Emotional processing, cognition and well-being in ALS: evidence from behavioral and physiological level and professional medical encounter

In addition to physical impairment, patients with amyotrophic lateral sclerosis (ALS) may present with some extra-motor symptoms such as changes in emotional processing, behavior and cognition. So far it has not been fully understood how these symptoms interfere with patients’ well-being and how they influence medical encounter with professionals. Therefore, the aim was to study extra-motor manifestations including emotional processing, signs of pathological laughing and crying and cognition. Additionally, impact of these on patients’ decision status for or against therapeutic interventions and professionals’ perspective on the influence of these interventions on patients’ well-being were studied. Emotional processing in ALS was studied by asking patients (N=30) and healthy controls (N=29) to rate basic facial emotions on a computer screen and view the facial expressions in a functional magnetic resonance image scanner (subset of N=15 patients and N=14 healthy controls) while their cortical activity was recorded. Furthermore, patients (N=10) and healthy controls (N=10) were asked to rate emotionally laden and neutral pictures while listening to happy or sad music extracts. This was correlated with their emotional lability score. Influence of patients’ cognition on their therapy preferences was studied by including N=169 patients who filled out a questionnaire on their decision making regarding life prolonging measures. This was correlated with patients’ cognitive status. Finally, physicians perspectives were studied by asking N=105 neurologists to estimate depressiveness and quality of life of ALS patients with life prolonging measures and by comparing these with patients’ subjective ratings. Emotional processing, especially of negative material, was impaired: ALS patients recognized disgust and fear less than healthy controls and showed decreased activity in cortical areas related to these emotions. Especially when viewing sad faces patients presented with decreased activity in hippocampus bilaterally together with increased activity in the right inferior frontal gyrus, which was positively correlated with the patient’s social activity. This suggests a compensatory cortical function that might be enhanced by positive influence of social activity in life. ALS patients with pathological laughing and/or crying tended to rate neutral pictures accompanied by sad music more negatively. Patients with a high positive emotional lability score rated happy pictures more positively and sad pictures more negatively, while patients with a high negative lability score rated happy pictures more negatively. Therefore, our results suggest that ALS patients with pathological laughing and/or crying might exhibit reduced inhibitory mechanisms and are in general more sensitive to the negative component of emotion regulation. Patients’ decisions regarding life prolonging measures, hypothetical ideation to turn off these measures or patients’ wish for hastened death were not associated with their cognitive status or behavioral changes. Therefore, decision making by proxies should not necessarily be rushed. Furthermore, with regards to patients’ psychological well-being with life prolonging measures, highly experienced neurologists estimated quality of life and depressiveness closer to patients’ subjective estimation than neurologists with low experience on ALS. Thus, neurologists with significant experience are able to correctly recognize patients’ affective state and sympathize with their life with certain therapeutic measures. Similarly, in contrast to those without palliative care training, neurologists with palliative care training estimated depressiveness and quality of life of patients similarly with patients’ subjective ratings. Some in ALS occurring extra-motor symptoms might be counteracted by positive social contacts and they are not necessarily associated with patients’ psychosocial adaptation and decision making regarding life prolonging measures. Patients’ well-being might often be underestimated by social environment with insufficient knowledge on the disease. On the other hand, patients’ good psychosocial adaptation is probably well understood among highly experienced neurologists. Therefore, patients with a rare disease such as ALS would be ideally treated by experienced neurologists, when they might receive both more advanced medical expertise and better understanding for their condition with regard to psychological well-being

Screening for Cognitive Function in Complete Immobility Using Brain–Machine Interfaces: A Proof of Principle Study

Background: In many neurological conditions, there is a combination of decline in physical function and cognitive abilities. For far advanced stages of physical disability where speaking and hand motor abilities are severely impaired, there is a lack of standardized approach to screen for cognitive profile.Methods:N = 40 healthy subjects were included in the study. For proof of principle, N = 6 ALS patients were additionally measured. For cognitive screening, we used the Edinburgh cognitive and behavioral ALS screen (ECAS) in the standard paper-and-pencil version. Additionally, we adapted the ECAS to a brain–machine interface (BMI) control module to screen for cognition in severely advanced patients.Results: There was a high congruency between BMI version and the paper-and-pencil version of the ECAS. Sensitivity and specificity of the ECAS-BMI were mostly high whereas stress and weariness for the patient were low.Discussion/Conclusion: We hereby present evidence that adaptation of a standardized neuropsychological test for BMI control is feasible. BMI driven neuropsychological test provides congruent results compared to standardized tests with a good specificity and sensitivity but low patient load

Disease progression but not physical state per se determines mental wellbeing in ALS

Background!#!Studies provide inconclusive results on the question whether loss of mental wellbeing is actually associated with decline in physical function in amyotrophic lateral sclerosis (ALS). The purpose of this study was to determine predictors of mental wellbeing in ALS.!##!Methods!#!In total, n = 330 ALS patients were interviewed on parameters of mental wellbeing to evaluate the patients' capacity of psychosocial adaptation. These parameters were global and subjective quality of life (QoL), and depressiveness. A subsample of n = 82 ALS patients were interviewed again within approximately a year (mean 14.34 ± 5.53 months).!##!Results!#!Both global and subjective QoL were stable, whereas depressiveness increased within the course of 1 year after diagnosis. Physical function decline was associated with mental wellbeing. Progression of physical disabilities and symptom duration were significant predictors of wellbeing in the sense that fast progression and short time since symptom onset (both indicating short time to adapt) were associated with low wellbeing.!##!Conclusions!#!There is evidence for subsamples in ALS with regard to mental wellbeing, which are mainly determined by clinical parameters. Those subjects being reported in the literature to present with high mental wellbeing are often long survivors. High progression rate and low physical function when attending the clinic for the first time should be red flags and need special attention in clinical counseling

Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective

Abstract Objective To investigate intercultural neurologists' perception of well‐being in patients with amyotrophic lateral sclerosis (ALS) using gastrostomy (PEG), non‐invasive, and/or invasive ventilation (NIV/IV) and to analyse the determinants and impact on the management of the above medical interventions (MIs). Methods The study was based on anonymous questionnaires addressing the clinical approach and personal attitude towards the use of PEG, NIV and IV in ALS patients completed by 465 neurologists: 228 from Germany and 237 from Poland. Results The German and Polish neurologists estimated the quality of life in ALS patients using PEG and NIV as neutral, whilst low in individuals using IV. A regression model revealed an independent influence of palliative care training (PCT) and age on that attitude in the German group. Higher values of estimated patients' depressiveness on PEG, NIV and IV were found amongst the Polish neurologists. Marital status, experience in ALS and being a parent independently influenced the perception of patients' depressiveness in the German, whilst marital status, age and PCT were factors in the Polish group. Amongst German neurologists, a higher perception of patients' depressiveness in individuals using PEG, NIV and IV was linked to the later timing of the MIs discussion. In the Polish group, it was a lower estimation of QoL in patients using PEG. Conclusion Neurologists' perception of ALS patients' well‐being on MIs reflects their demographic status, professional experience and potentially their cultural background. This perception plays an important role in the timing of MIs discussion, possibly influencing the decision‐making process

One third of physicians discuss exit strategies with patients with amyotrophic lateral sclerosis: Results from nationwide surveys among German and Polish neurologists

Abstract Objective This paper examines neurologists’ approaches to exit strategies (ESs), such as euthanasia and physician‐assisted suicide, in patients with amyotrophic lateral sclerosis (PALS) in two European countries. Methods In a nationwide anonymous survey, we collected responses from 237 Polish and 228 German neurologists, focusing on their practices and beliefs about ESs, as well as their viewpoints on life‐sustaining measures (LSMs) (percutaneous endoscopic gastrostomy, non‐invasive, and invasive ventilation). To analyze the data, we employed statistical methods, including Mann–Whitney U, Kruskal–Wallis, chi‐square tests, Spearman's rank correlation, and multiple regression analysis. Results One third of the neurologists initiated the discussion about ESs with PALS. Half were ready to have this conversation upon patient's request. Age, gender, religiousness, and nationality were closely associated with this approach. One in 9 neurologists received a request to terminate an LSM, whereas 1 in 10 to implement an ES. German neurologists and palliative care trainees acquired both demands more commonly. Neurologists quoted a low quality of life, decreased mood, and being a burden to the family/closest ones as primary reasons for a wish to hasten death among PALS. Although the majority expressed a willingness to terminate an LSM at a request of the patient, most opposed the legalization of euthanasia. Younger and less religious individuals were more likely to favor accepting euthanasia. Conclusion Neurologists vary significantly in their approaches to terminal care. Complex relationships exist among personal indices, shared beliefs, and current practices