Marcus Gunn syndrome | Overview
Marcus Gunn Jaw Winking Syndrome consists of elevation or depression of the eyelid on chewing/suckling and may occur either in one or both eyes, with or without congenital ptosis. It is hypothesized that axons, intended to travel within the motor branch of the trigeminal nerve (Cranial Nerve V) to innervate the ipsilateral pterygoid muscle, aberrantly (deviating from the normal course) innervate myofibers of the Levator Palpebrae Superioris (LPS)-the muscle that elevates and retracts the upper eyelid, which is normally innervated by a branch of Cranial Nerve III.
Marcus Gunn Jaw Winking Syndrome can occur in association with complex strabismus conditions such as Duane syndrome and CFEOM1,2. The boy to the right has bilateral congenital ptosis, upgaze limitation and, prior to extrocular muscle surgery, exhibited the Marcus Gunn phenomenon.
The majority of cases of this disorder are reported to be sporadic (isolated), although there are a few reports in the scientific literature of familial Marcus Gunn syndrome3.
Online Mendelian Inheritance in Man (OMIM). Victor A. McKusick, Editor, Johns Hopkins University, last updated 11/6/1994 (entry number #154600) Home page: http://www3.ncbi.nlm.nih.gov/Omim/.
- Brodsky, M. Hereditary external ophthalmoplegia synergistic divergence, jaw winking, and oculocutaneous hypopigmentation: a congenital fibrosis syndrome caused by deficient innervation to extraocular muscles. Ophthalmology 105, 717-725 (1998).
- Abeloos, M.-C. et al. Fibrose Congenitale des muscles oculaires: un diagnostic pour plusieurs tableaux cliniques. Bull Soc Belge Ophtalmol 239, 61-74 (1990).
- Kirkham, T. H. Familial Marcus Gunn phenomenon. Brit. J. Ophthal. 53: 282-283, 1969.