Eye Bobbing Associated with Jaw Movement CAROLYN S. OESTERLE, MD,* WILLIAM J. FAULKNER, MD,t RICHARD CLAY, MD,t EUGENE R. FOLK, MD,* NEIL LUCCHESE, MD,* ALLEN M. PUTTERMAN, MD*
Abstract: Two patients were treated for a previously unreported disorder believed to be related to Marcus Gunn jaw-winking. The first, a 9-month-old infant with left congenital blepharoptosis without jawwinking and with normal ocular motility had an up and down movement of the left globe synchronous with nursing movements of the jaw. The second was a 5-year-old girl with left blepharoptosis, jaw-winking, and left double elevator palsy who had up and down movements of both the left upper lid and the left globe synchronous with chewing. The left globe movements were most prominent in the field of vertical action of the superior rectus muscle and persisted after levator excision and fascia lata sling procedures. The possible etiology of the Marcus Gunn jawwinking phenomenon is discussed and related to our cases of eye bobbing. The eye bobbing probably is caused by abnormal innervation to the superior rectus muscle produced by jaw movements in a manner analogous to the abnormal stimulation of the levator muscle in jawwinking. The similar embryologic development and innervation of the levator and superior rectus muscles add credence to this theory. [Key words: blepharoptosis, double elevator palsy, eye bobbing, jawwinking.] Ophthalmology 89:63-67, 1982
Jaw-winking was first described by Marcus Gunn in 1883. 1 In this phenomenon, which is usually associated with ptosis ofthe affected lid, the upper eyelid elevates involuntarily with movements of the jaw. Ocular motility is usually normal. At times the Marcus Gunn phenomenon has been associated with paralysis of the superior rectus muscle 2 and double elevator palsy3 and with esotropia,4-6 ipsilateral external ophthalmoplegia,3
and Duane's syndrome in isolated cases.
motion of the affected eye that occurred with nursing. In the second case, jaw-winking was associated with ptosis, double elevator palsy, esotropia, and an elevation of the globe produced involuntarily when the patient closed her mouth. To our knowledge, no similar cases have been reported.
We treated two patients with an atypical type of jaw movement phenomenon. In the first case, ptosis without jaw-winking was accompanied by an up and down
From the Departments of Ophthalmology, University of Illinois Hospital Eye and Ear Infirmary, Chicago,* and University of Oklahoma, Oklahoma City. t Supported in part by grants EY 7038 and EY 1792 from the National Institutes of Health, Bethesda, Maryland.
Reprint requests to Carolyn S. Oesterle, MD, 1600 Lake Avenue, Wilmette, IL 60091.
Case 1. A 9-month-old white female infant was the 2440-gm product of a pregnancy complicated by a mild toxemia that otherwise was unremarkable. Labor was induced at eight months and delivery was uncomplicated. Blepharoptosis of the left eyelid was present at birth, and she often tilted her head backwards. On nursing, the mother noted a bobbing of the left eye. The infant was otherwise normal in growth and development. Two older siblings were reported as normal.
The infant followed objects with either eye and had normal extraocular motility and pupillary reaction. Hand light and dilated fundus examinations were normal. Palpebral fissures
63
OPHTHALMOLOGY • JANUARY 1982 • VOLUME 89 • NUMBER 1
measured 8 to 9 mm on the right and 3 to 4 mm on the left. She preferred a backward head tilt for fixating. Atropine refraction was +0.50 +2.00 x 45° in the right eye and plano +2.00 x 90° in the left eye. The motion of the left globe while nursing consisted of a regular up and down movement that occurred with the same apparent frequency as the infant's sucking. Each suck was accompanied by an upward jerk of the eye. After a pause, the eye returned to its previous position. The ptotic left lid was unaffected by nursing.
Case 2. A 6-year-old white girl was referred to the Illinois Eye and Ear Infirmary motility clinic in July 1978. Blepharoptosis of the left eyelid with jaw-winking was present since birth; a left esotropia was first noticed at 2 years of age. Her previous medical history was unremarkable. Family history disclosed that the mother also had esotropia as a child that resolved without treatment. An uncle was reported to have ptosis and an aunt was reported to have ptosis and esotropia.
Ocular examination of the girl disclosed a visual acuity of 20/30 in the right eye and 20/60 in the left eye. Refraction was + 1.50 +0.75 x 90° in the right eye and +0.75 + 1.75 x 90° in the left eye. Her palpebral fissures measured 8 mm in the right eye and 2 mm in the left eye , with a levator function of 12 mm in the right eye and 2 to 3 mm in the left eye (Fig I) . Margin reflex distance -I was 2.5 mm on the right and -3.5 on the left. Motility examination (Fig 2) revealed 7° of left
hypotropia at 6 M ; and 7° of esotropia and 10° of left hypotropia at 1/3 M; an A-pattern deviation with 16° of esotropia in upgaze and orthophoria in downgaze. Double elevator palsy of the left eye was manifested by limited upgaze in both adduction and abduction. Chewing produced vertical movement of both the left upper lid and the left globe; opening the mouth was associated with raising of the lid and eye; closing the mouth lowered the lid and eye. This vertical movement of the left eye and upper lid was most prominent with levoversion. Slit-lamp and fundus examination results were unremarkable.
In September 1978, bilateral 30-mm levator excision and bilateral fascia lata sling procedures were performed (Fig 3). After operation, the left eye amblyopia increased to 20/200 but improved to 20/80 with patching; the motility examination remained unchanged (Fig 4) except for reduction of the vertical movements of the left globe associated with chewing.
DISCUSSION
To our knowledge, this is the first report of elevation and depression of the globe in association with jaw movements. We believe this phenomenon
. Fig 1. A, left, preoperative photograph of patient in second case with left ptosis, left esotropia, and left hypertropia. B, right, improvement of left ptosis with closure of mouth during chewing. Left globe also elevates with closure of mouth.
64
OESTERLE, et al • EYE BOBBING
Fig 2. Patient in second case shows left ptosis and limited elevation of left eye before operation. Esotropia is greater on upgaze.
is related to the more common Marcus Gunn jawwinking phenomenon.
The Marcus Gunn jaw-winking phenomenon is usually congenital and often improves with age. 8 However, some cases are apparently acquired. 2,3 Jawwinking may be inherited in an irregular autosomal dominant pattern. 3 Most cases are unilateral, but bilateral cases have been reported. 9 The left upper lid is affected more frequently than the right. 2 In both of our cases of eye bobbing, the left eye was involved.
Walsh and Hoyt lO state that the upward jerk of the upper lid usually occurs simultaneously with contraction of the ipsilateral external pterygoid muscle, which is produced when the mandible is moved to the oppo-
Fig 3. Patient in second case after ptosis surgery.
site side or the jaw is opened. However, the jawwinking can be associated with contraction of the internal pterygoid when closing the jaw, clenching the teeth, or protruding the tongue. 3 This paper reports upward movement of the globe on opening the mouth in case 1 and, in case 2, upward movement of both the lid and globe on closing the jaw.
The ocular movement in our two patients is probably a variant of the Marcus Gunn jaw-winking phenomenon. Marcus Gunn attributed the original case to abnormal innervation; the levator muscle receiving innervation from both the nucleus of the 3rd cranial nerve and the motor nucleus of the 5th cranial nerve, which also innervates the external pterygoid muscle. l
Spaeth 11 offers a different innervational theory, noting that the motor nuclei of the 3rd and 5th cranial nerves appear at a similar embryologic period, antedating the appearance of the sensory root of the 5th cranial nerve. He envisions a reflex arc from the motor division of the 5th cranial nerve to the trigeminal ganglion, continuing by intraneuronal connections to the oculomotor nucleus and on to the levator muscle.
A different theory proposed by Wartenberg 12•l3 explains jaw-winking as a release phenomenon. Movements of the lid occur in lower animals with opening of the mouth, but phylogenetically and ontogenetically, these associated movements become inhibited by newer supranuclear connections. A lesion in the supranuclear pathways could result in jaw-winking by release of the more recently acquired inhibition or even cause hyperexcitability of the denervated structure. l4 Sano iS offers evidence in support of Warten-
65
OPHTHALMOLOGY • JANUARY 1982 • VOLUME 89 • NUMBER 1
Fig 4. Patient in second case showing double elevator palsy of left eye after operation.
berg ' s theory by demonstrating simultaneous electromyographic activity in the levator and the external pterygoid muscles among normal cases that is enhanced in jaw-winking.
None of the above theories explain why only the levator muscle is affected in Marcus Gunn jawwinking. The levator muscle develops embryologically from the superior rectus muscle anlage, and both are innervated by the superior division of the oculomotor nerve. In an 8-year-old boy with jaw-winking, FericSeiwerth et al. 16 demonstrated electromyographically that both the levator and the superior rectus muscles become active during stimulation of the jaw muscles. The most likely explanation for eye bobbing is abnormal stimulation of the superior rectus muscle in association with movements of the jaw. In case 1, the superior rectus muscle contraction was associated with typical jaw-winking; in the other the vertical movement of the globe associated with chewing was accentuated in the vertical field of action of the superior rectus muscle. The palsy of the superior rectus in case 2 substitutes for the ptosis usually associated with jaw-winking, How the lid surgery in case 2 improved the eye bobbing is not understood.
The cause of the phenomenon observed in our cases remains as speculative as the explanation for Marcus Gunn jaw-winking. Since jaw-winking occurs in 2 to 13% of all congenital ptosis and because of the close
66
embryologic and anatomic association of the levator and the superior rectus muscles, it is surprising that eye bobbing is not reported more frequently.
REFERENCES
1. Gunn MR. Congenital ptosis with peculiar associated movements of the affected lid . Trans Ophthalmol Soc UK 1883; 3283-7.
2. Lutz A. The jaw winking phenomenon and its explanation. Arch Ophthalmol 1919; 48144-58.
3. Duke-Elder S, ed. System of Ophthalmology. Vol. 3, pt 2, Normal and Abnormal Development: Congenital Abnormalities. St Loui s: CV Mosby, 1963; 900 - 6.
5. Garkal RS. Marcus Gunn phenomenon associated with synkinetic oculo-palpebral movements. Br J Ophthalmol 1961; 45566- 7.
6. Mosavy SH, Horiat M. Marcus Gunn phenomenon associated with synkinetic oculopalpebral movements. Br Med J 1976; 2:675-6.
7. Takeuchi M, Maruo 1. Duane's retraction syndrome associated with the Marcus Gunn phenomenon. Jpn J Clin Ophthalmol 1969; 231189-93.
8. Iliff CEo The optimum time for surgery in the Marcus Gunn phenomenon. Trans Am Acad Ophthalmol Otolaryngol 1970; 741005-10.
OESTERLE, et al • EYE BOBBING
9. Musson KH, Newton CA. Bilateral Marcus Gunn phenomenon; report of a case. J Pediatr Ophthalmol 1970; 7:14-6.
10. Walsh FB, Hoyt WF, eds. Clinical Neuro-ophthalmology, 3rd ed. Baltimore: Williams and Wilkins, 1969; 3, 309-18.
11. Spaeth EB. The Marcus Gunn phenomenon; discussion, presentation of four instances and consideration of its surgical correction. Am J Ophthalmol 1947; 30:143-58.
12. Wartenberg R. Winking-jaw phenomenon. Arch Neurol Psychiat 1948; 59734-53.
13. Wartenberg R. Associated movements in the oculomotor and facial muscles. Arch Neural Psychiat 1946; 55:439-88.
14. Richwien R. Zur Pathophysiologie des Marcus-GunnPhiinomens. Klin Monatsbl Augenheilkd 1970; 157:365-74.
15. Sano K. Trigemino-oculomotor synkineses. Neurol Med Chir (Tokyo) 1959; 129-51.
16. Ferie-Seiwerth F, Celie M, Domljan Z. Marcus-Gunn-syndrom. Klin Monatsbl Augenheilkd 1969; 154:519-24.
