Neuropsychological deficits have only limited impact on psychological well-being in amyotrophic lateral sclerosis.

To investigate the association between neuropsychological deficits and psychological well-being in amyotrophic lateral sclerosis (ALS). Subjective (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. ALSbi 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. The 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.