Quality of life in adult patients with limb-girdle muscular dystrophies.

Abstract

Although limb-girdle muscular dystrophies (LGMD) can cause permanent disability, to date there are no studies that examined quality of life (QoL) in these patients. Our aim was to evaluate QoL in patients with LGMD, and to identify the most significant predictors of QoL. The study comprised 46 patients with diagnosis of limb-girdle muscular weakness. QoL in patients was evaluated using two scales-SF-36 questionnaire and the Individualized Neuromuscular Quality of Life questionnaire (INQoL). Following scales were also applied: Epworth Sleepiness Scale (ESS), Hamilton Scale for Depression (HamD), and Krupp's Fatigue Severity Scale (FSS). Mean SF-36 score was 52.4±23.5, and physical composite score was worse than mental. Total INQoL score was 46.1±20.4, with worst results obtained for weakness, fatigue and independence, while social relationships and emotions showed better results. Significant predictors of worse SF-36 score in LGMD patients were higher fatigue level (β=-0.470, p<0.01) and use of assistive device (β=-0.245, p<0.05). Significant predictors of worse INQoL score were higher fatigue level (β=0.514, p<0.01) and presence of cardiomyopathy (β=-0.385, p<0.01). It is of special interest that some of the identified factors that correlated with worse QoL in LGMD patients were amenable to treatment.