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非行走型脊髓性肌萎缩症患者的哈默史密斯功能运动量表和运动功能测量-20

Hammersmith Functional Motor Scale and Motor Function Measure-20 in non ambulant SMA patients.

作者信息

Mazzone E, De Sanctis R, Fanelli L, Bianco F, Main M, van den Hauwe M, Ash M, de Vries R, Fagoaga Mata J, Schaefer K, D'Amico A, Colia G, Palermo C, Scoto M, Mayhew A, Eagle M, Servais L, Vigo M, Febrer A, Korinthenberg R, Jeukens M, de Viesser M, Totoescu A, Voit T, Bushby K, Muntoni F, Goemans N, Bertini E, Pane M, Mercuri E

机构信息

Department of Paediatric Neurology, Catholic University, Rome, Italy.

Dubowitz Neuromuscular Centre, Institute of Child Health, University College, London, UK.

出版信息

Neuromuscul Disord. 2014 Apr;24(4):347-52. doi: 10.1016/j.nmd.2014.01.003. Epub 2014 Jan 16.

Abstract

The aim of this prospective longitudinal multi centric study was to evaluate the correlation between the Hammersmith Functional Motor Scale and the 20 item version of the Motor Function Measure in non ambulant SMA children and adults at baseline and over a 12 month period. Seventy-four non-ambulant patients performed both measures at baseline and 49 also had an assessment 12 month later. At baseline the scores ranged between 0 and 40 on the Hammersmith Motor function Scale and between 3 and 45 on the Motor Function Measure 20. The correlation between the two scales was 0.733. The 12 month changes ranged between -11 and 4 for the Hammersmith and between -11 and 7 for the Motor Function Measure 20. The correlation between changes was 0.48. Our results suggest that both scales provide useful information although they appeared to work differently at the two extremes of the spectrum of abilities. The Hammersmith Motor Function Scale appeared to be more suitable in strong non ambulant patients, while the Motor Function Measures appeared to be more sensitive to capture activities and possible changes in the very weak patients, including more items capturing axial and upper limb activities. The choice of these measures in clinical trials should therefore depend on inclusion criteria and magnitude of expected changes.

摘要

这项前瞻性纵向多中心研究的目的是评估在非行走型脊髓性肌萎缩症儿童和成人中,哈默史密斯功能运动量表与20项运动功能测量量表在基线及12个月期间的相关性。74名非行走型患者在基线时进行了这两项测量,49名患者在12个月后也进行了评估。在基线时,哈默史密斯运动功能量表的得分在0至40分之间,20项运动功能测量量表的得分在3至45分之间。两个量表之间的相关性为0.733。哈默史密斯量表12个月的变化范围在-11至4分之间,20项运动功能测量量表的变化范围在-11至7分之间。变化之间的相关性为0.48。我们的结果表明,尽管这两个量表在能力谱的两个极端似乎工作方式不同,但它们都提供了有用的信息。哈默史密斯运动功能量表似乎更适合于较强的非行走型患者,而运动功能测量量表似乎对捕捉非常虚弱患者的活动和可能变化更敏感,包括更多捕捉轴向和上肢活动的项目。因此,在临床试验中选择这些测量方法应取决于纳入标准和预期变化的幅度。

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