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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.

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Correspondence to Vidosava Rakocevic-Stojanovic.

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Authors declare no conflict of interest.

Funding

The study was funded by the Ministry of Education, Science and Technological Development of the Republic of Serbia (Grant # 175083).

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the Ethical Board of the Neurology Clinic, Clinical Center of Serbia, and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Peric, M., Peric, S., Stevanovic, J. et al. Quality of life in adult patients with limb–girdle muscular dystrophies. Acta Neurol Belg 118, 243–250 (2018). https://doi.org/10.1007/s13760-017-0857-9

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  • DOI: https://doi.org/10.1007/s13760-017-0857-9

Keywords

  • Limb–girdle muscular dystrophies
  • Quality of life
  • Fatigue
  • Cardiomyopathy
  • Assistive device
  • Weakness