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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. |
