- Type of congenital cranial dysinnervation disorders (CCDD)
MGJWS was first described by a Scottish ophthalmologist Dr.Robert Marcus Gunn in the year 1883. This syndrome was initially reported in a 15-year-old girl as unilateral ptosis associated with the upper eyelid contraction on the same side. Other names of MGJWS include Marcus-Gunn jaw winking phenomenon (MGP), Marcus Gunn ptosis, Marcus Gunn jaw winking trigemino-oculomotor synkinesis, Maxillopalpebral synkinesis, and Pterygoid-levator synkinesis.
- Cleft lip/cleft palate
- Olfactory nerve disturbance
- CHARGE syndrome (coloboma, heart defects, choanal atresia, retardation of growth and development, genital abnormalities, and ear abnormalities)
- Renal calculi
Jaw-wink reflex:Momentary retraction/elevation of the upper eyelid (which was ptotic) upon stimulation of the ipsilateral pterygoid muscle, which is followed by a rapid return to a lower position
- Movement of one upper eyelid in a rapid rising motion each time the jaw moves
Unilateral blepharoptosis:Normal, low-lying upper eyelid margin
- Strabismus (50-60% cases)
- Anisometropia (5-25% cases)
- Amblyopia (30-60%), almost always secondary to strabismus/anisometropia
- Defined as a decrease in vision of 2 or more lines on the Snellen chart
- Superior rectus palsy (25% cases)
- Double elevator palsy (25% cases)
- Morning Glory anomaly (rare)
- Duane retraction syndrome (rare)
Inverse Marcus Gunn phenomenon:Ptosis in primary gaze is worsened with mouth opening as apposed to MGJWS where the ptosis improves with mouth opening
Marin Amat syndrome:Abnormal connection between the motor branch of CN V nerve and frontal, zygomatic branches of CN VII. Here, both eyes close momentarily like blepharospasm with mouth opening secondary to the action of orbicularis oculi muscle.
Other differential diagnoses:
- Chronic progressive external ophthalmoplegia
- Congenital fibrosis syndrome
- Myasthenia gravis
Treatment of Marcus Gunn phenomenon is usually not needed because the condition tends to grow less noticeable with age.
- Aggressive treatment with occlusion therapy and/or correction of anisometropia before any consideration of ptosis surgery if amblyopia is encountered.
- In severe cases: Surgery with unilateral levator excision and frontalis brow suspension may be used