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ARTICLES
Year : 1978  |  Volume : 26  |  Issue : 2  |  Page : 46-48

Oculopharyngeal muscular dystrophy with tuberculous meningitis (A case report)


Department of Neurosurgery, Kurnool Medical College, Kurnool (A.P.), India

Correspondence Address:
J Vimala
Department of Neurosurgery, Kurnool Medical College, Kurnool (A.P.)
India
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Source of Support: None, Conflict of Interest: None


PMID: 721245

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How to cite this article:
Vimala J, Seetharam W, Dinakar I. Oculopharyngeal muscular dystrophy with tuberculous meningitis (A case report). Indian J Ophthalmol 1978;26:46-8

How to cite this URL:
Vimala J, Seetharam W, Dinakar I. Oculopharyngeal muscular dystrophy with tuberculous meningitis (A case report). Indian J Ophthalmol [serial online] 1978 [cited 2020 Jun 1];26:46-8. Available from: http://www.ijo.in/text.asp?1978/26/2/46/31477

Victor, Hayes and Adams[15] identified cases of ocular myopathy with dysphagia as a sepa­rate group to which they gave the name oculo­pharyngeal myopathy. There are a certain number of disorders of which the predominant clinical feature is the disturbance of extraocular muscles. These include ocular myopathy[5], descending ocular myopathy[6], oculopharyngeal muscular dystrophy[15], Kearns-Shy Syndrome (oculocraniosomatic neuro-muscular disease with ragged fibers)[4],[9] and oculopharyngo distal myopathy[11],[12] Ocular involvement in Tuber­culous meningitis has been reported from time to time[8],[13],[14]. There has been no report of this type and hence the following case report.


  Case Report Top


G.V.R. a 30 year old male was admitted to the N.S. Ward on 18-2-73 for difficulty in swallowing and ptosis of 2 years duration. The difficulty in swallow­ing was more for solids. Ptosis was constant with no diurnal variation and unaffected by activity. There was no history of nasal regurgitation of fluids. There were no constitutional disturbances. He had one brother and one sister, both of them were healthy. He was not married. General examination revealed a moderately nourished and thin built individual, with normal pulse rate and blood pressure. Systemic examination of cent­ral nervous system revealed dysarthria, bilateral partial ptosis [Figure - 1], fixed eye balls, weakness of facial, masseter and palatal muscles. Fundi were normal. Fasciculations, myotonia, cataract, retinitis pigmentosa, pyramidal tract signs, sensory disturbances were absent,

Cardiovascular system was clinically normal. ECG was normal. There was no evidence of cardiomyopathy. A clinical diagnosis of oculopharyngeal muscular dystrophy was made. Routine laboratory investigations were normal. C.S.F. was normal i.e. globulin-ve, protein 40 mg%, Sugar 50 mg% and chlorides 680 mg%, Myasthenia Gravis was excluded by means of the prostigmine and Tensilon tests. Fields of vision, X-rays of skull and Pneumoencephalogram were normal. He was discharged on 12-12-7 3.

He was readmitted on 10-8-75 for fever, headache and vomiting of 15 days duration. Difficulty in swallo­wing and ptosis were persisting. There was history of evening rise of temperature associated with night sweats.

He had anorexia and loss of weight. General exami­nation revealed a moderately nourished thin built indivi­dual running temperature of 100'F ; Pulse rate of 112/ mt. and normal blood pressure. Neurological exami­nation revealed signs of meningeal irritation in addition to the previous clinical features i.e. dysarthria, bilateral fixed eye balls, bilateral partial ptosis, dysphagia, weak­ness of palatal muscles. Fundi were normal. Clinical diagnosis of tuberculous meningitis with oculopharyn­geal muscular dystrophy was made this time. Haemo­gram revealed ESR of 50'mm at the end of 1st hour and total count of 10,200, 1c.mm. The cerebrospinal fluid was under high tension, showed positive globulin, pro­tein content of 500 mg%, sugar of 39 mg% chlorides 520 mg% and 200 lymphocytes /c. mm. The diagnosis of tuberculous meningitis was confirmed. Patient was started on Inj. Streptomycin 1 gm i m. INH 100 mg three times a day orally, PAS 12 gms a day. Patient improved remarkably. Signs of meningeal irritation disappeared though oculopharyngeal weakness persisted. He was discharged on 8-9-75 with an advice to continue antituberculous treatment for 1½ years. To date he is free from features of meningitis but the ptosis and dysphagia are persisting.


  Discussion Top


The present case fits into the oculopharyn­geal muscular dystrophy described by Victor et al.[15] The illness presented in late 3rd decade in the present case. The mean age of onset was 40 yrs. according to Bray, Kaarsoo and Ross[2] Many of the reported cases have been familial and were of the French Canadian stock.[1] Sporadic cases have been reported.[3] The present case is a sporadic one as other member of the family has suffered. The inheritance in familial cases is said to be dominant[3]. The disorder bears some slight resemblance's to dystrophia myotonica in its clinical, genetic features in probable involve­ment of smooth muscle[7], and reports of abnor­malities of immunoglobulins[10]. In the present case, there were no features of dystrophia myotonica.

Ocular complications, facial weakness, dysphagia have been described in tuberculous meningitis. In the present case tuberculosis developed about 4 yrs. after the onset of the myopathic symptoms. There were no clinical or biochemical changes suggesting tuberculous meningitis on his first admission in 1973.


  Summary Top


Oculopharyngeal muscular dystrophy is a rare congenital anomaly especially presenting as a sporadic case. Ocular involvement in tuberculous meningitis has been reported from time to time. A combination of both diseases with tuberculous meningitis developing in a known patient of oculopharyngeal muscular dystrophy has not been reported hitherto.

 
  References Top

1.
Barbeau, A., cited by Brain et. al.  Back to cited text no. 1
    
2.
Bray, G.M., Kaarsoo, M , and Ross, R.T., 1965, cited by Brain et al.  Back to cited text no. 2
    
3.
Brain, L., and Walton, J.G., 1969 Brain's Diseases of the Nervous system, seventh Edition., Oxfo University Press, London.  Back to cited text no. 3
    
4.
Karpati, G., Carpenter, S., Larbrisseau, A., et al cited by Satoyoshi et al.  Back to cited text no. 4
    
5.
Kiloh, L.G., Neven, S., Cited by Satoyoshi et al.  Back to cited text no. 5
    
6.
Lees, F., Liversedge, L.A., (1962) Descending Ocelar Myopathy Brain 85, 701.  Back to cited text no. 6
    
7.
Lewis, I., 1966, cited by Brain et al.  Back to cited text no. 7
    
8.
Mishra, R.K., and Gupta., S.P., (1962) Ocular complications of Tuberculous Meningitis J. Ind. Med. Ass. 38, 10, 513.  Back to cited text no. 8
    
9.
Oslon, W., Engel, W.K., Walso, G.O. et al cited by Satoyoshi et al.  Back to cited text no. 9
    
10.
Russe, H., Busey H., and Barbeau, A., cited by Brain et al.  Back to cited text no. 10
    
11.
Satoyoshi, E.. Murakami, K., Kowa, H, Kinoshita, M., and Toril, J. 1965, Distal involve­ment of the Extremities in ocular myopathy, Am. J. Ophrhal., 59, 669 .  Back to cited text no. 11
    
12.
Satovoshi, E., Kinoshita, M., 1977, Oculo­pharyngo distal myopathy, Report of Four Families, Arch. Neurol 4, 99-92.  Back to cited text no. 12
    
13.
Tvapar, R.K., Dayal, R.S., and Singhal, K.C., 1968 Ocular complications in Tuberculous Meningitis, Ind. Pead. 5, 10  Back to cited text no. 13
    
14.
Thomas, M.D., Chopra, J.S., Banerjee, A.K., and Singh M.S., (1977). Tuberculous Meningitis (A clinico-pathological study) Neurol., Ind. 25 1, 26.  Back to cited text no. 14
    
15.
Victor, Hayes, R., Admas RD. (1962) Oculo. pharyngeal muscular dystrophy. A familial disease of late life characterised by dysphagia and progressive ptosis of eyelids, New England, Med 267, 1967.  Back to cited text no. 15
    


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