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本文引用的文献

1
Perceived fatigue in myotonic dystrophy type 1: a case-control study.1型强直性肌营养不良患者的感知疲劳:一项病例对照研究。
BMC Neurol. 2019 Mar 28;19(1):45. doi: 10.1186/s12883-019-1280-z.
2
Tracking the brain in myotonic dystrophies: A 5-year longitudinal follow-up study.肌强直性营养不良症患者的大脑追踪:一项 5 年的纵向随访研究。
PLoS One. 2019 Mar 7;14(3):e0213381. doi: 10.1371/journal.pone.0213381. eCollection 2019.
3
Disease burden of myotonic dystrophy type 1.1 型肌强直性营养不良的疾病负担。
J Neurol. 2019 Apr;266(4):998-1006. doi: 10.1007/s00415-019-09228-w. Epub 2019 Feb 20.
4
Myotonic Dystrophy-A Progeroid Disease?强直性肌营养不良——一种早老性疾病?
Front Neurol. 2018 Jul 25;9:601. doi: 10.3389/fneur.2018.00601. eCollection 2018.
5
Cognitive behavioural therapy with optional graded exercise therapy in patients with severe fatigue with myotonic dystrophy type 1: a multicentre, single-blind, randomised trial.1 型先天性肌营养不良症患者严重疲劳采用认知行为疗法联合选择性分级运动疗法治疗:一项多中心、单盲、随机试验。
Lancet Neurol. 2018 Aug;17(8):671-680. doi: 10.1016/S1474-4422(18)30203-5. Epub 2018 Jun 19.
6
Core Clinical Phenotypes in Myotonic Dystrophies.强直性肌营养不良的核心临床表型
Front Neurol. 2018 May 2;9:303. doi: 10.3389/fneur.2018.00303. eCollection 2018.
7
The proposal of a clinical protocol to assess central and peripheral fatigue in myotonic dystrophy type 1.一项评估1型强直性肌营养不良症中枢和外周疲劳的临床方案提案。
Arch Ital Biol. 2017 Jul 1;155(1-2):43-53. doi: 10.12871/000398292017125.
8
Further evidence for the reliability and validity of the Fatigue and Daytime Sleepiness Scale.疲劳与日间嗜睡量表可靠性和有效性的进一步证据。
J Neurol Sci. 2017 Apr 15;375:23-26. doi: 10.1016/j.jns.2017.01.032. Epub 2017 Jan 10.
9
Sleepiness and Sleep-related Breathing Disorders in Myotonic Dystrophy and Responses to Treatment: A Prospective Cohort Study.肌强直性营养不良症的嗜睡和与睡眠相关的呼吸障碍,以及对治疗的反应:一项前瞻性队列研究。
J Neuromuscul Dis. 2016 Nov 29;3(4):529-537. doi: 10.3233/JND-160191.
10
Disease awareness in myotonic dystrophy type 1: an observational cross-sectional study.1型强直性肌营养不良症的疾病认知:一项观察性横断面研究。
Orphanet J Rare Dis. 2016 Apr 4;11:34. doi: 10.1186/s13023-016-0417-z.

1型强直性肌营养不良症中的疲劳:一项为期七年的前瞻性研究。

Fatigue in myotonic dystrophy type 1: a seven-year prospective study.

作者信息

Peric Stojan, Bjelica Bogdan, Bozovic Ivo, Pesovic Jovan, Paunic Teodora, Banovic Marija, Brkusanin Milos, Aleksic Ksenija, Basta Ivana, Pavicevic Dusanka Savic, Stojanovic Vidosava Rakocevic

机构信息

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.

出版信息

Acta Myol. 2019 Dec 1;38(4):239-244. eCollection 2019 Dec.

PMID:31970322
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6955629/
Abstract

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 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.

摘要

目的

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。

材料与方法

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)。

结果

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。

结论

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。