Myasthenic Ptosis With Synkinetic Override in a Child With Congenital Oculomotor Nerve Palsy

Abstract

Congenital myasthenia gravis usually involves the ocular motor system in predictable ways. The purpose of this report is to describe a child who had a congenital oculomotor nerve palsy with aberrant regeneration and fatigable ptosis associated with congenital myasthenia gravis. During periods of levator fatigability, gaze activation of the ipsilateral superior or medial rectus muscles produced instantaneous levator retraction with no fatigability. The ability of this child’s synkinetic neuromuscular connections to override her myasthenic levator fatigability may provide insight into the establishment of normal and abnormal neuromuscular connections in the setting of congenital myasthenia gravis.