Describing and assessing behavioural symptoms in amyotrophic lateral sclerosis with and without frontotemporal dementia: a scoping review.

PURPOSE OF REVIEW: Alongside motor and cognitive symptoms, amyotrophic lateral sclerosis (ALS) and ALS with frontotemporal dementia (ALSFTD) present with behavioural symptoms, which can be challenging for all affected by the disease. A scoping review of studies published between 2011 and 2024 was conducted to present the breadth of behavioural symptoms in ALS and ALSFTD, explore how they are described and assessed, and identify patterns in the literature. FINDINGS: This scoping review identified 3939 articles, with 111/3939 meeting eligibility criteria. Most studies were from Australia (23.22%), Italy (16.94%) and the UK (14.29%); 75.67% were cross-sectional. Sample size ranged from 1 to 1013, as case studies were included. Overall mean age (100/111 studies) was 61.32 (SD = 4.15). Proportion of male patients (reported 102/111 studies) was 61.49%; mean disease duration (reported in 86/111 records) was 32.63 months (SD = 24.72). Papers described a broad range of behavioural symptoms (465 examples), which were thematically collated into seven categories: disinhibition (27.74%), apathy (25.16%), perseverative/compulsive behaviours (17.42%), hyperorality (10.53%), loss of sympathy or empathy (8.6%), psychotic symptoms (7.74%), and loss of insight about disease and changes (2.8%). Most studies (78.37%) used validated behavioural assessments that elicited carer's perspectives. SUMMARY: Despite extensive evidence of behavioural symptoms in ALS, implementation of assessments and management of behavioural symptoms in clinical care remain limited. Clinicians must assess behavioural symptoms, as these can negatively affect disease prognosis, patient treatment engagement and increase family distress. Measures capturing carers' perspectives through interviews are ideal as they can reveal anosognosia, lack of sympathy and lack of empathy.

Factors affecting anticipatory grief of family carers supporting people living with Motor Neurone disease: the impact of disease symptomatology.

OBJECTIVE: To investigate the effect of carer- and disease-related factors on anticipatory grief (AG) in family carers supporting people living with Motor Neurone Disease. METHODS: Seventy-five carers from the UK and USA participated in this cross-sectional study, between July 2021 and February 2023. Participants completed assessments on: anticipatory grief (MMCGI-SF, comprising three sub-scales: Personal Sacrifice Burden, Heartfelt Sadness and Longing, Worry and Felt Isolation); person with MND (pwMND) behavioral changes (MiND-B) and disease severity (ALSFRS-R); carer-pwMND emotional bond (Relationship Closeness Scale), familism levels (Familism Scale), and reported hours of care provided. Multiple linear regression analyses were conducted to explore factors impacting carer AG. RESULTS: AG total scores showed that 50.7% of carers were experiencing common grieving reactions, 22.6% presented intense grieving emotions, and 26.7% presented low grieving responses.Disease severity (regression coefficient, ß = -0.31, p = 0.01, 95%CI -0.91 to -0.13) and behavioral changes (ß = -0.34, p = 0.002, 95%CI -1.45 to -0.33) predicted AG total scores (proportion of explained variation, R2=0.38, p < 0.001).Regarding AG subscales, Personal Sacrifice Burden (R2=0.43, p < 0.001) was predicted by disease severity (ß = -0.39, p < 0.001, 95%CI -0.42 to -0.11). Behavioral changes predicted Heartfelt Sadness and Longing (ß = -0.27, p = 0.03, 95%CI -0.49 to -0.03; R2 = 0.21, p = 0.01) and Worry and Felt Isolation (ß = -0.42, p < 0.001, 95%CI -0.63 to -0.20; R2=0.33, p < 0.001). CONCLUSION: This study suggests that disease-related factors may be the strongest predictors of carer AG. Interventions addressing carers' understanding and management of MND symptoms seem crucial to support their experiences of loss and their acceptance of MND. Evidence-based support for carers in MND services is required.

Factors associated with grief in informal carers of people living with Motor Neuron Disease: A mixed methods systematic review.

The purpose of this mixed methods systematic review was to identify factors associated with anticipatory grief, post-death grief, and prolonged grief in informal carers of people living with Motor Neuron Disease (MND) to inform future research and practice. Six electronic databases were searched and two quantitative and eight qualitative studies were identified. Five overarching themes were generated through thematic synthesis. The findings suggest that there are factors that may affect different grieving processes. It might be particularly important to target some factors prior and after the death of the person living with MND such as the knowledge about the progression of the disease, changes in relationships, anxiety and depressive symptoms of carers, and planning for death of the care recipient. Factors that may affect all three grieving processes were also identified such as negative experiences of caregiving, experiences of losses, end of life and psychological support, and emotional avoidance coping.

Navigating an emotional journey: A qualitative study of the emotional experiences of family carers currently supporting people living with motor neurone disease.

BACKGROUND: Family carers of people living with motor neurone disease (MND) face continuous changes and losses during the progression of the disease, impacting on their emotional wellbeing. Carers' emotions might affect their engagement in everyday activities and their caring role. However, how carers manage their emotions and which strategies they identify as useful to cope with them while caring is under researched. OBJECTIVE: To identify the emotional experiences and coping strategies of MND family carers while caring the person living with MND. METHODS: We conducted 14 semi-structured interviews with family carers currently supporting people living with MND living in the UK. Interviews were audio/video recorded and professionally transcribed verbatim. We analyzed data inductively within an interpretive descriptive approach, using reflexive thematic analysis. RESULTS: Three key themes were generated from the analysis. Destabilization of diagnosis reflected the devastating impact the diagnosis had on carers, characterized by initial overwhelming emotions. Adapting to new circumstances and identifying coping strategies captured how carers experienced everyday changes and losses and how they gradually adjusted to the situation by identifying coping strategies to be able to manage arising emotions. Maintaining emotional coping encompassed how carers used individual strategies they had tried before and had worked for them to cope emotionally with the continuous changes and losses while preserving their emotional wellbeing. SIGNIFICANCE OF RESULTS: Our findings suggest that carers of people living with MND embark on an emotional journey from the diagnosis of the disease. As the disease progresses, carers adopt coping strategies that best work for them to manage their emotions (e.g., living day by day and seeking support). Understanding the key strategies used to support emotional coping during the caring journey and how carers re-construct their emotional life around MND could help inform future practice and research to better support carers of this population.

Contributions of Caregiver Management Styles to the Discrepancy Between Reported and Observed Task Performance in People with Dementia.

BACKGROUND: The identification and understanding of the discrepancy between caregivers' reports of people with dementia's (PwD) performance of activities of daily living (ADLs) and observed performance, could clarify what kind of support a PwD effectively needs when completing tasks. Strategies used by caregivers have not been included in the investigation of this discrepancy. OBJECTIVE: To (1) investigate if caregivers' report of PwD's ADL performance are consistent with PwD's observed performance; (2) explore if caregiver management styles, depression, and anxiety, contribute to this discrepancy. METHODS: PwD (n = 64) were assessed with standardized performance-based (Assessment of Motor and Process Skills, AMPS) and informant-based (Disability Assessment for Dementia, DAD) ADL assessments. Caregivers completed depression (PHQ-9), anxiety (GAD-7), and dementia management style (DMSS: criticism, active-management, and encouragement) questionnaires. Cohen's kappa determined agreement/disagreement in ADL performance. To investigate the potential discrepancy between the DAD and AMPS, a continuous variable was generated: comparative ADL score. Multiple linear regression analysis explored whether caregivers' management styles, depression or anxiety could explain the ADL discrepancy. RESULTS: Poor level of agreement between observed and reported ADL performance [k = -0.025 (95% CI -0.123 -0.073)] was identified, with most caregivers underestimating ADL performance. The combined model explained 18% (R2 = 0.18, F (5,55) â€Š= 2.52, p≤0.05) of the variance of the comparative ADL score. Active-management (ß= -0.037, t (60) â€Š= -3.363, p = 0.001) and encouragement (ß= 0.025, t (60) â€Š= 2.018, p = 0.05) styles made the largest and statistically significant contribution to the model. CONCLUSION: Encouragement style could be advised for caregivers who underestimate ADL performance, while active management style for those who overestimate it. Findings have scope to increase caregivers' abilities to support PwD activity engagement in daily life.