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在肌萎缩侧索硬化症治疗试验中使用电生理测试来测量疾病进展。

Use of electrophysiologic tests to measure disease progression in ALS therapeutic trials.

作者信息

Kelly J J, Thibodeau L, Andres P L, Finison L J

机构信息

Dept of Neurology, Tufts-New England Medical Center, Boston, Massachusetts 02111.

出版信息

Muscle Nerve. 1990 Jun;13(6):471-9. doi: 10.1002/mus.880130602.

DOI:10.1002/mus.880130602
PMID:2195338
Abstract

A battery of electrophysiologic tests was developed to assess the relative degree of lower and upper motor neuron (spasticity) deficit in a group of ALS patients enrolled in a therapeutic trial. Test results were correlated with strength in the tibialis anterior muscle as determined by measurement of maximum voluntary isometric contraction (MVIC), using strain gauge tensiometers, and were also correlated with a clinical spasticity rating scale. Patients were tested every 6 to 8 weeks over more than 1 year. Compound muscle action potential amplitude (CMAPa) from tibialis anterior correlated best with MVIC and also showed a strong linear correlation with time, as did MVIC. Other tests correlated poorly with MVIC on the average, although individual patients did show high correlations. In those patients where correlation between CMAPa and MVIC was low, MVIC did not show a high linear correlation with time and was also highly variable. This study suggests that the addition of CMAPa should be considered in ALS therapeutic trials if MVIC is not available. In addition, CMAPa can be useful in study samples where MVIC deterioration is not linear over time or is highly variable.

摘要

开发了一系列电生理测试,以评估参与治疗试验的一组肌萎缩侧索硬化症(ALS)患者上下运动神经元(痉挛)缺陷的相对程度。测试结果与通过使用应变片张力计测量最大自主等长收缩(MVIC)来确定的胫前肌力量相关,并且还与临床痉挛评定量表相关。患者在超过1年的时间里每6至8周接受一次测试。胫前肌的复合肌肉动作电位幅度(CMAPa)与MVIC的相关性最佳,并且与时间也呈现出很强的线性相关性,MVIC也是如此。平均而言,其他测试与MVIC的相关性较差,尽管个别患者确实显示出高度相关性。在那些CMAPa与MVIC之间相关性较低的患者中,MVIC与时间没有呈现出高线性相关性,并且变化也很大。这项研究表明,如果无法获得MVIC,则在ALS治疗试验中应考虑增加CMAPa测量。此外,在MVIC随时间的恶化不是线性的或变化很大的研究样本中,CMAPa可能会很有用。

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