Levator-Medial Rectus Synkinesis

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Pang et al. (1986) described 2 sisters with lid retraction on adduction, the classic sign of levator-oculomotor synkinesis. In both women the left eye was involved. There was no history of trauma or ocular surgery and there were no complaints of diplopia. Both sisters had good visual acuity and full ocular motility. Each exhibited 2 mm of left upper lid elevation on adduction and 2 to 6 mm with elevation on adduction with depression. One sister showed 1 mm of ptosis in straight ahead gaze in the involved eye; this increased to almost 2 mm on abduction but full levator function was observed. Four other family members in 3 generations in a pattern consistent with autosomal dominant inheritance were described. The family showed 1 instance of male-to-male transmission and 2 instances of failure of penetrance in transmitting females. The levator muscle is involved in 2 other forms of congenital synkinesis: the Duane retraction syndrome (126800) and the Marcus Gunn jaw-winking phenomenon (154600).