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ARTICLE
Year : 1966  |  Volume : 14  |  Issue : 5  |  Page : 217-222

Marcus gunn phenomenon


Medical College, Nagpur, India

Date of Web Publication17-Jan-2008

Correspondence Address:
A Naidu
Medical College, Nagpur
India
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How to cite this article:
Naidu A, Khandelwal M K. Marcus gunn phenomenon. Indian J Ophthalmol 1966;14:217-22

How to cite this URL:
Naidu A, Khandelwal M K. Marcus gunn phenomenon. Indian J Ophthalmol [serial online] 1966 [cited 2021 Oct 19];14:217-22. Available from: https://www.ijo.in/text.asp?1966/14/5/217/38658

Marcus Gunn phenomenon com­prises a synkinetic, involuntary eleva­tion of a congenitally ptotic eyelid on opening the mouth. The typical pheno­menon is congenital, unilateral and associated with ptosis only, but a re­view of literature reveals exceptions to each of these generalities (Duke-Elder, 1952). The first case was reported by Marcus Gunn in 1883. Since then only about a hundred and odd cases have been reported. From this coun­try the only published reports are those of Patel (1951), Krishnaswamy (1954), Prabhu et al (1958) and Bhargava and Choubey (1960). Five more cases of Marcus Gunn phenomenon are being reported here, three being typical and two showing slight variations from the classical description.

CASE No. 1: A six year old boy came for acute pharyngitis. It was then dis­covered that he had partial ptosis of the right eye since birth [Figure - 1] and also that the ptotic eyelid used to "shoot" upwards beyond the corneal margin whenever he opened his mouth wide [Figure - 2] and also on chewing. There was no family history of a simi­lar complaint. Movements of the eye­ball were normal. The pupillary re­action to light and accommodation were normal. There was no evidence of Horner's syndrome. Voluntarily the child could not elevate the right upper eyelid. Vision was normal in both eyes. Systemic examination revealed nothing abnormal except for the sore throat for which the patient had come. Fundoscopy revealed nothing abnor­mal,

CASE No. 2: A young girl aged eighteen years came for secondary amenorrhea. Incidentally it was dis­covered that she had partial ptosis of the right eyelid [Figure - 3]. This condi­tion was present since birth and there was no history of a similar complaint in any other member of the family. On being asked to open the mouth volun­tarily there was no change in the degree of ptosis, but on closer observation it was detected that from time to time especially when she became animated and spoke aloud, the ptotic eyelid would bat upwards beyond the corneal margin momentarily and then return to its position of rest. Baring the teeth. had a similar effect but to a much lesser extent. The degree of ptosis though slight varied from time to time.

One peculiar phenomenon noted in this case was an asymmetrical see-saw movement between the two lids i.e. whenever the right eye-lid drooped ex­cessively the left upper eyelid went up slightly beyond the position of rest, and whenever the right eyelid moved excessively upwards the left eyelid would droop slightly. Besides this there was infrequent blinking of the right eye and whenever the patient looked downwards and more so on looking downwards and inwards there was slight retraction of the ptotic eyelid -Pseudo Grafe sign [Figure - 4]. There was no evidence of weakness of any of the external ocular muscles. The pupils were normal. Systemic ex­amination revealed nothing abnormal. The fundi were normal and blood tests for Kahn's reaction and V.D.R.L. were negative.

CASE No. 3: A young girl aged ten years was brought for involuntary movements and excessive volubility. She was a full term normal delivered child, but there was a history of pro­longed labour, and she did not cry not about fifteen minutes after birth. At the age of two months the mother noticed that the child had squint. All the milestones of development were delayed. At the age of four years it was noticed that she had involuntary movements which were generalised, non-repetitive, non-purposeful and jerky. The severity and type of invo­luntary movements were subject to variation though at a particular time one specific type of involuntary move­ment predominated over the others. They were absent during sleep. An­other important complaint was, that for the last four years the patient was talking excessively and hyperkinetic all the time. There was no history sug­gestive of rheumatic arthritis in the past. There was no family history of similar complaints, nor history sugges­tive of syphilis in the mother.

The patient was fairly intelligent, extremely talkative and hyper-excit­able. She had concommitant internal squint of the left eye [Figure - 5] and in­voluntary movements of the chorei­form type. Besides, she had one pecu­liar movement in the form of involun­tary opening of the mouth with pro­trusion of the jaw to the right and downwards accompanied by bilateral retraction of both upper eye lids last­ing for about a second [Figure - 6]. This did not occur on voluntarily opening the mouth. There was no ptosis. Movements of the eyeballs were nor­mal. Pupils reacted normally to light and accommodation. There was no hypotonia. Systemic examination re­vealed nothing abnormal. Ocular fundi were normal.

CASE No. 4: M.S,, a fifty year old Hindu male sought admission for cirr­hosis of liver. During routine examina­tion ptosis of the right eye was noticed [Figure - 7]. On being asked to open the mouth typical Marcus Gunn pheno­menon was observed [Figure - 6]. Other movements of the jaw like protrusion and side to side movements did not affect the ptosis. Pupillary reactions and movements of the eyeball were normal. Systemic examination reveal­ed pallor, oedema over the feet, as­cities and an enlarged firm liver. Vision and eye grounds were normal. Blood K.T. and V.D.R.L. tests were nega­tive.

CASE No. 5: S.T., a sixteen year old school boy, attended the hospital for upper respiratory infection. Besides congestion of the throat, the patient showed typical Marcus Gunn pheno­menon on the right side viz. slight ptosis of the right eyelid which used to be corrected only momentarily on opening the mouth. There was no paresis of the ocular muscles. Eye grounds and pupils were normal. Sero­logical tests for syphilis were negative.


  Discussion Top


Case Nos. 1, 4 and 5 had typical Marcus Gunn phenomenon in that they had congenital ptosis with in­voluntary elevation of the ptotic eyelid on opening the mouth. Lateral devia­tion of the jaw did not bring about any significant change in the degree of ptosis. However, in case No. 1 and case No. 4 the elevation of the eyelid was a sustained one, and the ptotic eye­lid used to go up more than the normal lid, whereas in case No. 5 the eleva­tion of the lid was momentary and not more than the normal lid.

The second case is an atypical case for here the ptotic eyelid went up only when the patient spoke aloud and became excited, ordinary opening of the mouth without speaking having no effect on the ptosis. This patient also had associated pseudo-Grafe pheno­menon which has been reported by Bielchowsky (1932) and Manzitti et al (1948). This is usually found in cases recovering from third nerve palsy, but in this case it was congenital. One more interesting and uncommon finding in this patient was the presence of occa­sional see-saw movement of the two eyes which was more marked when the patient became animated. Spaeth (1947) described a similar case.

The third case is also an atypical case of Marcus Gunn phenomenon for, there was no ptosis at all and there was bilateral retraction of both the eyelids and it was always the involun­tary opening of the mouth with the protrusion of the jaw to the right side which led to excessive retraction of both eyelids, but not voluntary opening of the mouth. Bilateral retraction of the lids in Marcus Gunn phenomenon is extremely rare. Two cases have been reported so far by Marin Amat (1930) and Beaumont (1893).

It is uncommon for cases of Marcus Gunn syndrome to have no ptosis at all. But cases without ptosis have been described (Sinclair, 1895). None of these cases had any weakness of the external ocular muscles, the one most commonly associated with Marcus Gunn phenomenon, being superior rec­tus palsy. The one feature that is com­mon to all the cases is levator­pterygoid synkinesis.

Marcus Gunn phenomenon is usual­ly a congenital condition, but it may set in later life. From a review of 1.01 cases Grant (1936) concluded that it was more common in males and af­fected the left eye more than the right. It constitutes 2 per cent of all cases of congenital ptosis. A familial tendency has been noted in some, the heredity having the pattern of an irregular do­minant (Krause, Cotterman and Falls, 1949). All degrees of ptosis are pre­sent and in some ptosis may be entirely absent (Duke-Elder, 1952). The com­monest movement precipitating eleva­tion of the ptotic eyelid is opening the mouth, but other movements described are manifold namely closing the mouth, chewing, sucking, clenching the teeth, deep respiration, protrusion of the jaw and lateral movement of the jaw, emotional movements of face etc. Protrusion of the jaw to the opposite side leads to maximal retraction of the lid (Cooper, 1937).

Various hypotheses have been ad­vanced to explain this synkinetic movement. The most commonly ac­cepted one is that there is an abnor­mal nervous connection between the nerve supply of the levator and the pterygoids. The exact level of this connection is disputed: According to some it is (1) supranuclear (Lutz, 1919); (2) intranuciear (Buschke. (3) infranuclear (Gowers)--some of the fibres of the third nerve arising from the external pterygoid part of the fifth nucleus; (4) Marin Amat postulated that some anomalous fibres from the fifth nerve nucleus travel to the leva­tor via the auriculo temporal nerve: (5) Spaeth suggested a faulty distribu­tion of fibres in the posterior longitudi­nal bundle: (6) Another school of thought is that anatomical explanations do not explain the acquired cases and according to them it is due to an altera­tion of the functional relationship be­tween cranial nerves (Menoche): (7) Lewy, Grant and Groff consider it to he due to a proprioceptive reflex arc, the afferent path being the sensory fibres of the trigeminal nerve and the efferent path the autonomic fibres along the ophthalmic division of the fifth nerve.

Contrary to all these views is that of Bishop Harman (1903) who con­siders Marcus Gunn phenomenon to be an atavistic reversion to the relation­ship which exists between the mouth and gills found in fishes. When the mouth is opened the operculum opens and the spiracle dilates, the deep muscles contracting and the super­ficial relaxing, which are represented in man by the external pterygoid and orbicularis oculi respectively. In the presence of ptosis the weak levator can lift the lid only when its antagonist the orbicularis oculi is reflexely relaxed.


  Summary Top


Five cases of Marcus Gunn pheno­menon have been reported. Case Nos. 1, 4 and 5 were typical cases of Marcus Gunn phenomenon, in that they had partial ptosis of one eyelid and the ptotic eyelid used to shoot up, on opening the mouth. The other two cases were variants of the typical.

Case No. 2 showed retraction of the ptotic eyelid on animation and speak­ing aloud, and also on baring the teeth. It also showed pseudo-graefe phenomenon.

Case No. 3 had no ptosis at all, but showed bilateral retraction of the eye­lids and movement of the lower jaw downwards and to the right on involun­tary opening of the mouth.

A discussion involves the etiological aspects and comparison with previous­ly recorded cases.[21]

 
  References Top

1.
Beaumont: Quoted by Ref. No. 6.  Back to cited text no. 1
    
2.
Bhargava, H. S. and Choubey. B. S. (1960): Current Medl. Practice, 4: 297.  Back to cited text no. 2
    
3.
Bielchowsky (1932): Quoted by Ref. No. 6.  Back to cited text no. 3
    
4.
Buschke: Quoted by Ref. No. 6.  Back to cited text no. 4
    
5.
Cooper, E. L. (1937): Arch. of Ophthal. 18: 198.  Back to cited text no. 5
    
6.
Duke-Elder (1952): Textbook of Oph­thalmology Vol. V. p. 4686.  Back to cited text no. 6
    
7.
Gowers: quoted by Ref. No. 6.  Back to cited text no. 7
    
8.
Grant, F. C. (1936): Arch. Neurol. Psychiat. 35: 487.  Back to cited text no. 8
    
9.
Harman, N. B. (1903): Quoted by Ref. No. 11.  Back to cited text no. 9
    
10.
Krause Cotterman and Falls (1949): Quoted by Ref. No. 6.  Back to cited text no. 10
    
11.
Krishnaswamy, K. V. (1954): Neurol. 1: 45.  Back to cited text no. 11
    
12.
Lewy, F. H.: Grant, F. C. and Groff, R. A. (1937): Arch. Neural. Psychiat. 37: 1289.  Back to cited text no. 12
    
13.
Lutz (1919): Quoted by Ref. No. 6.   Back to cited text no. 13
    
14.
Manzitti: Quoted Ref. No. 6.  Back to cited text no. 14
    
15.
Marcus Gunn (1883): Quoted by Ref. No. 6.  Back to cited text no. 15
    
16.
Marin Amat: Quoted by Ref. No. 6.   Back to cited text no. 16
    
17.
Menoche: Quoted by Ref. No. 6.  Back to cited text no. 17
    
18.
Patel, V. P. (1950): Jour. Ind. Med. Assn. 19: 371.  Back to cited text no. 18
    
19.
Prabhu, M. C. and Rajan, G. (1958): Neurol. 6: 15.  Back to cited text no. 19
    
20.
Sinclair (1895): Quoted by Ref. No. 6  Back to cited text no. 20
    
21.
Spaeth, E. B. (1947): Am. Jour. Ophthal. 30: 143.  Back to cited text no. 21
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]



 

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