Bérard Carole, Payan Christine, Hodgkinson Isabelle, Fermanian Jacques
Department of Paediatric Rehabilitation, l'Escale, Centre Hospitalier Lyon-Sud, France.
Neuromuscul Disord. 2005 Jul;15(7):463-70. doi: 10.1016/j.nmd.2005.03.004.
A new scale for motor function measurement has been developed for neuromuscular diseases. The validation study included 303 patients, aged 6-62 years. Seventy-two patients had Duchenne muscular dystrophy, 32 Becker muscular dystrophy, 30 limb-girdle muscular dystrophy, 39 facio-scapulo-humeral dystrophy, 29 myotonic dystrophy, 21 congenital myopathy, 10 congenital muscular dystrophy, 35 spinal muscular atrophy and 35 hereditary neuropathy. The scale comprised 32 items, in three dimensions: standing position and transfers, axial and proximal motor function, distal motor function. Agreement coefficients for inter-rater reliability were excellent (kappa=0.81-0.94) for nine items, good (kappa=0.61-0.80) for 20 items and moderate (kappa=0.51-0.60) for three items. High correlations were found between the total score and other scores: Vignos (r=0.91) and Brooke (r=0.85) grades, Functional Independence Measure (r=0.91), the global severity of disability evaluated with visual analog scales by physicians (r=0.88) and physiotherapists (r=0.91). This scale is reliable, does not require any special equipment and is well-accepted by patients. Its sensitivity to change is being assessed to permit its use in clinical trials of neuromuscular diseases.
一种用于测量神经肌肉疾病运动功能的新量表已被开发出来。验证研究纳入了303名年龄在6至62岁之间的患者。其中72名患有杜氏肌营养不良症，32名患有贝克肌营养不良症，30名患有肢带型肌营养不良症，39名患有面肩肱型肌营养不良症，29名患有强直性肌营养不良症，21名患有先天性肌病，10名患有先天性肌营养不良症，35名患有脊髓性肌萎缩症以及35名患有遗传性神经病。该量表包含32个项目，分为三个维度：站立姿势与转移、轴向和近端运动功能、远端运动功能。评分者间信度的一致性系数在9个项目中为优（kappa = 0.81 - 0.94），20个项目中为良（kappa = 0.61 - 0.80），3个项目中为中等（kappa = 0.51 - 0.60）。在总分与其他评分之间发现了高度相关性：Vignos评分（r = 0.91）和Brooke评分（r = 0.85）、功能独立性测量（r = 0.91）、医生用视觉模拟量表评估的残疾总体严重程度（r = 0.88）以及物理治疗师评估的结果（r = 0.91）。该量表可靠，不需要任何特殊设备，且患者接受度良好。目前正在评估其对变化的敏感性，以便能用于神经肌肉疾病的临床试验。
