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用于II型和III型脊髓性肌萎缩症患者的哈默史密斯功能运动量表扩展版。

An expanded version of the Hammersmith Functional Motor Scale for SMA II and III patients.

作者信息

O'Hagen Jessica M, Glanzman Allan M, McDermott Michael P, Ryan Patricia A, Flickinger Jean, Quigley Janet, Riley Susan, Sanborn Erica, Irvine Carrie, Martens William B, Annis Christine, Tawil Rabi, Oskoui Maryam, Darras Basil T, Finkel Richard S, De Vivo Darryl C

机构信息

Columbia University, New York, NY, USA.

出版信息

Neuromuscul Disord. 2007 Oct;17(9-10):693-7. doi: 10.1016/j.nmd.2007.05.009. Epub 2007 Jul 19.

Abstract

PURPOSE

To develop and evaluate an expanded version of the Hammersmith Functional Motor Scale allowing for evaluation of ambulatory SMA patients.

PROCEDURES

Thirty-eight patients with SMA type II or III were evaluated using the Gross Motor Function Measure and the Hammersmith Functional Motor Scale. Based on statistical and clinical criteria, we selected 13 Gross Motor Function Measure items to develop an expanded HFMS. The expanded Hammersmith Functional Motor Scale was validated by comparison with the Gross Motor Function Measure minus the 13 items (GMFM-75) and an assessment of clinical function. The reliability of the expanded Hammersmith Functional Motor Scale in 36 patients was established.

FINDINGS

The expanded Hammersmith Functional Motor Scale was highly correlated with the GMFM-75 and the clinical function assessment (p=0.97, and p=0.90). The expanded Hammersmith Functional Motor Scale showed excellent test-retest reliability (International Coordinating Committee = 0.99).

CONCLUSIONS

The expanded Hammersmith Functional Motor Scale allows assessment of high functioning SMA type II and III patients. Ease of administration and correlation with established motor function measures justify use in future SMA clinical trials.

摘要

目的

开发并评估汉默史密斯功能运动量表的扩展版本,以用于评估能行走的脊髓性肌萎缩症(SMA)患者。

程序

使用粗大运动功能测量量表和汉默史密斯功能运动量表对38例II型或III型SMA患者进行评估。基于统计学和临床标准,我们选择了13项粗大运动功能测量量表项目来开发扩展版的汉默史密斯功能运动量表(HFMS)。通过与减去这13项后的粗大运动功能测量量表(GMFM-75)进行比较以及对临床功能进行评估,对扩展版汉默史密斯功能运动量表进行了验证。确定了扩展版汉默史密斯功能运动量表在36例患者中的可靠性。

结果

扩展版汉默史密斯功能运动量表与GMFM-75以及临床功能评估高度相关(p = 0.97和p = 0.90)。扩展版汉默史密斯功能运动量表显示出出色的重测信度(国际协调委员会 = 0.99)。

结论

扩展版汉默史密斯功能运动量表能够评估功能较高的II型和III型SMA患者。其易于实施以及与既定运动功能测量方法的相关性证明了它在未来SMA临床试验中的应用价值。

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