An eight-year-old female child presented to the pediatric emergency with complaints of generalized muscle weakness and ptosis with diplopia. A patient complained of painless fatiguability of bulbar and limb musculature resulting in dysphonia and difficulty in swallowing. The patient also had proximal limb weakness manifesting as difficulty in getting up from the floor, running, going upstairs, and lifting her arms above her head. The weakness was fluctuant, which became more pronounced on activity and improved on rest. The patient was being worked up for the cause of such weakness when suddenly the patient went into myasthenic crisis in the form of worsening dyspnoea, drowsiness, fatigue, worsening bulbar dysfunction, and marked global weakness with impending respiratory failure. The patient was kept on NIV support and was given intramuscular neostigmine after atropinization. The patient was also started on intravenous immunoglobulin along with steroids. Dramatic improvement was seen in the patient’s clinical status, and subsequently, the patient was weaned off from NIV support. Serum AchR antibodies were sent, which came out to be positive, and the patient was clinically diagnosed as a case of Juvenile Myasthenia Gravis with supporting lab evidence. CT chest was done to look for thymus enlargement, which came out to be expected. Now the patient has been shifted to a steroid-sparing agent – Mycophenolate mofetil, along with pyridostigmine, and the patient has shown significant improvement in symptoms.
Conclusion
Myasthenic crisis is a complication of myasthenia gravis characterized by worsening muscle weakness resulting in respiratory failure. Common precipitants of the myasthenic crisis include infections, including aspiration pneumonia, any stress, or any recent change in medication. The outcome of Juvenile myasthenia gravis has improved significantly over the last decade with better recognition, diagnosis, and more effective therapies with good long-term prognosis. Children with JMG exhibit higher rates of remission than adults, including spontaneous remission and remission following a period of drug therapy, with prepubertal children showing the highest rates of spontaneous remission.