Brandt N J, Buchthal F, Ebbesen F, Kamieniecka Z, Krarup C
J Neurol Neurosurg Psychiatry. 1977 Sep;40(9):920-5. doi: 10.1136/jnnp.40.9.920.
The tension produced by the cramp evoked in the adductor pollicis muscle by repetitive stimuli to the nerve (20/s for 50 s) and by full voluntary effort in the brachial biceps was measured in a patient with McArdle's disease. The contracture was 17% of the peaktetanic tension, and was not associated with action potentials. Twitches superimposed on the contracture were at most diminished to half, as were their action potentials. Both slow and fast muscle fibres participated in the contracture. The contraction time of the twitches elicited after the tetanus was prolonged more in the patient than in a normal subject of the same age. There was evidence of delayed firing, first observed 90 seconds after the peak of the contracture. The patient had electromyographic and histological signs of myopathy.
在一名患有麦克尔代尔氏病的患者中,测量了通过对神经进行重复刺激(每秒20次,持续50秒)在拇收肌中诱发的痉挛以及肱二头肌完全自主用力所产生的张力。挛缩为强直张力峰值的17%,且与动作电位无关。叠加在挛缩上的抽搐最多减弱至一半,其动作电位也是如此。慢肌纤维和快肌纤维都参与了挛缩。与同龄正常受试者相比,该患者在强直收缩后引发的抽搐收缩时间延长得更多。有延迟放电的证据,在挛缩峰值后90秒首次观察到。该患者有肌病的肌电图和组织学迹象。