目的 探讨杜氏肌营养不良(DMD)儿童的运动功能与全身肌肉力量及年龄的相关性,为更好地进行该病管理提供理论依据。方法 对2017年1月-2021年3月在深圳市儿童医院就诊且符合纳入标准的DMD患儿进行运动功能测试(MFM-32)、计时测试(仰卧站起测试、上4级阶梯测试、10 m走/跑测试)、Vignos评分、Brooke评分及全身的徒手肌力测试。采用Spearman相关性检验分析MFM各项分值与其他指标之间相关性,同时分析全身运动功能及肌力与年龄之间的关系。结果 共140名4~12岁DMD儿童患者被纳入研究,平均年龄(8.0±1.87)岁,MFM-32总分及D1区分值与下肢肌肉力量、计时测试、Vignos评分呈强-极强相关(r=-0.887~0.677,P<0.05);D2区分值与计时测试中等相关(r=-0.478~-0.423,P<0.05)),D3区的分值与其他指标不相关(P>0.05)。年龄与MFM-32总分及D1区分值、全身肌肉力量呈中等相关(r=-0.585~-0.468,P<0.05)),年龄与MFM-32 D2区分值不相关(P>0.05),与D3区呈弱相关(r=0.317,P<0.05)。结论 MFM-32的各项分值与全身肌肉力量、计时测试、Vignos评分及Brooke评分具有不同程度的相关性,可反映DMD患者的运动功能。儿童时期的DMD患者的下肢运动功能下降较明显,应首要加强其下肢运动功能及肌肉力量。
Abstract
Objective To investigate the correlation of motor function with muscle strength and age in children with Duchenne muscular dystrophy (DMD), so as to provide theoretical reference for better management of DMD. Methods DMD Children in Shenzhen Children's Hospital from January 2017 to March 2021 were enrolled in this study, and were assessed by Motor Function Measure (MFM-32), timing tests (supine standing test, upper 4-step step test, 10 meter walk/run test), Vignos score, Brooke score and Manual Muscle Testing.Spearman correlation test was used to analyze the relationship between scores of MFM-32 and other indexes.Besides, the association of age with motor function and muscle strength was analyzed. Results A total of 140 DMD children aged 4 to 12 years were enrolled in this study, with an average age of (8.0±1.87) years old.The total score and D1 subscale of the MFM-32 were strongly correlated with muscle strength, timing test and Vignos score (r=-0.887 - 0.677,P<0.05).The score of D2 subscale was moderately correlated with timing test (r=-0.478 - -0.423,P<0.05), while the score of D3 subscale was not correlated with these measures (P＞0.05).Children's age was moderately associated with MFM-32 score, score of D1 subscale and the whole body muscle strength (r=-0.585 - -0.468,P<0.05).However, it appeared to be not correlated with the score of D2 subscale (P＞0.05), and was slightly correlated with the score of D3 subscale (r =0.317, P<0.05). Conclusions The scores of MFM-32 correlate with whole body muscle strength, timing test, Vignos score and Brooke score to different degrees, which can reflect the motor function of DMD children.The lower limb motor function of DMD children during childhood decreases significantly, and should be given priority to strengthen their lower extremity motor function and muscle strength.
关键词
杜氏肌营养不良症 /
运动功能 /
运动功能测试 /
肌肉力量
Key words
Duchenne muscular dystrophy /
motor function /
Motor Function Measure /
muscle strength
{{custom_sec.title}}
{{custom_sec.title}}
{{custom_sec.content}}
参考文献
[1] Crisafulli S, Sultana J, Fontana A, et al.Global epidemiology of Duchenne muscular dystrophy: An updated systematic review and meta-analysis[J].Orphanet J Rare Dis, 2020,15(1):141.
[2] 杨茹莱.杜氏肌营养不良临床特点及诊治进展[J].中国儿童保健杂志, 2018,26(3):233-235.
[3] 杨书婷.进行性肌营养不良的最新诊疗与评估进展[J].国际儿科学杂志, 2020,47(2):87-90.
[4] Bérard C, Payan C, Fermanian J, et al.A motor function measurement scale for Neuromuscular diseases- description and validation study[J].Rev Neurol, 2006,162(4):485-93.
[5] Huang M, Cao J, Sun J, et al.Cross-cultural adaptation and multi-centric validation of the Motor Function Measure Chinese version (MFM-32-CN) for patients with neuromuscular diseases[J].Dev Neurorehabil, 2020,23(4):210-217.
[6] Trundell D, Le Scouiller S, Gorni K, et al.Validity and reliability of the 32-Item Motor Function Measure in 2- to 5-Year-Olds with Neuromuscular disorders and 2- to 25-Year-Olds with Spinal muscular atrophy[J].Neurol Ther, 2020,9(2):575-584.
[7] Martini J,Caromano FA, Carvalho EV, et al.Boys with Duchenne muscular dystrophy: 1-year locomotor changes in relation to a control group[J].Percept Mot Skis, 2018,125(1):40-56.
[8] 张昊, 周宁, 吴士文.Duchenne型肌营养不良患者运动功能分析[J].中国康复理论与实践, 2018,24(2):214-218.
[9] Kim J, Jung IY, Kim SJ, et al.A new functional scale and ambulatory functional classification of Duchenne muscular dystrophy: Scale development and preliminary analyses of reliability and validity[J].Ann Rehabil Med, 2018,42(5):690-701.
[10] Bohannon, Richard W.Reliability of manual muscle testing: A systematic review[J].Isokinet Exerc Sci, 2018,26(4):245-252.
[11] Muntoni F, Domingos J, Manzur AY,et al.Categorising trajectories and individual item changes of the North Star Ambulatory Assessment in patients with Duchenne muscular dystrophy[J].PLoS One, 2019,14(9):e0221097.
[12] 秦伦, 李一芳, 赵希文, 等.北极星移动评价量表在Duchenne型肌营养不良患儿中的信度研究[J].中国实用儿科杂志, 2019,34(11):922-926.
[13] 陈土容, 黄美欢, 曹建国.神经肌肉病运动功能评估量表的应用进展[J].中国康复理论与实践, 2018,24(9):1043-1047.
[14] 史惟, 李惠, 李西华, 等.复旦中文版神经肌肉疾病运动功能评估量表在儿童和青少年中的信度和效度研究[J].中国循证儿科杂志, 2018,13(1):35-39.
[15] Nagy S, Schmidt S,Hafner P, et al.Measurements of motor function and other clinical outcome parameters in ambulant children with Duchenne muscular dystrophy[J].J Vis Exp, 2019,12(143):e58784.
[16] Diniz GP, Lasmar LM, Giannetti JG.Motor assessment in patients with Duchenne muscular dystrophy[J].Arq Neuropsiquiatr, 2012,70(6):416-21.
[17] Vuillerot C, Girardot F, Payan C, et al.Monitoring changes and predicting loss of ambulation in Duchenne muscular dystrophy with the motor function measure[J].Dev Med Child Neurol, 2010,52(1):60-65.
[18] Nagy S,Schädelin S, Hafner P, et al.Longitudinal reliability of outcome measures in patients with Duchenne muscular dystrophy[J].Muscle Nerve, 2020,61(1):63-68.
[19] Schmidt S,Hafner P, Klein A, et al.Timed function tests, motor function measure, and quantitative thigh muscle MRI in ambulant children with Duchenne muscular dystrophy: A cross-sectional analysis[J].Neuromuscul Disord, 2018,28(1):16-23.
[20] Hukuda ME, Favero FM, Oliveira AB, et al.Relationship between muscle strength and motor function in Duchenne muscular dystrophy[J].Arq Neuropsiquiatr, 2016,74(7):530-535.
基金
深圳市残疾人综合服务应用研究(JB2017-16-3)
PDF(461 KB)
