Juvenile myasthenia gravis: From diagnosis to treatment

Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction at the postsynaptic motor end plate, resulting in damage to the structure or function of the acetylcholine receptors. Juvenile myasthenia gravis presents with fluctuating muscle weakness and fatigue before the age of 19 years. These symptoms can affect the oculomotor musculature, bulbar system or have a generalized involvement. The diagnosis is similar to that in adults, made using electrophysiological techniques and antibody testing. Treatment is divided into medical and surgical. The medical management is oriented towards acetylcholinesterase inhibitors, immunosuppression and immunomodulation, including the use of steroids, intravenous immunoglobulin and plasmapheresis. Steroid sparing agents can be used with caution in refractory cases. Surgical approach is based on thymectomy. Future research, ideally prospective randomized controlled data, is needed to understand the differences between the adult and pediatric population, efficacy of therapies and outcomes.