短时间运动及短时间运动加冷却试验在肌强直性营养不良症（DM1 和 DM2）诊断中的价值。
Value of short exercise and short exercise with cooling tests in the diagnosis of myotonic dystrophies (DM1 and DM2).
机构信息
Department of Neurology, Medical University of Warsaw, Banacha 1A, Warsaw, 02-097, Poland.
出版信息
Muscle Nerve. 2014 Feb;49(2):277-83. doi: 10.1002/mus.23908. Epub 2013 Jul 3.
INTRODUCTION
Standard electromyography (EMG) is useful in the diagnosis of myotonic dystrophy type 1 (DM1) and type 2 (DM2), but it does not differentiate between them. The aim of this study was to estimate the utility of the short exercise test (SET) and short exercise test with cooling (SETC) in differentiating between DM1 and DM2.
METHODS
SET and SETC were performed in 32 patients with DM1 (mean age 35.8 ± 12.7 years) and 28 patients with DM2 (mean age 44.5 ± 12.5 years).
RESULTS
We observed a significant decline in compound motor action potential (CMAP) amplitude in DM1 with both SET and SETC immediately after effort. In DM2, there was no marked change in CMAP amplitude with either SET or SETC.
CONCLUSIONS
SET and SETC may serve as useful tools for clinical differentiation between DM1 and DM2, and they may be used as a guide for molecular testing.
简介
标准肌电图（EMG）在 1 型肌强直性营养不良（DM1）和 2 型肌强直性营养不良（DM2）的诊断中很有用，但无法区分两者。本研究旨在评估短运动试验（SET）和短运动试验加冷却（SETC）在区分 DM1 和 DM2 中的效用。
方法
对 32 例 DM1 患者（平均年龄 35.8±12.7 岁）和 28 例 DM2 患者（平均年龄 44.5±12.5 岁）进行 SET 和 SETC 检查。
结果
我们观察到 DM1 患者在 SET 和 SETC 后即刻出现复合运动动作电位（CMAP）幅度明显下降。而在 DM2 患者中，SET 和 SETC 均未引起 CMAP 幅度的明显变化。
结论
SET 和 SETC 可作为临床区分 DM1 和 DM2 的有用工具，并可作为分子检测的指导。
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