Aim and Objectives: The aims were to study health-related quality of life, anxiety and depression in patients with amyotrophic lateral sclerosis and their next of kin and to compare these results with a subset of the general Swedish population. Thirty-five pairs of patients with amyotrophic lateral sclerosis and their next of kin were studied., Background: Life changes in many ways when a person is diagnosed with amyotrophic lateral sclerosis. Comparison between patients, next of kin and the general population are needed to get a wider understanding of their quality of life, anxiety and depression., Design: A descriptive study., Methods: All participants were studied with the SF-36 and the Hospital Anxiety and Depression Scale. Patients' physical function was assessed by the amyotrophic lateral sclerosis Functional Rating Scale-Revised and the Norris scale., Results: A correlation was found in both the mental component summary in SF-36 and in anxiety between the pairs of patients and their next of kin, while no correlation was found in the physical component summary or depression. These results were not related to the patients' physical function. Both patients and their next of kin had some poorer ratings in SF-36 and in anxiety and depression than the general Swedish population. Gender or age did not affect the estimates in any of the scales., Conclusions: The results indicate that both the patient and the next of kin are affected by the disease independent of physical disability. In most pairs of patient and next of kin, the mental component summary and anxiety were affected to a similar extent., Relevance to Clinical Practice: The results emphasise the need for medical and nursing support to both the patient and the next of kin soon after the diagnosis and during the course of the disease., (© 2010 Blackwell Publishing Ltd.)