|Year : 1966 | Volume
| Issue : 5 | Page : 217-222
Marcus gunn phenomenon
A Naidu, MK Khandelwal
Medical College, Nagpur, India
|Date of Web Publication||17-Jan-2008|
Medical College, Nagpur
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Naidu A, Khandelwal M K. Marcus gunn phenomenon. Indian J Ophthalmol 1966;14:217-22
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|| |
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|| |
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.
| References|| |
Beaumont: Quoted by Ref. No. 6.
Bhargava, H. S. and Choubey. B. S. (1960): Current Medl. Practice, 4: 297.
Bielchowsky (1932): Quoted by Ref. No. 6.
Buschke: Quoted by Ref. No. 6.
Cooper, E. L. (1937): Arch. of Ophthal. 18: 198.
Duke-Elder (1952): Textbook of Ophthalmology Vol. V. p. 4686.
Gowers: quoted by Ref. No. 6.
Grant, F. C. (1936): Arch. Neurol. Psychiat. 35: 487.
Harman, N. B. (1903): Quoted by Ref. No. 11.
Krause Cotterman and Falls (1949): Quoted by Ref. No. 6.
Krishnaswamy, K. V. (1954): Neurol. 1: 45.
Lewy, F. H.: Grant, F. C. and Groff, R. A. (1937): Arch. Neural. Psychiat. 37: 1289.
Lutz (1919): Quoted by Ref. No. 6.
Manzitti: Quoted Ref. No. 6.
Marcus Gunn (1883): Quoted by Ref. No. 6.
Marin Amat: Quoted by Ref. No. 6.
Menoche: Quoted by Ref. No. 6.
Patel, V. P. (1950): Jour. Ind. Med. Assn. 19: 371.
Prabhu, M. C. and Rajan, G. (1958): Neurol. 6: 15.
Sinclair (1895): Quoted by Ref. No. 6
Spaeth, E. B. (1947): Am. Jour. Ophthal. 30: 143.
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8]