OBJECTIVE: To assess patient quality-of-life and utilities for health states associated with different levels of amyotrophic lateral sclerosis (ALS) severity. METHODS: A structured interview was conducted with 77 patients, in 2 UK centres, covering four different clinical stages of ALS severity. Patients completed a disease specific health status measure (ALSAQ-40), a generic health status measure (EuroQol EQ-5D), visual analog scale (VAS) rating of current health and a standard gamble (SG) exercise to provide health state utilities for their own health state. RESULTS: The ALSAQ-40 was sensitive to disease severity, with scores for later stage disease states showing a lower level of patient rated health status than the scores for early stage disease states. Patients’ mean VAS rating of their own health ranged from 0.74 (95% CI: 0.64-0.85) for stage 1 (early) disease severity (n=15), to 0.37 (95% CI: 0.29-0.46) for stage 4 (late stage) disease severity (n=19). The mean VAS rating for the entire group was 0.55 (95% CI: 0.50-0.60), (n=76). Utilities elicited via SG were systematically higher than VAS scores and ranged from a mean of 0.79 (95% CI: 0.71-0.88) for stage 1 disease severity (n=15) to a mean of 0.45 (95% CI: 0.28-0.61) for stage 4 disease severity (n=19). The mean SG score for the entire group was 0.65 (95% CI: 0.58-0.71), (n=76). The EQ-5D was not sensitive to the different levels of disease severity. CONCLUSIONS: This study demonstrates that it is feasible and practical to obtain ALS patients’ valuations of their current health state. VAS and SG values associated with each of the 4 stages of disease severity fell as disease severity progressed through stages 1 to 4. ALS patients’ rating of their health state and the SG scores provide evidence that patients value their health-related quality-of-life highly, even in cases where health status is very poor.