Affiliation:
1. Department of Pediatrics, University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India
2. Department of Pediatrics, All India Institute of Medical Sciences, Guwahati, Assam, India
Abstract
Background:
Juvenile myasthenia gravis (MG) is a rare autoimmune neuromuscular disorder and has a varied presentation in childhood making it a diagnostic challenge.
Clinical Description:
An 8-year-old girl presented to the pediatric emergency with multiple episodes of falling, drooling of saliva, dysphagia, and bilateral ptosis. Within a few hours of admission, she had acute deterioration, developed respiratory failure, and was put on ventilatory support.
Management and Outcome:
A myasthenic crisis was suspected and a neostigmine challenge test was done. She was managed in the emergency with atropine, neostigmine, intravenous immunoglobulin, and supportive care. Subsequently, she achieved complete remission with steroids, physostigmine, and oral mycophenolate mofetil over the next 6 weeks.
Conclusion:
Juvenile MG may rarely be the cause of generalized muscular weakness. A strong suspicion of myasthenic crisis must be kept in case of sudden deterioration involving bulbar and respiratory muscles, in a patient with neurologic weakness. Early intervention in this rare condition can be lifesaving.