Marcus Gunn phenomenon

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Marcus Gunn phenomenon (a.k.a. Marcus Gunn Jaw-Winking or Trigemino-oculomotor Synkineses) is an autosomal-dominant condition with incomplete penetrance, in which nursing infants will have rhythmic upward jerking of their ptotic eyelid. This condition is characterized as a synkinesis: when two or more muscles that are independently innervated have either simultaneous or coordinated movements.

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, 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%).

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.

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).

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

  • Jaw thrust to opposite side (homolateral external pterygoid)
  • Jaw is projected forward (bilateral external pterygoid)
  • Mouth is opened widely

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

External pterygoid-levator synkinesis is the more common group.

Treatment

Treatment is usually unnecessary but in severe cases, surgery with a bilateral levator excision and frontalis brow suspension may be used.

Inverse Marcus Gunn Phenomenon

Inverse Marcus Gunn phenomenon is a rare condition 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.

References

  • Sano K: Trigemino-oculomotor synkinesis. Neurologia 1:29, 1959
  • Glaser J, Neuro-ophthalmology. 3rd Ed. Philadelphia: Lippincott Williams & Wilkins 1999; 474-475.
  • Ophthalmology Secrets 2nd Edition. Vander JF, Gault JA; Hanley & Belfus, Inc., 2002. ISBN 1-56053-429-X

External links

de:Marcus-Gunn-Syndrom



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