|Year : 1995 | Volume
| Issue : 3 | Page : 136-137
Childhood myasthenia gravis in a toddler
SG Jaison, Alexander P Abraham
Department of Ophthalmology, Christian Medical College & Hospital, Ludhiana, India
S G Jaison
Department of Ophthalmology, Christian Medical College & Hospital, Ludhiana 141 008
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Jaison S G, Abraham AP. Childhood myasthenia gravis in a toddler. Indian J Ophthalmol 1995;43:136-7
Myaesthenia gravis is defined as an acquired autoimmune disorder in which there is abnormal fatiguability of muscles due to deficiency of acetylcholine receptors caused by circulating antibodies directed against them. It can occur at any age but usually occurs after the age of five years.
There are not many reports of childhood myasthenia gravis. Of the 13 cases of childhood myaesthenia gravis reported by Verma et al, the youngest was 2 years at the onset of the illness. This case of childhood myaesthenia is being reported in view of its onset at 17 months of age.
| Case report|| |
An 18-month-old female child presented with complaints of partial bilateral ptosis and inability to move the eyeballs, noted by the parents one month prior to reporting to us. The onset of the symptoms were gradual, and not preceded by any acute febrile illness. There was no history of difficulty in swallowing, chewing, or physical activity. No history of snake bite was given. The child was born normally at full-term to a healthy young mother and the perinatal period was uneventful. The milestones of the child were also normal. None of the siblings had similar complaints.
General physical examination revealed a female child of normal physical and mental development. She had bilateral partial ptosis. Visual axes were parallel. There was total bilateral external ophthalmoplegia [Figure - 1]. The cornea was normal. There was no cataract or pigmentary changes in the retina. There was no heart block, ataxia, areflexia, motor weakness or any other neurological deficits.
Based on the history and physical examination, a provisional diagnosis of childhood myaesthenia (Stage I) was made. A repetitive stimulation of the right median nerve at 3/sec demonstrated a significant decremental response in the amplitude of muscle action potential (18%), confirming the diagnosis of myaesthenia gravis. The child was subjected to a Neostigmine test which was unequivocally positive. Chest X-ray and other routine tests were normal. The child was started on tablet neostigmine 3 mg twice daily and tablet prednisolone 10 mg daily orally. At follow-up 3 weeks later, ocular motility was found to be fully restored and there was no ptosis. At six months follow-up the child had recurrence of symptoms while off treatment, but on restarting the treatment the child was restored to normal and is on regular follow-up till date [Figure - 2].
| Discussion|| |
As this child presented with myaesthenia at an age below two years, familial infantile myaesthenia was considered; it was ruled out in absence of family history and involvement of extraocular muscles.
Congenital myaesthenia gravis described by Bowman and Levin was another possible diagnosis as the extraocular muscles were predominantly involved but congenital myaesthenia usually has familial occurrence and does not respond to steroid therapy.
Our patient was diagnosed to have juvenile myaesthenia, a variant of adult myaesthenia with similar clinical presentation and autoimmune mechanism. The remarkable response of the child to systemic steroid therapy confirms the autoimmune mechanism. This case of childhood myaesthenia of the juvenile subtype in a female child is being reported for its rare occurrence at the early age of 17 months. To our knowledge, this is the youngest patient of childhood myaesthenia of the juvenile subtype reported so far.
| References|| |
Desai AD, Raju TNR, Deshpande DH. Muscles and their disorders. In: Textbook of Paediatrics, Vol. I, Udani PM, (ed). New Delhi, Jaypee Brothers, 1991, pp. 2232-2244.
Verma AK, Behari M, Ahuja GK. Myasthenia gravis in young. Indian J Paediatr 23:363-369, 1986.
Bowman JR. Myasthenia gravis in young children. Paediatrics 1:472-477, 1948.
Levin PM. Congenital myasthenia in siblings. Arch Neurol 62:745-748, 1949.
Millichap JG, Dodge PR. Diagnosis and treatment of myasthenia gravis in infancy, childhood and adolescence. Neurology 11:1007-1014,1960.
[Figure - 1], [Figure - 2]