van der Hoeven J H, Links T P, Zwarts M J, van Weerden T W
Department of Clinical Neurophysiology, University Hospital Groningen, The Netherlands.
Muscle Nerve. 1994 Aug;17(8):898-905. doi: 10.1002/mus.880170809.
Muscle fiber conduction velocity (MFCV) in the brachial biceps muscle was determined in a large family of patients with hypokalemic periodic paralysis (HOPP) by both a surface and an invasive method. Other surface EMG parameters and the muscle force were also determined. Both the surface and the invasive method showed a significantly lower mean MFCV in the proven gene carriers but only the invasive method showed a lower MFCV in all proven carriers. It can be concluded that MFCV determination is a reliable method to detect the membrane defect in HOPP carriers and that the invasive method is not only easy to perform, but also more sensitive. The muscle force and the integrated EMG at maximal voluntary contraction were lower in the carrier group. A positive correlation between the surface MFCV and the neuromuscular efficiency (the quotient of force and integrated EMG) was found in the controls but not in the HOPP carriers. Since type II fibers have a higher neuromuscular efficiency, this suggests a preferential involvement of type II fibers in HOPP.
通过表面和侵入性两种方法,在一大组低钾性周期性麻痹(HOPP)患者家族中测定了肱二头肌的肌纤维传导速度(MFCV)。还测定了其他表面肌电图参数和肌力。表面法和侵入性方法均显示,在已证实的基因携带者中,平均MFCV显著降低,但只有侵入性方法在所有已证实的携带者中显示出较低的MFCV。可以得出结论,MFCV测定是检测HOPP携带者膜缺陷的可靠方法,并且侵入性方法不仅易于操作,而且更敏感。携带者组的肌力和最大自主收缩时的肌电图积分较低。在对照组中发现表面MFCV与神经肌肉效率(力与肌电图积分的商)之间呈正相关,但在HOPP携带者中未发现。由于II型纤维具有较高的神经肌肉效率,这表明II型纤维在HOPP中优先受累。