The case of a 1 month-old boy with a severe form of Thomsen-Becker myotonia is reported. Resistance to regular antimyotonic treatment over a 3 month-period of time led to use mexiletine, an antiarrhythmic drug. This treatment was responsible for a dramatic clinical and electromyographic improvement. No adverse effect has been observed over a 16 month-period of continuous treatment.
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