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Prednisone eliminates the electrophysiological decrement of myotonia congenita

Thomas Chelimsky1, Peggy Neal, Edward Westbrook

  • 1From the Department of Neurology, Case Western Reserve University and University Hospitals of Cleveland, Cleveland, Ohio.

Journal of Clinical Neuromuscular Disease
|December 17, 2008
PubMed

The physiological effect of corticosteroids on myotonia is not reported. We studied a 44-year-old woman with myotonia congenita whose myotonic symptoms improved on prednisone for an unrelated facial palsy. Ten-hertz repetitive stimulation showed a 46% decrement between the 7th and 20th responses at baseline. Prednisone reduced the decrement in a dose-related fashion, to 14% on 20 mg per day and to 5% on 40 mg per day. The short exercise test also improved considerably. Corticosteroids could be further investigated for symptomatic myotonia.

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