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ARTICLES |
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Year : 1973 | Volume
: 21
| Issue : 1 | Page : 36-39 |
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A case of acquired facial diplegia, macular oedema and lingua plicata ("Melkersson-Rosenthal syndrome")
PK Mukherjee, RC Dongre
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
Source of Support: None, Conflict of Interest: None | Check |
PMID: 4793007
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 |
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 | | |
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 | | |
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 | | |
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 | | |
A case of bilateral facial palsy and angioneurotic oedema of the face, and lingua plicata ("Melkersson-Rosenthal Syndrome") has been reported.
References | | |
1. | Cockayne, E. A.: Inherited Abnormalities of the skin and its Appendages, pp. 95, 209, Oxford University Press, New York. 1933. |
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. |
3. | Hellet, J. W. and Mitchell Barbara: Melkersson-Rosenthal Syndrome, Am. J. Ophth. 65: 542-544. 1968. |
4. | Melkersson, E.: Cited in 7, pp. 112. |
5. | Paton, D.: The Melkersson-Rosenthal Syndrome, A case report, Am. J. Ophth.. 59: 705, 1965. |
6. | Rosenthal, C.: Cited in 3 and 5. |
7. | Walsh, F. B.: Clinical Neuro Ophthalmology, 2nd edition pp. 112, Williams and Wilkins Co. Baltimore. 1957. |
[Figure - 1], [Figure - 2], [Figure - 3]
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