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| ARTICLE |
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| Year : 1960 | Volume
: 8
| Issue : 2 | Page : 52-54 |
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Myasthenia gravis in children
Roy Ebenezer
Sckell Eye Hospital. C.M.C., Vellore, India
| Date of Web Publication | 6-May-2008 |
Correspondence Address:
Roy Ebenezer
Sckell Eye Hospital. C.M.C., Vellore
India
 Source of Support: None, Conflict of Interest: None  | Check |
How to cite this article:
Ebenezer R. Myasthenia gravis in children. Indian J Ophthalmol 1960;8:52-4 |
The manifestation of the disease are practically the same in adults and children. The special features of the disease may be considered.
Myasthenia is a progressive and chronic disease with a tendency to remissions. There is abnormal muscular fatigue leading sometimes to permanent paralysis. In some cases there is a familiar tendency. The greater incidence is among women than men in the third and fourth decades though cases have been reported in children at it months and in men at 75 years. The disease often manifests itself after respiratory illness.
1t may be localised to particular groups of muscles for many years or become generalized leading to a fatal termination. The pathological changes are formed in the muscles with accumulation of small round cells. These are called lymphorrhages.
Ptosis is the most obvious and prominent sign; but a partial or complete ophthalmoplegia externa may be seen. In about 35-80 per cent of cases diplopia may be present. The pupillary reflexes and accommodation are usually normal; but there is associated convergence insufficiency. Reading becomes more difficult or impossible.
The weakness starts with muscles of the face and neck. Facial weakness is usually bilateral. Weakness of the jaws, soft palate and pharynx produce difficulties in speech and swallowing. Weakness of chest muscles and diaphragm may cause dyspnoea and respiratory embarrassment, and in severe cases respiratory failure. There may be weakness of muscles of the extremities. Sometimes there is close resemblance to supra nuclear paralysis. Though the external muscles of the eye are involved in 50 per cent of the cases, intrinsic muscles are not affected.
Horizontal phorias or tropias are the commonest deviations but vertical and torsional imbalance may occur. The weakness is least in the morning and increases with fatigue in the evening after work. Complete remissions and relapses are common.
The Myasthenia Syndrome can arise not only in Myasthenia Gravis but also under the influence of curare, in association with certain tumours of the Thymus and in a state of Thyrotoxicosis.
In the diagnosis of the disease, certain tests are found useful. The X-ray of the chest may not show any abnormality; on the other hand there may be an increase in the shadow in the region of the Thymus. Electromyography is used to elicit Myasthenia Gravis reaction, on stimulation with repeated faradic stimuli, the muscles respond worse and worse with increase of ptosis. There is absence of muscular atrophy.
When a test dose of 1.5 ingms. of prostigmine methyl sulphate with 0.6 mgm, of Atropine Sulphate is given as an intramuscular injection, improvement is noted within 10 minutes. This is absolutely diagnostic for Myasthenia Gravis. Administration of 'quinine intensifies the symptoms of the disease.
When prostigmine is injected, ptosis is improved but the extraocular muscle palsy may show little improvement; permanent structural changes may be found in such cases.
| Treatment | |  |
Prostigmine has to be injected 4-5 times daily, or may be given orally in 15 mgm. dose. Glycine or aminoacetic acid has been claimed to give good results. Prostigmine methyl sulphate is rapid in action but brief in duration. The Bromide salt taken arally is more suitable for maintenance therapy the oral dosage being about 3 times that of the injection.
Antimyasthenic drugs that may be used in conjunction with prostigmine are potassium chloride and ephedrine sulphate. Tetra ethyl Pyrophosphate, Octa Methyl Pyro Phosphoramide, Edrophonium are of diagnostic and therapeutic value. Di-iso-propyl Flouro-Phosphate has been used with some benefit.
It is not possible to correct the diplopia with prisms because of the extreme variability. If a thymic tumour is present removal of the tumour brings good relief.
Myasthenia Gravis among children is rare compared to incidence among adults. Out of 10,208 children examined in II years at the Christian Medical College, Hospital there were only 3 cases which, work out at 0.03 per cent.
| Case Report | | |
Valliamma, a female child of 9 years was admitted on 8-1-1953 . She had bilateral ptosis which was more severe later in the day and was associated with almost complete bilateral external ophthalmoplegia with Myasthenia facies. Pupils were not involved.
There was nothing special in the family history. She had an attack of conjunctivitis about a month before, which lasted for 4 days. When it subsided she developed fever. After a week she was free of the fever but noticed she could not look at the light or move the eye ball.
Systemic findings were normal. There was cliplopia, but no dysphagia or dysarthria. Symptoms were worse in the evenings. Electromyography was done. X-ray showed no increase in the Thymus shadow.
| Treatment | | |
An injection of i mg. of Prostigmine with 1/200 grain of atropine test dose was given and within 15 minutes the ptosis was very much improved. She volunteered she could see much better, but there was hardly any change in the ophthalmoplegia with persistence of diplopia. When 2 mgm. of prostigmine with 1/150 grain of atropine was injected, the improvement was noticed within io minutes. Ptosis was almost completely relieved for about 3 hours. Maintenance close was given 4-5 times a day.
To try the effect of Thymectomy, on the 19th day the gland was excised by splitting the sternum. There was no pathology of the gland on microscopic section, but her ptosis improved to some extent. 30 mgm. of prostigmine orally was given four hourly. This had the same effect as of a 2 mgm. prostigmine injection subcutaneously.
Since there was no appreciable amelioration of her ptosis, except with prostigmine injections crutch spectacles were specially made and fitted. The patient was happy and was discharged to her village.[4]
| References | | |
| 1. | Vail D: 1949, Amer. J. Ophth. 32, 1414-15.  |
| 2. | Lisman Jack V.; 1949, Am. J. Ophth. 32, 565-571. |
| 3. | Lloyd I.: 1955, Br.J. Ophth., 39, 48-49. |
| 4. | Orgen C.: 1953, Br. J. Ophth., 37, 365. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]
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