Lomonaco M, Milone M, Valente E M, Padua L, Tonali P
Department of Neurology, Università Cattolica del Sacro Cuore, Rome, Italy.
Muscle Nerve. 1996 Dec;19(12):1523-9. doi: 10.1002/mus.880191204.
Five patients with muscle glycogenoses (Gly), 30 normal subjects (NS), and 52 disease controls received 3 Hz repetitive stimulation of the ulnar nerve for 4 min (P-LRNS) during regional ischemia and during normal circulation. During regional ischemia, the compound muscle action potential (CMAP) of NS showed 8.5% (SE: 1.0) facilitation after 1 min of P-LRNS and 4.8 +/- 1.5% facilitation after 4 min. Gly showed 8.1 +/- 1.0% facilitation after 1 min of P-LRNS but -58.6 +/- 6.9% depression after 4 min of P-LRNS. During normal circulation, in 18 NS and 4 Gly tested, the facilitation detected after 1 min of P-LRNS was unchanged until the end of stimulation. Compared with the mean +/- 2 SD of NS, individual values of CMAP depression were abnormal in all Gly patients already by the 3rd min of ischemic P-LRNS. Of disease controls, myasthenia gravis patients only showed a CMAP depression during ischemic P-LRNS which was distinguishable from that detected in Gly, being mainly induced by neuromuscular transmission block.
5例肌肉糖原贮积病（Gly）患者、30名正常受试者（NS）和52名疾病对照者在局部缺血期间及正常循环期间接受了尺神经3Hz重复刺激4分钟（P-LRNS）。在局部缺血期间，正常受试者的复合肌肉动作电位（CMAP）在P-LRNS刺激1分钟后显示出8.5%（标准误：1.0）的易化，4分钟后显示出4.8±1.5%的易化。糖原贮积病患者在P-LRNS刺激1分钟后显示出8.1±1.0%的易化，但在P-LRNS刺激4分钟后出现-58.6±6.9%的抑制。在正常循环期间，在测试的18名正常受试者和4例糖原贮积病患者中，P-LRNS刺激1分钟后检测到的易化在刺激结束前保持不变。与正常受试者的平均值±2倍标准差相比，在缺血性P-LRNS刺激的第3分钟时，所有糖原贮积病患者的CMAP抑制个体值均异常。在疾病对照者中，重症肌无力患者仅在缺血性P-LRNS期间出现CMAP抑制，这与在糖原贮积病患者中检测到的情况不同，主要是由神经肌肉传递阻滞引起的。
