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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 26  |  Issue : 1  |  Page : 92-94

Trigemino-oculomotor synkinesis: Report of a rare case


1 Department of Oral Medicine and Radiology, Manubhai Patel Dental College of Bhavnagar University, Vadodara, Gujarat, India
2 Department of Physiology, Gujarat Medical Education and Research Society (GMERS) Medical College,Vadodara, Gujarat, India

Date of Submission13-Aug-2014
Date of Acceptance22-Aug-2014
Date of Web Publication26-Sep-2014

Correspondence Address:
Manjiri Joshi
Department of Oral Medicine and Radiology, Manubhai Patel Dental College of Bhavnagar University and Sir Seewoosagur Ramgoolam Hospital (SSR), Vadodara - 390 011, Gujarat
India
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Source of Support: None, Conflict of Interest: None


Read associated Erratum: Erratum with this article

DOI: 10.4103/0972-1363.141870

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   Abstract 

Trigeminal-oculomotor synkinesis also known as the Marcus Gunn jaw winking phenomenon was first described by the ophthalmologist, Marcus Gunn, in 1883. It is one of the common congenital oculofacial synkinesis, and accounts for approximately 5% of all congenital ptosis. It is characterized by the involuntary winking of eyes during jaw movement, due to an aberrant connection between the mandibular division of the trigeminal nerve and one of the divisions of the occulomotor nerve. Here we report an unusual case of the Marcus Gunn jaw winking phenomenon, which was diagnosed accidentally.

Keywords: Jaw winking, Marcus Gunn phenomenon, ptosis, synkinesis


How to cite this article:
Joshi M, Tailor M, Ghugare B. Trigemino-oculomotor synkinesis: Report of a rare case . J Indian Acad Oral Med Radiol 2014;26:92-4

How to cite this URL:
Joshi M, Tailor M, Ghugare B. Trigemino-oculomotor synkinesis: Report of a rare case . J Indian Acad Oral Med Radiol [serial online] 2014 [cited 2020 May 27];26:92-4. Available from: http://www.jiaomr.in/text.asp?2014/26/1/92/141870


   Introduction Top


Ocular aberrant synkinesis syndromes are a complex group of disorders involving an abnormal connection of cranial nerves to the extraocular and jaw muscles. The Marcus Gunn jaw-winking phenomenon (MGJWP) is a rare synkinetic syndrome characterized by the retraction of the ptotic eyelid with jaw movements. [1],[2] In 1883, Robert Marcus Gunn first described this syndrome in a 15-year-old girl, with a peculiar type of congenital ptosis that included an associated winking motion of the affected eyelid on the movement of the jaw. [3] This phenomenon is also known as trigemino-oculomotor synkinesis, jaw winking syndrome, and pterygoid-levator synkinesis. The term, synkinesis refers to a simultaneous movement or a coordinated sequence of movements of muscles, which are supplied by different nerves or by separate peripheral branches of the same nerve. The MGJWP and Duane retraction syndrome are infrequent synkineses characterized by the interconnection between different cranial nerves. [4] It is usually associated with congenital blepharoptosis, with an incidence of 4-6%. [5],[6],[7] Acquired forms have been described after eye surgery, trauma, post Bell's palsy, and pontine tumors. [5] Spontaneous remission of the acquired form have been reported, whereas, the congenital form persists with no improvement. [8] Here we discuss a case of the Marcus Gunn jaw winking phenomenon, which was diagnosed in the Department of Oral Medicine as an accidental finding during an oral examination.


   Case Report Top


A 65-year-old male reported to the Department of Oral Medicine with a chief complaint of deposits and stains over teeth. Oral examination revealed regressive alterations of teeth and pseudo-partial anodontia. When performing an extraoral examination, we noted abnormal movements of the right upper eyelid with respect to jaw movements. We elicited the past history of patient, which revealed that he had this problem since birth. The extraoral findings revealed ptosis of the right upper eyelid and elevation of the concomitant upper eyelid with mouth opening [Figure 1] and [Figure 2]. The pupils were normal in both eyes and reacting well to light. No other neurological or congenital abnormality was noted in the patient or in the family. The systemic history revealed nothing significant.
Figure 1: Ptotic eyelid when jaw is closed

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Figure 2: Elevation of concomitant ptotic eyelid on jaw opening

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   Discussion Top


The MGJWP, characterized by involuntary winking of the eyes during chewing, is believed to be due to an aberrant connection between the mandibular division of the trigeminal nerve (innervating the external pterygoid muscle) and one of the divisions of the occulomotor nerve (innervating the levator palpebralis superioris of the upper eyelid). [9] From the reported literature it can be seen that males are affected more than females and the left upper eyelid is involved more frequently than the right. [10]

Etiology and pathophysiology

The Marcus Gunn phenomenon (MGP) is often first observed in the infant by the mother during breastfeeding, when the eye movements are noted, associated with sucking activity. The elevation of the upper eyelid may be stimulated by a variety of movements, including mandibular movements, tongue movements, chewing, speaking, sucking, swallowing, smiling, and whistling. [11] The MGJWP have been excellently reviewed by Brooks, Del-Core et al., Pratt et al. and Simpson.

There are various hypothesis put forth to describe the phenomenon: [12],[13],[14]

  1. An abnormal nervous connection, which is the most favored hypothesis, may be cortical, subcortical or internuclear. There might be faulty distribution in the posterior longitudinal bundle and infranuclear fibers. Some fibers of the third nerve may arise from the fifth nucleus or some fibers of the fifth nerve may reach the levator palpebrae via the auriculotemporal branch. Some believe that it may be due to double innervation of the levator, that is, by the third and fifth nerves.
  2. Functional interference - Duke Elder (1952) explained it on a physiological basis and postulated that it may be due to any of the following:
    1. Irritation of the normally dormant connection
    2. Release of inhibitions
    3. Spread of impulses by radiation.
  3. Atavistic reversion - In fish there is a strong associated movement of jaw opening and eye opening, that is, deep muscle contraction and superficial muscle relaxation. Thus, a weak levator may only elevate the lid when the orbicularis is relaxed by jaw opening.


This hypothesis is supported by synkinesis that has been observed during electromyographic studies. [12] Hence, it is also known as trigemino-oculomotor synkinesis. Also, it has been hypothesized that the release of phylogenetically older neural mechanisms, due to a lesion in the brainstem, may be responsible for the occurrence of this trigemino-ocular synkinesis, as seen with other synkinetic movements like the palmomental and primitive grasp feeding reflexes. Evidence favoring this is the co-firing of the occulomotor innervated extraocular muscles along with the muscles of mastication (innervated by the trigeminal nerve) on electromyography (EMG) in normal individuals. [9],[15]

It was postulated earlier in literature that injury to the trigeminal and occulomotor nerves, due to poorly controlled diabetes of a long duration and a subsequent recovery, may lead to an aberrant connection between the mandibular division of the trigeminal nerve and occulomotor nerve, leading to MGJWS. [9]


   Conclusion Top


Establishing a diagnosis of the Marcus Gunn phenomenon (MGP) is within the domain of the oral and maxillofacial diagnostician. Therefore, a comprehensive medical evaluation of the patient and a detailed history are mandatory. Apart from this, one must have sound knowledge about the various medical conditions and diseases that are encountered in routine dental practice.


   Acknowledgment Top


The authors express deep gratitude and acknowledge Dr. Rajendrasinh Rathod, Dean, Manubhai Patel Dental College for his cooperation and support.

 
   References Top

1.
Siatkowski RM, Glaser JS. Pediatric neuro-ophthalmology: General considerations and congenital motor and sensory anomalies. In: Glaser JS, editor. Neuro-Ophthalmology. Philadelphia: Lippincott Williams & Wilkins; 1999. p. 461-88.  Back to cited text no. 1
    
2.
Angmo D, Bajaj MS, Pushker N, Ghose S. Marcus Gunn jaw-winking phenomenon: A review. Delhi Journal of Ophthalmology 2011;21:19-22.  Back to cited text no. 2
    
3.
Gunn RM. Congenital ptosis with peculiar associated movements of the affected lid. Trans Ophthal Soc UK 1883;3:283-7.  Back to cited text no. 3
    
4.
Balaban H, Yildiz OK, Eliaçik S, Bolayir E, Topaktaº S. A case with a rare type of trigemino-occulomotor synkinesis: Clinical and blink reflex study. Acta Neurol Belg 2010;110:337-9.  Back to cited text no. 4
    
5.
Demirci H, Frueh BR, Nelson CC. Marcus Gunn jaw-winking synkinesis: Clinical features and management. Ophthalmology 2010;117:1447-52.  Back to cited text no. 5
    
6.
Park DH, Choi WS, Yoon SH. Treatment of the jaw-winking syndrome. Ann Plast Surg 2008;60:404-9.  Back to cited text no. 6
    
7.
Khwarg SI, Tarbet KJ, Dortzbach RK, Lucarelli MJ. Management of moderate-to-severe Marcus Gunn jaw-winking ptosis. Ophthalmology 1999;106:1191-6.  Back to cited text no. 7
    
8.
Doucet TW, Crawford JS. The quantification, natural course, and surgical results in 57 eyes with Marcus Gunn (jaw-winking) syndrome. Am J Ophthalmol 1981;92:702-7.  Back to cited text no. 8
[PUBMED]    
9.
Dutta D, Maisnam I, Ghosh S, Mukhopadhyay P, Mukhopadhyay S, Chowdhury S. Marcus-Gunn jaw winking syndrome and gustatory sweating in long standing poorly controlled diabetes: A case report. Int J Clin Med 2012;3:40-2.  Back to cited text no. 9
    
10.
Rajendran R. Diseases of nerve and muscles. In: Rajendran R, Sivapathasundaram S, editors. Shafer's Textbook of Oral Pathology. 7 th ed. New Delhi: Elsevier India; 2012. p. 863.  Back to cited text no. 10
    
11.
Brooks JK. The Marcus Gunn phenomenon. Discussion and report of a case. Oral Surg Oral Med Oral Pathol 1987;64:687-92.  Back to cited text no. 11
[PUBMED]    
12.
Duke ES. Normal and abnormal development: Congenital deformities. In: Duke ES, editor. System of Ophthalmology. Vol. 3. St. Louis: CV Mosby; 1963. p. 900-5.  Back to cited text no. 12
    
13.
Bowyer JD, Sullivan TJ. Management of Marcus Gunn jaw winking synkinesis. Ophthal Plast Reconstr Surg 2004;20:92-8.  Back to cited text no. 13
    
14.
Shukla SP. Marcus Gunn phenomenon. J All India Ophthalmol Soc 1966;14:223-4.  Back to cited text no. 14
[PUBMED]    
15.
Sano K. Trigemino-oculomotorsynkinesis. Neurologica 1959;1:29-51.  Back to cited text no. 15
    


    Figures

  [Figure 1], [Figure 2]



 

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