Bilateral Marcus Gunn jaw-winking syndrome

Abstract

A 10-year-old boy presented with alternating elevation of his eyelids upon chewing, present since birth (video on the Neurology ® Web site at Neurology.org). Examination showed mild left ptosis but no ophthalmoparesis. This represents Marcus Gunn jaw-winking syndrome (MGJWS), also called trigemino-oculomotor synkinesis or pterygoid-levator synkinesis. It likely results from aberrant connections in the midbrain between trigeminal mesencephalic nucleus and oculomotor nucleus, although others have postulated connections between the mandibular division of trigeminal nerve innervating lateral pterygoid and the superior division of oculomotor nerve innervating levator palpebrae superioris. MGJWS occurs in 2%-13% of patients with congenital ptosis.

The rare phenomenon of Marcus-Gunn jaw winking without ptosis: Report of 14 cases and review of the literature

“…MGJWP usually presents unilaterally, but rarely can be seen bilaterally. [ 7 8 9 ] All 14 patients demonstrated typical upper eyelid elevation on chewing or sucking. Although lid elevation is commonly associated with chewing or sucking movements, it has been reported with other movements such as lateral mandibular movement, yawning, smiling, sternocleidomastoid contraction, tongue protrusion, Valsalva maneuver, or even during inspiration.…”

Marcus Gunn syndrome without ptosis

“…"jaw-winking" феномен, е рядко конгенитално заболяване, описано за първи път през 1883 г. от Robert Marcus Gunn (1). Обозначава се още като тригемино-окуломоторна синкенезия или птериголеваторна синкенезия и е най-честата форма на конгенитална синкинетична птоза (2). Синдромът се характеризира с наличието на конгенитална птоза на горния клепач и синкинетично повдигане и дори ретракция на засегнатия клепач под формата на примигване при движение на долната челюст (3).…”

“…По последни литературни данни синдромът на Marcus Gunn съставлява 8.5% от всички случаи на конгенитална птоза (4), като за българската популация честотата му е 5.4% (5). По правило феноменът се наблюдава едностранно, макар че са описани и изяви на двуоочна патология (2,6,7). В повечето случаи се касае за спорадично засягане, като рядко описаните фамилни случаи имат АД механизъм на унаследяване (8).…”

“…1); нормален ортоптичен статус; рефракция в нормите за възрастта, без наличието INTRODUCTION Marcus Gunn syndrome or the so-called "jaw-winking" phenomenon is a rare congenital disorder, described for a first time by Robert Marcus Gunn in 1883 (1). It is known as trigemino-oculomotor synkinesis or pterygoid-levator synkinesis and is the most common form of congenital synkinetic ptosis (2). The syndrome is characterized by congenital ptosis and synkinetic elevation and even retraction of the affected eyelid as winking triggered by jaw movement (3).…”

“…An updated literature data reveal that Marcus Gunn phenomenon is responsible for up to 8.5% of all cases with congenital ptosis (4), with an incidence rate of 5.4% for the Bulgarian population (5). In most cases it is an unilateral disease, although bilateral cases have also been described (2,6,7). It is usually considered a sporadic condition, though familial cases have been documented (8).…”