Peric Stojan, Bjelica Bogdan, Bozovic Ivo, Pesovic Jovan, Paunic Teodora, Banovic Marija, Brkusanin Milos, Aleksic Ksenija, Basta Ivana, Pavicevic Dusanka Savic, Stojanovic Vidosava Rakocevic
Neurology Clinic, Clinical Centre of Serbia, School of Medicine, University of Belgrade, Belgrade, Serbia.
Faculty of Biology, Centre for Human Molecular Genetics, University of Belgrade, Belgrade, Serbia.
Acta Myol. 2019 Dec 1;38(4):239-244. eCollection 2019 Dec.
Cross-sectional studies reported fatigue in 50-90% of patients with myotonic dystrophy type 1 (DM1). The aim of this research was to assess frequency of fatigue in DM1 patients during a seven-year period.
Study included 64 DM1 patients at baseline (50% males, age 42 ± 12 years), and 38 after seven years. Following scales were used: Muscular Impairment Rating Scale (MIRS), Fatigue Severity Scale (FSS, score equal to or greater than 36 indicates significant fatigue), and Daytime Sleepiness Scale (DSS, score of more than six is considered significant).
At baseline, 54% of DM1 patients had fatigue and 46% had excessive daytime sleepiness (EDS). Ten (32%) patients with fatigue had no EDS. At the baseline, patients with fatigue were older, were more likely to had adult-onset DM1, worse MIRS and DSS compared to the patients without fatigue. After seven years, FSS score increased (34 ± 15 48 14, p < 0.01), fatigue was found in 82% of patients, and EDS in 60%. Still eight (26%) patients with fatigue had no EDS. Fatigue progression did not parallel MIRS increase.
Fatigue is a common symptom of DM1 and its progression during time did not correlate with the progression of muscle weakness.
横断面研究报告称,1型强直性肌营养不良(DM1)患者中有50%-90%存在疲劳症状。本研究的目的是评估DM1患者在七年期间疲劳的发生率。
研究纳入了64例基线期的DM1患者(50%为男性,年龄42±12岁),以及七年后的38例患者。使用了以下量表:肌肉损伤评定量表(MIRS)、疲劳严重程度量表(FSS,得分等于或大于36表明存在显著疲劳)和日间嗜睡量表(DSS,得分超过6被认为是显著的)。
基线期,54%的DM1患者有疲劳症状,46%有日间过度嗜睡(EDS)。10例(32%)有疲劳症状的患者没有EDS。基线期,有疲劳症状的患者年龄更大,更有可能患成人型DM1,与无疲劳症状的患者相比,MIRS和DSS更差。七年后,FSS评分增加(34±15 48±14,p<0.01),82%的患者有疲劳症状,60%有EDS。仍有8例(26%)有疲劳症状的患者没有EDS。疲劳进展与MIRS增加不平行。
疲劳是DM1的常见症状,其随时间的进展与肌肉无力的进展无关。