Comparison of Plasmapheresis and Intravenous Immunoglobulin as Maintenance Therapies for Juvenile Myasthenia Gravis | Cardiothoracic Surgery | JAMA Neurology | JAMA Network
[Skip to Navigation]
Access to paid content on this site is currently suspended due to excessive activity being detected from your IP address Please contact the publisher to request reinstatement.
Mullaney  P, Vajsar  J, Smith  R, Buncic  JR.  The natural history and ophthalmic involvement in childhood myasthenia gravis at the hospital for sick children.  Ophthalmology. 2000;107(3):504-510.PubMedGoogle ScholarCrossref
Lindstrom  JM, Seybold  ME, Lennon  VA, Whittingham  S, Duane  DD.  Antibody to acetylcholine receptor in myasthenia gravis: prevalence, clinical correlates, and diagnostic value: 1975.  Neurology. 1998; 51(4):933-939. PubMedGoogle Scholar
Hoch  W, McConville  J, Helms  S, Newsom-Davis  J, Melms  A, Vincent  A.  Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies.  Nat Med. 2001;7(3):365-368.PubMedGoogle ScholarCrossref
Vincent  A, Leite  MI.  Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravis.  Curr Opin Neurol. 2005;18(5):519-525.PubMedGoogle ScholarCrossref
Chiang  LM, Darras  BT, Kang  PB.  Juvenile myasthenia gravis.  Muscle Nerve. 2009;39(4):423-431.PubMedGoogle ScholarCrossref
Niks  EH, Kuks  JB, Verschuuren  JJ.  Epidemiology of myasthenia gravis with anti-muscle specific kinase antibodies in the Netherlands.  J Neurol Neurosurg Psychiatry. 2007;78(4):417-418.PubMedGoogle ScholarCrossref
Zhang  X, Yang  M, Xu  J,  et al.  Clinical and serological study of myasthenia gravis in HuBei Province, China.  J Neurol Neurosurg Psychiatry. 2007;78(4):386-390.PubMedGoogle ScholarCrossref
Andrews  PI, Massey  JM, Howard  JF  Jr, Sanders  DB.  Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis.  Neurology. 1994;44(7):1208-1214.PubMedGoogle ScholarCrossref
Andrews  PI, Massey  JM, Sanders  DB.  Acetylcholine receptor antibodies in juvenile myasthenia gravis.  Neurology. 1993;43(5):977-982.PubMedGoogle ScholarCrossref
Pevzner  A, Schoser  B, Peters  K,  et al.  Anti-LRP4 autoantibodies in AChR- and MuSK-antibody-negative myasthenia gravis.  J Neurol. 2012;259(3):427-435.PubMedGoogle ScholarCrossref
Barth  D, Nabavi Nouri  M, Ng  E, Nwe  P, Bril  V.  Comparison of IVIg and PLEX in patients with myasthenia gravis.  Neurology. 2011;76(23):2017-2023.PubMedGoogle ScholarCrossref
Evoli  A, Batocchi  AP, Bartoccioni  E, Lino  MM, Minisci  C, Tonali  P.  Juvenile myasthenia gravis with prepubertal onset.  Neuromuscul Disord. 1998;8(8):561-567.PubMedGoogle ScholarCrossref
Pitt  M.  Neurophysiological strategies for the diagnosis of disorders of the neuromuscular junction in children.  Dev Med Child Neurol. 2008;50(5):328-333.PubMedGoogle ScholarCrossref
Tracy  MM, McRae  W, Millichap  JG.  Graded response to thymectomy in children with myasthenia gravis.  J Child Neurol. 2009;24(4):454-459.PubMedGoogle ScholarCrossref
Millichap  JG, Dodge  PR.  Diagnosis and treatment of myasthenia gravis in infancy, childhood, and adolescence: a study of 51 patients.  Neurology. 1960;10:1007-1014.PubMedGoogle ScholarCrossref
Seybold  ME, Baergen  RN, Nave  B, Lindstrom  JM.  Anti-acetylcholine-receptor antibody concentrations after thymectomy in patients with myasthenia gravis.  Br Med J. 1978;2(6144):1051-1053.PubMedGoogle ScholarCrossref
Snead  OC  III, Benton  JW, Dwyer  D,  et al.  Juvenile myasthenia gravis.  Neurology. 1980;30(7, pt 1):732-739.PubMedGoogle ScholarCrossref
Zinman  L, Ng  E, Bril  V.  IV immunoglobulin in patients with myasthenia gravis: a randomized controlled trial.  Neurology. 2007;68(11):837-841.PubMedGoogle ScholarCrossref
Gajdos  P, Chevret  S, Toyka  K.  Intravenous immunoglobulin for myasthenia gravis.  Cochrane Database Syst Rev. 2008;(1):CD002277.PubMedGoogle Scholar
McMillan  HJ, Darras  BT, Kang  PB.  Autoimmune neuromuscular disorders in childhood.  Curr Treat Options Neurol. 2011;13(6):590-607.PubMedGoogle ScholarCrossref
Ashraf  VV, Taly  AB, Veerendrakumar  M, Rao  S.  Myasthenia gravis in children: a longitudinal study.  Acta Neurol Scand. 2006;114(2):119-123.PubMedGoogle ScholarCrossref
Kaminski  HJ, Cutter  G, Ruff  R.  Practice parameters and focusing research: plasma exchange for myasthenia gravis.  Muscle Nerve. 2011;43(5):625-626.PubMedGoogle ScholarCrossref
Selcen  D, Dabrowski  ER, Michon  AM, Nigro  MA.  High-dose intravenous immunoglobulin therapy in juvenile myasthenia gravis.  Pediatr Neurol. 2000;22(1):40-43.PubMedGoogle ScholarCrossref
Mandawat  A, Kaminski  HJ, Cutter  G, Katirji  B, Alshekhlee  A.  Comparative analysis of therapeutic options used for myasthenia gravis.  Ann Neurol. 2010;68(6):797-805.PubMedGoogle ScholarCrossref
Miller  RG, Barohn  RJ, Dubinsky  R.  Expanding the evidence base for therapeutics in myasthenia gravis.  Ann Neurol. 2010;68(6):776-777.PubMedGoogle ScholarCrossref
Mandawat  A, Mandawat  A, Kaminski  HJ, Shaker  ZA, Alawi  AA, Alshekhlee  A.  Outcome of plasmapheresis in myasthenia gravis: delayed therapy is not favorable.  Muscle Nerve. 2011;43(4):578-584.PubMedGoogle ScholarCrossref
Skeie  GO, Apostolski  S, Evoli  A,  et al; European Federation of Neurological Societies.  Guidelines for treatment of autoimmune neuromuscular transmission disorders.  Eur J Neurol. 2010;17(7):893-902.PubMedGoogle ScholarCrossref
Bril  V, Barnett-Tapia  C, Barth  D, Katzberg  HD.  IVIG and PLEX in the treatment of myasthenia gravis.  Ann N Y Acad Sci. 2012;1275:1-6.PubMedGoogle ScholarCrossref
Adams  C, Theodorescu  D, Murphy  EG, Shandling  B.  Thymectomy in juvenile myasthenia gravis.  J Child Neurol. 1990;5(3):215-218.PubMedGoogle ScholarCrossref
Gronseth  GS, Barohn  RJ.  Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology.  Neurology. 2000;55(1):7-15.PubMedGoogle ScholarCrossref
Rodriguez  M, Gomez  MR, Howard  FM  Jr, Taylor  WF.  Myasthenia gravis in children: long-term follow-up.  Ann Neurol. 1983;13(5):504-510.PubMedGoogle ScholarCrossref
Gadient  P, Bolton  J, Puri  V.  Juvenile myasthenia gravis: three case reports and a literature review.  J Child Neurol. 2009;24(5):584-590.PubMedGoogle ScholarCrossref
Romi  F, Gilhus  NE, Aarli  JA.  Myasthenia gravis: disease severity and prognosis.  Acta Neurol Scand Suppl. 2006;183:24-25.PubMedGoogle ScholarCrossref
Original Investigation
May 2014

Comparison of Plasmapheresis and Intravenous Immunoglobulin as Maintenance Therapies for Juvenile Myasthenia Gravis

Author Affiliations
  • 1Department of Neurology, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts
  • 2Clinical Research Center, Boston Children’s Hospital, Boston, Massachusetts
  • 3Joint Program in Transfusion Medicine, Department of Laboratory Medicine, Boston Children’s Hospital, Boston, Massachusetts
  • 4Department of Pathology, Harvard Medical School, Boston, Massachusetts
  • 5Department of Surgery, Boston Children’s Hospital, Harvard Medical School, Boston, Massachusetts
  • 6Division of Pediatric Neurology, University of Florida College of Medicine, Gainesville
JAMA Neurol. 2014;71(5):575-580. doi:10.1001/jamaneurol.2014.17

Importance  Juvenile myasthenia gravis (MG) is a relatively rare autoimmune disorder. The comparative efficacy of plasmapheresis (PLEX) vs immunoglobulin as maintenance therapy is unclear for this childhood disease.

Objective  To determine whether PLEX or intravenous immunoglobulin (IVIG) therapy is more effective as maintenance therapy in this disease.

Design, Setting, and Participants  This retrospective analysis over a 33-year period involved 54 children and adolescents with juvenile MG at a specialized neuromuscular clinic and electromyography laboratory at a tertiary care academic pediatric hospital.

Interventions  Plasmapheresis and IVIG.

Main Outcomes and Measures  Response to treatment was measured by both improvement in objective physical examination findings and the patients’ reported improvement in symptoms and functional abilities.

Results  Subjective and objective outcomes correlated well. Both PLEX and IVIG had high response rates. Of the 27 patients with generalized juvenile MG receiving PLEX, IVIG, or both treatments, 7 of 7 patients treated with PLEX alone responded, 5 of 10 patients treated with IVIG alone responded, and 9 of 10 patients who received both responded. There was a significant difference in response rates between patients who received PLEX vs IVIG (P = .04). The youngest age at which PLEX was initiated via peripheral venous access was 9 years, while the youngest child who received IVIG was 9 months old. Thymectomy was performed in 17 children, of whom 11 experienced significant postoperative improvement.

Conclusions and Relevance  This study provides class III evidence that PLEX and IVIG both have high response rates as maintenance therapies and are reasonable therapeutic options for juvenile MG. Plasmapheresis may have a more consistent response rate than IVIG in this setting. These findings will provide some guidance regarding the approach to therapy for juvenile MG, especially as the results differ somewhat from those of studies focusing on adult MG.