Update on juvenile myasthenia gravis

• Published in: Current opinion in pediatrics Vol. 25; no. 6; pp. 694 - 700
• Main Authors: Liew, Wendy K M, Kang, Peter B
• Format: Journal Article
• Language: English
• Published: United States 01-12-2013
• Subjects: Age of Onset; Child; Child, Preschool; Cholinesterase Inhibitors - therapeutic use; Electromyography; Female; Glucocorticoids - therapeutic use; Humans; Immunoglobulins, Intravenous - therapeutic use; Immunosuppression; Infant; Male; Myasthenia Gravis, Neonatal - diagnosis; Myasthenia Gravis, Neonatal - drug therapy; Myasthenia Gravis, Neonatal - physiopathology; Myasthenia Gravis, Neonatal - surgery; Myasthenic Syndromes, Congenital - diagnosis; Myasthenic Syndromes, Congenital - drug therapy; Myasthenic Syndromes, Congenital - physiopathology; Plasmapheresis; Pyridostigmine Bromide - therapeutic use; Randomized Controlled Trials as Topic; Thymectomy - methods
• DOI: 10.1097/MOP.0b013e328365ad16
• EISSN: 1531-698X
• ISSN: 1040-8703

Juvenile myasthenia gravis is a relatively rare autoimmune neuromuscular disorder. The pathophysiology of juvenile myasthenia gravis is similar to that of adult myasthenia gravis, though there remain important differences regarding presentation and therapeutic options. We review the pathophysiology, clinical presentation, and treatment options for juvenile myasthenia gravis.
Randomized clinical studies of myasthenia gravis have been carried out primarily in adult populations. As juvenile myasthenia gravis is rare, it has been difficult to collect prospective randomized controlled data to evaluate treatment outcomes and efficacy. A recent retrospective series suggests that, as in adult myasthenia gravis, thymectomy is a viable therapeutic option for selected cases of generalized juvenile myasthenia gravis. This is corroborated by the clinical experience of the authors in a referral center with a cohort of patients affected by juvenile myasthenia gravis over a number of years.
Recent studies illustrate that some, but not all, adult research on myasthenia gravis is applicable to children and adolescents with juvenile myasthenia gravis. Adult research can inform pediatric studies, but should not be regarded as a substitute for dedicated research in those populations.