Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy
Longitudinal functional and NMR assessment of upper limbs in Duchenne muscular dystrophy
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DOI：
10.1212/wnl.0000000000002464
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发表时间：
2016-03-15
期刊：
影响因子：
9.9
通讯作者：
Servais, Laurent
中科院分区：
文献类型：
--
作者：
Hogrel, Jean-Yves;Wary, Claire;Servais, Laurent
目的：探讨核磁共振（NMR）和功能评估在Duchenne型肌营养不良症（DMD）患者随访中的价值。所有患者进行了临床和功能评估，每6个月使用的运动功能测量（MFM），手握和关键捏强度，MoviPlate，和NMR光谱和imaging studies.Results：上肢远端力量下降在卧床患者在1年期间，门诊患者在同一时期表现出改善。这同样适用于几个NMRS指标，如磷酸肌酸/三磷酸腺苷，在老年患者中下降，但在年轻的门诊患者中增加。上肢脂肪浸润随年龄增长呈线性增加。几乎所有的NMR和功能评估结果correlated.Conclusions：我们的研究结果强调DMD患者的功能和NMR评估的互补性。对各种指标变化的敏感性可根据疾病阶段而不同。
Objective:To explore the value of nuclear magnetic resonance (NMR) and functional assessments for follow-up of ambulatory and nonambulatory patients with Duchenne muscular dystrophy (DMD).Methods:Twenty-five 53-skippable patients with DMD were included in this study; 15 were nonambulatory at baseline. All patients underwent clinical and functional assessments every 6 months using the Motor Function Measure (MFM), hand grip and key pinch strength, MoviPlate, and NMR spectroscopy and imaging studies.Results:Upper limb distal strength decreased in nonambulatory patients over the period of 1 year; ambulatory patients showed improvement during the same period. The same applied for several NMRS indices, such as phosphocreatine/adenosine triphosphate, which decreased in older patients but increased in younger ambulatory patients. Fat infiltration in the upper limbs increased linearly with age. Almost all NMR and functional assessment results correlated.Conclusions:Our results underscore complementarity of functional and NMR assessments in patients with DMD. Sensitivity to change of various indices may differ according to disease stage.