Sequential spread of TDP-43 load in the brain may be a pathological characteristic of amyotrophic lateral sclerosis (ALS). Diffusion tensor imaging (DTI) is a magnetic resonance imaging (MRI) based marker of this pathological feature. Cognitive deficits known to be present in a subset of ALS patients might act as an additional in vivo clinical marker of disease spread. N = 139 patients with ALS were tested with the Edinburgh Cognitive and Behavioural ALS screen (ECAS) in addition to DTI brain measures of pathological spread. Executive function, memory and disinhibited behaviour were selected for Cognitive-Staging criteria, as these cognitive functions are attributed to cerebral areas analogous to the pattern of MRI markers of TDP-43 pathology. ROC curve analyses were performed to define cut-off scores for cognitive stages 2 (executive function), stage 3 (disinhibited behaviour) and stage 4 (memory), and staging was performed according to the cognitive profile subsequently. Associations of Cognitive-Staging (stage 2-4) and MRI-Staging measures were determined. In total, 77 patients (55%) performed below ROC cut-off scores in either executive function or memory or both and/or were reported to have disinhibited behaviour which permitted Cognitive-Staging. The cognitive profile of patients with discrete MRI stages 2-4 correlated significantly with DTI parameters. For those patients with cognitive impairment, there was a high congruency between MRI and Cognitive-Staging with high specificity and sensitivity of executive functions for MRI stage 2, disinhibited behaviour for MRI stage 3 and moderate of memory for MRI stage 4. Cognitive impairment follows specific patterns in ALS and these patterns can be used for Cognitive-Staging with a high specificity compared to MRI-Staging. For the individual, cognitive screening is a fast and easy to apply measurement of cerebral function giving valuable information in a clinical context.
ObjectiveThis is an observational study on well-being and end-of-life preferences in patients with amyotrophic lateral sclerosis (ALS) in the locked-in state (LIS) in a Polish sample within the EU Joint Programme–Neurodegenerative Disease Research study NEEDSinALS (NEEDSinALS.com).MethodsIn this cross-sectional study, patients with ALS in LIS (n = 19) were interviewed on well-being (quality of life, depression) as a measure of psychosocial adaptation, coping mechanisms, and preferences towards life-sustaining treatments (ventilation, percutaneous endoscopic gastroscopy) and hastened death. Also, clinical data were recorded (ALS Functional Rating Scale–revised version). Standardized questionnaires (Anamnestic Comparative Self-Assessment [ACSA], Schedule for the Evaluation of Individual Quality of Life-Direct Weighting (SEIQoL-DW), ALS Depression Inventory–12 items [ADI-12], schedule of attitudes toward hastened death [SAHD], Motor Neuron Disease Coping Scale) were used, which were digitally transcribed; answers were provided via eye-tracking control. In addition, caregivers were asked to judge patients' well-being.ResultsThe majority of patients had an ACSA score >0 and a SEIQoL score >50% (indicating positive quality of life) and ADI-12 <29 (indicating no clinically relevant depression). Physical function did not reflect subjective well-being; even more, those with no residual physical function had a positive well-being. All patients would again choose the life-sustaining techniques they currently used and their wish for hastened death was low (SAHD <10). Caregivers significantly underestimated patient's well-being.InterpretationSome patients with ALS in LIS maintain a high sense of well-being despite severe physical restrictions. They are content with their life-sustaining treatments and have a strong will to live, which both may be underestimated by their families and public opinion.
The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) has been developed to assess cognition and behaviour in patients with amyotrophic lateral sclerosis (ALS). Cognitive impairments of ALS-specific and ALS-non-specific functions can be determined using cut-off scores based on performance of healthy subjects. However, detailed analyses show that older healthy subjects perform worse than younger ones, whereas highly-educated individuals perform better than those with lower education levels. As a consequence, this study presents new age and education matched cut-off scores for the revised German/Swiss-German version of the ECAS based on the performance of 86 healthy subjects.
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