Rumination in dementia and its relationship with depression, anxiety, and attentional biases.

Rumination (self-referential and repetitive thinking), attentional biases (AB), and impaired cognitive control are theorized as being integral factors in depression and anxiety. Yet, research examining the relationship between rumination, mood, and AB for populations with reduced cognitive control, e.g., people living with dementia (PwD), is lacking. To explore whether literature-based relationships are demonstrated in dementia, PwD (n = 64) and healthy controls (HC) (n = 75) completed an online self-report survey measuring rumination and mood (twice), and a telephone cognitive status interview (once). Rumination was measured as an emotion-regulation style, thinking style, and response to depression. We examined the test-retest reliability of PwD's (n = 50) ruminative-scale responses, ruminative-scale internal consistency, and correlations between rumination, age, cognitive ability, and mood scores. Also, nine participants (PwD = 6, HC = 3) completed an AB measure via eye-tracking. Participants fixated on a cross, naturally viewed pairs of facial images conveying sad, angry, happy, and neutral emotions, and then fixated on a dot. Exploratory analyses of emotional-face dwell-times versus rumination and mood scores were conducted. Except for the HC group's reflective response to depression measure, rumination measures were reliable, and correlation strengths between rumination and mood scores (.29 to .79) were in line with literature for both groups. For the AB measure subgroup, ruminative thinking style scores and angry-face metrics were negatively correlated. The results of this study show that literature-based relationships between rumination, depression, and anxiety are demonstrated in dementia, but the relationship between rumination and AB requires further investigation.

Sexual activity (re-)engagement following spinal cord injury: Identifying behavior change targets for intervention development.

OBJECTIVES: This study used the behavior change wheel to identify the priority behavioral factors for a tailored intervention to support (re-)engagement in sexual activity following a spinal cord injury (SCI). RESEARCH METHOD/DESIGN: Forty-eight semistructured interviews were conducted with participants from three outpatient/carer/clinician populations in the United Kingdom: people living with SCI, their partners/spouses, and healthcare professionals working in SCI rehabilitation. To identify potential strategies to facilitate (re-)engagement in sexual activity, the behavior change wheel (BCW) and behavior change technique taxonomy Version 1 were applied to code behavior change techniques (BCTs) present in interview transcripts. RESULTS: Six intervention functions, three policy categories, and 21 BCTs were identified as primary targets for interventions to support sexual (re-)engagement post-SCI. Increasing physical-related skills and training would promote physical capability, while sex-related knowledge and the understanding of sex-related health consequences would elevate psychological capability. A supportive healthcare team, alongside peer support and targeted environmental resources about sexual activity/well-being facilitate physical and social opportunities for sex. Motivation to (re-)engage in sexual activity comprised goal-driven reflective motivation to enhance beliefs about capabilities, and automatic motivation via emotional support and reward-based reinforcement. CONCLUSIONS: This study outlines the key BCW and theoretically-derived intervention targets which now provide the foundation for innovative future interventions in SCI and sexual activity. Targeting these highly specific BCTs increases the likelihood that sexual satisfaction can become universally accessible after SCI. (PsycInfo Database Record (c) 2024 APA, all rights reserved).

Psychotherapy and professional psychological support in multiple sclerosis: Uncovering patients' patterns of access and preferences.

BACKGROUND: Whilst there is research on psychotherapy and professional psychological support (PPS) in people with Multiple Sclerosis (pwMS) in discrete randomised controlled trials, little is known about the different types of PPS pwMS access throughout the trajectory of their illness and their perceived helpfulness. Additionally, research on what pwMS's preferences are with regard to PPS is lacking. METHOD: In an online cross-sectional survey study with 565 pwMS, we asked about the types of PPS pwMS had accessed and their preferences. RESULTS: Although 88% of the sample wanted PPS, only 53% of the sample had managed to access PPS. 40% of the entire sample currently wanted PPS but did not access it previously. The most common reason for this was because they were happy with the support they received from other sources (51%) and/or they were unaware of what was available to them (33%). 59% of those who had accessed PPS had accessed more than one type. The perceived level of helpfulness from PPS was rated as higher amongst those who had accessed more types of PPS. The most common combination of PPS accessed was a mixture of counselling with either cognitive behavioural therapy and/or mindfulness-based therapies. Counselling was the single-most accessed type of PPS. Most pwMS wanted PPS as a proactive means to either preserve and boost psychological well-being (37%) or learn skills to manage future difficulties as and when they arise (23%), rather than as a reaction to immediate pressing concerns (18%). The majority of pwMS showed a broad range of preferences regarding PPS and selected more than 5 types, with high interest in programmes with a self-management component, counselling and mindfulness-based interventions in particular. CONCLUSIONS: Patient preferences for PPS highlight the need to take a proactive and preventative approach to preserve psychological wellbeing rather than only being provided in response to mental health crises. Psychological support should be made more readily available early on to fulfil this presently unmet need.

Sexual function and sexual satisfaction following spinal cord injury: an interpretative phenomenological analysis of partner experiences.

PURPOSE: This study explored how partners/spouses of people with Spinal Cord Injury (SCI) experienced intimacy, sexual function, and sexual satisfaction post-SCI. MATERIALS AND METHODS: Qualitative, semi-structured interviews were conducted with the partners/spouses of people with SCI living in the community in the United Kingdom. Twelve participants (7 females; 5 males) were recruited using purposive sampling. A nine-item semi-structured interview design was used. Interviews were transcribed verbatim and analysed via Interpretative Phenomenological Analysis (IPA). RESULTS: Three superordinate themes were demonstrated: (1) Stolen sex through unpreparedness; (2) Redefining sex; and (3) Compromised commitment. Partners struggled to come to terms with the shock of radically altered post-SCI sexual relationships, questioning how their changed sexual relationship and sexual identity conflicted with caring requirements. To minimise post-SCI relationship changes, some partners engaged in strategies to protect against, distract from and avoid sexual intimacy, whereas others were able to retain adapted pre-injury patterns of intimate behaviour. CONCLUSION: Compromised sexual function and satisfaction significantly disrupt relationship dynamics post-SCI, initiate voluntary celibacy, and limit the perceived viability of continued sexual intimacy. Specialist partner-support provisions are urgently needed, recognising that partner support needs are not restricted to the inpatient rehabilitation phase but importantly extend long past discharge into the community.

Sexual function and satisfaction are highly challenging areas for partners post-spinal cord injury (SCI).Support and education for the partner must be incorporated into SCI rehabilitation to help with both adjustment and acceptance to changes in sexual functioning post-SCI.Partner contributions to and experiences of sexual function and satisfaction should be given more value, as these aid in the sustainability of healthy sexual relationships and psychological wellbeing following SCI.

Internet-facilitated interventions for informal caregivers of patients with neurodegenerative disorders: Systematic review and meta-analysis.

Objective: This systematic review explored the effectiveness of internet-delivered interventions in improving psychological outcomes of informal caregivers for neurodegenerative-disorder (ND) patients. Methods: We searched seven databases for English-language papers published from 1999 to May 2021. Study-eligibility required that interventions used a minimum 50% internet-facilitation, targeting unpaid, adult informal caregivers of community-based ND-patients. We included randomised controlled trials (RCTs) and pre-post evaluative studies reporting outcomes for at least one-time point post-intervention. Independent quality checks on abstract and full-text screening were completed. Data extraction encompassed interventions' features, approaches, theoretical bases and delivery-modes. The Integrated quality Criteria for the Review Of Multiple Study designs (ICROMS) framework assessed risk of bias. Alongside narrative synthesis, we calculated meta-analyses on post-intervention using outcome measures from at least two RCTs to assess effectiveness. Results: Searches yielded 51 eligible studies with 3180 participants. In 48 studies, caregivers supported a dementia-diagnosed individual. Intervention-durations encompassed four weeks to 12 months, with usage-frequency either prescribed or participant-determined. The most frequently-used approach was education, followed by social support. We calculated meta-analyses using data from 16 RCTs. Internet-delivered interventions were superior in improving mastery (g = 1.17 [95% CI; 0.1 to 2.24], p = 0.03) and reducing anxiety (g = -1.29 [95% CI; -1.56 to -1.01], p < 0.01), compared to all controls. Findings were equivocal for caregivers' quality of life, burden and other outcomes. High heterogeneity reflected the multifarious combinations of approaches and delivery-modes, precluding assessment of the most efficacious intervention features. Analyses using burden and self-efficacy outcomes' follow-up data were also non-significant compared to all comparator-types. Although 32 studies met the ICROMS threshold scores, we rated most studies' evidence quality as 'very-low'. Conclusions: This review demonstrated some evidence for the efficacy of internet-delivered interventions targeting informal ND-caregivers. However, more rigorous studies, with longer follow-ups across outcomes and involving NDs other than dementia, are imperative to enhance the knowledge-base.

Supporting Sexual Functioning and Satisfaction During Rehabilitation after Spinal Cord Injury: Barriers and Facilitators Identified by Healthcare Professionals.

OBJECTIVE: To explore healthcare professionals' perspectives on the barriers and facilitators impacting provision of support for sexual functioning/satisfaction during spinal cord injury rehabilitation. DESIGN: Qualitative, semi-structured interview design. METHODS: Sixteen healthcare professionals working in spinal cord injury rehabilitation settings were recruited (14 females, 2 males). Semi-structured interviews were conducted using a 9-item interview guide. Interviews were transcribed verbatim and inductively analysed following Braun and Clarke's (2006) 6 phases of thematic analysis. RESULTS: Five inductive themes were generated describing healthcare professional-perceived barriers and facilitators impacting upon care delivery postspinal cord injury: (1) Integrating sexual wellbeing in rehabilitation; (2) Sex-informed multi-disciplinary teams; (3) Acknowledging awkwardness; (4) Enhancing approachability; and (5) Recognizing the partner. CONCLUSION: Sexual functioning and satisfaction are priority areas for rehabilitation, yet they are persistently side-lined in multi-disciplinary team (MDT) rehabilitation agendas. Healthcare professionals do not feel supported to engage with their patients to improve and manage sexual functioning/satisfaction. Ensuring that healthcare professionals are equipped and made aware of sexuality-specific guidelines and operational frameworks, which can be easily interpreted, structured and implemented as a standard part of spinal cord injury rehabilitation is key. This would be instrumental in enabling healthcare professionals to be more informed and comfortable in creating an atmosphere in which sexual topics can be openly discussed to support individuals with spinal cord injury.

Uncovering patterns of real-world psychological support seeking and the patient experience in multiple sclerosis.

BACKGROUND: With the rate of psychological disorder being disproportionately high in people with Multiple Sclerosis (pwMS), it is important that they receive adequate psychological support. While there are informal and more formal sources of psychological support for pwMS available, there is a paucity of research in understanding the actual pattern of support that pwMS interact with in a real-world context. We aimed to understand this by examining the pattern of access across different sources of psychological support in the same large cohort of pwMS, and their experiences of support received. We also explore this in the context of different MS symptom profiles and demographics. METHOD: In an online survey, we asked 565 pwMS to report on their actual pattern of usage and their experience of receiving psychological support from four key sources - friends/family/peers, MS organisations/charities, MS specialist nurses and mental health professionals. Demographic and clinical data was also gathered about their MS profile and symptoms. RESULTS: Friends/family/peers were rated as the most common, helpful and easy to access source of psychological support. However, most participants received psychological support from multiple sources, almost always in conjunction with support from friends/family/peers. Demographic and MS related factors predicted whether patients accessed each source or not. Younger pwMS and those more recently diagnosed were more likely to avail of support from friend/family/peers. The more patients were bothered by their symptoms the more likely they were to avail of psychological support from more sources. In particular, pwMS who are more bothered by fatigue and psychological symptoms were more likely to avail of support from mental health professionals. Overall, the helpfulness of support depended largely on how well the support provider knew the pwMS as a person and MS as a condition, as well as the level of emotional and practical skills support provided. CONCLUSION: People with MS need to access multiple sources of support to meet the full spectrum of psychological needs as and when needed; friends/family/peers and mental health professionals for emotional support, and MS organisations/charities and specialist MS nurses for learning skills to manage their MS. This points towards the need to take a collaborative approach amongst the different sources of support to ensure all needs can be most effectively met.

"Data is the new oil": citizen science and informed consent in an era of researchers handling of an economically valuable resource.

As with other areas of the social world, academic research in the contemporary healthcare setting has undergone adaptation and change. For example, research methods are increasingly incorporating citizen participation in the research process, and there has been an increase in collaborative research that brings academic and industry partners together. There have been numerous positive outcomes associated with both of these growing methodological and collaborative processes; nonetheless, both bring with them ethical considerations that require careful thought and attention. This paper addresses the ethical considerations that research teams must consider when using participatory methods and/or when working with industry and outlines a novel informed consent matrix designed to maintain the high ethical standard to which academic research in the healthcare arena has traditionally adhered.

The COVID-19 Pandemic Experience in Multiple Sclerosis: The Good, the Bad and the Neutral.

INTRODUCTION: While the current COVID-19 pandemic has affected the lives of many, there is a paucity of information on the impact on people with multiple sclerosis (MS). This study sought to gain insight into the impact of the current situation on people with MS and the factors that influence this. METHODS: 324 MS patients participated in this online cross-sectional survey during the COVID-19 lockdown period. A mixed methods design was used, with quantitative information collected on MS-related factors as well as COVID-19 impact and an open-ended, qualitative response looking for reasons behind the self-reported COVID-19 impact. RESULTS: We found that 48% of the participants reported that COVID-19 had a neutral impact on their lives and 16% reported a positive impact. However, 36% reported a negative impact, and had greater levels of MS- and non-MS-related worries, and higher levels of bother related to psychological and cognitive symptoms and fatigue than the groups reporting a neutral or positive impact. Significant predictors of this adversely affected group were age, type of MS and presence of psychological symptoms. Antidepressant medication use, time since diagnosis, gender, location, living arrangements or employment status did not predict impact. Open-ended responses explaining personal COVID-19 impact indicate that coping strategies may contribute to these findings. In particular, active, problem-focused approaches were reported by the majority of people who reported a positive impact, as well as a third of those who reported a neutral impact. CONCLUSION: These findings suggest that younger people, those with progressive types of MS, and those with psychological symptoms are particularly vulnerable to the negative effects of a COVID-19 pandemic induced lockdown. Coping strategies provide further insight into these findings with those reporting active problem-focused approaches seemingly faring better than those who do not state any coping strategies. These results also have implications for understanding other like neurological conditions that share many similarities with MS and how best to direct support.

People with Multiple Sclerosis (MS) are more vulnerable to psychological disorders such as anxiety. Consequences of the COVID-19 pandemic could potentially heighten these levels due to the impact on health and healthcare. An online survey was completed by 324 people with MS during the COVID-19 pandemic to identify the level of COVID-19 impact (positive, neutral or negative) and the reasons why they felt this way. Findings show that just under half of people who completed the survey reported a neutral impact, with a negative impact reported by just over a third of participants. Having a progressive diagnosis of MS, being younger and having psychological symptoms predicted a negative impact. Coping strategies also appear to play a part in the COVID-19 experience for people with MS, with active, problem-focused strategies being an approach used by nearly all who reported a positive impact and over a third of people who reported a neutral impact. These findings can be used to direct support to the most vulnerable people within this population as well as those with other neurological conditions.

An International Validation of a Clinical Tool to Assess Carers' Quality of Life in Huntington's Disease.

Family carers of individuals living with Huntington's disease (HD) manage a distinct and unique series of difficulties arising from the complex nature of HD. This paper presents the validation of the definitive measure of quality of life (QoL) for this group. The Huntington's Disease Quality of Life Battery for Carers (HDQoL-C) was expanded (n = 47) and then administered to an international sample of 1716 partners and family carers from 13 countries. In terms of the psychometric properties of the tool, exploratory analysis of half of the sample demonstrated good internal consistency and reliability. Some items on the full version did not meet psychometric thresholds and a short version (HDQoL-Cs) (n = 23) was developed based on more stringent criteria. This was achieved using standard psychometric item reduction techniques to both increase reliability and reduce the burden of carers completing the scale. Confirmatory factor analysis of the model structure showed a good fit for all factors and indicated that the HDQoL-C and HDQoL-Cs are psychometrically robust measures of QoL. We found that carers who lived with and looked after their spouse/partner had reduced sense of coping, hope for the future, and overall QoL. Carers with children who were at risk carried the gene or were symptomatic also had poorer QoL outcomes. Findings indicated the HDQoL-C and HDQoL-Cs are valid in multiple languages and across varied cultures as measures of self-reported QoL in family carers of individual's living with HD. These psychometrically validated tools can aid and guide the implementation of therapeutic interventions to improve life quality in this population and research into international and cross-cultural carer experiences. The HDQoL-Cs is recommended as the definitive international measure of HD carer QoL.

Meaningful and Measurable Health Domains in Huntington's Disease: Large-Scale Validation of the Huntington's Disease Health-Related Quality of Life Questionnaire Across Severity Stages.

BACKGROUND: Although health-related quality of life is key for patients with long-term neurodegenerative conditions, measuring this is less straightforward and complex in Huntington's disease (HD). OBJECTIVES: To refine and validate a fully patient-derived instrument, the Huntington's Disease health-related Quality of Life questionnaire (HDQoL), and to elucidate health domains that are meaningful to patients' lived experience. METHODS: Five-hundred forty-one participants, from premanifest to end-stage disease, completed the HDQoL, together with generic quality-of-life measures and in-person motor, cognitive, and behavioral assessments. The psychometric properties of the HDQoL were examined using factor analysis and Rasch analysis. RESULTS: Four HDQoL domains emerged, reflecting the classical triad of HD features; they were Physical-Functional, Cognitive, and 2 different behavioral aspects, that is, the Mood-Self domain and a distinct Worries domain. These domains clarify the behavioral sequelae as experienced by patients, and all showed good to excellent internal consistency. Known-groups analyses illustrated significant and graded changes in clinical assessments and corresponding HDQoL domains across disease severity levels. Convergent and discriminant validity was demonstrated by the expected pattern of correlations between specific HDQoL domains and corresponding domain-relevant clinical assessments as well as patient-reported measures. The data demonstrate robust support for the refined HDQoL across disease stages. CONCLUSIONS: The HDQoL, with its 2 distinct behavioral domains of Mood-Self and Worries as well as the Physical-Functional and Cognitive domains, is a relevant, reliable, and valid patient-derived instrument to measure the impact of HD across all severity stages.

Cognitive rehabilitation, self-management, psychotherapeutic and caregiver support interventions in progressive neurodegenerative conditions: A scoping review.

BACKGROUND: Despite its potentially significant impact, cognitive disability may be overlooked in a number of progressive neurodegenerative conditions, as other difficulties dominate the clinical picture. OBJECTIVE: We examined the extent, nature and range of the research evidence relating to cognitive rehabilitation, self-management, psychotherapeutic and caregiver support interventions in Parkinsonian disorders, multiple sclerosis (MS), frontotemporal dementias (FTD), motor neuron disease and Huntington's disease. METHODS: Scoping review based on searches of MEDLINE and CINAHL up to 15 March 2016. RESULTS: We included 140 eligible papers. Over half of the studies, and almost all the randomised controlled trials, related to MS, while a number of single case studies described interventions for people with FTD. CR interventions addressed functional ability, communication and interaction, behaviour or memory. The majority of psychotherapy interventions involved cognitive behavioural therapy for depression or anxiety. Self-management interventions were mainly available for people with MS. There were few reports of interventions specific to caregivers. Numerous methodological challenges were identified. CONCLUSIONS: The limited range of studies for all conditions except MS suggests a need firstly to synthesise systematically the available evidence across conditions and secondly to develop well-designed studies to provide evidence about the effectiveness of CR and other psychological interventions.

Rating Scales and Performance-based Measures for Assessment of Functional Ability in Huntington's Disease: Critique and Recommendations.

Limitation of functional ability is a major feature of Huntington's disease (HD). The International Parkinson and Movement Disorder Society (MDS) commissioned the appraisal of the use and clinimetric properties of clinical measures of functional ability that have been applied in HD studies and trials to date, to make recommendations regarding their use based on standardized criteria. After a systematic literature search, we included a total of 29 clinical measures grouped into two categories: (1) performance-based measures (e.g., balance, walking, and reaching/grasping), and (2) rating scales. Three performance-based measures are rated as "recommended": the Tinetti Mobility Test for screening of fall risk and for severity assessment of mobility in patients with manifest HD (up to stage III); the Berg Balance Scale for severity of balance impairment; and the Six-Minute Walk Test for assessment of walking endurance (severity) in HD subjects with preserved ambulation. No rating scale targeting functional ability reached a "recommended" status either for screening or severity measurement. The main challenges identified in this review include applying widely accepted conceptual frameworks to the identified measures, the lack of validation of clinical measures to detect change over time, and absence of validated measures for upper limb function. Furthermore, measures of capacity or ability to perform activities of daily living had ceiling effects in people with early and pre-manifest HD. We recommend that the MDS prioritize the development of new scales that capture small, but meaningful changes in function over time for outcome assessment in clinical trials, particularly in earlier stages of HD.

Quality of Life in Huntington's Disease: Critique and Recommendations for Measures Assessing Patient Health-Related Quality of Life and Caregiver Quality of Life.

The compromise of quality of life in Huntington's disease is a major issue, both for individuals with the disease as well as for their caregivers. The International Parkinson and Movement Disorder Society commissioned a review of the use and clinimetric validation status of measures used in Huntington's disease to assess aspects related with quality of life and to make recommendations on their use following standardized criteria. We included both patient-centered measures (patient health-related quality-of-life measures) and caregiver-centered measures (caregiver quality-of-life measures). After conducting a systematic literature search, we included 12 measures of patient health-related quality of life and 2 measures of caregiver quality of life. Regarding patient-centered measures, the Medical Outcomes Study 36-Item Short-Form Health Survey is "recommended" as a generic assessment of health-related quality of life in patients with Huntington's disease. The 12-Item Short Form Health Survey, the Sickness Impact Profile, the 12-item World Health Organization Disability Assessment Schedule, and the Huntington's Disease Health-Related Quality of Life questionnaire are "suggested." No caregiver-centered quality-of-life measure obtained a "recommended" status. The Alzheimer's Carer's Quality of Life Inventory and the Huntington's Disease Quality of Life Battery for Carers are "suggested." Recognizing that the assessment of patient health-related quality of life can be challenging in Huntington's disease, as patients may lack insight and there is insufficient clinimetric testing of these scales, the committee concluded that further validation of currently available health-related quality-of-life measures should be undertaken, namely, those Huntington's disease-specific health-related quality-of-life measures that have recently been reported and used. © 2018 International Parkinson and Movement Disorder Society.

The long-term safety and efficacy of bilateral transplantation of human fetal striatal tissue in patients with mild to moderate Huntington's disease.

Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disease involving progressive motor, cognitive and behavioural decline, leading to death approximately 20 years after motor onset. The disease is characterised pathologically by an early and progressive striatal neuronal cell loss and atrophy, which has provided the rationale for first clinical trials of neural repair using fetal striatal cell transplantation. Between 2000 and 2003, the 'NEST-UK' consortium carried out bilateral striatal transplants of human fetal striatal tissue in five HD patients. This paper describes the long-term follow up over a 3-10-year postoperative period of the patients, grafted and non-grafted, recruited to this cohort using the 'Core assessment program for intracerebral transplantations-HD' assessment protocol. No significant differences were found over time between the patients, grafted and non-grafted, on any subscore of the Unified Huntington's Disease Rating Scale, nor on the Mini Mental State Examination. There was a trend towards a slowing of progression on some timed motor tasks in four of the five patients with transplants, but overall, the trial showed no significant benefit of striatal allografts in comparison with a reference cohort of patients without grafts. Importantly, no significant adverse or placebo effects were seen. Notably, the raclopride positron emission tomography (PET) signal in individuals with transplants, indicated that there was no obvious surviving striatal graft tissue. This study concludes that fetal striatal allografting in HD is safe. While no sustained functional benefit was seen, we conclude that this may relate to the small amount of tissue that was grafted in this safety study compared with other reports of more successful transplants in patients with HD.

Health-related quality of life in Huntington's disease patients: a comparison of proxy assessment and patient self-rating using the disease-specific Huntington's disease health-related quality of life questionnaire (HDQoL).

Huntington's disease (HD) is a fatal, neurodegenerative disease for which there is no known cure. Proxy evaluation is relevant for HD as its manifestation might limit the ability of persons to report their health-related quality of life (HrQoL). This study explored patient-proxy ratings of HrQoL of persons at different stages of HD, and examined factors that may affect proxy ratings. A total of 105 patient-proxy pairs completed the Huntington's disease health-related quality of life questionnaire (HDQoL) and other established HrQoL measures (EQ-5D and SF-12v2). Proxy-patient agreement was assessed in terms of absolute level (mean ratings) and intraclass correlation. Proxies' ratings were at a similar level to patients' self-ratings on an overall Summary Score and on most of the six Specific Scales of the HDQoL. On the Specific Hopes and Worries Scale, proxies on average rated HrQoL as better than patients' self-ratings, while on both the Specific Cognitive Scale and Specific Physical and Functional Scale proxies tended to rate HrQoL more poorly than patients themselves. The patient's disease stage and mental wellbeing (SF-12 Mental Component scale) were the two factors that primarily affected proxy assessment. Proxy scores were strongly correlated with patients' self-ratings of HrQoL, on the Summary Scale and all Specific Scales. The patient-proxy correlation was lower for patients at moderate stages of HD compared to patients at early and advanced stages. The proxy report version of the HDQoL is a useful complementary tool to self-assessment, and a promising alternative when individual patients with advanced HD are unable to self-report.

Assessment of cognitive symptoms in prodromal and early huntington disease.

The Functional Rating Scale Taskforce for pre-Huntington Disease (FuRST-pHD) is a multinational, multidisciplinary initiative with the goal of developing a data-driven, comprehensive, psychometrically sound, rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease (HD) gene expansion carriers. The process involves input from numerous sources to identify relevant symptom domains, including HD individuals, caregivers, and experts from a variety of fields, as well as knowledge gained from the analysis of data from ongoing large-scale studies in HD using existing clinical scales. This is an iterative process in which an ongoing series of field tests in prodromal (prHD) and early HD individuals provides the team with data on which to make decisions regarding which questions should undergo further development or testing and which should be excluded. We report here the development and assessment of the first iteration of interview questions aimed to assess cognitive symptoms in prHD and early HD individuals.

Assessment of Day-to-Day Functioning in Prodromal and Early Huntington Disease.

The Functional Rating Scale Taskforce for pre-Huntington Disease (FuRST-pHD) is a multinational, multidisciplinary initiative with the goal of developing a data-driven, comprehensive, psychometrically sound, rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease (HD) gene expansion carriers. The process involves input from numerous sources to identify relevant symptom domains, including HD individuals, caregivers, and experts from a variety of fields, as well as knowledge gained from the analysis of data from ongoing large-scale studies in HD using existing clinical scales. This is an iterative process in which an ongoing series of field tests in prodromal (prHD) and early HD individuals provides the team with data on which to make decisions regarding which questions should undergo further development or testing and which should be excluded. We report here the development and assessment of the first iteration of interview questions aimed to assess functional impact in day-to-day activities in prHD and early HD individuals.

Assessing behavioural manifestations prior to clinical diagnosis of huntington disease: "anger and irritability" and "obsessions and compulsions".

The Functional Rating Scale Taskforce for pre-Huntington Disease (FuRST-pHD) is a multinational, multidisciplinary initiative with the goal of developing a data-driven, comprehensive, psychometrically sound, rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease (HD) gene expansion carriers. The process involves input from numerous sources to identify relevant symptom domains, including HD individuals, caregivers, and experts from a variety of fields, as well as knowledge gained from the analysis of data from ongoing large-scale studies in HD using existing clinical scales. This is an iterative process in which an ongoing series of field tests in prodromal (prHD) and early HD individuals provides the team with data on which to make decisions regarding which questions should undergo further development or testing and which should be excluded. We report here the development and assessment of the first iteration of interview questions aimed to assess "Anger and Irritability" and "Obsessions and Compulsions" in prHD individuals.

Assessment of depression, anxiety and apathy in prodromal and early huntington disease.

The Functional Rating Scale Taskforce for pre-Huntington Disease (FuRST-pHD) is a multinational, multidisciplinary initiative with the goal of developing a data-driven, comprehensive, psychometrically sound, rating scale for assessing symptoms and functional ability in prodromal and early Huntington disease (HD) gene expansion carriers. The process involves input from numerous sources to identify relevant symptom domains, including HD individuals, caregivers, and experts from a variety of fields, as well as knowledge gained from the analysis of data from ongoing large-scale studies in HD using existing clinical scales. This is an iterative process in which an ongoing series of field tests in prodromal (prHD) and early HD individuals provides the team with data on which to make decisions regarding which questions should undergo further development or testing and which should be excluded. We report here the development and assessment of the first iteration of interview questions aimed to assess Depression, Anxiety and Apathy in prHD and early HD individuals.