Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease characterised by progressive motor disability. Cognitive and behavioural impairment is increasingly recognised, affecting up to 50% of patients, with 15% developing Frontotemporal Dementia (FTD). While cognitive screening tools like the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) exist, they are not routinely implemented in ALS care. Patient and caregiver perspectives on cognitive testing remain underexplored, limiting understanding of how best to facilitate acceptance and integration into clinical pathways. This study explores attitudes toward cognitive screening in ALS, identifying barriers, facilitators, and perceived impacts to inform patient-centred approaches. Semi-structured interviews were conducted with ALS patients ( n  = 10) and caregivers ( n  = 9) recruited from Sheffield Teaching Hospitals and the UK Motor Neuron Disease Association network. Participants represented a range of disease stages and cognitive-behavioural symptom severity. Interviews were conducted separately by three researchers. Reflexive Thematic Analysis was used, with iterative coding refinement to develop and finalise an interpretative framework capturing diverse perspectives. Perceptions of cognitive testing in ALS were highly individual and context-dependent. While some participants saw early screening as a means of preparedness, others feared it would threaten autonomy. Emotional responses varied, with individuals balancing denial, fear, and acceptance. Caregivers often advocated for screening to aid future planning but faced tensions in respecting patient autonomy. Misattributions of cognitive and behavioural symptoms created additional strain. Practical barriers, including travel, fatigue, and accessibility, further influenced decision-making. Clinician communication played a crucial role. Clear, empathetic discussions facilitated engagement, whereas overwhelming or unclear information led to hesitancy. Testing was valued when it provided actionable insights for care planning, but concerns arose when results lacked practical application. Cognitive screening in ALS requires a patient-centred approach that considers emotional readiness, individual preferences, and logistical challenges. Adaptive clinical strategies were recommended, which personalise communication, offer flexible testing delivery, and ensure results lead to meaningful, actionable outcomes. Findings support the development of remote cognitive screening tools that maintain clinician involvement while reducing testing burden. This could improve acceptance and accessibility of screening, facilitating timely support for those affected.

BACKGROUND AND OBJECTIVES:The assessment of health-related quality of life (HRQoL) in patients with amyotrophic lateral sclerosis (ALS) is heterogeneous and inconsistent. The objectives of this study were (1) to develop a comprehensive conceptual framework of HRQoL in ALS and (2) map the content of existing patient-reported outcome measures (PROMs) used in ALS to this novel framework. METHODS:Our model of HRQoL in ALS (Health-related Quality of life in Amyotrophic Lateral Sclerosis, QuALS) was developed from a systematic literature review and consultative input from key stakeholders (patients, carers, and health care professionals). Five electronic databases were searched in April 2022. Primary studies of any design that assessed HRQoL in ALS by using a multi-item PROM and/or qualitative methods were identified. Using an a priori framework, HRQoL themes were extracted and iteratively modified from the content of each PROM and qualitative study quotations identified in the literature. The conceptual framework was ratified by stakeholders with lived experience and clinical experts. The QuALS framework was used to map the content of identified PROMs and qualitative studies based on thematic coverage. RESULTS:QuALS covers 3 high-level domains of HRQoL (physical, psychological, and social functioning) and consists of 7 themes (Activities; Physical Health; Autonomy; Cognition; Feelings and Emotions; Self-identity; Relationships), characterized by 42 subthemes. Of 8,220 studies identified, 274 were included in the review that informed QuALS. In these studies, 111 PROMs were used to assess at least 1 aspect of HRQoL, and 11 studies used qualitative methods. Of the 3 high-level domains, physical functioning was the most commonly assessed, particularly within ALS-specific PROMs where almost one-quarter of PROMs exclusively assessed physical functioning. None of the PROMs or qualitative studies identified assessed all aspects of HRQoL in the QuALS framework. DISCUSSION:This study presents a new comprehensive conceptual framework of HRQoL in ALS (QuALS), informed by a robust systematic review of existing literature and stakeholder input, incorporating lived experience. QuALS provides a valuable resource for researchers and clinicians interested in taking a holistic approach to assessing and understanding the full impact of ALS on HRQoL and how this may be affected by treatments.

Background and ObjectivesThe assessment of health-related quality of life (HRQoL) in patients with amyotrophic lateral sclerosis (ALS) is heterogeneous and inconsistent. The objectives of this study were (1) to develop a comprehensive conceptual framework of HRQoL in ALS and (2) map the content of existing patient-reported outcome measures (PROMs) used in ALS to this novel framework.MethodsOur model of HRQoL in ALS (Health-related Quality of life in Amyotrophic Lateral Sclerosis, QuALS) was developed from a systematic literature review and consultative input from key stakeholders (patients, carers, and health care professionals). Five electronic databases were searched in April 2022. Primary studies of any design that assessed HRQoL in ALS by using a multi-item PROM and/or qualitative methods were identified. Using an a priori framework, HRQoL themes were extracted and iteratively modified from the content of each PROM and qualitative study quotations identified in the literature. The conceptual framework was ratified by stakeholders with lived experience and clinical experts. The QuALS framework was used to map the content of identified PROMs and qualitative studies based on thematic coverage.ResultsQuALS covers 3 high-level domains of HRQoL (physical, psychological, and social functioning) and consists of 7 themes (Activities; Physical Health; Autonomy; Cognition; Feelings and Emotions; Self-identity; Relationships), characterized by 42 subthemes. Of 8,220 studies identified, 274 were included in the review that informed QuALS. In these studies, 111 PROMs were used to assess at least 1 aspect of HRQoL, and 11 studies used qualitative methods. Of the 3 high-level domains, physical functioning was the most commonly assessed, particularly within ALS-specific PROMs where almost one-quarter of PROMs exclusively assessed physical functioning. None of the PROMs or qualitative studies identified assessed all aspects of HRQoL in the QuALS framework.DiscussionThis study presents a new comprehensive conceptual framework of HRQoL in ALS (QuALS), informed by a robust systematic review of existing literature and stakeholder input, incorporating lived experience. QuALS provides a valuable resource for researchers and clinicians interested in taking a holistic approach to assessing and understanding the full impact of ALS on HRQoL and how this may be affected by treatments.