Theme 11 - Cognitive and Psychological Assessment and Support

 

 

COG-01 Making sense: an online meaning centred psychological intervention for individuals with MND

S. de Moree[email protected]

Abstract

Background

Stress and dysphoria often follow the diagnosis MND, with some adapting quickly and finding a new balance, while others struggle to come to terms with the diagnosis and continue experiencing high levels of emotional distress. As observed in our previous study in patients with MND and their partners, re-appraisal and finding meaning play a significant role in the process of psychological adjustment. Psychological counselling can be offered to those in need, but is often not available or not specifically enough.

Meaning Centred Psychotherapy

Breitbart’s Meaning Centred Psychotherapy (BMCP) has been found to be well-accepted and effective in advanced cancer patients. BMCP is a time-limited, manualized group psychotherapy, focusing on sense of meaning and thereby relieving distress and promoting psychological well-being and it has been proven effective in multiple RCT’s, also in the Netherlands. Most themes addressed in BMCP seem in clinical practice also suitable for MND patients, who are faced with similar complex issues related to the prospect of progressive physical decline and early death. Patients are supported and guided in a directive manner to focus on what is meaningful to them - by reflecting on their life and to thereby gaining sharper focus on what is important to them in the remaining time ahead of them. To date, BMCP has not been studied in MND patients. Physical barriers can be overcome by offering this therapy individually and online, where patients follow the programme from home with e-mail coaching and face to face video-consulting with the psychologist.

Methods

We have adapted the Dutch group protocol for the purpose of such an online approach specifically for individuals diagnosed with ALS or PMA patients and this resulted in the Making Sense training. At this moment we are testing acceptability, feasibility and patient satisfaction, while we document changes in distress over time. We hypothesize that offering 8-weeks the internet-based Making Sense training for distressed MND patients, is acceptable, feasible and helpful in reducing emotional MND related distress with subsequent improvement in quality of life. A mixed method approach, collecting both quantitative and qualitative data, is used. For pragmatic and ethical reasons, we chose the best alternative for the RCT design namely a randomized Single-Case Experimental (SCE) design that requires only a small sample (N = 5). This study aims to answer the following questions: 1. Is the Making Sense training acceptable, feasible and appreciated by patients with MND? 2. What are the effects on emotional MND related distress (primary outcome), perceived quality of life and sense of meaning (secondary outcomes)?

Results

At this moment three patients are included and started the training. Two new participants will be included and randomised soon. We will be able to present our first results in December 2022.

COG-02 Clinicoanatomical substrates of selfish behaviour in amyotrophic lateral sclerosis – an observational cohort study

D. LuleS. MichelsJ. FinselH. BraakK. Del TrediciJ. StrobelA. BeerI. UttnerH. MüllerJ. KassubekF. JuenglingA. Ludolph[email protected]

Abstract

Background

ALS primarily affects motor functions, but cognitive functions, including social understanding, may also be impaired. Von Economo neurons (VENs) are part of the neuronal substrate of social understanding and these cells are histopathologically altered in ALS.

Objective

We investigated whether activity in areas including VENs is associated with an impairment of cognitive tasks that mirror social functioning.

Methods

In this observational prospective study, ALS patients (N = 26) were tested for cognitive behavioural function, encompassing different aspects of empathetic understanding (interpersonal reactivity index, IRI), social behaviour (ultimatum game), recognition of faux-pas situations, and general cognitive functioning (Edinburgh Cognitive and Behavioural ALS Screen, ECAS). For in vivo pathological staging according to Brettschneider/Braak, DTI-MRI was performed to determine those ALS patients with expected pathological involvement of VENs (B/B ALS stages 3 + 4) compared to those without (B/B ALS stages 1 + 2). Expected hypometabolism of cerebral areas was determined with 18F-FDG PET in N = 20 ALS patients and compared to N = 20 matched healthy controls. Volume of interest analysis was performed in the anterior cingulate cortex (ACC) and the anterior insular cortex (AIC), which contain high numbers of VENs.

Results

Compared to those without expected pathological involvement of VENs (B/B ALS stages 1 + 2), ALS patients with anticipated pathological involvement of VENs (B/B ALS stages 3 + 4) presented with significantly reduced fantasy to understand the mindset of others (IRI) and, social behaviour was more selfish (ultimatum game) despite the fact that cognitive understanding of socially inappropriate behaviour of others (faux-pas) was unimpaired. 18F-FDG-PET showed hypometabolism in ACC and AIC in ALS patients with anticipated pathological involvement of VENs compared to those without and this was significantly correlated to cognitive-behavioral functions in certain tasks.

Discussion

Here, we present evidence of altered social behaviour in ALS patients associated with regional 18FDG-PET hypometabolism in areas with a high density of VENs, thereby suggesting a possible causal association.

COG-03 A novel EEG paradigm to quantify the cortical network dysfunction underpinning verbal fluency impairments in ALS

R. McMackinS. PlaitanoM. MetzgerV. SirenkoE. GigliaP. MehraY. TadjineS. BistaN. PenderO. HardimanB. Nasseroleslami[email protected]

Abstract

Background

Deficits in verbal fluency are among the most common non-motor impairments which occur in ALS. Such symptomatic changes can be quantified by the Boston Naming Task (BNT) (1). However, performance in this task may not capture early or subtle impairments in verbal fluency, despite underlying pathophysiology. Further, performance in the BNT may be impaired by deficits in language/speech-generating cortical networks, in addition to those required for executive functioning (2).

Objectives

We aimed to design a novel paradigm to enable direct and distinct measurement the cortical network activities involved in language and executive functioning during BNT performance. We intend to use these measures to objectively, sensitively and non-invasively measure the pathophysiology underpinning verbal fluency impairment in ALS. Such measures may provide an early biomarker of verbal fluency disruption in ALS.

Methods

A modified version of the BNT is performed during recording of 128-channel EEG. The average cortical activation (event related potential, ERP) which occurs during correct naming of objects is compared to that captured during matching, non-naming trials. Recruitment is ongoing, with datasets from 3 controls and 14 people with ALS collected. Analyses based on datasets from 15 controls and 25 people with ALS will be presented at the 2022 MNDA Symposium.

Results

Preliminary examination of ERPs collected to date indicate that naming-associated ERP peaks over the lateral temporal and parietal cortices, as well as the right inferior frontal cortex, are smaller in those with ALS patients.

Discussion

A completed analysis comparing the BNT ERP between those with ALS and controls, as well as correlational analysis investigating the relationship between ERP components and symptomatic verbal fluency impairment in ALS, will be reported at the Symposium.

Acknowledgments

We thank the MNDA for funding this work.

COG-04 Premorbid psychological resilience is protective against cognitive deterioration in motor neuron diseases

P. FerraroE. GervinoG. MeoM. CilleraiM. PardiniL. RoccatagliataA. SchenoneC. Caponnetto[email protected]

Abstract

Background

Psychological resilience (PR) is defined as the human capacity to overcome adverse events using individual and social resources, and to use crises as an incentive for personal growth. Recent investigations have observed that PR is significantly associated with more well-preserved cognitive function in healthy subjects, but a specific investigation of such phenomenon in patients with motor neuron diseases (MNDs) is still lacking.

Objectives

To evaluate PR in MNDs, and to explore its relationship with cognitive, behavioral and mood symptoms in order to test the hypothesis that greater PR might be protective against extra-motor clinical manifestations of MND.

Methods

72 MND patients and 62 age and gender matched healthy controls (HCs) were included in the study. PR was assessed using the 10-items Italian version of the Connor-Davidson Resilience Scale (CD-RISC-10), patients were asked to provide responses they would have given to the statements before their diagnosis. MND cases were further evaluated for cognitive and behavioral disturbances using the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and for mood symptoms using the Hospital Anxiety and Depression Scale (HADS). Performances on CD-RISC-10 were compared between patients and HCs using the Mann–Whitney U test, and linear regression models were then applied to evaluate the role of CD-RISC-10 scores in predicting cognitive performance and severity of behavioral and mood symptoms in MND patients.

Results

MND cases showed significantly greater premorbid PR compared to HCs (CD-RISC-10 total score: p = 0.009, CD-RISC-1: p = 0.04, CD-RISC-3: p = 0.002, CD-RISC-5: p = 0.003, CD-RISC-6: p = 0.02, CD-RISC-7: p = 0.003, CD-RISC-8: p < 0.001, CD-RISC-10: p = 0.03).

In MND patients higher premorbid PR levels were significant predictors of greater overall cognitive performance (β values ranging from 2.21 to 2.39 and p values ranging from 0.04 to 0.05 for the ECAS total score) and more preserved ALS non-specific functions (β values ranging from 0.75 to 0.88 and p values ranging from 0.006 to 0.03 for the ECAS ALS non-specific functions score), memory in particular (β values ranging from 0.68 to 0.79 and p values ranging from 0.01 to 0.05 for the ECAS memory score). Additionally, greater premorbid PR levels were significant predictors of less severe depressive and anxiety symptoms (β values ranging from 0.16 to 1.20 and p values ranging from <0.001to 0.01 for the HADS depression score, and β values ranging from 0.12 to 1.21 and p values ranging from 0.002 to 0.05 for the HADS anxiety score) while no association was observed with the presence or severity of behavioral disturbances.

Discussion

Our results suggest that PR is an important protective factor against cognitive deterioration in MNDs, and provide preliminary evidence for the potential usefulness of resilience enhancement psychological interventions as a future strategy to prevent or delay the onset of cognitive disturbances in these neurodegenerative conditions.

COG-05 Quantifying network dysfunction underpinning social cognition impairments in ALS with EEG

R. McMackinS. PlaitanoM. MetzgerV. SirenkoE. GigliaP. MehraY. TadjineS. BistaM. SabbaghN. PenderO. HardimanB. Nasseroleslami[email protected]

Abstract

Background

Deficits in recognition of emotions is part of the cognitive impairment which occurs in ALS. Such symptomatic changes in social cognition can be quantified by the Reading the Mind in the Eyes Task (RMET) (1). However, performance in this task may not capture early or subtle impairments in social cognition, despite underlying pathophysiology. It has previously been demonstrated that social cortical network engagement during the RMET can be quantified using electroencephalography (EEG) (2).

Objectives

To determine if dysfunction in cortical networks driving social cognition can be directly captured and quantified in ALS using EEG.

Methods

A modified version of the RMET is performed during recording of 128-channel EEG. The average cortical activation (event related potential, ERP) which occurs during correct recognition of individuals’ emotional state is compared to that captured during recognition individuals’ sex, as a non-social control. Recruitment is ongoing, with datasets from 4 controls and 14 people with ALS collected to date. Analyses based on datasets from 15 controls and 25 people with ALS will be presented at the 2022 MNDA Symposium.

Results

Preliminary examination of ERPs collected to date and comparison to control data reported previously (2) indicate that the N240-400 ERP associated with social cognition network activation is smaller in those with ALS compared to controls.

Discussion

A completed analysis comparing the N240-400 ERP between those with ALS and controls, as well as correlational analysis investigating the relationship between this ERP and symptomatic social cognitive impairment in ALS, will be reported at the Symposium.

Acknowledgments

We thank the MNDA for funding this work.

COG-06 Experiences, coping strategies and support needs of family caregivers of ALS-patients post-bereavement

S. PujicL. Knudsen[email protected]

Abstract

Background

Family caregivers are an important source of support for patients with ALS. It is well-documented that family caregivers’ own physical and mental health and everyday life can be negatively affected by the course of the disease. Less is known about the long-term consequences post-bereavement for the caregivers, but there are indications that they may have an increased risk of anxiety, depression and prolonged grief disorder (1,2). The aim was to investigate the post-bereavement experiences and coping strategies of family caregivers and the types of support they would find beneficial.

Method

A qualitative study using semi-structured focus group interviews with family caregivers of patients with ALS post-bereavement. The methodology used was Interpretive Description and the theoretical framework was Antonovsky’s salutogenic theory of Sense of Coherence.

Results

Data collection is expected to be finished mid-August after which results will be analyzed and presented at the International Symposium on ALS in December.

Discussion

We expect the findings to point towards important support initiatives for bereaved family caregivers.

Funding

Funding for the study was received from Helsefonden, Familien Hede Nielsens Fond, Aage and Johanne Louis-Hansens Fond as well as Dansk Selskab for ALS.

Acknowledgements

The authors wish to thank the family caregivers for their participation in the study as well as Associate Professor Charlotte Handberg for her advice.

COG-07 Cognition in the course of ALS – a meta-analysis

J. FinselI. UttnerC. Vazquez MedranoA. LudolphD. Lule[email protected]

Abstract

Background

The question of cognitive decline in the course of ALS has been controversially discussed.

Objective

The goal of this meta-analysis is to improve insight into the development of cognition over the course of ALS and to assess predictors of cognitive performance.

Method

A literature search was conducted in Pubmed and Web of Science on 29 July 2019 and 16 March 2021. Data were screened in Endnote® Version X9. Meta-analyses and meta-regressions were calculated for cross-sectional data using Rstudio®. Studies were assigned to temporal and physical categories and Hedges’ g was calculated for the respective categories to provide an estimate of a cognitive course based on cross-sectional data. Due to low numbers and heterogeneity in reporting, longitudinal studies were analysed descriptively.

Results

A total of N = 45 cross-sectional and N = 13 longitudinal studies were included. Impairments in all cognitive domains, except verbal IQ, were found in ALS patients (PALS). PALS showed stable cognitive performances in cross-sectional and in most longitudinal studies. PALS with symptoms for 18–24 months and PALS who had an ALSFRS-R score of 40–36 were the most frequently reported subgroup regarding neuropsychology. Age was related to visuospatial functioning, and depressiveness to attention. In longitudinal studies, impact of site of onset and cognitive status at baseline on cognitive course was found.

Discussion

Despite vast evidence for cognitive impairment at disease onset in different domains, evidence for evolution of these deficits is rather limited, suggesting that PALS present with cognitive impairment early in the course possibly in a sense of disease trait.

COG-08 Disease onset as a motor determinant of social cognition performances in amyotrophic lateral sclerosis

F. PalumboB. IazzolinoL. PeottaA. CanosaU. ManeraM. GrassanoF. CasaleG. PellegrinoR. VastaC. MogliaA. ChiòA. Calvo[email protected]

Abstract

Background

Social Cognition (SC) deficits may be a distinctive feature of Amyotrophic Lateral Sclerosis (ALS) patients since the early stages of the disease (1). Up to date, however, cognitive and motor correlates of SC deficits in ALS patients are still unknown.

Objective

To assess SC performances in Amyotrophic Lateral Sclerosis patients, stratified by cognitive profile and onset site.

Methods

105 ALS patients attending the Turin ALS Center underwent neurological assessment, cognitive evaluation with a comprehensive battery of tests and SC assessment through the Ekman-60 Faces test (EK-60F), the Reading the Mind in the Eyes-Test (RMET-36) and the Story-Based Empathy Task (SET). Patients were classified into five cognitive categories according to Strong’s consensus criteria (2): Cognitively normal (ALS-CN), patients with cognitive impairment, behavioural impairment, cognitive and behavioural impairment, fronto-temporal dementia. For the aim of this study we considered only ALS-CN patients and we stratified them according to onset site (spinal vs bulbar). 42 healthy sex-, age- and education-matched controls were enrolled for comparative analysis in all SC tests.

Results

ALS-CN patients (n = 60) performed significantly worse than controls in RMET-36 (p < 0.001), EK-60F Global Score (p < 0.001), recognition of disgust, fear, and sadness (p < 0.001). Both ALS-CN patients with spinal and bulbar onset performed significantly worse than controls in the EK-60 Global Score (p = 0.04; p < 0.001 respectively), recognition of fear (p = 0.024; p = 0.002) and sadness (p = 0.008; p = 0.002), SET-Emotion Attribution and SET-Global Score (p < 0.001; p < 0.001). However, ALS-CN with bulbar onset performed significantly worse than controls in RMET-36 (p = 0.03) and SET-Intention attribution (p < 0.02), while ALS-CN patients with spinal onset did not differ from control in the same two tests (p = 0.09; p = 0.12, respectively).

Conclusions

Our results show that bulbar-onset ALS-CN patients are more impaired in RMET-36 and SET-IA than spinal-onset ALS-CN patients, compared to controls. This differentiated pattern of impairment in affective and cognitive ToM (RMET-36 and SET-IA, respectively) related to the onset site, may be due to the presence of a subtle affective and/or cognitive impairment, present to a greater extent in bulbar-onset patients, also when categorized as ALS-CN (3). Further longitudinal studies are needed to verify these findings; however our results emphasize that deficits in Theory of Mind abilities, requiring the integration and convergence of both emotional and cognitive abilities, may be a potential marker of an early cognitive impairment in ALS patients.

COG-09 Screening for visuospatial abilities in amyotrophic lateral sclerosis: a pilot study using the Battery for Visuospatial Abilities (BVA)

M. SharbafshaaerM. SicilianoC. PassanitiG. TedeschiL. TrojanoF. Trojsi[email protected]

Abstract

Introduction

Cognitive and behavioural deficits are common in Amyotrophic Lateral Sclerosis (ALS), which are mostly related to frontotemporal dysfunction. Conversely, less impairment of visuospatial abilities has been described in ALS. However, this multi-faceted domain of cognition could be better explored in ALS using a battery specifically devised for assessing visuospatial abilities with minimal or no requirement of verbal mediation, tapping basic or complex visuospatial processing.

Objectives

Our pilot study aims at screening visuospatial abilities in a group of ALS patients without an executive and/or language dysfunction, compared to healthy controls (HC) using the Battery for Visuospatial Abilities (BVA).

Method

Twenty-three ALS patients and twenty-three age-matched HC underwent a BVA battery, which comprises eight tasks, evaluating both visuo-spatial “perceptive” and “spatial cognition” abilities. Multivariate Analysis of Covariance (MANCOVA) test, using education as a covariate, was used to compare the ALS group to HC. All included patients had unimpaired executive and language functions at the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).

Results

Between-group analyses showed that, when compared to HC, ALS obtained lower scores in Line Orientation Judgment (LOJ) and Point Position Identification (PPI) tasks, both measuring visuo-spatial “perceptive” abilities.

Conclusions

Our findings suggest that ALS is a multisystem disorder and emphasizes the importance of visuospatial evaluations in the multidisciplinary assessment of ALS patients to address impairment of this domain early in the disease process.

Acknowledgments

The authors thank all the participants in this research.

COG-10 People of color living with and impacted by ALS and community created intervention

K. ByrdJ. ReyesL. McMullen[email protected]

Abstract

Background

An ALS diagnosis presents many physical, social and psychological challenges. But, an ALS diagnosis in people of color presents another set of challenges. According to Racial and ethnic differences among amyotrophic lateral sclerosis in the United States, “Of the 5,883 unique ALS cases identified, 74% were White, 9.3% were African-American Black, 3.6% were Asian, 12.0% were of an unknown race and 0.3% were marked as some other race.” The article focuses on differences in terms of numbers. It doesn’t explore real world questions like, How does systemic racism impact people living with ALS? I AM ALS is a community-led US non-profit revolutionizing ALS Advocacy. Community members self-organize into six community teams. The newest, Many Shades of ALS, formed as a result of I AM ALS’ inclusion initiative. A little over a year old, the team has partnered with organizations serving people of color and Many Shades of ALS has hosted several mental health events.

Objectives

To understand, raise awareness and share challenges an ALS diagnosis presents for people of color.

Methods

I Am ALS hosted several listening sessions where issues specific to people of color diagnosed with or impacted by ALS, were discussed. As a result, the Many Shades of ALS team formed with the goal of addressing issues like racial inequities in health care, accessibility and mental health.

Results

I AM ALS worked with people living with ALS, caregivers and mental health professionals to build a team that identifies issues specific to people of color and develops strategies to resolve those issues.

Discussion

Within the ALS landscape, ALS is discussed in terms of its impact on finances, the body, and the family. It is rare to hear ALS discussed in terms of its impact on mental health, race and bias in diagnosis. The Many Shades of ALS team continues to explore these issues to help bring about a change.

Acknowledgements

We would like to thank the people living with ALS, caregivers and people impacted by ALS for sharing their candid stories and ideas.

COG-11 Peripheral immunity involvement in the cognitive impairment of sporadic amyotrophic lateral sclerosis

T. YangQ. WeiC. LiB. CaoR. OuY. HouL. ZhangK. LiuJ. LinY. ChengY. XiaoH. Shang[email protected]

Abstract

Background

Recent studies have suggested that immune activation plays important roles in amyotrophic lateral sclerosis (ALS) (1). However, the effects of peripheral immunity on cognitive impairment in sporadic ALS remains poorly characterized.

Objectives

To evaluate the association between peripheral immune parameters and cognitive impairment in patients with sporadic ALS.

Methods

A case-control study of 289 patients with sporadic ALS was conducted. All patients underwent cognitive assessment and blood immune parameters measurements. The main outcomes were the adjusted odds ratio (OR) in multivariate logistic regression analysis and the adjusted coefficient in multivariate linear regression model. Sensitivity analysis was performed restricting by the King’s clinical stage.

Results

Cognitive impairment was detected in 98 (33.9%) patients. Higher counts of leukocyte (OR, 0.53; 95% CI, 0.29–0.95; p = 0.03), neutrophil (OR, 0.48; 95% CI, 0.26–0.88; p = 0.02), and monocyte (OR, 0.33; 95% CI, 0.18–0.60; p < 0.001) were significantly associated with better cognitive preformence in sporadic ALS, specifically in patients who were in King’s clinical stage 1 and 2. Whereas higher percentage of CD4+ T cell was found to increase the risk of cognitive impairment (OR, 2.79; 95% CI, 1.52–5.09; p = 0.001), especially in patients who were in King’s clinical stage 3.

Discussion

These findings highlight that peripheral immunity is involved in the cognitive impairment of sporadic ALS and might play dynamic and complex roles — beneficial and damaging — according to the disease stages. Clarification of associations between immunity and ALS helps to inform the pathophysiological mechanisms responsible for and potential immunotherapies for this fatal neurodegenerative disease.

Acknowledgments

We would like to thank the patients and their families for taking part in the study.

COG-12 The Italian version of the Hospital Anxiety and Depression Scale for use in Motor Neurone Disease (HADS-MND): detecting mood disorder in a large sample of MND patients

M. ConsonniV. FaltraccoD. PainA. TelescaE. Dalla BellaE. BersanoG. MoraG. Lauria[email protected]

Abstract

Background

The Hospital Anxiety and Depression Scale for use in motor neurone disease (HADS-MND) is used to assess symptoms of anxiety and depression in patients with motor neurone disease. This scale has never been specifically validated in Italian for use within this population.

Objectives

This study aimed at investigating the utility of the Italian Version of the HADS-MND (itHADS-MND) as a screening instrument for anxiety and depression in MND. The study also aimed at exploring the interplay between itHADS-MND and cognitive, behavioural and motor-functional impairment.

Methods

Two-hundred seventy-five patients with MND were administered the itHADS-MND and cognitive (Edinburgh Cognitive and Behavioural ALS Screen, ECAS), behavioural (Frontal Behavioural Inventory [FBI], Dimensional Apathy Scale [DAS]) and psychological assessment. Depression was evaluated using the Beck Depression Inventory (BDI-II >20), anxiety with the State-Trait Anxiety Inventory (STAI >50). Motor functional disability was measured with the revised ALS Functional Rating Scale (ALFRS-R). ItHADS-MND factorial structure and internal consistency were assessed with principal component analyses (PCA) and Cronbach’s alpha. Receiver operating characteristics (ROC) analysis was used to test the discriminant validity for anxiety and depressive disorders. Finally, correlational analyses were performed to test convergent and divergent validity.

Results

PCA identified two correlated components clustering anxiety and depression subscale items, with adequate internal consistency (0.73 and 0.82, respectively). The comparison of the areas under the curve (AUC) between the two subscales didn’t show any difference in identifying anxiety (p = 0.935) and depression (p = 0.152). The total scale showed a high internal consistency (0.853) and a high discriminating power for moderate levels of anxiety and depression (AUC =0.83; 95% CI =0.78–0.87). The cut-off point maximising sensitivity (73%) and specificity (77%) was >10; accordingly, 89 patients had altered affective state. ItHADS-MND total scores were highly related to BDI-II, STAI, emotional lability and emotional dysregulation (p < 0.001). No significant correlations were found between itHADS-MND, ECAS and DAS (p > 0.05). ItHADS-MND was positively related to FBI (p < 0.05) and negatively associated with ALSFRS-R scores (p < 0.005).

Discussion

The outcome of the PCA confirmed the structure of the original version of the HADS-MND, however the use of the anxiety and depression subscales as outcome measures should be considered with caution. ROC analyses suggest to use the total itHADS-MND score as a screening for psychiatric disorders in MND. Correlation analyses confirmed the scale as a higher-order measure of psychological distress, affecting one-third of patients. The paucity of association with cognitive and behavioural symptoms suggests that the HADS-MND is partially independent from frontotemporal spectrum disorders, but likely related to motor functional disability.

Funding

The study was supported by Fondazione Regionale per la Ricerca Biomedica, Regione Lombardia [2015-0023], Fondo Europeo di Sviluppo Regionale, Regione Lombardia [POR FESR 2014-2020; 1157625], and Ricerca Corrente from the Italian Ministry of Health.

COG-13 Apathy in amyotrophic lateral sclerosis, and how it influences trial participation

J. FoucherR. RadakovicJ. HellqvistC. Ingre[email protected]

Abstract

Background

Apathy appears to be present in several neurodegenerative disorders, both as a syndrome as well as a symptom. Apathy was for a long time considered to be unidimensional, but is now acknowledged to be divided in three apathy subtypes: “cognitive apathy”, “emotional-affective apathy” and “auto-activation apathy” (also called “initiation apathy”) (1), which can be captured by the dimensional apathy scale (DAS) (2). When discussing eligibility for clinical trial participation for ALS patients, we believe apathy influences the patient’s willingness to participate and thus hampers the patients trial participation at an early stage.

Objectives

To validate the DAS in Swedish and investigate the extent of apathy among the Swedish ALS cohort, and if the amount of apathy influences participation and compliance in clinical trials.

Methods

During a clinical visit, we have collected detailed information including an exam performed by a neurologist with extensive experience in ALS. In about 4 weeks after diagnosis, we conduct tests for ALS specific cognitive symptoms, Edinburgh Cognitive and behavioural ALS Screen (ECAS), as well as for depression, anxiety, linguistic ability, QoL, and activities of daily living including apathy (using the DAS scale). The collection of information has been performed at diagnosis, and then again, every 6 months thereafter. A control group (matched healthy controls consisting of spouses and siblings) will contribute with the same questionnaires as the patients. We aim to include 125 patients over a period of 2 years, and will also gather information about if the patients were approached for clinical trial participation, the screening and enrolment procedures, compliance when in a trial and any pre- termination issues.

Results

Over 20 patients and 10 controls have been included in this project in only a bit over 2 months, April 2022. Recruitment of new patients will be ongoing until April 2024.

Conclusions

This study is the first to describe the extent of apathy in a Swedish ALS cohort, described by the different apathy subtypes, investigating their potential impact on trial participation.

COG-14 Italian adaptation of the Beaumont Behavioural Inventory (BBI): psychometric properties and clinical usability

L. PeottaB. IazzolinoD. PainM. GallucciA. RadiciF. PalumboA. CanosaC. MogliaA. CalvoG. MoraA. Chiò[email protected]

Abstract

Objective

ALS is a neurodegenerative disease affecting motor neurons and, in 50% of cases, showing cognitive and behavioural alterations within the spectrum of frontotemporal degeneration (FTD). Behavioural dysfunctions in ALS patients are crucial to be early identified and they negatively impact on management, prognosis and survival (1). This study aimed at investigating psychometric properties and feasibility of the Beaumont Behavioural Inventory (BBI).

Materials

The BBI is a self-explanatory questionnaire to be administered to caregivers to grade ALS patients’ FTD-like behaviours (2). It includes 41 items assessing a variety of behavioural changes typical of fronto-temporal degeneration, cognitive changes and psychotic symptoms. The original BBI was translated in Italian by two independent bilingual Authors. ALS patients underwent clinical, cognitive, mood/anxiety and further behavioural (Frontal System Behaviour Scale, FrSBe; Frontal Behavioural Inventory, FBI) evaluation.

Methods

Ninety ALS patients were compared to 100 healthy participants (HPs) on the BBI. Validity, reliability, sensitivity and specificity of the BBI were assessed. Patients were classified throughout full neuropsychological assessment in cognitively normal (CN), cognitively impaired (ALS-ci), behaviourally impaired (ALS-bi), or both (ALS-cbi), and FTD (ALS-FTD) (3). Correlations were assessed between BBI and demographic measures, FrSBe, FBI, FAB, ECAS, ALS-FRS and HADS.

Results

The BBI was significantly related to FrSBe and FBI scores, whereas not to other measures. A Principal Component Analysis yielded a mono-component structure; Cronbach’s α was 0.93. By addressing the FrSBe as the gold standard, the BBI reached optimal sensitivity (85.7%) and specificity (79.7%) at a cut-off of 10.5. BBI-after scores proved to significantly differ across groups: ALS-ci/bi/cbi/FTD patients reported higher scores than both ALS-CN and health controls (z = 3.49; p = 0.002 and z = 4.49; p < 0.001, respectively) although no difference was detected between these two last groups (z = 0.981; p = 0.979).

Discussion

The BBI proved to be sensitive to changes in behaviour as well as to discriminate between different degrees of dysfunction. The BBI showed divergent validity with cognitive measures (ECAS, FAB) and no associations were found with mood measures and disease staging. Moreover, the BBI proved to be more accurate than the FrSBe and the FBI in clinical classifications. Its clinical usability is encouraged in ALS patients as being able to sensitively and specifically detect FTD-like behavioural changes.

COG-15 Stigma experienced by ALS patients and their caregivers: a mixed-methods study

M. Sommers-SpijkermanM. KavanaughE. Kruitwagen-van ReenenA. Zwarts-EngelbertA. Visser-MeilyA. Beelen[email protected]

Abstract

Background

In absence of a cure, optimizing quality of life is a major focus in the care for those living with amyotrophic lateral sclerosis (ALS). A currently little explored psychological factor that may be relevant to a more comprehensive understanding of quality of life among people living with ALS is health-related stigma. Health-related stigma can be defined in terms of enacted and felt stigma. Enacted stigma involves the actual experience of discrimination or social exclusion. Felt stigma refers to shame of being deviant and the feeling that discrimination or social exclusion will happen. Felt stigma may not only impact patients with ALS, but also their caregivers, referred to as affiliate stigma.

Objectives

To explore stigmatising experiences faced by Dutch ALS patients and their caregivers as well as their impact on psychological health and distress.

Methods

Cross-sectional surveys were completed by 193 ALS patients and 87 caregivers. Outcomes included stigma (Stigma Scale for Chronic Illness, Affiliate Stigma Scale), psychological health (WHOQoL-BREF), psychological distress (HADS) and caregiver burden (ZBI-12). Manifestations of stigma were explored in more depth through semi-structured interviews with 8 ALS patients and 11 caregivers. The impact of stigma on psychological health/distress was estimated using hierarchical multiple regression analysis.

Results

More than half of patients indicated experiencing enacted stigma (n = 120, 62%). Two manifestations of enacted stigma emerged from the interviews: (1) experiences of social exclusion (e.g. relationship distancing, being excluded in conversations) and (2) stigmatising attitudes and behaviours displayed by other people (e.g. staring, patronizing). In addition, 78% of patients (n = 150) and caregivers (n = 68) reported felt stigma. Felt stigma manifested itself in three ways, including (1) experiences of alienation (e.g. feeling ashamed/embarrassed, inferior or left-out), (2) perceived discrimination (e.g. feeling ignored, judged or misunderstood) and (3) anticipated stigma (e.g. fear of being excluded). Patients and caregivers were found to engage in both concealing responses (e.g. social withdrawal, maintaining secrecy) and resisting responses (e.g. disclosure, deflecting) to stigma. In both patients and caregivers, felt stigma but not enacted stigma was found a significant predictor of psychological health and distress. Felt stigma also significantly predicted caregiver burden.

Discussion

Our findings suggest that the majority of ALS patients and their caregivers have experiences of stigma, which, if not addressed in healthcare, may adversely impact on their psychological adjustment and quality of life. ALS care professionals may play an important role in supporting and promoting stigma-resisting strategies among patients and caregivers through creating awareness of stigmatising social experiences that patients and caregivers may encounter and discussing such experiences. Our findings highlight a range of perceptions and experiences underlying stigma that may serve as conversation topics for professionals.

Acknowledgements

We thank the ALS Association for financially supporting this study.

COG-16 A systematic review of factors associated with grief in informal carers of people living with motor neurone disease

A. TruccoT. BackhouseE. MioshiN. Kishita[email protected]

Abstract

Background

Family carers of people living with Motor Neurone Disease (MND) are at a higher risk of experiencing clinically significant levels of grief. Grieving processes can start before the care recipient’s death, a phenomenon known as anticipatory grief (1). When distressing grieving experiences remain untreated following the death of the care recipient (post-death grief), it can lead to a persistent grief reaction (complicated/prolonged grief) (2). Currently, evidence on interventions that can effectively target different types of grief is scarce. Therefore, identifying factors affecting grieving processes in this population is important to inform future research and practice.

Objective

This review aimed to identify factors associated with anticipatory grief, post-death grief and complicated/prolonged grief in family carers of people with MND.

Methods

A search of six electronic databases (Scopus, MEDLINE, CINAHL, PsycINFO, SciELO and LILACS) was conducted from their inception to 21 November 2021. Quantitative and qualitative studies reporting factors associated with grief and published in English, Spanish and Portuguese were included. Findings were synthesized using thematic analysis.

Results

Two quantitative studies and eight qualitative studies were identified. The majority of carers included in the identified studies were female spouses of the person living with MND. Five overarching themes were generated to illustrate factors associated with different types of grief: (1) nature of MND (e.g. unpredictability of the condition, negative experiences of caregiving), (2) familial and social life (e.g. changes in relationships, loss of purpose in life), (3) support (e.g. lack of bereavement support and psychological support), (4) carers’ emotional reactions (e.g. anxiety/depression, emotional avoidance) and (5) perceptions and experiences of death (e.g. accepting the need for death, planning for death).

Discussion

The findings suggest that there may be factors particularly important to target during the early grieving processes (anticipatory/post-death grief) such as the knowledge about the progression of the disease, change in relationships, anxiety and depressive symptoms of carers and planning for death. Factors that may affect all three types of grief, including prolonged grief, were also identified such as negative experiences of caregiving, getting caught in loss experiences, end of life and psychological support and emotional avoidance coping. Future research could investigate whether targeting such potential factors may lead to improved grieving experiences at different stages among carers. The number of included studies in the review was small, and thus the findings need to be interpreted with caution. More studies focusing on modifiable factors affecting grief experiences are needed to provide further clinical implications.

Funding

APT’s PhD studentship is funded by MND Scotland.