Intermittent Esotropia Associated with Rippling Muscle Disease

Abstract

We report a rare myopathy known as rippling muscle disease, with the unique feature of extraocular muscle involvement, presenting as a variable esotropia. Chart review with a review of the literature. Neurologic and neuro-ophthalmic examinations as well as electromyography and muscle biopsy confirm that this case closely resembles those described in the literature with the additional feature of a variable esotropia. Rippling muscle disease may be associated with intermittent esotropia. The pathophysiology of this disorder is unknown, but the intermittent esotropia is likely related to "rippling" of the medial recti.