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.

'A lightbulb moment': carers' experiences of behavioural symptoms in motor neurone disease before and after MiNDToolkit.

BACKGROUND: To explore carers' experiences of behavioural symptoms in Motor Neurone Disease (MND), before and after using the MiNDToolkit, a novel internet-based psychoeducational intervention to support management of behavioural symptoms (BehSymp) in MND. The study also investigated carers' views and acceptability of MiNDToolkit. METHODS: A qualitative process evaluation of carers engagement with, and acceptability of, the MiNDToolkit conducted using semi-structured interviews with carers (n = 11). All interviews were audio-recorded, professionally transcribed verbatim and analysed thematically. RESULTS: Five themes were identified: (1) In the dark: carers' experiences and reactions to BehSymp; (2) Others can see: the role of HCPs in identifying symptoms - and perceived opportunities for carers to receive support; (3) Shedding light: carers implementation and perceived impact of the MiNDToolkit content; (4) Acceptability and carers' engagement with MiNDToolkit; (5) Future implementation. Carers' experience of BehSymp was particularly distressing when symptoms were apparently out of context. MiNDToolkit appeared to support learning that BehSymp were part of MND. Content resonated with carers, who reported learning about the full picture of MND, which led to acceptance and use of newly learned strategies. Engagement with the platform was good, with varied input from HCPs. Greater and nuanced involvement from HCPs seem important to support management of BehSymp. Recommendations for a full-scale trial emerged, including adding a paper booklet to accompany the intervention and creation of new modules on emotional lability, changes in relationships, and transitioning to a care home. CONCLUSIONS: MiNDToolkit was acceptable to carers overall. Recommended improvements should be actioned in a full-scale trial.

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.

Examining the Impact of Different Components of Sleep Quality on Anxiety Among Family Carers of People with Dementia.

Existing interventions for family carers of people with dementia tend to be less effective for anxiety than for depression. Therefore, identifying factors affecting carer anxiety is important to inform future interventions. This study conducted 2 multiple regression analyses using a sample of 91 family carers. The first regression model (∆R2 = .24), exploring the impact of demographic variables and carer stressors, demonstrated that hours of caring (ß = .33) and overall sleep quality (ß = .28) were significant predictors of anxiety. To further investigate the impact of sleep quality, the second model (∆R2 = .24) focussed on exploring the differential impact of various components of sleep quality on anxiety. Findings demonstrated that subjective sleep quality (ß = .33) and sleep disturbances (ß = .22) were significant predictors. Hours of caring per week, subjective sleep quality and sleep disturbances seem to be critical for treating anxiety in family carers. Future studies should investigate whether targeting these variables could improve carer anxiety.

What are the factors associated with people with advanced dementia refusing assistance with personal care?

BACKGROUND: People with dementia sometimes refuse assistance with personal care activities such as washing or dressing. We aimed to investigate the factors associated with refusals of care in advanced dementia. METHODS: A cross-sectional study using informant-based measures. Participants were people with advanced dementia and their caregivers (family carers or care-home staff) (n = 260, 130 dyads) in the UK. Mixed effects linear models were used to examine the effects of neuropsychiatric behaviours, ability with activities of daily living, professional input, co-morbidities, psychotropic medications, environment modifications, and caregiver factors including type and training status on refusals of care. The Refusal of Care Informant Scale was used, range 1-13; higher scores indicate more refusal behaviours. RESULTS: Higher independence in activities of daily living was associated with less refusal behaviours (coefficient = -0.11, p < 0.001 [95% confidence interval -0.15, -0.07]). Higher agitation was associated with more refusal behaviours (0.11, p < 0.001 [0.06, 0.15]). No other statistically significant differences were found. There was no demonstrable evidence of differences in number of refusals of care between family and care-home caregivers or between dementia-trained or -untrained caregivers. CONCLUSIONS: Results suggest refusals of care have similar prevalence regardless of caregiver type (family or care home) or dementia training status, indicating that current dementia training has no impact on refusals of care or may not be implemented as intended. Improving independence in activities of daily living and reducing agitations may help prevent refusals of care. To establish causality, future research should consider embedding these factors into interventions targeting refusal of care.

Factors affecting the quality of life of family carers of people with dementia: the role of carer anxiety.

The role of anxiety on the quality of life of family carers of people with dementia is somewhat neglected in the carer literature. The current study aimed to investigate the impact of common risk factors (i.e., care recipient's neuropsychiatric symptoms, carer depression, and burden) and anxiety on QoL. This cross-sectional study recruited 89 family carers. Most of the participants were looking after a spouse with Alzheimer's or mixed dementia. A multiple regression analysis was conducted with carer QoL as a dependent variable. All risk factors (i.e., people with dementia's neuropsychiatric symptoms, carer depression, anxiety, and burden) were entered into the model simultaneously as independent variables. The model's R2 was 33%. The results demonstrated that anxiety was the only significant independent variable predicting carer QoL (ß = - 0.34, p = 0.03, 95% CI: - 0.64 to - 0.04). These results indicated that having more symptoms of anxiety was associated with worse QoL as measured by the ICEpop CAPability measure for Older people (ICECAP-O). These findings suggested that improving carer's anxiety may be particularly important in promoting QoL among family carers of people with dementia. Future interventions should target this variable to achieve the desired result of improving carer QoL.

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.

Examining the presence and nature of delusions in Alzheimer's disease and frontotemporal dementia syndromes.

OBJECTIVES: Abnormal beliefs and delusions have been reported in some people with dementia, however, the prevalence of delusions, and their neurocognitive basis has been underexplored. This study aimed to examine the presence, severity, content and neural correlates of delusions in a large, well-characterised cohort of dementia patients using a transdiagnostic, cross-sectional approach. METHODS: Four-hundred and eighty-seven people with dementia were recruited: 102 Alzheimer's disease, 136 behavioural-variant frontotemporal dementia, 154 primary progressive aphasia, 29 motor neurone disease, 46 corticobasal syndrome, 20 progressive supranuclear palsy. All patients underwent neuropsychological assessment and brain magnetic resonance imaging, and the Neuropsychiatric Inventory was conducted with an informant, by an experienced clinician. RESULTS: In our cohort, 48/487 patients (10.8%) had delusions. A diagnosis of behavioural-variant frontotemporal dementia (18.4%) and Alzheimer's disease (11.8%) were associated with increased risk of delusions. A positive gene mutation was observed in 11/27 people with delusions. Individuals with frequent delusions performed worse on the Addenbrooke's Cognitive Examination (p = 0.035), particularly on the orientation/attention (p = 0.022) and memory (p = 0.013) subtests. Voxel-based morphometry analyses found that increased delusional psychopathology was associated with reduced integrity of the right middle frontal gyrus, right planum temporale and left anterior temporal pole. CONCLUSION: Our results demonstrate that delusions are relatively common in dementia and uncover a unique cognitive and neural profile associated with the manifestation of delusions. Clinically, delusions may lead to delayed or misdiagnosis. Our results shed light on how to identify individuals at risk of neuropsychiatric features of dementia, a crucial first step to enable targeted symptom management.

A New and Tidier Setting: How Does Environmental Clutter Affect People With Dementia's Ability to Perform Activities of Daily Living?

BACKGROUND: The relationship between the physical environment and the person with dementia's (PwD) activities of daily living (ADLs) task performance is controversial. Although the general assumption is that this population benefits from their home environment when performing ADLs, very few experimental studies have been conducted to date. OBJECTIVES: The aim was to investigate the influence of the environment (home vs. Research-lab) and the role of clutter on ADL performance. METHODS: Sixty-five PwD were evaluated with a performance-based ADL assessment (at home and clutter-free Research-lab). Paired t tests compared ADL performance and level of clutter in both environments. Multiple regression analysis investigated factors associated with better ADL performance. RESULTS: Overall, PwD performed better at home even though clutter was significantly lower in the Research-lab. When stratified by dementia stage, PwD in the moderate stage of the disease performed better at home. CONCLUSION: Absence of clutter in the Research-Lab did not appear to play a beneficial role in ADLs. When stratified by dementia stage, only PwD in the moderate stage appeared to benefit from their home environment when performing ADL tasks. Future studies are required to elucidate the wider role of the environment in supporting engagement in daily activities in different dementia stages.

Factors Related to the Quality of Life in Family Carers of People With Dementia: A Meta-Analysis.

OBJECTIVES: This meta-analysis aimed to (1) quantitatively synthesize evidence of factors related to the quality of life (QoL) of family carers of people with dementia and (2) explore moderating factors that may influence the strength of the relationship between such potential predictive factors and carer QoL. METHODS: Studies that investigated correlations between patient/carer factors and QoL in unpaid family carers of people with dementia and were published in English, Spanish, Portuguese, or Japanese were included. RESULTS: Thirty-three studies were identified. The pooled correlations with carer QoL (effect size) were significantly large for depression (-0.58), significantly moderate for subjective burden (-0.47), and significantly small for people with dementia's neuropsychiatric symptoms (-0.24). These results indicated to be robust in the context of publication bias. The results of subgroup analyses demonstrated the social and economic development status of the country where study participants resided did not moderate these effects. CONCLUSION: Carer depression, subjective burden, and people with dementia's neuropsychiatric symptoms may play a critical role in maintaining QoL of family carers regardless of the social and economic circumstances.

Distinguishing Frontotemporal Dementia From Alzheimer Disease Through Everyday Function Profiles: Trajectories of Change.

BACKGROUND: Different dementia syndromes display different patterns of everyday functioning. This article explored different patterns of functioning at baseline and trajectories of change in behavioral variant frontotemporal dementia (bvFTD) and Alzheimer disease (AD). METHODS: Data from the Uniform Data Set of the National Alzheimer's Coordinating Centre were employed. The Functional Assessment Questionnaire assessed functioning at up to 7 follow-up visits. Independent t tests assessed variations in functioning between syndromes at baseline. Linear mixed-effect modeling explored longitudinal functional trajectories between syndromes. RESULTS: Data from 3351 patients (306 bvFTD and 3,045AD) were analyzed. At baseline, patients with bvFTD performed all daily activities poorer than AD dementia. Linear mixed models showed a significant effect of syndrome and time on functioning, and evidence of interaction between syndrome and time, with bvFTD showing a steeper decline for using the stove and travel. CONCLUSIONS: Findings can help in the effective care planning of everyday functioning for bvFTD and AD dementia.

Disease Progression in Frontotemporal Dementia and Alzheimer Disease: The Contribution of Staging Scales.

INTRODUCTION: There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD). OBJECTIVES: To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale-frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR). METHODS: The sample consisted of 70 individuals, aged 40+ years, with at least 2 years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant), and 27 with AD. The FTD-FRS, the CDR, and the 2 additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke Cognitive Examination-Revised was completed by patients. After 12 months, the same protocol was applied. RESULTS: The FTD-FRS, CDR-FTLD, and CDR detected significant decline after 12 months in the 3 clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages. CONCLUSIONS: The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum.

How does carer management style influence the performance of activities of daily living in people with dementia?

INTRODUCTION: People with Dementia (PwD)'s performance of activities of daily living (ADLs) has been associated with apathy, cognitive deficits, carers' depression and burden. However, it is not known if the carers' management style affects ADL performance, particularly alongside PwD's cognitive deficits and apathy. Thus, the aim of this study was to explore the contribution of intrinsic (cognition, apathy) and extrinsic (carer management styles) dementia factors to ADL performance. METHODS: PwD (n = 143) were assessed on global cognition (ACE-III); apathy (CBI-R); ADLs (Disability Assessment for Dementia-DAD). Carers' (n = 143) criticism, encouragement and active-management styles were assessed with the Dementia Management Strategy Scale (DMSS). Multiple linear regression analysis investigated contributions of carer styles, cognition, apathy (independent variables) on ADLs (dependent variable). RESULTS: The best model explaining the variance of the DAD scores included cognition (ß = 0.413, t(142)  = 4.463, p = 0.001), apathy (ß = -0.365, t(142)  = -5.556, p = 0.001), carer criticism (ß = -0.326, t(142)  = -2.479, p = 0.014) and carer encouragement styles (ß = 0.402, t(142)  = 2.941, p = 0.004) accounting for 40% of the variance of the DAD scores. CONCLUSIONS: This novel study demonstrated that PwD's level of apathy and the carer's use of criticism negatively affected ADL performance while PwD's cognitive abilities and carer encouragement style improved ADL performance. These findings have critical implications for the development of novel multi-component non-pharmacological interventions to maintain function and delay disease progression in dementia, as well as direct relevance to current carers and families.

Positive behaviour support in frontotemporal dementia: A pilot study.

Frontotemporal dementia (FTD) is a progressive neurodegenerative brain condition clinically characterized by marked changes in behaviour that impact the individuals' relationships and community participation, and present challenges for families. Family carers of individuals with FTD find apathy and disinhibition particularly challenging leading to high levels of stress and burden. Positive behaviour support (PBS) as a behaviour intervention framework has never been trialled in FTD. This pilot study examined the functional basis of apathetic and disinhibited behaviours in four FTD dyads and explored the acceptability of a PBS intervention. The PBS programme was provided by an occupational therapist in the participants' homes. Measures collected at baseline and post-intervention (M = 3.9 months) assessed: function of behaviours, challenging behaviours, and qualitative outcomes pertaining to the acceptability of the PBS approach. PBS was an acceptable intervention for all four dyads. "Sensory" and "tangible" were the most common functions contributing to the maintenance of behaviour changes, and aspects of apathetic and disinhibited behaviours improved following intervention. This study demonstrates the acceptability and potential benefit of a PBS programme to provide support in FTD. A more rigorous trial will be an important next step in developing improved services tailored to the needs of this unique population.

Pronounced Impairment of Activities of Daily Living in Posterior Cortical Atrophy.

INTRODUCTION: The impact of several dementia syndromes on activities of daily living (ADLs) has been well documented, but no study has yet investigated functional ability in posterior cortical atrophy (PCA). The primarily visual nature of deficits in this condition is likely to have a pronounced impact on ADLs. OBJECTIVE: The aim of this study was to profile functional change in PCA and identify predictors of change. METHOD: Twenty-nine PCA patients and 25 patients with typical Alzheimer's disease (AD) and their caregivers were included in this cross-sectional study. ADLs were assessed using the Disability Assessment for Dementia (DAD), administered to caregivers, assessing basic ADLs (e.g., eating, dressing) and instrumental ADLs (e.g., managing finances, meal preparation). The predictive utility of cognitive domains (Addenbrooke's Cognitive Examination), behavioural impairment (Cambridge Behavioural Inventory-Revised) and demographic variables on ADL ability was also examined. RESULTS: PCA patients showed significantly reduced total ADL scores compared to AD patients (medium effect size, d = -0.7; p < 0.05), with significantly more impairment on basic ADLs (large effect size, d = -0.8; p < 0.05) but similar impairment on instrumental ADLs (medium effect size, d = -0.5; p > 0.05). A model combining patient mood, disinhibition, apathy, symptom duration, and memory and attention/orientation scores explained the variance of scores in functional decline (61.2%), but the key factor predicting ADL scores was attention/orientation (p = 0.048). CONCLUSION: This study shows the profound impact of PCA on ADLs and factors underpinning patients' disability. Attention/orientation deficits were found to correlate and contribute to variance in ADL scores. Future work to develop tailored interventions to manage ADL impairment in PCA should take these findings into account.

Nonpharmacological Interventions to Improve Depression, Anxiety, and Quality of Life (QoL) in People With Dementia: An Overview of Systematic Reviews.

This overview aimed to systematically synthesize evidence from existing systematic reviews to signpost practitioners to the current evidence base on nonpharmacological interventions to improve depression, anxiety, and quality of life (QoL) in people with dementia and to discuss priorities for future research. The databases MEDLINE, PsycINFO, Scopus, and Cochrane Central Register of Controlled Trials were searched in August 2017 with an updated search in January 2019. Fourteen systematic reviews of randomized controlled trials of nonpharmacological interventions were identified. Dementia stage was rated moderate or severe in the majority of the reviews and type of dementia varied. Interventions reported to be effective were cognitive stimulation (QoL: standardized mean difference [SMD] = 0.38), music-based therapeutic interventions (depression: SMD = -0.27, anxiety: SMD = -0.43, QoL: SMD = 0.32), and psychological treatments (mainly cognitive behavior therapy; depression: SMD = -0.22, anxiety: MD = -4.57). Although health-care professionals are recommended to continue using these approaches, future research needs to focus on the type and form of interventions that are most effective for different stages and types of dementia.

Antonovsky's sense of coherence and resistance resources reduce perception of burden in family carers of people with Alzheimer's disease.

Objectives: Taking care of people with dementia (PWD) has been associated with some degree of burden. The variability of the carer's burden can be partially explained by their personal characteristics. Antonovsky's model of health defined the resistance resources (RRs) as essential mechanisms to cope with stressors, and to shape the personal sense of coherence (SOC). This study identifies the RRs related with carer's SOC, and their implications in the perception of burden in family dementia carers.Methods: A sample of 308 participants from the 'SOC & DEM study' (154 carers and 154 PWD) was recruited from two memory clinics. Carer's personal characteristics of burden, SOC, self-efficacy, coping strategies, perceived social support, and depression were evaluated using standardized instruments. PWD's degree of dependence and behaviour and psychological symptoms of dementia (BPSD) were assessed too. A path analysis was used to test the relationship between caregiver burden and SOC including the personal RRs of the carers and clinical data of PWD.Results: The path model identified SOC as a major factor related to carer's burden perception (r = -.327). Self-efficacy (r = .285), two coping strategies, 'use instrumental support' (r = -.235) and 'behavioural disengagement' (r = -.219), and social support perceived (r = .304) were the main carer's personal characteristics directly related with SOC. Caring experience (r = -.281) was the main carer factor related with burden while dependence (r = .156) and BPSD (r = .157) were the dementia factors.Conclusion: The SOC has previously related with carer's burden. The results contributed to identify relevant and modifiable personal characteristics as RRs that could reduce this burden.

Deficits in Emotion Recognition as Markers of Frontal Behavioral Dysfunction in Amyotrophic Lateral Sclerosis.

OBJECTIVE: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with prominent motor symptoms. Patients with ALS may also manifest frontal behavior symptoms and cognitive decline, including impairment in facial emotion recognition. The authors aimed to investigate whether deficits in emotion recognition were associated with frontal behavior symptoms in ALS. METHODS: Participants were patients with probable or definite sporadic ALS (N=21; male:female ratio, 11:10; median age, 62 years; median disease duration, 3 years) and age-matched and education-matched healthy control subjects (N=25; male:female ratio, 14:11; median age, 61 years). The Facial Emotion Recognition Test (FERT) was administered to all participants. Patients with ALS were assessed using the Cambridge Behavior Inventory-Revised and were classified into two groups according to the presence of frontal behavioral symptoms: ALS with no behavioral symptom (ALSns; N=9) and ALS with at least one behavioral symptom (ALSbs; N=12). RESULTS: Apathy and mood symptoms were the most frequent neuropsychiatric symptoms in the patient group. Patients with ALS performed worse than control subjects in the recognition of sadness (p<0.004). There were no differences between control subjects and patients in the ALSns group in all FERT scores, but the ALSbs group had lower performance than control subjects in sadness (p<0.003). CONCLUSIONS: Emotion recognition deficit may be a marker of frontal behavior in ALS.

Social support and high resilient coping in carers of people with dementia.

High resilience is associated with improved carer outcomes. Both individual factors and the availability of social support have been linked with resilience. This study was conducted to compare socio-demographic characteristics and the availability of social support for carers with low and high resilient coping, and identify if any domain of social support predicted high resilient coping in informal carers of people with dementia. The participants in this cross sectional survey included 108 informal carers of people with dementia. Findings showed the availability of emotional/informational support was most likely to predict resilient coping and tangible support the least likely. However, when controlling for all covariates, only gender predicted high resilient coping, individual social support domains were no longer significant. Therefore, as no single domain of social support has a significantly greater influence on resilient coping, service providers should enable carers to build a wide, multi-function support network.

Validity and Reliability of the Frontotemporal Dementia Rating Scale (FTD-FRS) for the Progression and Staging of Dementia in Brazilian Patients.

INTRODUCTION: Few studies on instruments for staging frontotemporal dementia (FTD) have been conducted. OBJECTIVE: The objective of this study was to analyze the factor structure, internal consistency, reliability, and convergent validity of the Brazilian version of the Frontotemporal Dementia Rating Scale (FTD-FRS). METHODS: A total of 97 individuals aged 40 years and above with >2 years' education took part in the study, 31 patients diagnosed with behavioral variant FTD (bvFTD), 8 patients with primary progressive aphasia, 28 with Alzheimer disease, 8 with mild cognitive impairment, and a control group of 22 healthy subjects. The FTD-FRS was completed by family members or caregivers, and Neurologists completed the 8-item Clinical Dementia Rating for Frontotemporal Lobar Degeneration (CDR-FTLD) scale (6 original domains plus Language and Behavior). The Alzheimer disease and FTD patients had equivalent disease severity level. RESULTS: The internal consistency of the FTD-FRS, estimated by Cronbach α, was 0.975 whereas test-retest reliability was 0.977. Scree plot and exploratory factor (Varimax rotation) analyses revealed the existence of 4 factors, with eigenvalues >1, which together explained 77.13% of the total variance with values of 1.28 to 17.52. The domains of the Brazilian version of the FTD-FRS scale correlated with the domains of the CDR-FTLD. CONCLUSIONS: The present study is the first to document the factorial structure of the FTD-FRS and its convergent validity with the CDR-FTLD. These tools are key to determine dementia severity in FTD. The Brazilian FTD-FRS demonstrated adequate psychometric properties for use in Brazil. This instrument may contribute to disease staging in FTD and may help to document intervention-related changes.