Quality of life, cognitive and behavioural impairment in people with motor neuron disease: a systematic review.

PURPOSE: Motor neuron disease (MND) is a neurodegenerative disease, progressively impacting function and self-perceived quality of life (QoL). Up to 50% of people with MND can present with cognitive and behavioural impairment, with an associated increase in caregiver burden or strain. However, there has been no systematic exploration of the relationship between QoL and cognitive or behavioural impairment in MND. The aim was to determine if there is a relationship between QoL and cognitive/behavioural impairment in MND, while also supplementarily looking to determine the types of cognitive/behavioural and QoL measures utilised in these studies. METHODS: A systematic search was performed across multiple databases (PsychINFO, Embase, Medline, AMED) for research published up to the date of February 22, 2023. Studies utilising quantitative methods of measuring QoL, cognitive/behavioural functioning/impairment were included. Findings examining relationships between QoL-cognitive/behavioural impairment were extracted and synthesised. RESULTS: A total of 488 studies were identified, with 14 studies included in the systematic review. All 14 studies were observational (11 cross-sectional, 3 longitudinal). 13 studies utilised MND non-specific measures, particularly in relation to QoL and cognitive impairment. Of 8 studies measuring behavioural impairment 62.5% (N = 5) found either a lower QoL difference or association. Only 33.3% (N = 4) of 12 studies measuring cognitive impairment found a lower QoL difference or association. CONCLUSIONS: This systematic review shows that behavioural impairment may have an impact on QoL in MND. There is variability in types of assessments used to measure QoL and also cognitive/behavioural impairment, most of which are disease-non-specific. Recommendations for future research are to use comprehensive disease-specific, multidomain measures to further elucidate the QoL-cognitive/behavioural impairment relationship.

Are neuropsychiatric symptoms a marker of small vessel disease progression in older adults? Evidence from the Lothian Birth Cohort 1936.

BACKGROUND: Neuropsychiatric symptoms could form part of an early cerebral small vessel disease prodrome that is detectable before stroke or dementia onset. We aimed to identify whether apathy, depression, anxiety, and subjective memory complaints associate with longitudinal white matter hyperintensity (WMH) progression. METHODS: Community-dwelling older adults from the observational Lothian Birth Cohort 1936 attended three visits at mean ages 73, 76, and 79 years, repeating MRI, Mini-Mental State Examination, neuropsychiatric (Dimensional Apathy Scale, Hospital Anxiety and Depression Scale), and subjective memory symptoms. We ran regression and mixed-effects models for symptoms and normalised WMH volumes (cube root of WMH:ICV × 10). RESULTS: At age 73, 76, and 79, m = 672, n = 476, and n = 382 participants attended MRI respectively. Worse apathy at age 79 was associated with WMH volume increase (ß = 0.27, p = 0.04) in the preceding 6 years. A 1SD increase in apathy score at age 79 associated with a 0.17 increase in WMH (ß = 0.17 normalised WMH percent ICV, p = 0.009). In apathy subscales, executive (ß = 0.13, p = 0.05) and emotional (ß = 0.13, p = 0.04) scores associated with increasing WMH more than initiation scores (ß = 0.11, p = 0.08). Increasing WMH also associated with age (ß = 0.40, p = 0.002) but not higher depression (ß = -0.01, p = 0.78), anxiety (ß = 0.05, p = 0.13) scores, or subjective memory complaints (ß = 1.12, p = 0.75). CONCLUSIONS: Apathy independently associates with preceding longitudinal WMH progression, while depression, anxiety, and subjective memory complaints do not. Patients with apathy should be considered for enrolment to small vessel disease trials.

Multidimensional Apathy in Behavioral Variant Frontotemporal Dementia, Primary Progressive Aphasia, and Alzheimer Disease.

Apathy is prevalent in dementia, such as behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD). As a multidimensional construct, it can be assessed and subsumed under a Dimensional Apathy Framework. A consistent apathy profile in bvFTD and PPA has yet to be established. The aim was to explore apathy profiles and awareness in bvFTD, PPA, and AD. A total of 12 patients with bvFTD, 12 patients with PPA, 28 patients with AD, and 20 matched controls, as well as their informants/carers, were recruited. All participants completed the Dimensional Apathy Scale (DAS), assessing executive, emotional, and initiation apathy subtypes, a 1-dimensional apathy measure, depression measure, and functional and cognitive screens. Apathy subtype awareness was determined through DAS informant/carer and self-rating discrepancy. Apathy profile comparison showed patients with bvFTD had significantly higher emotional apathy than patients with AD (P < .01) and significantly higher apathy over all subtypes than patients with PPA (Ps < .05). Additionally, patients with bvFTD had significantly lower awareness for emotional apathy (P < .01) when compared to patients with AD and PPA. All patient groups had significant global apathy over all subtypes compared to controls. The emergent apathy profile for bvFTD seems to be emotional apathy (indifference or emotional/affective neutrality), with lower self-awareness in this subtype. Further, lower self-awareness for executive apathy (lack of motivation for planning, organization, or attention) differentiates bvFTD from PPA. Future research should investigate the cognitive and neural correlates as well as the practical impact of apathy subtypes.

A Road Map for Remote Digital Health Technology for Motor Neuron Disease.

Despite recent and potent technological advances, the real-world implementation of remote digital health technology in the care and monitoring of patients with motor neuron disease has not yet been realized. Digital health technology may increase the accessibility to and personalization of care, whereas remote biosensors could optimize the collection of vital clinical parameters, irrespective of patients' ability to visit the clinic. To facilitate the wide-scale adoption of digital health care technology and to align current initiatives, we outline a road map that will identify clinically relevant digital parameters; mediate the development of benefit-to-burden criteria for innovative technology; and direct the validation, harmonization, and adoption of digital health care technology in real-world settings. We define two key end products of the road map: (1) a set of reliable digital parameters to capture data collected under free-living conditions that reflect patient-centric measures and facilitate clinical decision making and (2) an integrated, open-source system that provides personalized feedback to patients, health care providers, clinical researchers, and caregivers and is linked to a flexible and adaptable platform that integrates patient data in real time. Given the ever-changing care needs of patients and the relentless progression rate of motor neuron disease, the adoption of digital health care technology will significantly benefit the delivery of care and accelerate the development of effective treatments.

Recommendations for the Nonpharmacological Treatment of Apathy in Brain Disorders.

Apathy is a common neuropsychiatric syndrome observed across many neurocognitive and psychiatric disorders. Although there are currently no definitive standard therapies for the treatment of apathy, nonpharmacological treatment (NPT) is often considered to be at the forefront of clinical management. However, guidelines on how to select, prescribe, and administer NPT in clinical practice are lacking. Furthermore, although new Information and Communication Technologies (ICT) are beginning to be employed in NPT, their role is still unclear. The objective of the present work is to provide recommendations for the use of NPT for apathy, and to discuss the role of ICT in this domain, based on opinions gathered from experts in the field. The expert panel included 20 researchers and healthcare professionals working on brain disorders and apathy. Following a standard Delphi methodology, experts answered questions via several rounds of web-surveys, and then discussed the results in a plenary meeting. The experts suggested that NPT are useful to consider as therapy for people presenting with different neurocognitive and psychiatric diseases at all stages, with evidence of apathy across domains. The presence of a therapist and/or a caregiver is important in delivering NPT effectively, but parts of the treatment may be performed by the patient alone. NPT can be delivered both in clinical settings and at home. However, while remote treatment delivery may be cost and time-effective, it should be considered with caution, and tailored based on the patient's cognitive and physical profile and living conditions.

Apathy dimensions in Parkinson's disease.

OBJECTIVE: Apathy is a prominent and disabling symptom in Parkinson's disease (PD) and is a multidimensional behaviour, but which dimensions are specifically affected is unclear. Therefore, the aim of this preliminary study was to determine the psychometric properties of the Dimensional Apathy Scale (DAS) and explore the multidimensional profile of apathy in PD patients. METHODS: Thirty-four PD patients, with 30 of their informants/carers, and 34 healthy controls, with 30 of their informants, completed the DAS, Apathy Evaluation Scale and the Geriatric Depression Scale Short Form. Motor staging and independent living status were recorded. RESULTS: Comparative group analyses revealed that PD patients were significantly more apathetic on self-rated executive (p = 0.01) and initiation (p = 0.03) dimensions than controls, where only executive apathy was significantly higher in ratings of patients' informants/carers compared with controls' informants (p = 0.02). A third of patients were impaired on at least one apathy dimension. Additionally, patients with apathy tended to have more impaired activities of daily living, while none of the apathy dimensions related to motor disability. CONCLUSION: Our findings show the DAS is a valid and reliable multidimensional apathy tool for use in PD. PD is characterised by an executive apathy profile as determined by informants/carers, although patients described both executive and initiation apathy. This indicates a lack of motivation for planning, organisation and attention and lack of initiation of thoughts or behaviours. Further research is needed to determine the cognitive underpinnings of this emerging apathy profile and the clinical impact in PD. Copyright © 2017 John Wiley & Sons, Ltd.

Multidimensional apathy in ALS: validation of the Dimensional Apathy Scale.

AIM: Apathy is a prominent symptom of amyotrophic lateral sclerosis (ALS), but measurement is confounded by physical disability. Furthermore, it has been traditionally measured as a unidimensional symptom despite research demonstrating a multifaceted construct. The new Dimensional Apathy Scale (DAS) has been specifically designed for patients with motor disability to measure 3 neurologically based subtypes of apathy: Executive, Emotional and Initiation. We aimed to explore this behavioural symptom by examining the substructure of apathy in ALS and to determine the reliability and validity of the DAS in patients and their carers. METHOD: Patients and carers were recruited through the national Scottish Motor Neurone Disease Register and were asked to complete the DAS, the standardised Apathy Evaluation Scale, and the Geriatric Depression Scale-Short Form. 83 patients with ALS, 75 carers and 83 sex-matched, age-matched and education-matched controls participated. RESULTS: When compared with healthy controls, patients showed a significant increase in apathy on the Initiation subscale, and were significantly less apathetic on the Emotional subscale. Scores on the DAS patient and carer versions did not significantly differ. Internal consistency reliability, convergent and discriminant validity were found to be good for the DAS subscales. There was no association between the DAS and functional disability using the ALS Functional Rating Scale. CONCLUSIONS: Apathy in ALS is characterised by a specific profile of increased initiation apathy and reduced emotional apathy. The DAS is a reliable and valid measure for the assessment of multidimensional apathy in ALS.

A systematic review of the validity and reliability of apathy scales in neurodegenerative conditions.

BACKGROUND: There are several scales used to detect apathy in disease populations. Since apathy is a prevalent symptom in many neurodegenerative diseases, this is an especially important context in which to identify and compare scales. AIMS: To provide an overview of apathy scales validated in generic and specific neurodegenerative disease populations, compare validation studies' methodological quality and the psychometric properties of the validated apathy scales. METHODS: A systematic review of literature was conducted of articles published between 1980 and 2013. The final articles selected for review were rated on methodological quality and the psychometric properties of the scales used were interpreted. RESULTS: Sixteen articles validating apathy scales were included in the review, five in a generic neurodegenerative sample and eleven in specific neurodegenerative samples. The methodological quality of specific studies varied from poor to excellent. The highest quality, which had psychometrically favorable scales, were the dementia apathy interview and rating (DAIR) and the apathy evaluation scale-clinical version (AES-C) in Alzheimer's disease and the Lille apathy rating scale (LARS) in Parkinson's disease. Generic neurodegenerative disease validation studies were of average methodological quality and yielded inconsistent psychometric properties. CONCLUSIONS: Several instruments can be recommended for use in some specific neurodegenerative diseases. Other instruments should either be validated or developed to assess apathy in more generic populations.

Psychometric properties of the Russian version of the Edinburgh cognitive and behavioral amyotrophic lateral sclerosis screen.

BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative condition with observable cognitive and behavioral impairment. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) is a tool developed specifically for people with ALS (pwALS) and previously translated into Russian, but the psychometric properties have not yet been explored. The aim was to explore and determine the psychometric properties of the Russian-version of ECAS (ECAS-R). METHODS: 56 Russian speaking pwALS, 32 of their caregivers and 26 healthy controls were recruited for the study. They completed the ECAS-R, Patient Health Questionnaire-9 (PHQ-9) and Montreal Cognitive Assessment (MoCA). King Staging System was also utilized. Internal consistency, divergent and convergent validity, as well as culturally-derived cutoff scores of ECAS-R were determined. RESULTS: The internal consistency of ECAS-R was good (Cronbach's alpha = 0.73). Convergent validity was observed though a strong correlation between the ECAS-R and MoCA scores. No correlation between ECAS-R and PHQ-9 were observed in terms of divergent validity. Based on culturally-derived cutoff scores, 64.2% (N = 36) of pwALS displayed cognitive impairment, with the most affected cognitive domains as executive function and language. Apathy was the most common behavioral impairment for pwALS followed by a loss of sympathy/empathy. CONCLUSIONS: The ECAS-R is valid and reliable tool for the screening for the cognitive and behavioral impairment in Russian-speaking pwALS, with culturally-derived cutoffs presented.

Exploring clinical management of cognitive and behavioural deficits in MND. A scoping review.

OBJECTIVES: Little is known about how cognitive and behavioural decline in MND is managed clinically. This review aimed to summarise clinical management approaches of cognitive and behavioural decline in MND reported in peer-reviewed and grey literature. METHODS: A scoping review was conducted across Embase, Medline, Psychinfo and Emcare in October 2022. Grey literature was also searched across Google Scholar and Google in October 2022. RESULTS: A total of N = 26 studies and 8 documents were included. Thematic analysis revealed six key areas of clinical management: i. Assessment, ii. Education, iii. Advance Care Planning, iv. Adaptation of Care Plan, v. Communication and vi. Carer Support. CONCLUSIONS: The literature on management of cognitive and behavioural decline in MND is sparse. Most peer-reviewed literature consists of expert commentary and there is a lack of primary data to guide practitioners and families on how to manage cognitive and behavioural change in MND. PRACTICE IMPLICATIONS: Determining as early as practicable the presence of cognitive and behavioural changes in pwMND will enable practitioners to make adaptations to communication, provide education and supported decision-making for forward planning. This will enable individualised care, planned in partnership with families with MND, which incorporates personal needs and wishes.

Sleep quality, perivascular spaces and brain health markers in ageing - A longitudinal study in the Lothian Birth Cohort 1936.

BACKGROUND: Sleep is thought to play a major role in brain health and general wellbeing. However, few longitudinal studies have explored the relationship between sleep habits and imaging markers of brain health, particularly markers of brain waste clearance such as perivascular spaces (PVS), of neurodegeneration such as brain atrophy, and of vascular disease, such as white matter hyperintensities (WMH). We explore these associations using data collected over 6 years from a birth cohort of older community-dwelling adults in their 70s. METHOD: We analysed brain MRI data from ages 73, 76 and 79 years, and self-reported sleep duration, sleep quality and vascular risk factors from community-dwelling participants in the Lothian Birth Cohort 1936 (LBC1936) study. We calculated sleep efficiency (at age 76), quantified PVS burden (at age 73), and WMH and brain volumes (age 73 to 79), calculated the white matter damage metric, and used structural equation modelling (SEM) to explore associations and potential causative pathways between indicators related to brain waste cleaning (i.e., sleep and PVS burden), brain and WMH volume changes during the 8th decade of life. RESULTS: Lower sleep efficiency was associated with a reduction in normal-appearing white matter (NAWM) volume (ß = 0.204, P = 0.009) from ages 73 to 79, but not concurrent volume (i.e. age 76). Increased daytime sleep correlated with less night-time sleep (r = -0.20, P < 0.001), and with increasing white matter damage metric (ß = -0.122, P = 0.018) and faster WMH growth (ß = 0.116, P = 0.026). Shorter night-time sleep duration was associated with steeper 6-year reduction of NAWM volumes (ß = 0.160, P = 0.011). High burden of PVS at age 73 (volume, count, and visual scores), was associated with faster deterioration in white matter: reduction of NAWM volume (ß = -0.16, P = 0.012) and increasing white matter damage metric (ß = 0.37, P < 0.001) between ages 73 and 79. On SEM, centrum semiovale PVS burden mediated 5% of the associations between sleep parameters and brain changes. CONCLUSION: Sleep impairments, and higher PVS burden, a marker of impaired waste clearance, were associated with faster loss of healthy white matter and increasing WMH in the 8th decade of life. A small percentage of the effect of sleep in white matter health was mediated by the burden of PVS consistent with the proposed role for sleep in brain waste clearance.

Validation of the self-rated Dimensional Apathy Scale in community stroke survivors.

Apathy, a disorder of motivation observed in up to 40% of stroke survivors, is negatively associated with stroke rehabilitation outcomes. Different apathy subtypes have been identified in other conditions, but there is currently no validated multidimensional measure of post-stroke apathy (PSAp). The Dimensional Apathy Scale (DAS) assesses apathy across three subtypes: Executive, Emotional and Initiation apathy. We aimed to determine if the DAS is a reliable and valid tool to detect and characterise apathy in stroke. Fifty-three stroke survivors, (45.3% males, median age 54), and 71 people without stroke (26.8% males, median age 45) completed measures of apathy (DAS, Apathy Evaluation Scale, AES), depression (Patient Hospital Questionnaire, PHQ-9) and anxiety (Generalised Anxiety Disorder scale, GAD-7) as part of an online survey. The DAS showed high internal consistency and convergent validity with the current gold standard unidimensional assessment for apathy (AES) and divergent validity with depression (PHQ-9) and anxiety (GAD-7). Stroke survivors scored significantly higher on the total score of the DAS and all subscales, compared with controls. There were however no significant differences on depression and anxiety scores between the two groups. Our results suggest the DAS is a reliable and valid screening tool to detect and characterise PSAp.

Psychometric Properties of the Revised Dysexecutive Questionnaire in a Non-clinical Population.

Aims: The aim of this study was to assess the psychometric properties of the revised self-rated version of the Dysexecutive Questionnaire (DEX-R) within a non-clinical sample. Methods: The study was hosted online, with 140 participants completing the DEX-R, GAD-2 and PHQ-2. Sixty participants also completed the FrSBe, with 99 additionally completing the DEX-R again 3 weeks later. Correlations with demographic factors and symptoms of anxiety and depression were conducted. Rasch and factor analysis were also used to explore underlying subconstructs. Results: The DEX-R correlated highly with the FrSBe, indicating sound concurrent validity. Internal consistency, split-half reliability and test-retest reliability were excellent. Age and symptoms of depression and anxiety correlated with DEX-R scores, with older age associated with less dysexecutive problems. The Rasch analysis confirmed the multidimensionality of the rating scale, and a three-factor structure was found relating to activation-self-regulatory, cognitive and social-emotional processes. Frequencies of responses on DEX-R items varied, many were not fully endorsed indicating specific relevance of most but not all items to patients. Conclusion: Interpretations of DEX-R ratings of dysexecutive problems should consider mood and individual variation. Systematic comparison of DEX-R responses between healthy and clinical groups could help identify a suitable cut off for dysexecutive symptoms.

I'm looking through you: Mentalizing in frontotemporal dementia and progressive supranuclear palsy.

Mentalizing and emotion recognition are impaired in behavioral variant frontotemporal dementia (bvFTD). It is not clear whether these abilities are also disturbed in other conditions with prominent frontal lobe involvement, such as progressive supranuclear palsy (PSP). Our aim was to investigate social cognition (facial emotion recognition, recognition of social norms violation and mentalizing) in bvFTD and PSP. The neural basis of these functions in PSP and bvFTD groups, by analysis of structural neuroimaging, were also investigated. Twenty-three bvFTD patients, 21 PSP patients and 23 healthy controls were included. All participants underwent 3T brain MRI and a full cognitive exam including the short version of Social and Emotional Assessment (Mini-SEA), which is composed of a facial emotion recognition test (FERT) and the faux pas test. Two components of the faux pas test were distinguished: a score assessing the recognition of social norms violation and a score assessing mentalizing. Compared to controls, bvFTD and PSP patients had significantly reduced scores in all tests of social cognition but did not differ on these measures. PSP and bvFTD had cerebral atrophy in critical regions for social cognition processes, when compared to controls. The cortical correlates of emotion recognition partially overlapped in bvFTD and PSP, with correlations retrieved within the frontal medial cortex, cingulate, insula and limbic structures. PSP and bvFTD patients also displayed similar patterns of brain correlations for the composite score of social norms, with a significant cluster in anterior temporal lobes. Mentalizing scores were associated with frontal and temporal poles bilaterally, in both bvFTD and PSP. These findings support previous observations that PSP patients exhibit impairment in complex cognitive abilities, such as mentalizing. Moreover, these data extend previous findings showing that PSP and bvFTD share key clinical, cognitive and neuroimaging features.

Prevalence of pain in amyotrophic lateral sclerosis: a systematic review and meta-analysis.

Objectives: Physical pain is a known symptom in amyotrophic lateral sclerosis (ALS), but no systematically derived prevalence estimate is available. The aim of this study was to determine the pooled prevalence of pain in ALS, relative to its method of measurement and pain characteristics. Methods: A systematic search across multiple databases was conducted on January 16, 2020. Random-effects meta-analyses of single proportions were performed on prevalence data. Heterogeneity was determined using the I2 statistic. Where available, pain location, intensity, and type or source were compared. Results: 2552 articles were identified. Twenty-one eligible studies were included. All studies used observational designs (14 cross-sectional, 6 cohort, 1 case-control). Pooled prevalence of pain in ALS across all studies was 60% (95% CI = 50-69%), with a high degree of heterogeneity (I2 = 94%, p < .001). Studies that used only validated measures had lower heterogeneity (I2 = 82%, p = 0.002), compared to those that used tailored measures, or tailored supplemented with validated measures (I2 = 90%, p < 0.001 and I2 = 83%, p < 0.001, respectively). In a subset of studies (N = 9), the most commonly reported pain location was the upper limbs including shoulders/extremities (41.5%). A further study subset (N = 7) showed moderate-severe intensity pain was most frequently reported. Type of pain was commonly related to cramp or spasm. Conclusions: Experiencing physical pain in ALS occurs with high prevalence. Deriving consensus on which specific tools should be used to assess, monitor and compare symptoms of pain in this population will reduce current heterogeneity in approaches and increase the likelihood of ameliorating distressing experiences more effectively.

Development of the MiNDToolkit for management of cognitive and behavioral impairment in motor neuron disease.

Aim: To develop structured guidance, recommendations and techniques for nonpharmacological management of cognitive and behavioral impairments in motor neuron disease, called the MiNDToolkit. Methods: A four-round-modified Delphi method was utilized (online and face-to-face meeting), supplemented by recent research, recommendations, expertise from allied health professionals, clinicians, researchers and clients. Results: Round 1 (N = 47) identified allied health professionals techniques. Round 2 (N = 23) and 3 (N = 19) used expert consensus, refining general focus, specific elements and techniques. Round 4 (N = 8) applied personal, lived and occupational experience, finalizing the general structure and content of specific techniques. Conclusion: The MiNDToolkit is composed of multiple tools to structure decision-making through flowcharts, decision trees and checklists, provide information about impairments, assessment recommendations and techniques or strategies for nonpharmacological management cognitive or behavioral impairments in motor neuron disease.

Spanish Adaptation of the Dimensional Apathy Scale (DAS) in Amyotrophic Lateral Sclerosis.

Aim: To adapt, translate, and utilize the Dimensional Apathy Scale (DAS) in Amyotrophic Lateral Sclerosis (ALS) to the Spanish population. Method: We recruited 104 ALS patients (67 of their caregivers) and 49 controls. Participants completed the Spanish-translated DAS, Geriatric Depression Scale- Short form. Patients were also administered the ALS Functional Rating Scale-Revised (ALSFRS-R). Caregivers additionally completed the informant/caregiver-rated Spanish-translated DAS. The DAS was translated to Spanish using a back-translation method. Test-retest and internal consistency reliability were examined. Divergent validity was assessed by comparing the DAS with the depression scale (GDS-15). Principal Component Analysis (PCA) was applied to explore the substructure of the Spanish DAS. Results: The internal consistency reliability of self-rated Spanish DAS was 0.72 and of the informant/caregiver-rated Spanish DAS was 0.84. Correlations between self-rated DAS subscales and GDS-15 were not statistically significant, with a good test-retest reliability. PCA analysis showed a similar substructure to the original DAS. ALS patients had significantly higher Initiation apathy than controls. Additionally, ALS patient informant/caregiver-rated DAS Emotional apathy was significantly higher than the self-rated, with no significant differences observed in the Executive and Initiation subscales. No association was found between DAS and functional impairment using the ALS Functional Rating Scale (ALSFRS-R). Conclusion: The Spanish translation of the DAS is valid and reliable for use in assessing multidimensional apathy in the Spanish population. Availability of the Spanish DAS will allow for future research to explore different apathy subtypes and their impact in ALS and other conditions.