Ocular Neuromyotonia
A child with a history of left exotropia secondary to a congenital third nerve palsy treated with strabismus surgery and ptosis repair presented with sudden onset intermittent left upper lid twitching and involuntary eye movements. Patient had no previous history of intracranial masses, brain radiation, thyroid abnormalities or demyelinating conditions. Upon examination patient had decreased visual acuity on the left secondary to amblyopia. Slit lamp exam showed left upper lid ptosis and a well healed surgical scar. Fundoscopic examination was normal in both eyes. Sensorimotor exam revealed small but variable angle exotropia in primary gaze that changed to a left esotropia with prolonged right gaze associated with nystagmoid movements and left upper lid twitching, most consistent with ocular neuromyotonia. There was significant limitation to supraduction and infraduction on the left eye due to a congenital left third nerve palsy and widening of the palpebral fissure in adduction due to aberrant regeneration. Brain MRI with and without intravenous contrast was performed and found normal. Patient was followed for 5 years and ocular motility remained stable. Treatment with Oxcarbazepine was recommended but patient and parents deferred.
Presentation Date: 06/03/2021
Issue Date: 06/18/2021
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