom living in the pre-manifest stage of Huntington's disease: An interpretative phenomenological analysis.

This paper explores the experience of ten individuals living in the UK who were in the pre-manifest stage of Huntington's disease (HD), a genetic neurodegenerative condition. Data were gathered using semi-structured interviews and analyzed using interpretative phenomenological analysis. Three themes were reported from the data: 'feeling limited by time', 'the perception of stalling time', and 'making the most of time', all highlighting the way in which time holds significant meaning when living in the pre-manifest stage of HD. This study has highlighted the difficulties experienced by individuals when adjusting to the pre-manifest stage of HD. Feeling able to manage their anxieties and dealing with ongoing uncertainty related to future deterioration was key to supporting their wellbeing, facilitated by factors such as positivity and hope. Individuals may benefit from counseling which supports them to develop proactive coping strategies to manage their anxieties and acceptance of an uncertain future.

Psychosocial work factors and sick leave risk after a terrorist bomb attack: a survey and registry-based longitudinal study of governmental employees in Norway.

OBJECTIVES: Studies show that social support may reduce the negative psychological effects of terror. The aim was to explore the effects of the psychosocial work environment on sick leave risk among governmental employees after a workplace bomb attack. DESIGN: We linked longitudinal survey data collected at 10 and 22 months after the bombing with registry data on doctor-certified sick leave collected from 42 months before the attack to 33 months after the attack. ORs and rate ratios were estimated with mixed effects hurdle models. SETTING: The bombing of the government ministries in Oslo, Norway, 22 July 2011. PARTICIPANTS: We identified 1625 participants from a cohort of 3520 employees working in the ministries during the bombing in 2011. RESULTS: After adjustment for confounders, social support from coworkers reduced the odds of sick leave (OR 0.80, 95% CI 0.68 to 0.93), and there was marginal evidence for reduced odds with support from superior (OR 0.87, 95% CI 0.87 to 1.03). A social work climate, an innovative climate and a human resource primacy climate (HRP) reduced the sick leave risk (eg, HRP OR 0.77, 95% CI 0.66 to 0.90). The hurdle model found no associations between psychosocial support at work and the duration of sick leave. CONCLUSIONS: Psychosocial support at work can enhance employees' work ability after terror and reduce the sick leave risk by more than 20%. However, a supportive psychosocial work environment did not reduce the duration of sickness absence. The protective role of psychosocial work factors on sick leave may be most significant when employees are at work and interact with their work environment.

Literature Review: Psychological Resilience Factors in People with Neurodegenerative Diseases.

BACKGROUND: Psychological distress is frequently observed in neurodegenerative diseases (NDDs) having a significant impact on function, quality of life (QOL), caregiver burden, and illness cost. As higher psychological resilience can protect against negative outcomes and aid in the successful adjustment to illness, identifying resilience factors is important. Understanding what enhances or lowers psychological resilience allows us to offer support/interventions to people with NDDs and their carers starting early in the disease process. OBJECTIVE: To investigate factors associated with psychological resilience in NDDs by completing a systematic review of relevant studies. METHOD: Five electronic databases were searched for studies relating to psychological resilience in NDDs. Eighteen articles were reviewed using a narrative synthesis approach. RESULTS: Studies with varied aims and methodologies were found. The identified factors were categorized into core, internal, and external resilience. Regarding core factors contradicting evidence was found about the relationship between resilience and physical function. Fatigue, however, appears to be associated with less resilience. A limited number of studies focused on pain and demographics. Of internal resilience factors, studies found positive associations between higher resilience and improved mental health, even over time. Resilience appears to correlate positively with various psychological and QOL factors. Importantly, external resilience factors (e.g., social connectedness and intervention) were discovered to be linked to resilience. The evidence for resilience-enhancing intervention suggests that resilience can be modified. CONCLUSION: Various factors were identified to be associated with psychological resilience in NDDs. As resilience appears to be modifiable, it is important to focus on resilience-enhancing interventions for people with NDDs.

Is perceived safety and threat after workplace terrorism linked to employee sick-leave? A registry-based longitudinal study of governmental employees in Norway.

BACKGROUND: A large body of research has shown that terrorism enhances fears and undermines perceived safety in a high proportion of both directly exposed individuals and individuals without any form of direct exposure (i.e. no geographical proximity to an attack). Some studies have further suggested that fear of terrorism may adversely affect health in those without direct exposure and that this may constitute an important public health burden because of the number who are indirectly exposed. Limited studies have investigated threat and safety perception after workplace terrorism and the possible consequences for employee health. OBJECTIVE: To explore whether perceived safety and threat in employees whose workplace was subjected to a terrorist attack are associated with subsequent sick-leave. METHOD: A longitudinal questionnaire survey on governmental employees' perceived safety and threat at work one (T1) and two (T2) years after the 2011 terrorist attack on the Norwegian ministries was linked to registry data on doctor-certified sick-leave for two 9-month periods following T1 and T2 (N = 1703). RESULTS: There was fairly strong evidence (0.004 < p < 0.034) that higher perceived safety was associated with a close to 30% reduction in sick-leave in fully adjusted models which included terror exposure and symptom-based PTSD. There was inconclusive evidence that lower perceived threat was associated with reduced sick-leave in the full models. CONCLUSIONS: Reduced perceived safety in employees following workplace terrorism may have adverse health consequences of public health significance given how prevalent this perception seems to be. The study supports that post-terrorism response plans should include strategies on how to address the potentially large number of individuals suffering ill health after terror even if they were not directly exposed and do not meet criteria for PTSD.

Antecedentes: Una gran cuerpo de investigación ha mostrado que el terrorismo incrementa el miedo y disminuye la percepción de seguridad en gran proporción tanto en individuos directamente expuestos como también en individuos sin ninguna forma de exposición directa (es decir, sin ninguna proximidad geográfica al ataque). Algunos estudios han sugerido que el miedo al terrorismo puede afectar en forma negativa la salud de aquellos sin exposición directa y que esto puede constituir una importante carga en la salud pública, debido al número de personas expuestas indirectamente. Un limitado número de estudios han investigado la percepción de amenaza y seguridad después de terrorismo en el lugar de trabajo y sus posibles consecuencias para la salud de los empleados.Objetivo: Explorar si es que la seguridad y amenaza percibida en empleados en los cuales su lugar de trabajo fue sujeto a un acto terrorista están asociadas con permiso de ausencia por enfermedad.Método: Se conectaron los resultados de un cuestionario longitudinal sobre la seguridad y amenaza percibida en empleados gubernamentales al primer (T1) y segundo (T2) año posterior al ataque terrorista de los ministerios noruegos del año 2011 con los datos registrados de permiso de ausencia por enfermedad certificados por un médico en dos períodos de nueve meses posteriores a T1 y T2 (N=1703)Resultados: Se encontró evidencia bastante fuerte (0.004< p < 0.034) que mayor seguridad percibida se asoció con una reducción cercana al 30 por ciento en permiso por enfermedad en modelos completamente ajustados que incluían exposición al terror y TEPT basado en síntomas. Hubo evidencia poco concluyente de que menos percepción de la amenaza fue asociada con disminución en permiso por enfermedad en los modelos completos.Conclusiones: La percepción reducida de seguridad en empleados posterior a terrorismo en el lugar de trabajo puede tener consecuencias de salud adversas de relevancia para la salud pública, debido a lo prevalente que es esta percepción. Este estudio apoya la tesis de que los planes de respuesta post-terrorista debiesen incluir estrategias sobre cómo lidiar con el número potencialmente grande de personas que sufren problemas de salud después del terror, incluso si no estuvieron expuestos directamente y no cumplen con los criterios para TEPT.

Psychological Interventions for People with Huntington's Disease: A Call to Arms.

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.

Assessing Mental Health Difficulties of Persons With Huntington's Disease: Does Informant Presence Make a Difference?

OBJECTIVE: Persons with Huntington's disease (HD) often have communication difficulties and cognitive impairments, making mental health assessment difficult. Informants close to the patient are often included in assessments. The authors investigated effects of informant presence during assessment of persons with HD. METHODS: Data for four subsamples from ENROLL-HD were examined: manifest for HD (N=4,109), premanifest (N=1,790), genotype negative (N=1,041), and family members with no genetic risk (N=974). Assessment interviews with and without an informant present were identified, and the subsamples were compared on three subscales of the short-form Problem Behaviors Assessment: affect, apathy, and irritability. Differences in scores between participant-only and informant-present interviews were examined via multiple regression, controlling for demographic, disease-related, and individual confounds. RESULTS: Significant differences in apathy and irritability scores were found between participant-only and informant-present conditions for the premanifest, manifest, and genotype-negative subsamples. Affect subscale scores were not influenced by informant presence. When the analysis controlled for confounds, informant presence significantly increased irritability scores in the manifest, and genotype-negative groups and significantly increased apathy scores in the manifest group. CONCLUSIONS: Apathy may have been systemically underreported in participant-only interviews, which supports previous findings that persons with HD underreport mental health symptoms. When an informant was present, irritability scores were higher for both HD and non-HD individuals, suggesting that underreporting via self-report may be attributable to non-HD factors. Informant contributions to apathy assessments may be particularly important for persons with HD. Clinicians should note potential underreporting regarding irritability and affect, which was not remediated by informant presence.

Post-traumatic stress reactions and doctor-certified sick leave after a workplace terrorist attack: Norwegian cohort study.

OBJECTIVE: To explore the association between the psychosocial work environment and the risk of sick leave among governmental employees with symptom-defined post-traumatic stress disorder (PTSD) after a workplace bomb attack. DESIGN: A prospective study on employees who met the symptom criteria for PTSD. Questionnaire data on the psychosocial work environment 10 months after the terrorist attack was linked to registry data on doctor-certified sick leave in the period 12-22 months after the attack. SETTING: The bombing of the government ministries in Oslo, Norway, 22 July 2011. PARTICIPANTS: The study sample consists of 94 Norwegian governmental employees, all with symptom-defined PTSD from the Norwegian version of the PTSD checklist (Post-traumatic Stress Disorder Checklist-Specific) measured 10 months after the attack. RESULTS: After adjustment for sex and severity of PTSD symptoms, predictability at work reduced the odds of sick leave (adjusted OR=0.62, 95% CI 0.40 to 0.98). Sense of control over decisions at work was associated with fewer absence days for employees with sick leave (adjusted rate ratio=0.61, 95% CI 0.38 to 0.98). CONCLUSIONS: Employees with PTSD after workplace terrorism would benefit from control over their workplace conditions and increased predictability to reduce the risk of sick leave. The findings suggest that the work environment can facilitate employees' work ability after stressful events, independent of severity of PTSD symptoms.

Validity of irritability in Huntington's disease: A scoping review.

PURPOSE: 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.

Intake of sucrose-sweetened soft beverages during pregnancy and risk of congenital heart defects (CHD) in offspring: a Norwegian pregnancy cohort study.

Studies report increased risk of congenital heart defects (CHD) in the offspring of mothers with diabetes, where high blood glucose levels might confer the risk. We explored the association between intake of sucrose-sweetened soft beverages during pregnancy and risk of CHD. Prospective cohort data with 88,514 pregnant women participating in the Norwegian Mother and Child Cohort Study was linked with information on infant CHD diagnoses from national health registers and the Cardiovascular Diseases in Norway Project. Risk ratios were estimated by fitting generalized linear models and generalized additive models. The prevalence of children with CHD was 12/1000 in this cohort (1049/88,514). Among these, 201 had severe and 848 had non-severe CHD (patent ductus arteriosus; valvular pulmonary stenosis; ventricular septal defect; atrial septal defect). Only non-severe CHD was associated with sucrose-sweetened soft beverages. The adjusted risk ratios (aRR) for non-severe CHD was 1.30 (95% CI 1.07-1.58) for women who consumed 25-70 ml/day and 1.27 (95% CI 1.06-1.52) for women who consumed ≥ 70 ml/day when compared to those drinking ≤ 25 ml/day. Dose-response analyses revealed an association between the risk of non-severe CHD and the increasing exposure to sucrose-sweetened soft beverages, especially for septal defects with aRR = 1.26 (95% CI 1.07-1.47) per tenfold increase in daily intake dose. The findings persisted after adjustment for maternal diabetes or after excluding mothers with diabetes (n = 19). Fruit juices, cordial beverages and artificial sweeteners showed no associations with CHD. The findings suggest that sucrose-sweetened soft beverages may affect the CHD risk in offspring.

Association of light-to-moderate alcohol drinking in pregnancy with preterm birth and birth weight: elucidating bias by pooling data from nine European cohorts.

Women who drink light-to-moderately during pregnancy have been observed to have lower risk of unfavourable pregnancy outcomes than abstainers. This has been suggested to be a result of bias. In a pooled sample, including 193 747 live-born singletons from nine European cohorts, we examined the associations between light-to-moderate drinking and preterm birth, birth weight, and small-for-gestational age in term born children (term SGA). To address potential sources of bias, we compared the associations from the total sample with a sub-sample restricted to first-time pregnant women who conceived within six months of trying, and examined whether the associations varied across calendar time. In the total sample, drinking up to around six drinks per week as compared to abstaining was associated with lower risk of preterm birth, whereas no significant associations were found for birth weight or term SGA. Drinking six or more drinks per week was associated with lower birth weight and higher risk of term SGA, but no increased risk of preterm birth. The analyses restricted to women without reproductive experience revealed similar results. Before 2000 approximately half of pregnant women drank alcohol. This decreased to 39% in 2000-2004, and 14% in 2005-2011. Before 2000, every additional drink was associated with reduced mean birth weight, whereas in 2005-2011, the mean birth weight increased with increasing intake. The period-specific associations between low-to-moderate drinking and birth weight, which also were observed for term SGA, are indicative of bias. It is impossible to distinguish if the bias is attributable to unmeasured confounding, which change over time or cohort heterogeneity.

Irritability in Huntington's Disease: Factor Analysis of Snaith's Irritability Scale.

BACKGROUND: Elevated levels of irritability are reported to occur in a number of neurological conditions, including Huntington's disease (HD), a genetic neurodegenerative disorder. Snaith's Irritability Scale (SIS) is used within HD research, but no psychometric evaluation of this instrument has previously been undertaken. Therefore, the current study aimed to analyze the factor structure of this scale among an HD population. METHODS: Exploratory and confirmatory factor analysis were used to examine the structural properties of SIS using responses from 1,264 HD gene expansion carriers, across 15 European countries, who were engaged in the REGISTRY 3 study. RESULTS: An exploratory factor analysis of a subsample of the data suggested a two-factor interpretation of the data comprising "temper" and "self-harm." Eight possible models were tested for goodness of fit using confirmatory factor analysis. Two bifactor models, testing general and group factors in the structure of the scale, provided an equivocal "good" fit to the data. The first comprised a general irritability factor and two group factors (as originally proposed using SIS): outward irritability and inward irritability. The second comprised a general irritability factor and two group factors (as proposed by the exploratory factor analysis): temper and self-harm. The findings from both models suggested that the loadings of items were higher on the general factor. CONCLUSIONS: Bifactor models are proposed to best consider the structure of the SIS, with findings suggesting that an overall score should be used to measure irritability within HD populations.

Huntington's Disease: Prevalence and Psychological Indicators of Pain.

BACKGROUND: Huntington's disease (HD) is a genetic neurodegenerative condition that involves impairments in movement, cognition, and mood. Research is lacking in HD with regard to the prevalence of pain and the relationships between psychological factors and pain. The aim of this research was to investigate the prevalence of pain and identify the psychological factors associated with pain severity in people with HD. METHODS: This data-mining study used data from 1474 people who participated in the European Huntington's Disease Network (EHDN) REGISTRY study. Pain severity was measured using the Medical Outcome Study 36-item short-form health survey. Separate ordinal regression analyses were conducted with participant-rated and interviewer-rated psychological measures (the Hospital Anxiety and Depression Scale-Snaith Irritability Scale and the Unified Huntington's Disease Rating Scale). The psychological factors considered were anxiety, depression, irritability, aggression, low self-esteem, and apathy. RESULTS: The prevalence of pain in the total sample was 41% (stage I, 42%; stage II, 44%; stage III, 39%; stages IV and V, 50%). After controlling for confounding variables, pain severity was significantly associated with participant-rated anxiety and depression. Interviewer-rated anxiety, depression, and irritability also were significantly associated with severity of pain after controlling for confounding variables. CONCLUSIONS: This research confirmed that pain is indeed an issue for people with HD, particularly during the later stages of the disease. Caregivers and health professionals should consider the possibility that people with HD might be experiencing pain, particularly if they are showing signs of anxiety, depression, or irritability.

Exploring the Structural Relationship Between Interviewer and Self-Rated Affective Symptoms in Huntington's Disease.

This study explores the structural relationship between self-report and interview measures of affect in Huntington's disease. The findings suggest continued use of both to recognize the multidimensionality within a single common consideration of distress.