Pediatric Myasthenia Gravis

Pediatric myasthenia gravis encompasses a heterogenous group of disorders that cause impaired neuromuscular junction transmission. Patients exhibit a characteristic fatigable weakness that can be life-threatening if respiration is affected. In children, myasthenia gravis can be grouped into 3 subtypes: transient neonatal myasthenia (TNM) arising from transplacental antibody transfer, congenital myasthenic syndromes (CMS) related to genetic defects, and juvenile myasthenia gravis (JMG) caused by antibodies to synaptic components. Diagnostic evaluation combines clinical evaluation with serologic and electrophysiologic studies. The condition is managed medically with acetylcholinesterase inhibitors and immunosuppressive agents. Thymectomy is an important part of the treatment of JMG, and intravenous immunoglobulin (IVIG) and plasma exchange (PLEX) play a role in acute, severe exacerbations. Management of the ophthalmic complications, including strabismus and ptosis, is necessary to prevent permanent vision loss from amblyopia.