Theme 11 - Cognitive and Psychological Assessment and Support

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 reflect-ing 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 pro- gramme 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 sub-sequent 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 ¼ 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 (second- ary 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. Background: ALS primarily affects motor functions, but cog- nitive functions, including social understanding, may also be impaired. Von Economo neurons (VENs) are part of the neur- onal 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 ¼ 26) were tested for cognitive behavioural function, encompassing different aspects of empathetic under- standing (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 stag- ing according to Brettschneider/Braak, DTI-MRI was performed to determine those ALS FDG PET in N 20 ALS patients and compared to N 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 þ 2), ALS patients with anticipated pathological involvement of VENs (B/B ALS stages 3 þ 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 antici- pated 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. 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, perform- ance 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 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 cogni- tive 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 pro- tective 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 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 dis- turbances in these neurodegenerative conditions. 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 patho- physiology. It has previously been demonstrated that social cortical network engagement during the RMET can be quan- tified using electroencephalography (EEG) (2). Objectives: To determine if dysfunction in cortical networks driving social cognition can be directly captured and quanti- fied in ALS using EEG. Methods: A modified version of the RMET is performed during recording of 128-channel EEG. The average cortical acti- vation (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) the post-bereavement experiences and coping were screened in Endnote V R Version X9. Meta-analyses and meta-regressions were calculated for cross-sectional data using Rstudio V R . Studies were assigned to temporal and physical categories and Hedges ’ g was calculated for the respect-ive categories to provide an estimate of a cognitive course based on cross-sectional data. Due to low numbers and het- erogeneity in reporting, longitudinal studies were analysed descriptively. Results: A total of N ¼ 45 cross-sectional and N ¼ 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 base-line 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 pre- sent with cognitive impairment early in the course possibly in a sense of disease trait. Background: Social Cognition (SC) deficits may be a distinct- ive feature of Amyotrophic Lateral Sclerosis (ALS) patients since the early stages of the Conclusions: Our results show that bulbar-onset ALS-CN patients are more impaired in RMET-36 and SET-IA than spi-nal-onset ALS-CN patients, compared to controls. This differ- entiated 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 co