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Marin-Amat Syndrome

A Jogiya and C Sandy¹ 

Department Of Ophthalmology, The Royal London Hospital, Whitechapel, London and ¹Department Of Ophthalmology, North Hampshire Hospital, Aldermaston Road, Basingstoke


Correspondence to: Dr Aryan Jogiya, Department Of Ophthalmology, The Royal London HospitalWhitechapelLondon E11BBEmail: aryanjogiya{at}aol.com

Accepted for publication: 1st March 2003

  Involuntary lid closure on jaw movement with Marin-Amat syndrome.
1) Patient was asked to repeatedly open and close her mouth. 
2) Patient was asked to repeatedly draw in her lips.
3) Patient was asked to repeatedly simulate the action of chewing.

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Background

Synkinesis is the presence of unintentional muscle activation in one area when intentionally performing movement in another area. Marin-Amat syndrome is a synkinesis which develops following facial nerve palsy and manifests as involuntary lid closure on jaw opening. The most commonly held theory is that it arises as a result of aberrant regeneration of the facial nerve.^1,2,3,4 We present a case of facial synkinesis in a patient recovering from Bell’s palsy. This manifests with involuntary narrowing of the ipsilateral palpebral fissure on volitional jaw movements.

  

Case Report

The patient presented 8 months following a left sided Bell’s palsy from which she had mostly recovered. Her initial presenting symptoms were those of blurred vision, and watering. She had a past history of sarcoidosis, migraine, ureteric calculus, thyrotoxicosis with thyroid eye disease treated with radioiodine rendering her hypothyroid. She was on thyroxine and had no known drug allergies.

Examination revealed left hemi-facial spasm and mild left facial palsy. She was reviewed 4 months later and she had noticed that the spasm worsened on opening her mouth or chewing. There was lid closure on opening her mouth or when she simulated chewing. She was offered Botulinum Toxintreatment⁵ for the spasm but declined in favour of more conservative measures.⁶ She underwent facial physiotherapy and at the last review her facial nerve function had improved but the involuntary lid closure was unchanged.

 

Discussion

The facial nucleus is somatotopically organized and this organization is continued from the facial nucleus through the facial nerve itself, and distally to the muscles. After facial nerve lesion, and regeneration, the topography of the facial nucleus is disturbed; and this has been illustrated in animal models. This proposed reorganization at the nuclear level would be accompanied by new synapses and, therefore result in the altered motor control that might be causal or at least contributory towards synkinesis.⁹

Several authorities have also suggested that synkinesis could also be a result of an increase in collateral branching. Other factors that proposed as contributing towards synkinesis are hyperexcitability of the facial nucleus following paresis and regeneration of the facial nerve. Ephaptic transmission is another mechanism implicated in synkinesis where neuroelectrical transmission can occur in the absence of anatomic coupling.^7,8,9

Although this condition has also been described by some as the inverse Marcus-Gunn phenomenon we agree with other authorities that the term inverse Marcus-Gunn phenomenon be reserved for a congenital lesion whose pathogenesis is quite different.² It has been suggested that the inverse Marcus-Gunn phenomenon is related to Wartenberg’s hypothesis of ‘release-phenomenon’ where a primitive reflex association between the deep and superficial facial muscles is re-established through failure of inhibition.¹²

 

References

1. Pavone, P; Garozzo, R; Trifiletti, R R; Parano, E. Marin-Amat syndrome: case report and review of the literature, Journal of Child Neurology, Volume 14, Issue 4, April 1999, Pages 266-268
2. Rana, P V; Wadia, R S. The Marin-Amat syndrome: an unusual facial synkinesia, Journal of Neurology, Neurosurgery, and Psychiatry, Volume 48, Issue 9, September 1985, Pages 939-941
3. Koike, M; Yoshioka, H. Case of Marin Amat syndrome, Ganka. Ophthalmology, Volume 11, Issue 3, March 1969, Pages 230-233
4. Kinoshita, M; Nakazato, H; Satoyoshi, E. A case of sarcoidosis with Marin-Amat syndrome (author's transl), [Zasshi] Journal. Nihon Naika Gakkai, Volume 65, Issue 12, December 10, 1976, Pages 1437-1440
5. Roggenkamper P, Laskawi R, Damenz W, Schroder M, Nussgens Z. .Orbicular synkinesis after facial paralysis: treatment with botulinum toxin.
   Doc Ophthalmol. 1994;86(4):395-402.
6. Brach JS, VanSwearingen JM, Lenert J, Johnson PC. Facial neuromuscular retraining for oral synkinesis.Plast Reconstr Surg. 1997 Jun;99(7):1922-31; discussion 1932-3.
7. Celik M, Forta H, Vural C. The development of synkinesis after facial nerve paralysis. Eur Neurol. 2000;43(3):147-51.
8. Moller AR. Symptoms and signs caused by neural plasticity.
   Neurol Res. 2001 Sep;23(6):565-72. Review.
9. Choi D, Raisman G. Somatotopic organization of the facial nucleus is disrupted after lesioning and regeneration of the facial nerve: the histological representation of synkinesis. Neurosurgery. 2002 Feb;50(2):355-62; discussion 362-3
10. S.R. Gifford. Paradoxic elevation of the lid. Arch Ophthalmol 22 (1939), pp. 252–256
11. Pavesi, G; Medici, D; Macaluso, G M; Ventura, P; Allegri, I; Gemignani, F
    Raverdy, P; Theron, H P; Souillard, C; Remy, A. Unusual synkinetic movements between facial muscles and respiration in hemifacial spasm, Movement Disorders: Official Journal of the Movement Disorder Society, Volume 9, Issue 4, July 1994, Pages 451-454
12. MVS Prakash, M Radhakrishnan, A Yogeshwari, W Nazir, K Maragatham, K Natarajan: Inverse Marcus Gunn Phenomenon. Indian J Ophthalmol 2002;50 142-144.