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Myasthenia Gravis

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Symptom-Based Approach to Pediatric Neurology
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Abstract

This chapter focuses on the clinical aspects of juvenile myasthenia gravis as may be encountered by a general pediatrician. Presenting symptoms and initial management strategies are discussed, with a particular focus on early detection of an impending myasthenic crisis. The key differentiating examination findings are reviewed, which can help narrow the broad differential of a lower motor neuron pattern of weakness to myasthenia gravis and guide appropriate testing. The aim of this chapter is to empower pediatricians to be able to recognize the symptoms of myasthenia gravis, elicit and interpret distinguishing findings of the disease on a neurological examination, monitor for signs of an evolving myasthenic crisis, and take prompt appropriate action when signs of a crisis are present.

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References

  1. Mansukhani SA, Bothun ED, Diehl NN, Mohney BG. Incidence and ocular features of pediatric myasthenias. Am J Ophthalmol. 2019;200:242–9. https://doi.org/10.1016/j.ajo.2019.01.004. Epub 2019 Jan 14.

    Article  Google Scholar 

  2. Evoli A, Batocchi AP, Bartoccioni E, Lino MM, Minisci C, Tonali P. Juvenile myasthenia gravis with prepubertal onset. Neuromuscul Disord. 1998;8(8):561–7.

    Article  CAS  Google Scholar 

  3. 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):386–90.

    Article  Google Scholar 

  4. Chiu HC, Vincent A, Newsom-Davis J, Hsieh KH, Hung T. Myasthenia gravis: population differences in disease expression and acetylcholine receptor antibody titers between Chinese and Caucasians Neurology. 1987;37(12):1854–7.

    Google Scholar 

  5. Andrews PI, Massey JM, Howard JF Jr, Sanders DB. Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis. Neurology. 1994 Jul;44(7):1208–14. https://doi.org/10.1212/wnl.44.7.1208.

    Article  CAS  Google Scholar 

  6. Anlar B, Ozdirim E, Renda Y, Yalaz K, Aysun S, Topçu M, Topaloğlu H. Myasthenia gravis in childhood. Acta Paediatr. 1996;85(7):838–42. https://doi.org/10.1111/j.1651-2227.1996.tb14162.x.

    Article  CAS  Google Scholar 

  7. Pevzner A, Schoser B, Peters K, Cosma NC, Karakatsani A, Schalke B, Melms A, Kröger S. Anti-LRP4 autoantibodies in AChR- and MuSK-antibody-negative myasthenia gravis. J Neurol. 2012;259(3):427–35. https://doi.org/10.1007/s00415-011-6194-7. Epub 2011 Aug 5

    Article  CAS  Google Scholar 

  8. Zhang B, Tzartos JS, Belimezi M, Ragheb S, Bealmear B, Lewis RA, Xiong WC, Lisak RP, Tzartos SJ, Mei L. Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis. Arch Neurol. 2012;69(4):445–51. https://doi.org/10.1001/archneurol.2011.2393. Epub 2011 Dec 12

    Article  Google Scholar 

  9. Hughes BW, Moro De Casillas ML, Kaminski HJ. Pathophysiology of myasthenia gravis. Semin Neurol. 2004;24(1):21–30. https://doi.org/10.1055/s-2004-829585.

    Article  Google Scholar 

  10. Huijbers MG, et al. MuSK myasthenia gravis. Proc Natl Acad Sci. 2013;110(51):20,783–8. https://doi.org/10.1073/pnas.1313944110.

    Article  CAS  Google Scholar 

  11. Limburg PC, The TH, Hummel-Tappel E, Oosterhuis HJ. Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1. Relation to clinical parameters in 250 patients. J Neurol Sci. 1983;58(3):357–70. https://doi.org/10.1016/0022-510x(83)90095-3.

    Article  CAS  Google Scholar 

  12. Andrews PI. Autoimmune myasthenia gravis in childhood. Semin Neurol. 2004;24(1):101–10. https://doi.org/10.1055/s-2004-829591.

    Article  CAS  Google Scholar 

  13. Afifi AK, Bell WE. Tests for juvenile myasthenia gravis: comparative diagnostic yield and prediction of outcome. J Child Neurol. 1993;8(4):403–11. https://doi.org/10.1177/088307389300800422.

    Article  CAS  Google Scholar 

  14. Batra P, Herrmann C Jr, Mulder D. Mediastinal imaging in myasthenia gravis: correlation of chest radiography, CT, MR, and surgical findings. AJR Am J Roentgenol. 1987;148(3):515–9. https://doi.org/10.2214/ajr.148.3.515.

    Article  CAS  Google Scholar 

  15. Pfeffer G, Povitz M. Respiratory management of patients with neuromuscular disease: current perspectives. Degener Neurol Neuromuscul Dis. 2016;6:111–8. Published 2016 Nov 18. https://doi.org/10.2147/DNND.S87323.

    Article  Google Scholar 

  16. Rønager J, Ravnborg M, Hermansen I, Vorstrup S. Immunoglobulin treatment versus plasma exchange in patients with chronic moderate to severe myasthenia gravis. Artif Organs. 2001;25(12):967–73. https://doi.org/10.1046/j.1525-1594.2001.06717.x.

    Article  Google Scholar 

  17. 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):2017–23. https://doi.org/10.1212/WNL.0b013e31821e5505.

    Article  CAS  Google Scholar 

  18. Misanovic V, Pokrajac D, Zubcevic S, et al. Plasmapheresis in Pediatric intensive care unit. Med Arch. 2016;70(5):332–5. https://doi.org/10.5455/medarh.2016.70.332-335.

    Article  Google Scholar 

  19. Peragallo JH. Pediatric myasthenia gravis. Semin Pediatr Neurol. 2017;24(2):116–21. https://doi.org/10.1016/j.spen.2017.04.003. Epub 2017 Apr 7.

    Article  Google Scholar 

  20. Ashraf VV, Taly AB, Veerendrakumar M, Rao S. Myasthenia gravis in children: a longitudinal study. Acta Neurol Scand. 2006;114(2):119–23. https://doi.org/10.1111/j.1600-0404.2006.00646.x.

    Article  CAS  Google Scholar 

  21. Seybold ME. Thymectomy in childhood myasthenia gravis. Ann N Y Acad Sci. 1998;841:731–41. https://doi.org/10.1111/j.1749-6632.1998.tb11010.x.

    Article  CAS  Google Scholar 

  22. Grob D, Brunner N, Namba T, Pagala M. Lifetime course of myasthenia gravis. Muscle Nerve. 2008;37(2):141–9.

    Article  Google Scholar 

  23. Mantegazza R, Baggi F, Antozzi C, Confalonieri P, Morandi L, Bernasconi P, Andreetta F, Simoncini O, Campanella A, Beghi E, Cornelio F. Myasthenia gravis (MG): epidemiological data and prognostic factors. Ann N Y Acad Sci. 2003;998:413–23. https://doi.org/10.1196/annals.1254.054.

    Article  Google Scholar 

  24. Rodriguez M, Gomez MR, Howard FM Jr, Taylor WF. Myasthenia gravis in children: long-term follow-up. Ann Neurol. 1983;13(5):504–10. https://doi.org/10.1002/ana.410130506.

    Article  CAS  Google Scholar 

  25. Finnis MF, Jayawant S. Juvenile myasthenia gravis: a paediatric perspective. Autoimmune Dis. 2011;2011:404101. https://doi.org/10.4061/2011/404101.

    Article  CAS  Google Scholar 

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Correspondence to Duygu Selcen .

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Christoffel, K., Selcen, D. (2022). Myasthenia Gravis. In: Kamat, D.M., Sivaswamy, L. (eds) Symptom-Based Approach to Pediatric Neurology . Springer, Cham. https://doi.org/10.1007/978-3-031-10494-7_34

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  • DOI: https://doi.org/10.1007/978-3-031-10494-7_34

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