Extraocular myotonia in families with dystrophia myotonica

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Abstract
It was shown that extraocular muscles are affected in dystrophia myotonica. Localized clinical myotonia of the muscles of ocular motility, without evidence of other systemic manifestations of dystrophia myotonica, could conceivably be another mark of the so-called carrier state. Possibly, long follow-up of such individuals will reveal that, in some cases, localized ocular myotonia is an early manifestation of dystrophia myotonica. In either instance, it is believed that the eye sign represents extraocular myotonia and is a forme fruste of dystrophia myotonica.

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