Peric Marina, Peric Stojan, Stevanovic Jelena, Milovanovic Sara, Basta Ivana, Nikolic Ana, Kacar Aleksandra, Rakocevic-Stojanovic Vidosava
Mother and Child Health Care Institute "Dr. Vukan Cupic", Radoja Dakica 6, 11000, Belgrade, Serbia.
Neurology Clinic, Clinical Center of Serbia, School of Medicine, University of Belgrade, 6, Dr Subotica Street, 11000, Belgrade, Serbia.
Acta Neurol Belg. 2018 Jun;118(2):243-250. doi: 10.1007/s13760-017-0857-9. Epub 2017 Nov 7.
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.
尽管肢带型肌营养不良症（LGMD）可导致永久性残疾，但迄今为止尚无研究对这些患者的生活质量（QoL）进行过调查。我们的目的是评估LGMD患者的生活质量，并确定生活质量最重要的预测因素。该研究纳入了46例被诊断为肢带型肌无力的患者。使用两种量表对患者的生活质量进行评估，即SF-36问卷和个体化神经肌肉生活质量问卷（INQoL）。还应用了以下量表：爱泼沃斯嗜睡量表（ESS）、汉密尔顿抑郁量表（HamD）和克虏伯疲劳严重程度量表（FSS）。SF-36平均得分为52.4±23.5，身体综合得分低于心理得分。INQoL总得分是46.1±20.4，其中在虚弱、疲劳和独立性方面得分最差，而社会关系和情绪方面得分较好。LGMD患者中SF-36得分较差的显著预测因素是较高的疲劳水平（β=-0.470，p<0.01）和使用辅助设备（β=-0.245，p<0.05）。INQoL得分较差的显著预测因素是较高的疲劳水平（β=0.514，p<0.01）和存在心肌病（β=-0.385，p<0.01）。特别值得关注的是，在LGMD患者中，一些与较差生活质量相关的已确定因素是可以治疗的。
