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An analysis of myotonia in paramyotonia congenita
  1. David Burke,
  2. Nevell F. Skuse,
  3. A. Keith Lethlean
  1. Unit of Clinical Neurophysiology, Division of Neurology, Prince Henry Hospital, Little Bay, N.S.W. 2036, Australia


    In two subjects with paramyotonia congenita myotonic delay in muscle relaxation, recorded electromyographically and with a displacement transducer, was found to increase with repeated forceful contractions. Myotonia was elicited readily in warm temperatures, was initially aggravated by cooling, but was invariably lost as muscle fatigue developed. The EMG evidence of myotonia usually subsided before complete muscle relaxation had occurred, suggesting that a defect of the contractile mechanism was present over and above any defect at membrane level.

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    • 1 Some of these findings were reported to the 1974 meeting of the Australian Association of Neurologists.