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ObjectiveQuantitative analysis of decision-making on therapeutic options in different sociocultural context in amyotrophic lateral sclerosis (ALS).MethodsALS patients (n = 244) were consecutively recruited in Germany (n = 83), Poland (n = 83), and Sweden (n = 78) in a prospective cross-cultural study (www.NEEDSinALS.com). They were interviewed on preferences for therapeutic techniques including invasive (IV) and non-invasive ventilation (NIV), as well as percutaneous endoscopic gastrostomy (PEG) and on hypothetical termination of these using quantitative questions. Using standardized questionnaires, religiousness, personal values, quality of life, and depressiveness were assessed.ResultsNIV was most frequently used in Germany and PEG in Sweden. Swedish patients were most liberal on initiation and termination of PEG, NIV and IV. Polish patients were mostly undecided and were least likely to consider discontinuing supportive management. Current use was partly associated with age, gender and state of physical function; also, financial support explained some variance. Future preferences on therapeutic options from the patient’s perspective were also closely associated with cultural factors. The more oriented towards traditional and conservative values, the less likely patients were to decide for invasive therapeutic devices (IV, PEG), the least likely to have ideations to discontinue any device and the more likely to have an undecided attitude.ConclusionsCurrent use of therapeutic options is determined by medical condition in analogy to clinical guidelines. For future considerations, other factors such as cultural background are crucial, yielding hurdles to be regarded in the implementation of advanced directives in a multicultural environment.

Amyotrophic lateral sclerosis (ALS) primarily impairs motor abilities but also affects cognition and emotional processing. We hypothesise that subjective ratings of emotional stimuli depicting social interactions and facial expressions is changed in ALS. It was found that recognition of negative emotions and ability to mentalize other's intentions is reduced. Processing of emotions in faces was investigated. A behavioural test of Ekman faces expressing six basic emotions was presented to 30 ALS patients and 29 age-, gender and education matched healthy controls. Additionally, a subgroup of 15 ALS patients that were able to lie supine in the scanner and 14 matched healthy controls viewed the Ekman faces during functional magnetic resonance imaging (fMRI). Affective state and a number of daily social contacts were measured. ALS patients recognized disgust and fear less accurately than healthy controls. In fMRI, reduced brain activity was seen in areas involved in processing of negative emotions replicating our previous results. During processing of sad faces, increased brain activity was seen in areas associated with social emotions in right inferior frontal gyrus and reduced activity in hippocampus bilaterally. No differences in brain activity were seen for any of the other emotional expressions. Inferior frontal gyrus activity for sad faces was associated with increased amount of social contacts of ALS patients. ALS patients showed decreased brain and behavioural responses in processing of disgust and fear and an altered brain response pattern for sadness. The negative consequences of neurodegenerative processes in the course of ALS might be counteracted by positive emotional activity and positive social interactions.

The syndrome of pathological laughing and crying (PLC) is characterized by episodes of involuntary outbursts of emotional expression. Although this phenomenon has been referred to for over a century, a clear-cut clinical definition is still lacking, and underlying pathophysiological mechanisms are not well understood. In particular, it remains ill-defined which kind of stimuli—contextually appropriate or inappropriate—elicit episodes of PLC, and if the phenomenon is a result of a lack of inhibition from the frontal cortex (“top-down-theory”) or due to an altered processing of sensory inputs at the brainstem level (“bottom-up-theory”). To address these questions, we studied ten amyotrophic lateral sclerosis (ALS) patients with PLC and ten controls matched for age, sex and education. Subjects were simultaneously exposed to either emotionally congruent or incongruent visual and auditory stimuli and were asked to rate pictures according to their emotional quality. Changes in physiological parameters (heart rate, galvanic skin response, activity of facial muscles) were recorded, and a standardized self-assessment lability score (CNS-LS) was determined. Patients were influenced in their rating behaviour in a negative direction by mood-incongruent music. Compared to controls, they were influenced by negative stimuli, i.e. they rated neutral pictures more negatively when listening to sad music. Patients rated significantly higher on the CNS-LS. In patients, changes of electromyographic activity of mimic muscles during different emotion-eliciting conditions were explained by frontal cortex dysfunction. We conclude that PLC is associated with altered emotional suggestibility and that it is preferentially elicited by mood-incongruent stimuli. In addition, physiological reactions as well as behavioural changes suggest that this phenomenon is primarily an expression of reduced inhibitory activity of the frontal cortex, since frontal dysfunction could explain changes in physiological parameters in the patient group. We consider these findings being important for the clinical interpretation of emotional reactions of ALS patients.

Despite the fatal outcome and progressive loss of physical functioning in amyotrophic lateral sclerosis (ALS), many patients maintain contentment in life. It has been shown that non-professionals tend to underestimate the well-being of patients with ALS, but professionals’ perspective is yet to be studied. In total, 105 neurologists with varying degrees of experience with ALS were included in an anonymous survey. They were asked to estimate the quality of life and depressiveness of ALS patients with artificial ventilation and nutrition. Physicians’ estimations were compared with previously reported subjective ratings of ALS patients with life-prolonging measures. Neurologists with significant experience on ALS and palliative care were able to accurately estimate depressiveness and quality of life of ALS patients with life-prolonging measures. Less experienced neurologists’ estimation differed more from patients’ reports. Of all life-prolonging measures neurologists regarded invasive ventilation as the measure associated with lowest quality of life and highest depressiveness of the patients. Experienced neurologists as well as neurologists with experience in palliative care are able to better empathize with patients with a fatal illness such as ALS and support important decision processes.

Amyotrophic lateral sclerosis (ALS) primarily affects motor and speech abilities. In addition, cognitive functions are impaired in a subset of patients. There is a need to establish an eye movement-based method of neuropsychological assessment suitable for severely physically impaired patients with ALS. Forty-eight ALS patients and thirty-two healthy controls matched for age, sex and education performed a hand and speech motor-free version of the Raven’s coloured progressive matrices (CPM) and the D2-test which had been especially adapted for eye-tracking control. Data were compared to a classical motor-dependent paper–pencil version. The association of parameters of the eye-tracking and the paper–pencil version of the tests and the differences between and within groups were studied. Subjects presented similar results in the eye-tracking and the corresponding paper–pencil versions of the CPM and D2-test: a correlation between performance accuracy for the CPM was observed for ALS patients ( p  < 0.001) and controls ( p  < 0.001) and in the D2-test for controls ( p  = 0.048), whereas this correlation did not reach statistical significance for ALS patients ( p  = 0.096). ALS patients performed worse in the CPM than controls in the eye-tracking ( p  = 0.053) and the paper–pencil version ( p  = 0.042). Most importantly, eye-tracking versions of the CPM ( p  < 0.001) and the D2-test ( p  = 0.024) reliably distinguished between more and less cognitively impaired patients. Eye-tracking-based neuropsychological testing is a promising approach for assessing cognitive deficits in patients who are unable to speak or write such as patients with severe ALS.

Cognitive deficits, especially in the domains of social cognition and executive function including verbal fluency, are common in amyotrophic lateral sclerosis (ALS) patients. There is yet sparse understanding of pathogenesis of the underlying, possibly adaptive, cortical patterns. To address this issue, 65 patients with ALS and 33 age-, gender- and education-matched healthy controls were tested on cognitive and behavioral deficits with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Using functional magnetic resonance imaging (fMRI), cortical activity during social cognition and executive function tasks (theory of mind, verbal fluency, alternation) adapted from the ECAS was determined in a 3 Tesla scanner. Compared to healthy controls, ALS patients performed worse in the ECAS overall (p < 0.001) and in all of its subdomains (p < 0.02), except memory. Imaging revealed altered cortical activation during all tasks, with patients consistently showing a hyperactivation in relevant brain areas compared to healthy controls. Additionally, cognitively high performing ALS patients consistently exhibited more activation in frontal brain areas than low performing patients and behaviorally unimpaired patients presented with more neuronal activity in orbitofrontal areas than behaviorally impaired patients. In conclusion, hyperactivation in fMRI cognitive tasks seems to represent an early adaptive process to overcome neuronal cell loss in relevant brain areas. The hereby presented cortical pattern change might suggest that, once this loss passes a critical threshold and no cortical buffering is possible, clinical representation of cognitive and behavioral impairment evolves. Future studies might shed light on the pattern of cortical pattern change in the course of ALS.

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.

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. = 40 healthy subjects were included in the study. For proof of principle, = 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. 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. 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.