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Post-tetanic mechanical tension and evoked action potentials in McArdle's disease
  1. N. J. Brandt,
  2. F. Buchthal,
  3. F. Ebbesen,
  4. Z. Kamieniecka,
  5. C. Krarup
  1. Laboratory of Clinical Neurophysiology, University of Copenhagen, Denmark
  2. The Department of Pediatrics, Section of Teratology of the Rigshospital, University of Copenhage, Denmark
  3. The Institute of Neurophysiology, University of Copenhagen, Denmark


    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.

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