Year : 1979 | Volume
: 27 | Issue : 4 | Page : 150--151
SVM Surya Rao, C Anjaneyulu, K Vengal Rao
Kurnool Medical College, Kurnool, India
SVM Surya Rao
Kurnool Medical College, Kurnool
|How to cite this article:|
Surya Rao S, Anjaneyulu C, Vengal Rao K. Ocular myopathy.Indian J Ophthalmol 1979;27:150-151
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Surya Rao S, Anjaneyulu C, Vengal Rao K. Ocular myopathy. Indian J Ophthalmol [serial online] 1979 [cited 2020 Sep 28 ];27:150-151
Available from: http://www.ijo.in/text.asp?1979/27/4/150/32607
Chronic progressive bilateral external ophthalmoplegia is a myopathy. The condition is usually bilateral and starts with ptosis which gradually becomes complete. Meanwhile slowly progressive paralysis of all the extraocular muscles appear and the eyes become practically immobile in 30-40 years. The pupil and accommodation are not affected. The disease usually appears first during adolescence although it may be noticed between 20-30 years.
The orbicularis oculi muscle is affected in about 25% of cases. In about 10% cases weakness of the face, neck, shoulder, girdle and lower limbs appears. Patient's lifespan is unaffected. The disease has a strong familial tendency. Associated conditions like retinitis pigmentosa, cataracts, hyperthyroidism, testicular atrophy and premature baldness have been discribed. Some cases of congenital ptosis may be myopathic.
Diagnosis and Investigations
The diagnosis depends on the Typical clinical picture of bilaterality, ptosis and external ophthalmoplegia without involvement of the pupils and accomodation. In some cases associated similar lesions are seen elsewhere in the body eg. dysphagia, degeneration of muscles of face, neck, shoulder and pelvic girdle.
1. Estimation of serum muscle enzymes which may not be always abnormal as the disease is restricted to a small group of muscles,
Serum creatinine phosphokinase-Normal 5-50 mU/ml
Serum Aldolase Normal 0-6 mU/ml
2. Electromyography-Extremely low amplitude of response.
3. Muscle biopsy is most reliable except in advanced cases. Histological examination of the affected muscles shows atrophy of muscle bundles and fatty and fibrous replacement. The sarcolemmal nuclei show focal areas of hyperplasia. There is an absence of myofibrils and cross striations in occasional fibres.
As ocular myopathy is a comparatively rare condition in this paper three cases are reported, The investigations include muscle biopsy and histopathological examination.
No. 1. David a 16 year old male attended the ophthalmic department of the Government General Hospital, Guntur with a complaint of drooping of both upper lids. The condition was noticed at the age of 15 years in both the eyes at the same time. There is no history of similar complaint in his family.
There were evidences of involvement of levator palpebral suspensions, all extra-ocular muscles and orbis cularis oculi. There was weakness and a slight wasting of the scapular muscles on the left side.
Neurological examination revealed no abnormality. Blood V.D.R.L. was negative. Muscle biospy of lateral rectus and oribicularis of the left eye and infraspinatus muscle on the left side showed changes consistant with those of myopathy.
No. 2. G. Kanakamma, a 60 year old female attended the Ophthalmic Department with the complaint of drooping of both upper lids since one year. She was having diplopia before, but not present now. There is no family history of similar condition. The ptosis started first in right eye. There were evidences of weakness of levator and all extraocular muscles. The pupils and accommodation are normal. There is no other abnormality elsewhere in the body. Blood V.D.R.L. was negative. Neurological examination revealed no abnormality. Muscles biopsy of lateral rectus and oribicularis of right eye. External rectus showed hyalinisation of tissue. Orbicularis showed muscle bundles with loss of striations and replacement by fibrous and fatty tissue.
Diagnosis muscular dystrophy.
No. 3. G. Maniamma, female aged sixteen years attended the Ophthalmic department with the complaint of drooping of the eye lids of three years duration. There was no history of diplopia. There is no other abnormality elsewhere in the body. There were evidences of varying degree of involvement of extraocular muscles in either eye.
Neurological examination--Nil Blood V.D.R.L. Negative. Muscle biopsy of lateral rectus and orbicularis showed typical myopathic changes.
Three cases of ocular myopathy are described. In all cases muscle biopsy showed characteristic myopathic changes. In the first case myopathic changes were present in the left infraspinatus muscle also. There was wasting of scapular muscles on the left side in this case. Except for this in all other cases there was no evidence of involvement of muscles of face, neck, pelvic girdle etc., Probably if these cases are followed up for years involvement of muscles in other parts of body may be clinically manifest. In a typical case of ocular myopathy there is bilateral ptosis and external Ophthalmoplegia (without involvement of pupils and accommodation). In early cases when the disease is in one eye only it may be mistaken for neurogenic palsy. As regards the differential diagnosis myasthenia gravis resembles closely this condition but can be differentiated by "Prostigmin Test" and in this condition the histological change in the affected muscle is different. Nuclear palsies are characterised by bilaterality, diplopia and with or without internal ophthalmoplegia. Here the muscles are normal and electrical reactions are normal.
The literature on ocular myopathy is briefly reviewed. Three cases of ocular myopathy are presented. The diagnosis was confirmed by muscle biospy in all these cases.
We are grateful to Dr. D. Bhaskara Reddy, Professor of Pathology for his report on Pathological specimens and the excellent Photomicrographs.