Melkersson-Rosenthal syndrome")">
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ARTICLES
Year : 1973  |  Volume : 21  |  Issue : 1  |  Page : 36-39

A case of acquired facial diplegia, macular oedema and lingua plicata ("Melkersson-Rosenthal syndrome")


Department of Ophthalmology, Pandit Jawaharlal Nehru Memorial Medical College, Raipur, India

Correspondence Address:
P K Mukherjee
Department of Ophthalmology, Pandit Jawaharlal Nehru Memorial Medical College, Raipur
India
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Source of Support: None, Conflict of Interest: None


PMID: 4793007

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How to cite this article:
Mukherjee P K, Dongre R C. A case of acquired facial diplegia, macular oedema and lingua plicata ("Melkersson-Rosenthal syndrome"). Indian J Ophthalmol 1973;21:36-9

How to cite this URL:
Mukherjee P K, Dongre R C. A case of acquired facial diplegia, macular oedema and lingua plicata ("Melkersson-Rosenthal syndrome"). Indian J Ophthalmol [serial online] 1973 [cited 2020 Jun 1];21:36-9. Available from: http://www.ijo.in/text.asp?1973/21/1/36/31419

Peripheral facial diplegia with swelling of face and lingua plicata is an uncommon disorder. The triad is known as "Melkersson-Rosenthal Syn­drome". Originally recurrent facial diplegia with swelling of face was re­ported by E. MELKERSSON [4] in 1928. Later C. RosENTHAL [6] added the fea­ture of Lingua plicata and since then the syndrome has been eponymed after the two authors.

Though the ocular involvement is a prominent feature yet the ophthalmic literature is scant on the subject. [2],[3],[7]

The following case is of interest that it appeared in an Indian girl.


  Case Report Top


An eight year old girl Gayitri was referred to the eye out patient depart­ment of the hospital on 5-5-70 with complaints of watering from both eyes of three months duration.

The patient's parent informed that the child was alright about three months ago. Since last three months the child has developed watering from both eyes. It was noticed that she was unable to close the eyes complete­ly. The eyes role up in an attempt to close the lids. The face of the child looked puffy. These disabilities gradually increased for the initial one month or so; but have remained stationary since then. The child was a fullterm normal delivery. Her mile­stones have been average except that she started speaking late and her voice has never been clear. Family history and past history did not reveal any­thing of particular significance.

General Examination:

An apparently healthy female child of normal build and normal intelli­gence, unable to articulate properly, prefers to keep mum and answers mostly by gestures. The face is puffy, the facial creases are absent and have an appearance of expression­less face. She was afebrile, she had no lymphadenopathy, no congenital anomaly, no oedema over the feet, abdomen or any other dependent part.

Examination of Central Nervous System:

It revealed bilateral infra nuclear Seventh (VII) Nerve palsy. (Photo­graph No. 1). Examination of the tongue showed that the tongue was furrowed. (Photograph No. 2, 3).

The child was unable to protrude the tongue out and could not move it from side to side. There were fascicu­lations on the tongue. The other cranial nerves, motor system, sensory system and reflexes were normal.

Ocular Examination:

Supra orbital furrows were absent. Palpebral aperture was wide. There was puffiness of the lids, incomplete clossure of the palpebral aperture. In an attempt to close the eye, Bell's phenomenon was positive, consequent­ly there was accumulation of tears, in the lower fornix and overflow of tears. Puncta, canaliculus and sac were normal, there was no regurgitation from the sac. Extra ocular muscles were normal. Other structures of the eye and fundi were normal.

Examination of the ear showed no abnormality. No tenderness over the region of the mastoid. Vocal cords normal.

Investigation:

Examination of the blood and Cerebro-spinal fluid did not reveal anything of significance.

E.S.R. 20 mm. fall at the end of first hour. (By Westergren's method).

Total serum protein-7.8 gm. % , Albumin-4.5 gm. % , globulin 3.3 gm. % .

C.S.F. Protein 30 mg. % , Sugar 60 mg. % , Chlorides 820 mg. % . Cells­-Nil.

Screening chest, X-Ray Skull A.P. and Lat. View and X-Ray Mastoid showed no abnormality.

Nasal scraping showed no A.F.B.


  Diagnosis Top


The child was diagnosed as a case of Melkersson-Rosenthal Syndrome and hospitalised for observation and skin biopsy which was refused and child was discharged on 2-5-70 with­out any change in the condition.

The child returned to the hospital after two months with added com­plaints of fever of 15 days duration which has not responded to treatment from the primary health centre.

The physical findings were the same except that the face was more swollen.

The investigations were repeated in view of pyrexia of unknown ori­gin. The Widal test, Urine and Blood culture were negative. The tempera­ture ranged between 99.0°F to 102.0°F and subsided without any specific treatment gradually over a period of ten days.

This time the parents agreed for a skin biopsy from the lids which reveal­ed evidence of angioneurotic oedema with lymphangiectasis, and lympho­cytic infiltration.

The various possibilities of bilate­ral facial palsy, with twelfth nerve in­volvement were excluded by clinical and laboratory datas. The commonest cause of facial palsy in childhood is chronic suppurative otitis media. This was ruled out by examination of the ear and X-ray mastoid. Leprosy, however, is the common cause of peri­pheral facial palsy in adults and is a late manifestation but is unknown in childhool, yet nasal scraping and cli­nical examination were done to ex­clude the possibilities. Involvement of the tongue and seventh nerve is possible only in wide spread lesion of central nervous system like polio, in­fective polyneuritis and diphtheria. These were given due thought and discarded as possible aetiological agent by process of exclusion.


  Discussion Top


The syndrome of Melkersson Ro­senthal usually manifests itself in childhood or early youth with an at­tack of peripheral facial palsy which may be recurrent and bilateral WALSH. [7] The oedema of the face is a very constant feature and was describ­ed by the original author in 1928 and has been reported regularly ever since. [2],[3],[5] Occurrence of oedema is variable and it may precede, accom­pany or follow. The oedema is non pitting in nature. Histology of the oedematous tissue is suggestive of an­gioneurotic oedema with lymphocytic infiltration.

There is a difference of opi­nion regarding lingua plicata, and is a most inconstant feature. Lingua plicata is congenital and is in some cases familial. COCKAYNE, [1] believes it to be of inherited trait transmitted in a dominated manner. PATON [5] suggested that furrowed ton­gue might be an indication of heredi­tary predisposition to development of facial paralysis and suggested that the appearance of the tongue be noted in all cases of Bell's palsy.

The exact etiology is not clear, it could be an allergic manifestation of some unknown factor. This is sup­ported by the histological appearance of angioneurotic oedema of the skin. The affection can well be a familial or inherited disorder.

The present case manifested all the triads of the syndrome and the skin biopsy was confirmatory of angioneu­rotic oedema, in accordance with Melkersson-Rosenthal Syndrome.


  Summary Top


A case of bilateral facial palsy and angioneurotic oedema of the face, and lingua plicata ("Melkersson-Rosenthal Syndrome") has been reported.

 
  References Top

1.
Cockayne, E. A.: Inherited Abnor­malities of the skin and its Appen­dages, pp. 95, 209, Oxford Univer­sity Press, New York. 1933.  Back to cited text no. 1
    
2.
Ford, F. R.: Disease of the Nervous System in infancy, childhood and adolescence, 5th edition pp. 385-386, Charles, C. Thomas Publishers. Springfield, Illinois, 1966.  Back to cited text no. 2
    
3.
Hellet, J. W. and Mitchell Barbara: Melkersson-Rosenthal Syndrome, Am. J. Ophth. 65: 542-544. 1968.  Back to cited text no. 3
    
4.
Melkersson, E.: Cited in 7, pp. 112.  Back to cited text no. 4
    
5.
Paton, D.: The Melkersson-Rosen­thal Syndrome, A case report, Am. J. Ophth.. 59: 705, 1965.  Back to cited text no. 5
    
6.
Rosenthal, C.: Cited in 3 and 5.  Back to cited text no. 6
    
7.
Walsh, F. B.: Clinical Neuro Oph­thalmology, 2nd edition pp. 112, Williams and Wilkins Co. Baltimore. 1957.  Back to cited text no. 7
    


    Figures

  [Figure - 1], [Figure - 2], [Figure - 3]



 

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