TY - JOUR
T1 - Update on juvenile myasthenia gravis
AU - Liew, Wendy K.M.
AU - Kang, Peter B.
PY - 2013/12
Y1 - 2013/12
N2 - PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.
AB - PURPOSE OF REVIEW: 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. RECENT FINDINGS: 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. SUMMARY: 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.
KW - acetylcholine receptor antibodies
KW - intravenous immunoglobulin
KW - juvenile myasthenia gravis
KW - plasmapheresis
KW - thymectomy
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U2 - 10.1097/MOP.0b013e328365ad16
DO - 10.1097/MOP.0b013e328365ad16
M3 - Review article
C2 - 24141560
AN - SCOPUS:84888127925
SN - 1040-8703
VL - 25
SP - 694
EP - 700
JO - Current Opinion in Pediatrics
JF - Current Opinion in Pediatrics
IS - 6
ER -