Dhananjay Shrikhande
: It is an acquired autoimmune mediated postsynaptic disorder that is rare in childhood with prevalence of 5 in 1,00,000. First case was reported in the year 1644 in USA of an Indian chief Opechankanough. The disease is due to circulating antibodies which bind to acetylcholine receptors at neuromuscular junction and prevent a normal action of acetylcholine in opening the calcium channel in muscle fi bers. There are two types of the disease: ocular (corresponding to 10% of cases) [1] or
more » ... ed (90% of cases), although both may occur under different temporal evolution in the same patient. Myasthenia gravis geoepidemiology shows that it is a rare disorder with similar incidence and prevalence in the world, except for infantile MG, which is more common in Asia. Methods : With progressive ptosis towards the end of the day, Juvenile Myasthenia gravis was suspected. Anti cholinesterase (AChE) test was done under ECG monitoring using intravenous injection of neostigmine (Prostigmine test) which showed a dramatic response i.e. immediate elevation of both upper eyelids with resolution of ptosis[Fig. 1 and 2 ]. Bed side icepack test was also performed which was positive. Computerized tomography (CT scan) of thorax was done to rule out thymic hyperplasia or thymoma. We diagnosed this child as a case of juvenile myasthenia gravis and treated with intravenous neostigmine(0.04mg/ kg/dose) 4 hourly followed by atropine(0.05mg/kg/dose) to counteract ill muscarinic side effects of neostigmine. Intravenous methylprednisolone was also given as pulse therapy (30mg/ kg/day for 3 consecutive days) with multipara monitoring (for hypertension and hyperglycemia). Plasma anti-AChR antibodies though advised was not affordable. Results : The cardinal symptom in MG is muscle weakness that is typically fl uctuating and fatigable. The most common initial presentation involves a specifi c muscle weakness, and not a generalized weakness. Symptoms are exacerbated with exercise (it may be caused by sustained muscle action) and
doi:10.21304/2018.0501.00317 fatcat:ljgqn2fy5vfdthfuu64d37i3sa