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Marcus Gunn Jaw-winking Syndrome

Background

In 1883, Marcus Gunn described a 15-year-old girl with a peculiar type of congenital ptosis that included an associated winking motion of the affected eyelid on the movement of the jaw.
This synkinetic jaw-winking phenomenon now bears his name. See the image below.

Congenital left upper eyelid ptosis.

Congenital left upper eyelid ptosis.

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Patients with Marcus Gunn jaw-winking syndrome have variable degrees of blepharoptosis in the resting, primary position. Although Marcus Gunn jaw-winking syndrome is usually unilateral, it can present bilaterally in rare cases.

The wink reflex consists of a momentary upper eyelid retraction or elevation to an equal or higher level than the normal fellow eyelid upon stimulation of the ipsilateral pterygoid muscle. This response is followed by a rapid return to a lower position. The amplitude of the wink tends to be worse in downgaze. This rapid, abnormal motion of the eyelid can be the most disturbing aspect of the jaw-winking syndrome.

The wink phenomenon may be elicited by opening the mouth, thrusting the jaw to the contralateral side, jaw protrusion, chewing, smiling, or sucking.
This wink phenomenon is often discovered early, as the infant is bottle-feeding or breastfeeding. See the image below.

Marcus Gunn jaw-winking with left upper eyelid ret

Marcus Gunn jaw-winking with left upper eyelid retraction on opening of the mouth.

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Jaw-winking ptosis is almost always sporadic, but familial cases with an irregular autosomal dominant inheritance pattern have been reported.

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