Marcus Gunn phenomenon

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Marcus Gunn phenomenon
Other namesMarcus Gunn jaw-winking or Trigemino-oculomotor synkinesis
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Nerves of the orbit, and the ciliary ganglion. Side view. (Trigeminal nerve and oculomotor nerve both visible)
Specialty Ophthalmology neurology

Marcus Gunn phenomenon is an autosomal dominant condition with incomplete penetrance, in which nursing infants will have rhythmic upward jerking of their upper eyelid. This condition is characterized as a synkinesis: when two or more muscles that are independently innervated have either simultaneous or coordinated movements. [1] [2]

Contents

Common physiologic examples of synkineses occur during sucking, chewing, or conjugate eye movements. There are also several abnormal cranial nerve synkineses, both acquired and congenital. Marcus Gunn jaw-winking is an example of a pathologic congenital synkinesis.

First described by the ophthalmologist Marcus Gunn in 1883, [3] this condition presents in approximately 5% of neonates with congenital ptosis. This condition has been associated with amblyopia (in 54% of cases), anisometropia (26%), and strabismus (56%).

Presentation

An affected young woman demonstrating the Marcus Gunn Jaw Wink Phenomenon. The woman cannot control her eyelids while sucking on a straw. This is a particularly mild case. Markusgunjawwinkwoman.jpg
An affected young woman demonstrating the Marcus Gunn Jaw Wink Phenomenon. The woman cannot control her eyelids while sucking on a straw. This is a particularly mild case.

Behavioral and social implications

Although treatment may be unnecessary, there may be social implications, especially in young children when venturing from a supportive home environment to a public environment (e.g., starting school). Continued support, including monitoring behavior and educating the child about his or her appearance as seen by others, is encouraged. Gradual or sudden withdrawal from interaction with others is a sign that may or may not be related to such behavior. Studies are being conducted to elucidate these implications.[ citation needed ]

Pathophysiology

It has been postulated that the synkinesis is due to damage to cranial nerve nuclei, caused by peripheral nerve injury and the nuclear lesion releases evolutionarily older [neural] mechanisms with their tendency toward associated movements, and so primitive reflexes are not inhibited.[ citation needed ]

Marcus Gunn jaw-winking is an exaggeration of a very weak physiologic co-contraction that has been disinhibited secondary to a congenital brain stem lesion. The stimulation of the trigeminal nerve by contraction of the pterygoid muscles of jaw results in the excitation of the branch of the oculomotor nerve that innervates the levator palpebrae superioris ipsilaterally (on the same side of the face), so the patient will have rhythmic upward jerking of their upper eyelid.[ citation needed ]

There are two major groups of trigemino-oculomotor synkineses:[ citation needed ]
1) External pterygoid-levator synkinesis is when the eyelid raises upon:

2) Internal pterygoid-levator synkinesis is when the eyelid raises upon teeth clenching

External pterygoid-levator synkinesis is the more common group.[ citation needed ]

Treatment

Treatment is usually unnecessary. In severe cases, surgery with a bilateral levator excision and frontalis brow suspension may be used. [4]

Inverse Marcus Gunn phenomenon

Inverse Marcus Gunn phenomenon is a rare condition [5] that causes the eyelid to fall upon opening of the mouth. In this case, trigeminal innervation to the pterygoid muscles of the jaw is associated with an inhibition of the branch of the oculomotor nerve to the levator palpebrae superioris, as opposed to stimulation in Marcus Gunn jaw-winking.[ citation needed ]

Related Research Articles

Articles related to anatomy include:

<span class="mw-page-title-main">Oculomotor nerve</span> Cranial nerve III, for eye movements

The oculomotor nerve, also known as the third cranial nerve, cranial nerve III, or simply CN III, is a cranial nerve that enters the orbit through the superior orbital fissure and innervates extraocular muscles that enable most movements of the eye and that raise the eyelid. The nerve also contains fibers that innervate the intrinsic eye muscles that enable pupillary constriction and accommodation. The oculomotor nerve is derived from the basal plate of the embryonic midbrain. Cranial nerves IV and VI also participate in control of eye movement.

<span class="mw-page-title-main">Supraorbital nerve</span> Branch of the frontal nerve

The supraorbital nerve is one of two branches of the frontal nerve, itself a branch of the ophthalmic nerve. The other branch of the frontal nerve is the supratrochlear nerve.

Blinking is a bodily function; it is a semi-autonomic rapid closing of the eyelid. A single blink is determined by the forceful closing of the eyelid or inactivation of the levator palpebrae superioris and the activation of the palpebral portion of the orbicularis oculi, not the full open and close. It is an essential function of the eye that helps spread tears across and remove irritants from the surface of the cornea and conjunctiva.

<span class="mw-page-title-main">Eyelid</span> Thin fold of skin that covers and protects the eye

An eyelid is a thin fold of skin that covers and protects an eye. The levator palpebrae superioris muscle retracts the eyelid, exposing the cornea to the outside, giving vision. This can be either voluntarily or involuntarily. "Palpebral" means relating to the eyelids. Its key function is to regularly spread the tears and other secretions on the eye surface to keep it moist, since the cornea must be continuously moist. They keep the eyes from drying out when asleep. Moreover, the blink reflex protects the eye from foreign bodies. A set of specialized hairs known as lashes grow from the upper and lower eyelid margins to further protect the eye from dust and debris.

<span class="mw-page-title-main">Duane syndrome</span> Rare congenital disease characterized by external gaze palsy

Duane syndrome is a congenital rare type of strabismus most commonly characterized by the inability of the eye to move outward. The syndrome was first described by ophthalmologists Jakob Stilling (1887) and Siegmund Türk (1896), and subsequently named after Alexander Duane, who discussed the disorder in more detail in 1905.

<span class="mw-page-title-main">Levator palpebrae superioris muscle</span> Muscle in orbit that elevates upper eyelid

The levator palpebrae superioris is the muscle in the orbit that elevates the upper eyelid.

<span class="mw-page-title-main">Extraocular muscles</span> Seven extrinsic muscles of the human eye

The extraocular muscles, or extrinsic ocular muscles, are the seven extrinsic muscles of the human eye. Six of the extraocular muscles, the four recti muscles, and the superior and inferior oblique muscles, control movement of the eye and the other muscle, the levator palpebrae superioris, controls eyelid elevation. The actions of the six muscles responsible for eye movement depend on the position of the eye at the time of muscle contraction.

<span class="mw-page-title-main">Orbicularis oculi muscle</span> Facial muscle that closes the eyelids

The orbicularis oculi is a muscle in the face that closes the eyelids. It arises from the nasal part of the frontal bone, from the frontal process of the maxilla in front of the lacrimal groove, and from the anterior surface and borders of a short fibrous band, the medial palpebral ligament.

<span class="mw-page-title-main">Tensor veli palatini muscle</span> Muscle of the soft palate

The tensor veli palatini muscle is a thin, triangular muscle of the head that tenses the soft palate and opens the Eustachian tube to equalise pressure in the middle ear.

<span class="mw-page-title-main">Superior tarsal muscle</span>

The superior tarsal muscle is a smooth muscle adjoining the levator palpebrae superioris muscle that helps to raise the upper eyelid.

<span class="mw-page-title-main">Ptosis (eyelid)</span> Drooping of the upper eyelid over the eye

Ptosis, also known as blepharoptosis, is a drooping or falling of the upper eyelid. This condition is sometimes called "lazy eye," but that term normally refers to the condition amblyopia. If severe enough and left untreated, the drooping eyelid can cause other conditions, such as amblyopia or astigmatism, so it is especially important to treat the disorder in children before it can interfere with vision development.

Blepharochalasis is an inflammation of the eyelid that is characterized by exacerbations and remissions of eyelid edema, which results in a stretching and subsequent atrophy of the eyelid tissue, leading to the formation of redundant folds over the lid margins. It typically affects only the upper eyelids, and may be unilateral as well as bilateral.

<span class="mw-page-title-main">Weber's syndrome</span> Medical condition

Weber's syndrome, also known as midbrain stroke syndrome or superior alternating hemiplegia, is a form of stroke that affects the medial portion of the midbrain. It involves oculomotor fascicles in the interpeduncular cisterns and cerebral peduncle so it characterizes the presence of an ipsilateral lower motor neuron type oculomotor nerve palsy and contralateral hemiparesis or hemiplegia.

<span class="mw-page-title-main">Oculomotor nerve palsy</span> Medical condition

Oculomotor nerve palsy or oculomotor neuropathy is an eye condition resulting from damage to the third cranial nerve or a branch thereof. As the name suggests, the oculomotor nerve supplies the majority of the muscles controlling eye movements. Damage to this nerve will result in an inability to move the eye normally. The nerve also supplies the upper eyelid muscle and is accompanied by parasympathetic fibers innervating the muscles responsible for pupil constriction. The limitations of eye movement resulting from the condition are generally so severe that patients are often unable to maintain normal eye alignment when gazing straight ahead, leading to strabismus and, as a consequence, double vision (diplopia).

Synkinesis is a neurological symptom in which a voluntary muscle movement causes the simultaneous involuntary contraction of other muscles. An example might be smiling inducing an involuntary contraction of the eye muscles, causing a person to squint when smiling. Facial and extraocular muscles are affected most often; in rare cases, a person's hands might perform mirror movements.

External pterygoid can refer to:

Bienfang's test is a clinical test used in the diagnosis of ocular myasthenia gravis. It is used in conjunction with other examination techniques such as Cogan's lid twitch test or enhancement of blepharoptosis from prolonged upward gaze. It is a simple, quick, and non-invasive test for ocular myasthenia gravis that can be performed not only by ophthalmologists or neurologists, but also by other physicians evaluating patients with ptosis, diplopia, or other symptoms of myasthenia gravis.

In ophthalmology, apraxia of lid opening (ALO) is an inability to initiate voluntary opening of the eyelid following a period of eyelid closure, with normal function at other times. Manual lifting of the eyelid often resolves the problem and the lid is able to stay open.

<span class="mw-page-title-main">Roots of the ciliary ganglion</span>

The ciliary ganglion is a parasympathetic ganglion located just behind the eye in the posterior orbit. Three types of axons enter the ciliary ganglion but only the preganglionic parasympathetic axons synapse there. The entering axons are arranged into three roots of the ciliary ganglion, which join enter the posterior surface of the ganglion.

References

  1. Yamada K, Hunter DG, Andrews C, Engle EC (September 2005). "A novel KIF21A mutation in a patient with congenital fibrosis of the extraocular muscles and Marcus Gunn jaw-winking phenomenon". Arch. Ophthalmol. 123 (9): 1254–9. doi:10.1001/archopht.123.9.1254. PMID   16157808.[ permanent dead link ]
  2. Cates CA, Tyers AG (2008). "Results of levator excision followed by fascia lata brow suspension in patients with congenital and jaw-winking ptosis". Orbit. 27 (2): 83–9. doi:10.1080/01676830701376072. PMID   18415867. S2CID   23268883.
  3. Gunn RM. Congenital ptosis with peculiar associated movements of the affected lid. Trans Ophthal Soc UK. 1883;3:283-7.
  4. Ibrahim HA (2007). "Use of the levator muscle as a frontalis sling". Ophthal Plast Reconstr Surg. 23 (5): 376–80. doi:10.1097/IOP.0b013e3181379e20. PMID   17881988. S2CID   13655363.
  5. Prakash MV, Radhakrishnan M, Yogeshwari A, Nazir W, Maragatham K, Natarajan K (June 2002). "Inverse Marcus Gunn phenomenon". Indian J Ophthalmol. 50 (2): 142–4. PMID   12194575.