Abstract
ObjectiveTo investigate the association between neuropsychological deficits and psychological well-being in amyotrophic lateral sclerosis (ALS).MethodsSubjective (Schedule for the Evaluation of the Individual Quality of Life-Direct Weighting, SEIQoL-DW) and global quality of life (QoL; Anamnestic Comparative Self-Assessment, ACSA) as well as depression (ALS-Depression-Inventory, ADI-12) as indicators for psychological well-being were measured in 214 patients with ALS and correlated with neurocognitive performance assessed by the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). Primary caregivers evaluated behaviour. Patients were classified to be cognitively (ALSci) or behaviourally impaired (ALSbi) according to Strong criteria.ResultsALSbi patients had poorer psychological well-being than patients without behavioural alterations, while the psychological well-being of patients with and without neurocognitive deficits was comparable.ConclusionThe study provides evidence that minor neuropsychological deficits do not interfere with psychological well-being of ALS in contrast to alterations on behavioural level. Thus, abnormalities in individual cognitive domains have limited relevance for the patients’ everyday life in comparison to the impact of behavioural alterations.
Highlights
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease leading to death because of respiratory insufficiency within 3–5 years
Male-to-female ratio was 1.55:1. 28.1 months have passed since onset of symptoms and mean ALS-FRS-R was 38.7. 51.9% of the study population were medicated with riluzole. 14% showed pathological laugher and crying
The most prominent neurocognitive deficits were in the domains of language function and verbal fluency (22.0%)
Summary
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease leading to death because of respiratory insufficiency within 3–5 years. The psychosocial adaptation model provides an explanatory approach for the fact that many ALS patients succeed in adapting to their new circumstances despite a fatal diagnosis [1]. The quality of life of ALS patients can be unexpectedly high; over time, it may be within the range of chronic ill patients without severe neurological impairment [2]. Neuropsychological deficits in ALS patients have been reported in up to 50% [4]. They affect either cognitive domains such as executive function or behavioural domains such as apathy and disinhibition. These deficits do not interfere with medical decision-making in ALS patients [5]
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