关键词: spinal muscular atrophy Hand duchenne muscular dystrophy patient outcome assessment

来  源:   DOI:10.3233/JND-230182

Abstract:
UNASSIGNED: Measurement of muscle strength and motor function is recommended in clinical trials of neuromuscular diseases, but the loss of hand strength at which motor function is impacted is not documented.
UNASSIGNED: To establish the relationship between hand strength and function, and to determine the strength threshold that differentiates normal and abnormal hand function in individuals with Duchenne Muscular Dystrophy (DMD) or Spinal Muscular Atrophy (SMA).
UNASSIGNED: Maximal handgrip and key pinch strength were measured with the MyoGrip and MyoPinch dynamometers, respectively. Hand function was assessed using the MoviPlate, the Motor Function Measure items for distal upper limb (MFM-D3-UL) and the Cochin Hand Function Scale (CHFS).
UNASSIGNED: Data from 168 participants (91 DMD and 77 SMA, age 6-31 years) were analyzed. Relationships between strength and function were significant (P < 0.001). Hand function was generally preserved when strength was above the strength threshold determined by Receiver-Operating Characteristic (ROC) analysis: For MFM-D3-UL, the calculated handgrip strength thresholds were 41 and 13% of the predicted strength for a healthy subject (% pred) and the key pinch strength thresholds were 42 and 26% pred for DMD and SMA, respectively. For the MoviPlate, handgrip strength thresholds were 11 and 8% pred and key pinch strength thresholds were 21 and 11% pred for DMD and SMA, respectively. For participants with sub-threshold strength, hand function scores decreased with decreasing strength. At equal % pred strength, individuals with SMA had better functional scores than those with DMD.
UNASSIGNED: Hand function is strength-dependent for most motor tasks. It declines only when strength falls below a disease-specific threshold. Therefore, therapies capable of maintaining strength above this threshold should preserve hand function.
摘要:
在神经肌肉疾病的临床试验中建议测量肌肉力量和运动功能,但运动功能受到影响的手的力量损失没有记录。
要建立手部力量与功能之间的关系,并确定区分Duchenne肌营养不良(DMD)或脊髓性肌萎缩(SMA)个体的正常和异常手功能的力量阈值。
用MyoGrip和MyoPinch测力计测量了最大的握力和钥匙捏合强度,分别。使用MoviPlate评估手功能,上肢远端运动功能测量项目(MFM-D3-UL)和Cochin手部功能量表(CHFS)。
来自168名参与者的数据(91DMD和77SMA,年龄6-31岁)进行分析。强度和功能之间的关系显着(P<0.001)。当强度高于接收器工作特性(ROC)分析确定的强度阈值时,通常会保留手功能:对于MFM-D3-UL,对于健康受试者,计算出的握力阈值为预测强度的41%和13%(%pred),对于DMD和SMA,键捏力阈值为42%和26%pred,分别。对于MoviPlate,对于DMD和SMA,握力阈值分别为11%和8%pred,键捏力阈值分别为21%和11%pred,分别。对于强度低于阈值的参与者,手功能评分随着力量的降低而降低。在相同的%pred强度下,SMA患者的功能评分优于DMD患者.
对于大多数运动任务,手的功能取决于强度。只有当力量低于疾病特异性阈值时,它才会下降。因此,能够将力量保持在该阈值以上的疗法应保留手功能。
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