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An electrophysiological investigation of limb-girdle and facioscapulohumeral dystrophy
  1. R. E. P. Sica1,
  2. A. J. McComas2
  1. Regional Neurological Centre, Newcastle General Hospital, Newcastle upon Tyne


    A detailed electrophysiological study has been made of the extensor digitorum brevis muscle and its motor innervation in 11 patients with limb-girdle or facioscapulohumeral dystrophy. In nine patients there were reductions in the populations of motor units and many surviving units appeared to be abnormally large. Most of the investigated muscles had slowed isometric twitches and decremental evoked potentials after repetitive nerve stimulation. The experimental observations have been interpreted in terms of a neuropathic process.

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    • 1 British Council Scholar; now at Hospital Ramos Mejia, Buenos Aires, Argentina.

    • 2 M.R.C. External Scientific Staff; now in Department of Medicine, McMaster University Medical Centre, Hamilton 16, Ontario.