Stojan Peric, Bogdan Bjelica, Ivo Bozovic, Jovan Pesovic, Teodora Paunic, Marija Banovic, Milos Brkusanin, Ksenija Aleksic, Ivana Basta, Dusanka Savic Pavicevic, Vidosava Rakocevic Stojanovic
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引用次数: 0
摘要
目的:横断面研究报告了50-90%的1型肌强直性营养不良(DM1)患者的疲劳。本研究的目的是评估DM1患者在7年期间的疲劳频率。材料和方法:研究纳入64例DM1患者(男性50%，年龄42±12岁)，7年后38例。使用以下量表:肌肉损伤评定量表(MIRS)、疲劳严重程度量表(FSS，分数等于或大于36表示明显疲劳)和日间嗜睡量表(DSS，分数大于6视为显著)。结果:基线时，54%的DM1患者有疲劳，46%有白天嗜睡(EDS)。10例(32%)疲劳患者无EDS。在基线时，与没有疲劳的患者相比，疲劳患者年龄较大，更有可能患有成人发病的DM1, MIRS和DSS更差。7年后FSS评分升高(34±15 vs 48 14, p < 0.01)，疲劳发生率82%，EDS发生率60%。还有8例(26%)疲劳患者没有EDS。疲劳进展不平行于MIRS增加。结论:疲劳是DM1的常见症状，其病程进展与肌无力进展无相关性。
Fatigue in myotonic dystrophy type 1: a seven-year prospective study.
Objectives: 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.
Materials and methods: 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).
Results: 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 vs 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.
Conclusions: Fatigue is a common symptom of DM1 and its progression during time did not correlate with the progression of muscle weakness.
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