Neuropalliative care for progressive neurological diseases: A scoping review on models of care and priorities for future research.

BACKGROUND: Neuropalliative care is a newly-defined subspeciality bringing specific aspects of fields of neurology and palliative care together to better meet the complex care needs of people with progressive neurological diseases. Examining these needs would help provide guidance about developing relevant models of care and identify gaps in research knowledge. AIM: To identify current models and approaches to neuropalliative care for people with progressive neurological diseases and the priorities for future research work. DESIGN: A scoping literature review following the methods described by the Joanna Briggs Institute. DATA SOURCES: An electronic search of the literature was undertaken from six sources including MEDLINE (Ovid), EMCARE, PsycINFO and CINAHL covering the years January 2011 to September 2021. RESULTS: Twenty-eight studies were found examining neuropalliative care from the perspectives of 4795 PND patients, 774 informal carers and 138 health professionals. All studies held themes of integrative care, with most studies employing outpatient models of multidisciplinary care. Topics discussed included: overcoming local system-issues, providing education for professionals, patients and carers, early referral and capturing outcome measures for quality-assurance and future research work. CONCLUSIONS: Most models of neuropalliative care described in the international literature are predominantly outpatient, multidisciplinary and integrative. Clinicians typically utilise existing neurology and palliative care infrastructure to provide care. More high-quality research and outcome tools are needed to guide the design of evidence-based palliative care for people with progressive neurological diseases.

Emotion Recognition Correlates With Social-Neuropsychiatric Dysfunction in Huntington's Disease.

OBJECTIVES: People with Huntington's disease (HD) experience poor social quality of life, relationship breakdown, and social withdrawal, which are mediated to some extent by socially debilitating neuropsychiatric symptoms, such as apathy and disinhibition. Social cognitive symptoms, such as impaired emotion recognition, also occur in HD, however, the extent of their association with these socially debilitating neuropsychiatric symptoms is unknown. Our study examined the relationship between emotion recognition and symptom ratings of apathy and disinhibition in HD. METHODS: Thirty-two people with premanifest or symptomatic-HD completed Part 1 of The Awareness of Social Inference Test (TASIT), which is a facial emotion recognition task. In addition, we obtained severity ratings for apathy and disinhibition on the Frontal Systems Behavior Scale (FrSBe) from a close family member. Our analyses used motor symptom severity as a proxy for disease progression. RESULTS: Emotion recognition performance was significantly associated with family-ratings of apathy, above and beyond their shared association with disease severity. We found a similar pattern for disinhibition ratings, which fell short of statistical significance. As expected, worse emotion recognition performance was correlated with higher severity in FrSBe symptom ratings. CONCLUSIONS: Our findings suggest that emotion recognition abilities relate to key socially debilitating neuropsychiatric symptoms in HD. Our results help to understand the functional significance of emotion recognition impairments in HD, and may have implications for the development of remediation programs aimed at improving patients' social quality of life. (JINS, 2018, 24, 417-423).

Characterizing social communication changes in amyotrophic lateral sclerosis.

BACKGROUND: Speech and language impairments are well-established in individuals with amyotrophic lateral sclerosis (ALS). However, knowledge about particular aspects of social communication and everyday conversational abilities is limited. AIMS: To investigate self- and informant-report ratings of social communicative abilities in ALS participants and matched healthy controls. METHODS & PROCEDURES: Thirty-two participants with ALS and 24 controls completed the La Trobe Communication Questionnaire (LCQ). Participants nominated a close other to provide an informant report on the LCQ. Demographic and clinical information was also collected. OUTCOMES & RESULTS: Informant ratings indicated greater difficulties in conversational initiation, effectiveness and partner sensitivity for ALS participants compared with controls. ALS participants did not rate their social communicative abilities as poorer than controls and self-reports only differed from informant ratings in the control group. LCQ scores were not significantly correlated with clinical/functional variables. CONCLUSIONS & IMPLICATIONS: Social communication can be reduced in ALS and individuals may lack insight into these difficulties. In order to understand and provide targeted interventions for such difficulties, clinical speech and language assessment should incorporate social communication assessment, including both a self- and informant-report format.

Which behaviours? Identifying the most common and burdensome behaviour changes in amyotrophic lateral sclerosis.

Behaviour change is increasingly recognised as a common feature of amyotrophic lateral sclerosis (ALS), and may be similar to that seen in frontotemporal dementia (FTD). The behaviours most disturbed in ALS, and those that relate most significantly to caregiver burden, however, have not been well established. Forty ALS participants and their caregivers, and 27 age- and gender-matched healthy controls and their relatives, participated in this study. ALS participants were assessed on a disease rating scale, and caregivers and control informants completed the revised version of the Cambridge Behaviour Inventory and a measure of burden. ALS caregivers reported significantly more disturbance than healthy control informants on the functional domains of everyday skills, self-care, and sleep, and in the behavioural domains of mood and motivation. There were no differences between groups in frequency of memory and orientation difficulties, or behaviours characteristic of FTD, such as changes to eating habits or stereotypic and motor behaviour, indicating that the behavioural profile in ALS may differ from FTD. In the ALS group, the domains with the strongest relationship to caregiver burden were everyday skills, motivation and memory, likely because poor motivation, memory dysfunction and difficulties completing activities of daily living require more carer support via direct supervision, prompting or hands on care. Services to support ALS patients and caregivers need to provide targeted interventions for those functional and behavioural changes which are most burdensome in the disease.

Using the Knowledge to Action framework to improve housing and support for people with Multiple Sclerosis.

Background People with Multiple Sclerosis (MS) have unique housing and support needs that are essential for maintaining independence at home; however, there is limited research to guide the design of community living options for this population. The aim of this study was to examine housing and support needs and preferences of people with MS with the intention to inform the planning of a co-designed intervention based on the study's findings. Methods Using the Knowledge to Action (KTA) framework, quantitative (n =79) and qualitative (n =6) data from people with MS were extracted and integrated from projects completed by the research team that explored the housing and support needs and preferences of people with disability. Results were synthesised and presented to a reference group for validation, contextualisation, and adaptation to the Australian context. Results High physical support needs were common across participants. People most commonly required home modifications to improve accessibility, such as ramps, equipment such as heating and cooling, and assistive technology. Many people required more than 8 hours per day of paid support. Moving into individualised housing facilitated independence and community reintegration. People reported gaps between what they wanted from support workers and what they received, citing individual and systemic barriers. Conclusion People with MS have support needs that require proactive and responsive funding arrangements, housing design and support provision. In line with KTA principles, findings will inform the planning of a co-designed intervention that involves people with lived experience of MS and other stakeholders to influence policy and improve home and living outcomes for this population.

A pilot evaluation of an 8-week mindfulness-based stress reduction program for people with pre-symptomatic Huntington's disease.

People with Huntington's disease (HD) face difficult emotional and practical challenges throughout their illness, including in the pre-symptomatic stage. There are, however, extremely limited psychosocial interventions adapted to or researched for HD. We adapted and piloted an 8-week mindfulness-based stress reduction (MBSR) program in people with pre-symptomatic HD to determine if the program (i) was feasible and acceptable to participants, (ii) resulted in increased mindfulness understanding and skills, and (iii) led to improved psychological adjustment. Quantitative measures of mindfulness, emotion regulation, mood, and quality of life were administered pre and post the MBSR program and at 3-month follow-up. Measures of mindfulness practice and session clarity were administered weekly. Qualitative participant feedback was collected with a post-program interview conducted by independent clinicians. Seven participants completed the 8-week course. The program's feasibility and acceptability was supported by excellent retention and participation rates and acceptable rates of home practice completion. In addition, qualitative feedback indicated participant satisfaction with the program structure and content. Two core mindfulness skills (observing and non-judgment) showed significant improvement from pre- to post-assessment. Participant qualitative feedback indicated increased confidence and capacity to use mindfulness techniques, particularly in emotionally challenging situations. Participant questionnaire data showed good psychological adjustment at baseline, which did not change after treatment. Psychological benefits of the program identified in qualitative data included fewer ruminations about HD, reduced isolation and stigma, and being seen by others as calmer. These findings justify expansion of the program to determine its efficacy in a larger, controlled study.

Enhancing the Clinical Utility of DriveSafe DriveAware for People with Huntington's Disease.

DriveSafe DriveAware (DSDA) is a cognitive screening tool assessing driving safety. Previously, we found DSDA categorised some HD participants as 'likely to pass' on-road assessments, despite displaying cognitive impairments in domains known to impact driving. As processing speed is affected early in HD, we examined whether DSDA completion time could provide supplementary cognitive information to support clinical decision-making. The HD group completed subtests significantly slower than controls, and completion times correlated with cognitive functions essential for driving. Considering DSDA completion time may tailor the assessment for people with HD so that it is more reflective of HD-related cognitive functioning.

Cognitive Fitness to Drive in Huntington's Disease: Assessing the Clinical Utility of DriveSafe DriveAware.

BACKGROUND: DriveSafe DriveAware (DSDA) has been validated as an off-road screening tool for predicting on-road driving performance in clinical populations, but its utility in people with Huntington's disease (HD) is unknown. OBJECTIVE: Our aim was to evaluate the utility of DSDA in people with HD by demonstrating sensitivity of DSDA scores to HD progression and exploring associations between DSDA performance and cognitive functions that are essential to driving and impaired in people with HD. METHODS: We administered the iPad application version of DSDA to 26 pre-symptomatic and symptomatic participants with HD. Disease progression was assessed via measures of motor impairment, disease burden and functional capacity. Standardised neuropsychological tests were used to assess cognitive function across several domains including attention, processing speed, planning, and visuoperception. RESULTS: Results underscore the sensitivity of DSDA to HD progression and cognitive impairment; that is, poorer DSDA performance was associated with greater HD severity and poorer cognitive ability across the domains of attention, processing speed, and planning. Nevertheless, we identified a proportion of participants with HD who were predicted to pass on-road testing based on DSDA, but scored in the impaired range on multiple cognitive tests. These participants tended to score closer to the cut-off score used by DSDA to categorise pass/fail outcomes. CONCLUSIONS: Our findings demonstrate potential for use of DSDA in the HD population, however, significant variability in cognitive performance among those predicted to 'pass' on-road driving assessment suggests the screening tool requires further development for use with HD drivers.

Evaluating the Utility of a Structured Clinical Protocol for Reducing the Impact of Behavioural and Psychological Symptoms of Dementia in Progressive Neurological Diseases: A Pilot Study.

OBJECTIVES: Behavioural and psychological symptoms of dementia (BPSD) cause significant distress to both aged care residents and staff. Despite the high prevalence of BPSD in progressive neurological diseases (PNDs) such as multiple sclerosis, Huntington's disease, and Parkinson's disease, the utility of a structured clinical protocol for reducing BPSD has not been systematically evaluated in PND populations. METHOD: Staff (n = 51) and individuals with a diagnosis of PND (n = 13) were recruited into the study, which aimed to evaluate the efficacy of a PND-specific structured clinical protocol for reducing the impact of BPSD in residential aged care (RAC) and specialist disability accommodation (SDA) facilities. Staff were trained in the clinical protocol through face-to-face workshops, which were followed by 9 weeks of intensive clinical supervision to a subset of staff ("behaviour champions"). Staff and resident outcome measures were administered preintervention and immediately following the intervention. The primary outcome was frequency and severity of BPSD, measured using the Neuropsychiatric Inventory-Nursing Home Version (NPI-NH). The secondary outcome was staff coping assessed using the Strain in Dementia Care Scale (SDCS). RESULTS: In SDA, significant reductions in staff ratings of job-related stress were observed alongside a statistically significant decrease in BPSD from T1 to T2. In RAC, there was no significant time effect for BPSD or staff coping; however, a medium effect size was observed for staff job stress. CONCLUSIONS: Staff training and clinical support in the use of a structured clinical protocol for managing BPSD were linked to reductions in staff job stress, which may in turn increase staff capacity to identify indicators of resident distress and respond accordingly. Site variation in outcomes may relate to organisational and workforce-level barriers that may be unique to the RAC context and should be systematically addressed in future RCT studies of larger PND samples.

Multimodal emotion processing deficits are present in amyotrophic lateral sclerosis.

OBJECTIVE: Emotion processing abilities might be reduced in amyotrophic lateral sclerosis (ALS). Previous studies of emotion processing in ALS have inconsistent results, and are limited by variations in task difficulty, modalities examined, and participants' cognitive status. The current study used a battery of emotion processing tasks at differing levels of difficulty and across different modalities (facial affect and voice prosody) to assess the extent of emotion processing deficits in nondemented ALS. METHOD: 33 ALS participants with intact basic cognition and 22 healthy controls completed the abbreviated Comprehensive Affect Testing System (CATS), which assesses simple and complex facial affect recognition, affective prosody recognition, cross-modal face-prosody integration, and semantic comprehension of affect. Participants also completed measures of executive function, mood, and functional impairment. RESULTS: ALS participants showed impairments on complex facial affect recognition, affective prosody recognition, and cross-modal integration. In contrast, simple facial affect recognition and semantic comprehension of affect were intact. ALS participants did not have significant mood symptoms, and neither mood nor functional impairment was related to emotion processing. Performance on the cross-modal composite was related to executive function, however, this relationship was not apparent for facial or prosody recognition within a single modality. CONCLUSIONS: These results indicate that people living with ALS without dementia often have subtle difficulties with recognizing emotions in both faces and voices, even in the context of intact basic cognition. Clinicians should be aware of the potential for these emotion processing difficulties to be present in ALS and to affect interpersonal behavior and quality of life. (PsycINFO Database Record

Families Affected by Huntington's Disease Report Difficulties in Communication, Emotional Involvement, and Problem Solving.

BACKGROUND: Family functioning in Huntington's disease (HD) is known from previous studies to be adversely affected. However, which aspects of family functioning are disrupted is unknown, limiting the empirical basis around which to create supportive interventions. OBJECTIVE: The aim of the current study was to assess family functioning in HD families. METHODS: We assessed family functioning in 61 participants (38 HD gene-expanded participants and 23 family members) using the McMaster Family Assessment Device (FAD; Epstein, Baldwin and Bishop, 1983), which provides scores for seven domains of functioning: Problem Solving; Communication; Affective Involvement; Affective Responsiveness; Behavior Control; Roles; and General Family Functioning. RESULTS: The most commonly reported disrupted domain for HD participants was Affective Involvement, which was reported by 39.5% of HD participants, followed closely by General Family Functioning (36.8%). For family members, the most commonly reported dysfunctional domains were Affective Involvement and Communication (both 52.2%). Furthermore, symptomatic HD participants reported more disruption to Problem Solving than pre-symptomatic HD participants. In terms of agreement between pre-symptomatic and symptomatic HD participants and their family members, all domains showed moderate to very good agreement. However, on average, family members rated Communication as more disrupted than their HD affected family member. CONCLUSION: These findings highlight the need to target areas of emotional engagement, communication skills and problem solving in family interventions in HD.

Emotion Evaluation and Social Inference Impairments in Huntington's Disease.

BACKGROUND: Huntington's disease (HD) is an inherited neurodegenerative disorder characterised by motor, cognitive and neuropsychiatric symptoms. Recent research has established that individuals with HD display reduced discrimination of emotional facial expressions and impaired higher-order social cognitive skills, including 'theory of mind'. OBJECTIVE: This study aimed to further characterise the emotion evaluation and theory of mind deficits in HD in an ecologically-valid context, and determine their impact on socially-relevant functional abilities. METHODS: A sample of 17 HD participants and 24 healthy controls were assessed using The Awareness of Social Inference Test (TASIT), together with additional self- and informant rated measures of cognition, social communication, empathy and neuropsychiatric symptoms. RESULTS: Participants with HD showed significant difficulties in evaluating negative emotions, and understanding sincere, sarcastic and 'paradoxical sarcastic' statements, compared with controls. The ability to evaluate positive emotions was negatively correlated with behavioural problems, but no other clinical, behavioural or communication measures correlated significantly with TASIT subscales. CONCLUSIONS: These findings suggest that social cognitive difficulties in symptomatic HD may be underpinned by more generalised impairments, related to deciphering social exchanges, as opposed to a selective theory of mind deficit. Such difficulties have the potential to place significant strain on interpersonal relationships, and thus warrant thorough clinical assessment, using ecologically-valid tools, to promote early detection and development of person-centred interventions.

Exploring sarcasm detection in amyotrophic lateral sclerosis using ecologically valid measures.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive condition involving degeneration of both upper and lower motor neurons. Recent research suggests that a proportion of persons with ALS show a profile similar to that of frontotemporal dementia (FTD), with this group of ALS patients exhibiting social cognitive deficits. Although social cognitive deficits have been partially explored in ALS, research has yet to investigate such changes using ecologically valid measures. Therefore, this study aimed to further characterize the scope of social cognitive and emotion recognition deficits in non-demented ALS patients using an ecologically valid measure of social cognition. A sample of 35 ALS patients and 30 age-and-education matched controls were assessed using the Addenbrooke's Cognitive Examination, the Brixton Spatial Anticipation Test, and The Awareness of Social Inference Test, where participants were required to discriminate between various emotions and decipher socially challenging scenarios enacted in video vignettes. Participants with ALS showed significant difficulties in recognizing both sarcastic and paradoxical sarcastic statements, but not sincere statements, when compared to controls. After controlling for executive difficulties, ALS patients still displayed significant difficulties on tasks that assessed their comprehension of both sarcastic and paradoxical sarcastic statements. The inability to read social cues and make social inferences has the potential to place significant strain on familial/interpersonal relationships in ALS. The findings of this study highlight the importance of employing a broader range of neuropsychological assessment tools to aid in early detection of frontal lobe impairment in non-demented ALS patients.

Home or Residential Care? The Role of Behavioral and Psychosocial Factors in Determining Discharge Outcomes for Inpatients with Huntington's Disease.

BACKGROUND: The progressive nature of Huntington's disease (HD) means that families often struggle to cope with increasing care needs of their affected family member. As a result, individuals with HD are likely to be at risk of hospitalization and subsequent early placement into residential care facilities. OBJECTIVE: To explore which factors were associated with early residential care placement in a group of hospitalized patients with HD. METHODS: A retrospective, systematic medical file audit of a neurological inpatient unit was conducted. Fifty-nine patients with HD were admitted from the community between January 2008 and December 2011; of these, 31 patients were discharged home while 28 patients required discharge to a residential care facility. These two groups were compared on a range of demographic, clinical and psychosocial variables identified as precipitating the hospital admission. Group comparisons were performed using t-tests and chi-square tests with Bonferroni correction for multiple comparisons. RESULTS: A higher proportion of men were placed in residential care than were able to return home (p = 0.045); and the group placed in residential care had longer inpatient hospital stays (p < 0.001). Groups did not differ in age, medications or disease duration. We found that psychosocial difficulties (p < 0.001) and behavioral problems (p = 0.001), but not physical, cognitive, or psychiatric factors, significantly differentiated the groups. CONCLUSIONS: Patients with HD discharged to residential care were more likely to have psychosocial and behavioral problems, and lengthy hospital stays. These findings indicate the need for community-based psychosocial and behavior management interventions aimed at preventing residential care admissions for persons with HD.