Theme 11 - COGNITIVE AND PSYCHOLOGICAL ASSESSMENT AND SUPPORT

E. Costello, M. Ryan, M. Doherty, C. McHutchinson, M. Heverin, S. Abrahams, O. Hardiman and N. Pender Academic Unit of Neurology, Trinity College Dublin, Dublin, Ireland; Psychology Deparment, Beaumont Hospital, Dublin, Ireland; Smurfit Institute of Genetics, Trinity College Dublin, Dublin, Ireland; School of Philosophy, Psychology and Language Sciences, University of Edinburgh, Edinburgh, United Kingdom

Background: Informal caregivers play a key role in the care of people with ALS. Research on caregiving generally focuses on the negative aspects including stress and burden (1). Consideration of positive aspects of caregiving is important also (2). Objectives: To explore the complex self-reported burden and well-being experiences of informal caregivers at two leading ALS centres in Europe. Methods: An exploratory cross-sectional study characterizes two groups of informal caregivers in Ireland (n ¼ 76) and the Netherlands (n ¼ 58). Data were collected as part of a European multi-centre study (3). In a semi-structured face-toface interview, standardized measures assessed psychological distress, quality of life and burden, and in an open-ended question, caregivers were asked to identify any positive aspects of their caregiving experience. Descriptive statistics summarized the socio-demographic and wellbeing measures of the two cohorts. T-tests, Mann-Whitney U tests and Chisquare tests compared psychological distress, burden, and quality of life between caregiver cohorts. Thematic analysis was used to analyse the qualitative responses. Results: Both caregiver cohorts were predominantly female and spouse/partners of the person with ALS. Higher levels of self-assessed burden were found among the Dutch caregivers (p ¼ 0.0002), and higher levels of quality of life among the Irish cohort (p ¼ 0.004). Themes generated through qualitative analysis identified caregiver satisfaction, ability to meet the patient's needs, the (re)evaluation of meaning and existential facets of life as positive aspects of caregiving. Existential factors were identified frequently by Irish caregivers (44%), and personal satisfaction and meeting the care recipient's needs by the Dutch cohort (33.3% and 28.7%, respectively). Discussion: It is important to generate an evidence base to support the psychosocial wellbeing and brain health of informal caregivers. Based on our findings, we suggest that both burden and the presence of positive factors should be evaluated and monitored. The possibility of concurrent positive and challenging experiences should be considered in the design and delivery of supportive interventions for informal caregivers. Further research will consider disease stage and the influence of cultural context, family configuration and coping strategies on the wellbeing of informal caregivers. mark.heverin@tcd.ie 1 Neuroscience Department Rita Levi Montalcini, University of Turin, Turin, Italy; 2 AOU City of Health and Science, Molinette Hospital, Torino, Italia Objective: to assess Theory of Mind (ToM) abilities in a cohort of Amyotrophic Lateral Sclerosis (ALS) patients at diagnosis. Secondly, to assess possible differences in ToM abilities depending on cognitive profile, motor phenotype and degree of severity of motor symptoms. Background: Affective and Cognitive ToM have been intensively studied in the last decades in several neurodegenerative diseases, including ALS (1). ToM deficits have been included in ALS-FTD revised diagnostic criteria (2). There is, however, remarkable variability in ToM impairment profile across different studies and this may be related to the high cognitive and motor phenotypic heterogeneity in ALS patients and variability in Neuropsychological tools employed (3). Methods: We included 85 patients attending the ALS Centre of Turin University Hospital between May 2019 and June 2021. All patients underwent neurological evaluation and cognitive evaluation. Affective ToM assessment was performed through Reading Mind in the Eyes task (RMET) and Story-Based Empathy task-Emotion Attribution (SET-EA), while cognitive ToM assessment was performed through Story-Based Empathy task-Intention Attribution (SET-IA). Both SET-IA and SET-EA were compared to a control condition of Causal Inference (SET-CI). Results: Out of 85 patients included, 4 (5%) were diagnosed with ALS-FTD, 9 (11%) ALScbi, 23 (27%) ALSci, 5 (6%) ALSbi and 44 (52%) were cognitively normal (ALS-CN), according to the revised ALS-FTD Consensus Criteria. Overall, 15 patients (18%) showed deficit in at least one of ToM tests. Specifically, RMET corrected scores showed a positive significant correlation with Category Fluency test (p 0.02), while SET-EA and SET-IA corrected score showed significant correlation with Trial Making Test B-A (p 0.02 and 0.006, respectively). Moreover, RMET showed significant correlation with onset site (p 0.004, with bulbar onset patients performing worse than spinal onset ones), and degree of severity of motor symptoms expressed through ALSFRS-S (p 0.009). Conclusions: Deficits in affective and cognitive ToM may be present in ALS patients since the early stages of the disease and show a significant correlation with degree of severity of motor symptoms and executive functions. The presence of ToM deficits in ALS patients since the initial disease stages suggests the need of a systematic assessment, both to provide a more accurate definition of cognitive profile across FTD-ALS spectrum, and also to help clinicians to elaborate better communication and care strategies taking into account such deficits in ALS patients.
Background: Motor neuron disease (MND) is a group of terminal, neurodegenerative diseases that cause progressive, and often rapid, paralysis. Unsurprisingly, diagnosis and disease progression are also very distressing for family members and friends, who often become informal carers, and studies of the experiences of MND carers often highlight the need for psychological support. Therefore, this project aims to explore psychological interventions that could be beneficial for informal carers of people living with MND to help inform further research and service improvements. Method: A scoping review was carried out to explore and describe psychological interventions for MND carers. Initially, search terms included MND, synonyms for MND, and variants of MND, as well as search terms for carers (including synonyms and terms for different relatives, such as 'family', 'spouse', 'partner', 'daughter', 'son', 'friend', etc.), and terms for psychological interventions. The goal was to be as inclusive as possible. Given the small amount of research available specifically focused on MND carers, the searches were then expanded to also include studies of psychological interventions for carers of people with related diseases, whose needs might partially overlap. This included carers of people with Parkinson's disease, Huntington's disease, and multiple sclerosis, and the search terms were expanded accordingly. The literature was collated from the databases: MEDLINE via OvidSP and PsycInfo (APA PsycInfo V R ) via Ovid and subsequently screened for relevance. Results: Interventions were generally delivered face-to-face; the majority of interventions were targeted towards current caregivers aged 18 years old or above. Outcome measures generally focused on anxiety and depression. Results of the studies were often inconclusive due to small numbers of participants, lack of control conditions, and high attrition rates. However, there were some positive results for interventions incorporating mindfulness and meditation. In addition, across all of the intervention types, participant feedback was positive, with many caregivers saying they would recommend the support to others and reporting that they valued the peer support offered by group interventions. Conclusion: This scoping review provides a useful starting point for future research to develop/adapt psychological interventions for MND carers. Carers who took part in the interventions generally provided positive feedback, and interventions including mindfulness and meditation show some promise. Future research should build on these findings, with a particular focus on refining interventions to support MND carers, and increasing accessibility to ensure more carers can fully participate in the interventions. Background: As many as 50% of ALS patients experience cognitive and/or behavioral symptoms, with approximately 15% developing dementia. Conversely, approximately 15% of FTD patients develop motor symptoms. Despite the growing understanding of this overlap between these two disorders, there remains a gap in readily assessing and uniformly quantifying cognitive dysfunction in this group of patients. Objectives: We aimed to utilize the Uniform Data Set (v3.0) executive function composite score (UDS3-EF) model (1) in an NIH ALS-FTD disease spectrum cohort to uniformly assess executive function. The UDS validation cohort included clinically normal, MCI, AD, and bvFTD patients. However, it did not include ALS patients or patients with other diagnoses in the ALS-FTD spectrum. Methods: Our cohort (n ¼ 78) is 52.6% female, 93.6% white, and 16.2 average years of education and includes 24 ALS and 36 FTD patients as well as 18 patients diagnosed with other neurodegenerative conditions. Because the demographics of the NIH cohort were well-matched to that of the cohort analyzed to develop the UDS model, we were able to utilize the demographics-adjusted model for EF scores. Parametric and non-parametric statistical tests were chosen based on variable distribution. Results: The median EF scores for ALS (À0.491; IQR À1.466, À0.012) were significantly higher than that of FTD (À2.868; IQR À3.697, À1.708); Mann-Whitney U ¼ 94, p < 0.0001, representing greater executive function. In 75% of ALS patients, the EF scores were under zero indicating executive function impairment. Patients across the full FTD spectrum, beyond bvFTD (including patients diagnosed with CBS, PPA, and PSP), had abnormal scores consistent with impaired executive function. Conclusion/Discussion: The UDS3-EF model may be used in ALS-FTD spectrum patients to allow for greater insight into the severity of executive dysfunction in these patients. These results indicate that a high proportion of ALS patients have impaired executive function similar to previous reports, and as expected, more severe impairment is present across the FTD disease spectrum. These results have important implications for recognizing impaired cognition in this group of patients, which impacts their daily lives, from self-care to management of finances and beyond. Objective: To identify potential clinical and biochemical biomarkers of cognitive impairment in patients with amyotrophic lateral sclerosis (ALS), we examined the differences in clinical and biochemical parameters between patients with cognitive impairment (ALS-ci) and those with no cognitive impairment (ALS-nci), and evaluated the predictive power of potential parameters. Methods: In this cross-sectional study, 124 ALS patients were assessed by the Edinburgh cognitive and behavioral screen (ECAS) (1) and categorized into the ALS-ci group and the ALS-nci group. Clinical, biochemical, and behavioral data were compared between the two groups. Parameters with significant difference were further included in a multivariate logistic regression analysis and assessed for their predictive power of cognitive impairment in ALS patients. Results: Up to 40% of ALS patients showed cognitive impairment. The ALS-ci group had lower education level (p < 0.001), older age at symptom onset (p ¼ 0.001), and older age at testing (p ¼ 0.001). Multivariable analysis showed that low plasma uric acid (p ¼ 0.037), older age at testing (p ¼ 0.002) and low education level (p ¼ 0.001) were independent predictors of cognitive impairment in ALS patients. The predictive model including these three parameters had an area under the curve value of 0.788 with a sensitivity of 79.2% and a specificity of 73.7%. Conclusion: Cognitive impairment was a relatively common feature in ALS patients. Plasma uric acid might help evaluate the risk of cognitive impairment in ALS patients when combined with education level and age at testing. Pseudobulbar affect is a disorder of emotional expression commonly observed in amyotrophic lateral sclerosis (ALS), presenting as episodes of involuntary laughter, or crying. The objective of the current study was to determine the association between the frequency of pathological laughter and crying (PLC) episodes with clinical features, cognitive impairment, and brainstem pathology. 35 sporadic ALS patients underwent neuropsychological assessment, with a subset also undergoing brain imaging. The Centre for Neurological Study Lability Scale (CNS-LS) was used to screen for the presence and severity of pseudobulbar affect (CNS-LS !13) and frequency of PLC episodes. Presence of pseudobulbar affect was significantly higher in bulbar onset ALS (p ¼ 0.02). Frequency of PLC episodes was differentially associated with cognitive performance and brainstem integrity. Notably pathological laughter frequency, but not crying, showed a significant positive association with executive dysfunction on the Trail Making Test B-A (R 2 ¼ 0.14, p ¼ 0.04 Background: Given the nature of MND, many family members and friends become informal carers, giving up their own time, including their own professional, personal, and social lives. Previous research has highlighted the unmet needs of MND informal carers; however, there remains a gap in our understanding of the needs (and how to address said needs) specific to MND carers.
Objectives: This study aims to explore the needs and support preferences of informal MND carers in more detail, including directly through 1:1 discussion, thereby informing the future development of a psychological intervention. Methods: Previously, we have collected mood scores of depression (PHQ-9), anxiety (GAD-7), and strain (MCSI) from informal MND carers using the Telehealth in MND (TiM) system. In the present study, this data was analysed using descriptive statistics. New participants, consisting of both current and bereaved informal carers, were recruited via an advert circulated on social media and disseminated by the MND Association. Carer experiences, challenges, unmet needs, and preferences for psychological support were explored further in semi-structured interviews, which were held remotely and transcribed. Thematic analysis from a grounded-inductive perspective was used to identify common themes and key elements, which may serve as therapeutic targets.
Results: Mood scores were collected from 35 caregiver participants across multiple time points; measures included the PHQ-9 and GAD-7. Thirty-one percent of participants (n ¼ 11) reported a clinical level of depression on at least one occasion, while forty percent (n ¼ 14) reported a clinical level of anxiety at least once. MCSI scores suggest a high level of caregiver burden. Thematic analysis is ongoing, but emerging observations include: participants almost unanimously in favour of a remote (video call) format for psychological support sessions; several suggestions for an 'emergency hotline' type of telephone support, either by a professional or knowledgeable volunteer; reports of feeling emotionally compelled to support the relative with MND, with some participants expressing discomfort at leaving their family member under formal care despite the strain their informal role creates; and most participants feeling inadequately supported by MDT members and healthcare professionals due to a lack of empathy, specialist knowledge, and/or training. Discussion: We have identified evidence of mood disturbance among informal MND carers; experiences of depression and anxiety fluctuated over time suggesting these factors may be ameliorable to an intervention. We identified additional areas of psychological distress, barriers to accessing support, and preferences for the type of support they require. The outcome of this pilot study will help to inform the development of a new psychological intervention tailored to informal carers of MND.
jebretten1@sheffield.ac.uk COG-10 'Seeing my wife gradually deteriorate and knowing where it is going makes me sad': a multicentre, exploratory study of burden and difficulties of informal ALS caregivers Background: It is essential to support the wellbeing of Informal caregivers, as they play a vital role in caring for the person with ALS (1). Objectives: To explore the burden and difficulties of caring for someone with ALS in three leading ALS centres in Europe.
Methods: This mixed-methods analysis describes the burden and self-defined difficulties of informal ALS caregivers from three multidisciplinary ALS centres -Dublin, Ireland (n ¼ 76), Utrecht, the Netherlands (n ¼ 58) and Sheffield, England (n ¼ 38). Data were collected as part of a European multicentre study (2) Objective: To explore clinical differences between MND-CBI and MND-motor patients both at disease onset and over disease course. Methods: 61 MND patients were followed longitudinally with mood and behavioral evaluations approximately every 3 months, and with cognitive and motor examinations approximately every 6 months, for up to 15 months. Cognitive and behavioral alterations were assessed using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS), raw scores were age and education corrected using corresponding normative values. Mood disturbances were evaluated using the Hospital Anxiety and Depression Scale (HADS) and motor impairment was evaluated using the ALS Functional Rating Scale Revised (ALSFRS-r) and its rate of progression at baseline. Individual slopes of decline for each clinical measure were generated and Chi-squared and Mann-Whitney U tests were then used to compare baseline and longitudinal features between MND-CBI and MND-motor patients.
Results: According to current criteria, 32.79% of patients (N ¼ 20) were classified as MND-CBI, while the remaining 67.21% of cases (N ¼ 41) as MND-motor. At baseline, no significant differences were observed between the two groups in terms of demographic and mood features, while MND-CBI cases showed higher baseline ALSFRS-r progression rate. MND-CBI patients performed poorer than MND-motor cases also in ALS non-specific measures (ALS non-specific total score p ¼ 0.04, memory p ¼ 0.05 and visuospatial abilities p ¼ 0.01). 59.01% of patients (N ¼ 36) had at least one followup at the time of the investigation. Longitudinally, no significant differences were observed in the rates of motor decline. 32.14% of MND-motor patients converted to MND-CBI and 25% of MND-CBI cases developed additional behavioral symptoms. Both groups declined in ALS specific functions, however, a more severe worsening was selectively observed in MND-CBI (p ¼ 0.02), mainly driven by a significant deterioration in the executive domain (p ¼ 0.003). Both groups declined in ALS non-specific functions, MND-motor patients in the memory domain and MND-CBI patients in the visuospatial domain, but these differences did not reach significance. Mood disturbances ameliorated in MND-CBI and worsened in MND-motor cases, without a significant effect. Background: There is an urgent need to develop and test psychological interventions to support people with MND and family members. We developed an online self-help psychological intervention called Coping and Living well with MND (CALM) to help people with MND and family members deal with the emotional impact of MND. This intervention/website draws on techniques from cognitive behaviour therapy (CBT), acceptance and commitment therapy (ACT) and mindfulness and was developed using insights from qualitative research with people with MND and family members. Methods: The website was evaluated in two phases. In Phase 1, we conducted 'think-aloud' interviews with people with MND (n ¼ 9) and family members (n ¼ 9) to get detailed feedback on the acceptability of the content and presentation of the website. Findings from Phase 1 were used to iteratively refine the website. In Phase 2, different people with MND (n ¼ 18) and family members (n ¼ 9) were given access to the website and asked to use it and try out the suggested activities. After 6 weeks of using the website, we conducted interviews to explore their experiences. Interview data was analysed using thematic analysis. Results: In Phase 1, feedback included optimising aspects of the language and presentation of the website and implementing minor changes to the activities and activity instructions. These changes were necessary to improve inclusivity for people with varying levels of physical and cognitive ability whilst ensuring it was also suitable for family members. The changes also helped reduce the possibility of the content triggering frustration and sadness or seeming off-putting or unfeasible. In Phase 2, the website was described as accessible and easy to use. Mindfulness activities, strategies to deal with difficult thoughts, and suggestions to do activities to stay positive were reported as useful to deal with difficult emotions. Some of the tips and activities were difficult for people with more severe symptoms and people whose symptoms were progressing quickly. There was mixed feedback about the use of images and videos, where some wanted more images or videos of people with MND and family members sharing their experiences, but some felt images or examples of people with certain symptoms or using certain equipment could be too confronting. Although few participants were professional caregivers (6%), for most, the time spent caregiving was equivalent to a parttime or fulltime career (76%). Home visits from nurses and occupational or physical therapists ranked as the most highly used (53%) and highly needed (49%) ALS program. Fewer caregivers reported utilizing trainings on general caregiving (34%), but these trainings ranked second (42%) in terms of mattering most to caregivers in their roles. Most often, caregivers said their family and loved ones' wellbeing was a top concern (61%). Time for self-care, decreased engagement in enjoyable activities, and depression also rankly highly among caregivers' concerns. Among current caregivers, 46% reported feeling unprepared for changes in caregiver responsibilities as ALS progresses, and nearly 37% rated their quality of life on the lower half-or poor-of a 10-point scale. We discuss implications for how to serve ALS caregivers and ensure their wellbeing as they focus on the wellbeing of people with ALS.
sparvanta@alsa-national.org COG-18 Subjective health perception prioritizes psychological well-being over physical function as ALS advances

B. Lapin and N. Thakore
Cleveland Clinic, Cleveland, OH, USA Background: Despite declining physical function, patients with ALS report relative preservation of overall individual quality of life (QoL). This paradoxical finding is attributed to psychological adaptation to deficits with reprioritization of the individual construct of QoL. A similar adaptation could also affect subjective health perception (HRQoL).
Objective: The aim of this cross-sectional study was to examine reprioritization of factors that determine HRQoL with disease progression using structural equation modeling (SEM). Methods: Consecutive ALS patients seen in the course of routine care self-reported ALSFRS-R (a measure of bulbar, motor, and respiratory function), PHQ-9 (a measure of depression), and EQ-5D-3L (a 5-dimensional utility index that also includes a visual analog scale [EVAS] asking about health perception on a line segment). ALS was staged by the FT9 method and classified into early (FT9 stages 0, 1 and 2) and late (FT9 stages 3 and 4) disease. Measurement models were constructed for the latent factors of physical and psychological well-being (PHY and PSY respectively), with appropriate indicators chosen from self-reported measures. Model fit was assessed by standard metrics. EVAS was used as the overall measure of HRQoL. Weights of PHY and PSY on EVAS for early and late disease were estimated to examine reprioritization.
Discussion: This study confirms the hypothesis that health perception (HRQoL) is more representative of psychological well-being and less representative of physical function as disease advances, in parallel with overall QoL. The effect of disease duration on this adaptation is explored. Subjective health status may differ from health utility, and may be of importance for health technology assessment. Greater allocation for psychological health would be the most effective strategy to maximize subjective health status as ALS advances.
thakorn@ccf.org COG-19 Schizotypal traits across the frontotemporal dementia-motor neuron disease spectrum: pathomechanistic insights Background: Psychiatric symptoms are highly prevalent across the frontotemporal dementia-motor neuron disease (FTD-MND) spectrum, present in up to 50% of cases (1). Despite this, schizotypal personality traits that are characterised by subclinical psychotic-like perception, thoughts and behaviours and have been independently associated with an increased risk for the development of clinical psychosis, poor psychosocial outcomes and functional impairment, have never been examined (2). Objective: The current study aimed to address this gap in knowledge by providing the first, detailed examination of all subtypes of schizotypal traits across the FTD-MND spectrum, as well as exploring the neural underpinnings to characterize potential underlying mechanisms. Methods: 109 participants were recruited to the study (37 MND, 10 FTD-MND, 45 behavioural variant FTD, and 17 age-, sex-and education-matched healthy controls) and were administered the comprehensive 74-item Schizotypal Personality Questionnaire to assess Schizotypal traits (3). The association between changes in grey matter volume density and severity of schizotypal traits was examined using voxelbased morphometry (VBM) analysis on T1-wieghted structural magnetic resonance imaging.
Results: Relative to controls, pervasive schizotypal personality traits across the domains of positive and negative schizotypy and disorganised thought disorders were present across all patient groups (all p 0.001), confirming a wide spectrum of subclinical schizotypal symptoms, beyond classic psychotic symptoms, across the FTD-MND continuum. Atrophy in frontal, anterior cingulate and insular cortices, and caudate and thalamus was implicated in positive schizotypy, while integrity of the cerebellum was associated with disorganised thought disorder traits (corrected for cluster-extent multiple comparisons at p < 0.05, with a cluster-forming threshold of p < 0.001).
Discussion: The frontal-striatal-limbic regions underpinning manifestation of schizotypy in the FTD-MND spectrum are similar to those widely established in schizophrenia neuroimaging research. This finding expands the concept of a psychiatric overlap in FTD-MND and schizophrenia-related conditions and suggests potentially common underlying mechanisms involving disruptions to frontal-striatal-limbic networks in the emergence of psychotic symptoms. This also holds implications for future psychiatric and neurodegenerative disorder research, warranting a transdiagnostic approach for investigation and interpretation of the clinical phenomena related to psychosis. Background: People with MND and their family members (FmMND) face significant psychological adjustment challenges related to increasing disability and a markedly shortened lifespan, including increased depression and anxiety. It is unclear whether psychological interventions should be targeted to those showing most distress, as is typical in resource stretched services, or offered more broadly, including to those with lower distress levels. Given the challenge of MND, even those with no overt mental health symptoms may benefit from a psychological intervention to support emotional wellbeing and psychological resilience. Mindfulness has been shown to be efficacious across clinical and non-clinical populations, and has demonstrated efficacy for those with MND (1). In MND, it is not known whether this intervention may differentially affect those with mental health symptoms or without. Objectives: The aim of this research was to identify people with MND and FmMND with high versus low depressive symptoms, and to compare these groups in relation to (i) baseline demographic and psychological features and (ii) engagement with and response to a mindfulness intervention. Methods: Potential participants with early to middle stage MND were identified from the patient database at a multidisciplinary MND clinic. Fifty seven eligible patients and FmMND agreed to participate in a fortnightly four-session mindfulness program adapted for MND. A single arm waitlist design was employed, with assessments pre-waitlist (T1), pre-intervention (T2) and post intervention (T3). Measures examined depression and anxiety, demoralisation, quality of life, mindfulness skills, and benefit-finding. Assessments of between-session practice were also included. Analyses involved parametric and non-parametric statistics. Results: Forty participants (22 male, age: M ¼ 63yrs, range 20-88) completed the program (retention rate ¼70%). Based on the modified Hospital Anxiety and Depression Scale scores (2), participants were divided into high depression (n ¼ 11) and low depression (n ¼ 28) groups. Group comparisons showed no differences in demographic features or disease status and strong differences in baseline psychological characteristics. The groups showed different patterns of engagement of in mindfulness practice over the course of the intervention. In response to the intervention, the high depression group showed decreased anxiety (p < 0.05) and positive changes in benefit-finding and quality of life (p < 0.1), while change was not evident in the low depression group. Discussion: Measurable benefit from a mindfulness-based intervention was shown amongst those with increased depression. Where there are resource constraints in the provision of psychological interventions, they should be targeted towards those with mental health symptoms. A longer follow-up period would be needed to examine whether mindfulness interventions are protective of future distress in later stages of MND. Background: Up to 35-40% of non-demented ALS patients show oral language impairment within the spectrum of frontotemporal degeneration (1), whose diagnostic and prognostic entailments have been highlighted (2). Central dysgraphic features, reflecting actual language deficits, have been also described (3), although their clinical relevance in ALS is yet to be proved, especially for western languages, as current literature is mostly focused on Japanese patients (2,3). Objectives: To describe clinical and cognitive features of clinically non-aphasic ALS patients displaying writing errors (WE). Methods: Eight consecutive ALS patients with no other major co-morbidities underwent clinical assessment (ALSFRS-R; MiToS and King's staging) and both brief and multidomain cognitive screening: the ALS Cognitive Behavioural Screen (ALS-CBS) (4) and the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) (5). Writing was assessed via a writing-to-dictation task of an ad-hoc list of words and nonwords (29 stimuli). Results: Mean age of the 8 patients was 61.3 ± 12.5; mean education 13.5 ± 6.1; four were males. Mean disease duration was 39.4 ± 27.4 months. ALS phenotype was heterogeneous (3 classical; 2 flail leg; 1 pyramidal; 1 PLS; 1 respiratory); six patients had a spinal onset; bulbar signs at evaluation were present in four. Mean ALSFRS-R was 32.5 ± 5.4. Median MiToS and King's stages were 1 and 3, respectively. WEs were present in 6/8 patients (Mdn ¼3, IQR ¼2; range: 0-7) that could not be accounted for motor disabilities. No defective ALS-CBS scores were detected. Patients were subdivided according to the number of WEs: WEþ (making !3 errors), and WE-(making <3 errors). Among WEþ (N ¼ 4), three had below-cut-off ECAS-total and -ALS-Specific scores. Among WE-no patient had impaired ECAS-total/-ALS-Specific scores. Bulbar sign prevalence was 3/4 in WE þ vs. 1/4 in WEpatients. King's stage 3 was more represented in WE þ patient (3/4 vs. 1/4 WE-patient in King's stage 4). Phenotype, onset and MiToS across-group distribution revealed no specific frequency patterns. Discussion: Some western non-aphasic ALS patients show central dysgraphic features, in agreement with the literature regarding eastern patients (3). These patients present with executive/language dysfunctions that are detectable by multi-domain screens (ECAS). Therefore, writing-to-dictation tasks might be valid to test for frontotemporal involvement in this population, particularly in severely dysarthric patients. WE in ALS patients are associated with bulbar involvement and advanced disease (as assessed by King's staging). Background: Amyotrophic lateral sclerosis (ALS) is increasingly recognized as a multisystem neurogenerative disease, mainly affecting motor neurons, although also characterized by cognitive and behavioral impairment. Fatigue is a symptom of exhaustion unexplained by drug effects, or psychiatric disorders, associated with other fatigue-related symptoms, such as reversible motor weakness and whole-body tiredness, which are only partially relieved by rest. We aim at investigating the clinical correlations between both "physical" and "mental" components of fatigue, measured by the Multidimentional Fatigue Inventory (MFI) scale, and motor and cognitive/behavioral disability in ALS. Moreover, we investigated the correlations between these symptoms and brain resting state functional magnetic resonance imaging (RS-fMRI) alterations in a cohort of patients with ALS. Methods: 130 ALS patients were assessed for motor disability (ALS Functional Rating Scale-Revised, ALFRS-R); cognitive and behavioral dysfunctions (Edinburgh Cognitive and Behavioral ALS Screen, ECAS); fatigue (MFI); anxiety; apathy and daytime sleepiness. Moreover, the collected clinical parameters were correlated with RS-fMRI connectivity changes in 30 ALS patients who underwent MRI. Results: Multivariate correlation analysis revealed that "mental" fatigue was positively related to memory and executive measures. RS-fMRI analysis showed inverse correlations between functional connectivity in the right and left insula ("salience" network) and in the left middle temporal gyrus (Deafault Mode Network) and mental fatigue. Conclusions: Although the "physical" component of fatigue may be influenced by the disease itself, in ALS the "mental" component of fatigue correlates with cognitive and behavioral impairment, as well as with alterations of functional connectivity in extra-motor areas. Background: Perceived social isolation, also termed loneliness, impacts on neurobiological architecture, ensuing significant consequences on mental and physical health. In elderly people, it represents a risk factor for dementia. In ALS, loneliness has been find to be related to poor psychological health, however, the possible backlash of loneliness on behavioural and cognitive profiles of ALS has been poorly investigated. Objectives: To explore the association of loneliness with behavioural and cognitive symptoms of frontotemporal syndromes of ALS. Methods: Loneliness was measured using the 3-item UCLA Loneliness Scale (range: 0-6 points; no loneliness: 0 points, mild/moderate loneliness: 1-3 points, high loneliness: 4-6 points) in 206 consecutive ALS patients. The Edinburgh Cognitive and Behavioural ALS Screen, the Story-based Empathy Task and the Ekman 60-Faces Test were used to evaluate cognitive efficiency and social cognition. Behavioural symptoms were measured with the Frontal Behavioural Inventory (FBI-ALS) and the Dimensional Apathy Scale (DAS). Self-reports of mood (Hospital Anxiety and Depression Scale for motor neuron disease -HADS-MND), alexithymia, Difficulty in Emotional Regulation (DERSF) and quality of life (QoL) were also used. Spearman rho and Jonckheere-Terpstra test examined factors mainly associated with UCLA scores. Significance level was set at 0.001. Results: In our cohort, 126 patients (62%) reported no loneliness, 70 (33%) were classified as low/moderately lonely; and 10 (5%) felt highly lonely. UCLA scores were significantly associated with FBI (N ¼ 161; Rho ¼ À0.338), DAS (N ¼ 147; Rho ¼ À0.376), depression (N ¼ 99; Rho ¼0.402), anxiety (n ¼ 199; Rho ¼0.297), alexithymia (n ¼ 167, Rho ¼0.303), DERSF (N ¼ 176, Rho ¼0.447) and QoL scores (N ¼ 186; Rho ¼ À0.413), but not with cognitive abilities including social cognition. Motor functional status and disease duration were not related to loneliness. A significant cross-sectional effect was observed (p < 0.001) across degree of loneliness for behavioural changes (Z ¼ 4.109), apathy (Z ¼ 4.469), mood (Z ¼ 5.457), anxiety (Z ¼ 3.855), alexithymia (Z ¼ 3.852), DERS (Z ¼ 5.793) and QoL (Z ¼ À5.038). Discussion: Our findings indicated that, in ALS, the satisfaction of social environment is associated with a sense of life well-being that is not limited to the functional motor status. Loneliness was strongly related to neurobehavioral functioning and not with cognitive abilities. Depressive mood, anxiety, disinhibition and apathy were higher in lonely patients. Feelings of loneliness were also accompanied by subjective emotion dysregulation and poor emotional awareness, but not with empathy and emotion recognition dysfunctions. The relationship of loneliness to the behavioural and psychiatric symptoms is likely bidirectional; in some situations, it may act as a risk factor and in others it may be the consequence of these symptoms. Paying attention to social isolation in patients with ALS will help clinicians to intervene at an early stage. monica.consonni@istituto-besta.it respondents checked the following optional responses: general discussion with the patient/carer (29%), clinical judgement based on a semi-structured interview about the specific decision (28.0%), using a standardised assessment/tool (14%), and clinical judgement based on discussion within clinic (13%). If a cognitive screening indicated cognitive impairment, only 55.6% said that this would prompt a capacity assessment to be carried out if a specific decision needed to be made. When questioned if life-prolonging interventions would still be offered to those with cognitive impairment, around a third said yes, whilst around two-thirds said that it would depend (most often on the nature and degree of cognitive impairment). Conclusions: The study provides insight into the current practices of using capacity assessments in the clinical care of pwMND in the UK.
debbie.gray@ed.ac.uk families with ALS. However, due to lacking guidelines, ALS professionals are often uncertain about how they can provide optimal care to parents and children in families living with ALS. Objectives: To provide ALS care professionals with guidance on how to support parents and children in families living with ALS. Methods: A practical guide for family-centered ALS care was developed, based on (1) a systematic review of the literature on support needs of families with ALS and other life-limiting illnesses; (2) experiences, struggles and needs of parents and children living with ALS, explored through semi-structured interviews with 21 parents (8 with ALS) and 15 children; (3) a survey among ALS care professionals (N ¼ 65) to map the support ALS care professionals provide to families living with ALS and to identify their needs, struggles and knowledge gaps with regard to ALS care for families; and (4) input from a workgroup including ALS care professionals, ALS patients and relatives. Results: A practical guide incorporating generic and specific guidance was developed to facilitate ALS care professionals in supporting parents and children in families with ALS. Generic guidance involves education and collaboration between family and professional. Specific guidance is clustered around four themes: (1) awareness of the family as a system, (2) attention to the parental role, (3) psycho-education of parents, and (4) involvement of children in ALS care. The guide contains practical recommendations and tools for identifying family support needs, starting a dialogue about parenting issues, assessment of family functioning, and referral. Discussion: The findings have important implications for current ALS care practices. The newly developed guide for family-centered ALS-care may contribute towards improving the quality of care provided to families living with ALS. Although this guide has been developed in the Netherlands, the themes identified may be applicable in other countries/cultures as well. m.p.j.spijkerman-6@umcutrecht.nl