Review Article

Juvenile Myasthenia Gravis: A Paediatric Perspective

Table 3

Treatment options in JMG.

TreatmentEvidence of efficacy in generalised JMGReference

Acetylcholinesterase inhibitorsFirst line therapy.May be sufficient in ocular JMG or mild generalised JMGSkeie et al., 2010 [29]
ThymectomyRecommended to increase remission rates in postpubertal, seropositive children. Not recommended in prepubertal childrenHennessey et al., 2011 [30]
Rodriguez et al., 1983 [31]
Tracy et al., 2009 [32]
Lindner et al., 1997 [20]
SteroidsOften used in combination with steroid sparing agents. Significant side-effect profile if used long-term at high doseSchneider-Gold et al., 2005 Cochrane review: one JMG study, others adult or unspecified age ranges [33]
Zhang et al., 1998 [6]
Steroid sparing agents
 AzathioprineUsually used in combination with corticosteroids. Occasionally used alone.Mertens et al., 1981 [34] includes some children but no subgroup analysis
Gold et al., 2008 [35]
Palace et al., 1998 [36] adult study
 Cyclosporin AAs monotherapy or in conjunction with corticosteroidsTindall et al., 1987 [37] adult study
Hart et al., 2009 (Cochrane) [38] series include some children
 CyclophosphamideDe Feo et al., 2002 [39] adult population
Hart et al., 2009 [38] (Cochrane) series include some children
 TacrolimusFurukawa et al., 2008 [40]
Ponseti et al., 2008 [41]
both include post pubertal children
Ishigaki et al., 2009 [42] prepubertal female
 MMFHehir et al., 2010 [43] includes some peri-/postpubertal children
 RituximabWylam et al., 2003 [44] pediatric case
IVIG/Plasma exchangeSelcen et al., 2000 [45]
Gajados et al., 2008, Cochrane database [46]