LAUSR

BACKGROUND Measuring health-related quality of life (HR-QOL) is vital for understanding the disease impact, but the complex relationship between clinical parameters and QOL remains unclear. The objective was to determine the demographic and clinical factors that influence the QOL in adults with inherited and acquired myopathies. METHODS The study was of cross-sectional design. Detailed demographic and clinical details were collected. Patients answered Neuro-QOLTM and Patient Reported Outcomes Measurement Information System (PROMIS®) short-form questionnaires. RESULTS Data was collected from 100 consecutive in-person patient visits. Mean age of the cohort was 49.5±20.1 (18-85) years and the majority were males; 53 (53%). Bivariate analysis between the various demographic and clinical features with the QOL scales revealed SSQ, hand grip strength, MRC sum score, female gender, and age to be non-uniformly associated with the QOL scales. There was no difference between inherited and acquired myopathies for any of the QOL scores except poorer lower limb function domain in inherited myopathies (36.7±7.3 vs 40.9±11.2, p 0.049). Linear regression models revealed lower SSQ, lower hand grip strength and lower MRC sum score to independently predict poor QOL. CONCLUSION Hand grip strength and SSQ serve as novel predictors of QOL in myopathies. Handgrip strength has a significant impact on physical, mental and social domains and deserves special attention with respect to rehabilitation. SSQ correlates well with QOL and can be employed as a quick and global assessment of a patient's well-being. Differences in QOL scores between patients with inherited and acquired myopathies were minimal.