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Evidence for the occurrence of myotonia in the extraocular musculature in patients with dystrophia myotonica

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<mark>Journal publication date</mark>1993
<mark>Journal</mark>Neuro-Ophthalmology
Issue number1
Volume13
Number of pages8
Pages (from-to)17-24
Publication StatusPublished
<mark>Original language</mark>English

Abstract

Dystrophia myotonica (DM) is a multisystem disorder which has been reported to affect eye movements. There is, however, disagreement in the literature as to whether the observed slowing of saccadic eye movements is due to a central abnormality or due to abnormalities of the extraocular muscles themselves. A ‘warm-up’ effect is well known to occur in the peripheral muscles in DM. This effect involves a progressive decrease in the degree of myotonia observed with each successive relaxation during a period of repetitive contraction and relaxation of a given muscle. The authors have studied seven patients using two different paradigms to provide evidence that the ‘warm-up’ phenomenon can be demonstrated in extraocular musculature in patients with DM. Such an effect is not seen in controls. The observation of a ‘warm-up’ phenomenon suggests that the disorder of saccadic eye movements is likely to be due to an abnormality of the extraocular muscles themselves rather than a central mechanism.