Skip to main content
SpringerLink
Log in

Myasthenia Gravis

  • Chapter
  • First Online:
Neuromuscular Disorders

Abstract

Myasthenia gravis is the most common immunological disease of the neuromuscular junction. It affects young females and elderly males and is associated with other immunological diseases. A proportion of patients have thymoma. External ocular muscles, eyelids, muscles of mastication and swallowing are preferentially affected by the disease. Myasthenic crisis forms a medical emergency and urgent intensive therapy is required. The course is fluctuating; remissions and exacerbations are known. The type of antibody has some bearing on the presentation, course and therapy options. Long-term immunosuppression and medical monitoring are required for most patients. Advances in the intensive care and judicious use of available options in immunosuppression have improved the quality of life.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution

Institutional subscriptions

References

  • Andersen JB, Erik Gilhus N, Sanders DB. Factors affecting outcome in myasthenia gravis. Muscle Nerve. 2016;54(6):1041–9.

    Article  CAS  PubMed  Google Scholar 

  • Barnett C, Katzberg H, Nabavi M, Bril V. The quantitative myasthenia gravis score: comparison with clinical, electrophysiological, and laboratory markers. J Clin Neuromuscul Dis. 2012;13(4):201–5.

    Article  PubMed  Google Scholar 

  • Bedlack RS, Simel DL, Bosworth H, Samsa G, Tucker-Lipscomb B, Sanders DB. Quantitative myasthenia gravis score: assessment of responsiveness and longitudinal validity. Neurology. 2005;64(11):1968–70.

    Article  CAS  PubMed  Google Scholar 

  • Benatar M. A systematic review of diagnostic studies in myasthenia gravis. Neuromuscul Disord. 2006;16:459–67.

    Article  PubMed  Google Scholar 

  • Binks S, Vincent A, Palace J. Myasthenia gravis: a clinical-immunological update. J Neurol. 2016;263(4):826–34.

    Article  CAS  PubMed  Google Scholar 

  • Bou Ali H, Salort-Campana E, Grapperon AM, Gallard J, Franques J, Sevy A, Delmont E, Verschueren A, Pouget J, Attarian S. New strategy for improving the diagnostic sensitivity of repetitive nerve stimulation in myasthenia gravis. Muscle Nerve. 2017;55(4):532–8.

    Article  PubMed  Google Scholar 

  • Bouwyn JP, Magnier P, Bédat-Millet AL, Ahtoy P, Maltête D, Lefaucheur R. Anti-MuSK myasthenia gravis with prolonged remission. Neuromuscul Disord. 2016;26(7):453–4.

    Article  PubMed  Google Scholar 

  • Burns TM, Sanders DB, Kaminski HJ, Wolfe GI, Narayanaswami P, Venitz J. Two steps forward, one step back: mycophenolate mofetil treatment for myasthenia gravis in the United States. Muscle Nerve. 2015;51(5):635–7.

    Article  CAS  PubMed  Google Scholar 

  • Carr AS, Cardwell CR, McCarron PO, et al. A systematic review of population based epidemiological studies in myasthenia gravis. BMC Neurol. 2010;18(10):46.

    Article  Google Scholar 

  • Chen P, Feng H, Deng J, Luo Y, Qiu L, Ou C, Liu W. Leflunomide treatment in corticosteroid-dependent myasthenia gravis: an open-label pilot study. J Neurol. 2016;263(1):83–8.

    Article  CAS  PubMed  Google Scholar 

  • Costa J, Evangelista T, Conceição I, de Carvalho M. Repetitive nerve stimulation in myasthenia gravis-relative sensitivity of different muscles. Clin Neurophysiol. 2004;115:2776–82.

    Article  PubMed  Google Scholar 

  • Cruz JL, Wolff ML, Vanderman AJ, Brown JN. The emerging role of tacrolimus in myasthenia gravis. Ther Adv Neurol Disord. 2015;8(2):92–103.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Dalakas MC. Future perspectives in target-specific immunotherapies of myasthenia gravis. Ther Adv Neurol Disord. 2015;8(6):316–27.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • De Feo LG, Schottlender J, Martelli NA, Molfino NA. Use of intravenous pulsed cyclophosphamide in severe, generalized myasthenia gravis. Muscle Nerve. 2002;26(1):31–6.

    Article  PubMed  Google Scholar 

  • Díaz-Manera J, Rojas García R, Illa I. Treatment strategies for myasthenia gravis: an update. Expert Opin Pharmacother. 2012;13(13):1873–83.

    Article  PubMed  Google Scholar 

  • Drachman DB. Myasthenia gravis. Semin Neurol. 2016a;36(5):419–24.

    Article  PubMed  Google Scholar 

  • Drachman DB. Treatment for refractory myasthenia gravis-new lymphs for old. JAMA Neurol. 2016b;73(6):624–6.

    Article  PubMed  Google Scholar 

  • El-Salem K, Yassin A, Al-Hayk K, Yahya S, Al-Shorafat D, Dahbour SS. Treatment of MuSK-associated myasthenia gravis. Curr Treat Options Neurol. 2014;16(4):283.

    Article  PubMed  Google Scholar 

  • Farrugia ME, Vincent A. Autoimmune mediated neuromuscular junction defects. Curr Opin Neurol. 2010;23(5):489–95.

    Article  CAS  PubMed  Google Scholar 

  • Fitzgerald MG, Shafritz AB. Distal myasthenia gravis. J Hand Surg Am. 2014;39(7):1419–20.

    Article  PubMed  Google Scholar 

  • Gajdos P, Chevret S, Toyka K. Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev. 2002;4:CD002275.

    Google Scholar 

  • Gajdos P, Chevret S, Toyka KV. Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev. 2012;12:CD002277.

    PubMed  Google Scholar 

  • Gilhus NE, Verschuuren JJ. Myasthenia gravis: subgroup classification and therapeutic strategies. Lancet Neurol. 2015;14(10):1023–36.

    Article  CAS  PubMed  Google Scholar 

  • Gilhus NE, Skeie GO, Romi F, Lazaridis K, Zisimopoulou P, Tzartos S. Myasthenia gravis - autoantibody characteristics and their implications for therapy. Nat Rev Neurol. 2016;12(5):259–68.

    Article  CAS  PubMed  Google Scholar 

  • Gotterer L, Li Y. Maintenance immunosuppression in myasthenia gravis. J Neurol Sci. 2016;369:294–302.

    Article  PubMed  Google Scholar 

  • Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve. 2011;44(1):36–40.

    Article  PubMed  Google Scholar 

  • Ha JC, Richman DP. Myasthenia gravis and related disorders: pathology and molecular pathogenesis. Biochim Biophys Acta. 2015;1852(4):651–7.

    Article  CAS  PubMed  Google Scholar 

  • Hehir MK, Burns TM, Alpers J, Conaway MR, Sawa M, Sanders DB. Mycophenolate mofetil in AChR-antibody-positive myasthenia gravis: outcomes in 102 patients. Muscle Nerve. 2010;41(5):593–8.

    Article  CAS  PubMed  Google Scholar 

  • https://www.uptodate.com/contents/clinical-manifestations-of-myasthenia-gravis

  • Hughes T. The early history of myasthenia gravis. Neuromuscul Disord. 2005;15(12):878–86.

    Article  PubMed  Google Scholar 

  • Iorio R, Damato V, Alboini PE, Evoli A. Efficacy and safety of rituximab for myasthenia gravis: a systematic review and meta-analysis. J Neurol. 2015;262(5):1115–9.

    Article  CAS  PubMed  Google Scholar 

  • Jayam Trouth A, Dabi A, Solieman N, Kurukumbi M, Kalyanam J. Myasthenia gravis: a review. Autoimmune Dis. 2012;2012:874680, 10 pages.

    PubMed  PubMed Central  Google Scholar 

  • Jolly F. UeberMyasthenia gravis pseudoparalytica, vol. 32. Berlin: Klin Wochenschr; 1895.

    Google Scholar 

  • Juel VC. Evaluation of neuromuscular junction disorders in the electromyography laboratory. Neurol Clin. 2012;30(2):621–39.

    Article  PubMed  Google Scholar 

  • Juel VC, Massey JM. Myasthenia gravis. Orphanet J Rare Dis. 2007;2:44.

    Article  PubMed  PubMed Central  Google Scholar 

  • Kalita J, Kohat AK, Misra UK. Predictors of outcome of myasthenic crisis. Neurol Sci. 2014;35(7):1109–14.

    Article  CAS  PubMed  Google Scholar 

  • Kerty E, Elsais A, Argov Z, Evoli A, Gilhus NE. EFNS/ENS guidelines for the treatment of ocular myasthenia. Eur J Neurol. 2014;21(5):687–93.

    Article  CAS  PubMed  Google Scholar 

  • Khadilkar SV, Chaudhari CR, Patil TR, Desai ND, Jagiasi KA, Bhutada AG. Once myasthenic, always myasthenic? Observations on the behavior and prognosis of myasthenia gravis in a cohort of 100 patients. Neurol India. 2014;62(5):492–7.

    Article  PubMed  Google Scholar 

  • Kubis KC, Danesh-Meyer HV, Savino PJ, Sergott RC. The ice test versus the rest test in myasthenia gravis. Ophthalmology. 2000;107(11):1995–8.

    Article  CAS  PubMed  Google Scholar 

  • Kumar R, Nagappa M, Sinha S, Taly AB, Rao S. MG-QoL-15 scores in treated myasthenia gravis: experience from a university hospital in India. Neurol India. 2016;64(3):405–10.

    Article  PubMed  Google Scholar 

  • Larner AJ. The place of the ice pack test in the diagnosis of myasthenia gravis. Int J Clin Pract. 2004a;58(9):887–8.

    Article  CAS  PubMed  Google Scholar 

  • Larner AJ. The place of the ice pack test in the diagnosis of myasthenia gravis. Int J Clin Pract. 2004b;58(9):887–8.

    Article  CAS  PubMed  Google Scholar 

  • Mantegazza R, Antozzi C, Peluchetti D, Sghirlanzoni A, Cornelio F. Azathioprine as a single drug or in combination with steroids in the treatment of myasthenia gravis. J Neurol. 1988;235(8):449–53.

    Article  CAS  PubMed  Google Scholar 

  • Melzer N, Ruck T, Fuhr P, Gold R, Hohlfeld R, Marx A, Melms A, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F, Meuth SG, Wiendl H. Clinical features, pathogenesis, and treatment of myasthenia gravis: a supplement to the guidelines of the German Neurological Society. J Neurol. 2016;263(8):1473–94.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Murthy JM. Thymectomy in myasthenia gravis. Neurol India. 2009;57(4):363–5.

    Article  CAS  PubMed  Google Scholar 

  • Murthy JM, Meena AK, Chowdary GV, Naryanan JT. Myasthenic crisis: clinical features, complications and mortality. Neurol India. 2005;53(1):37–40.

    Article  CAS  PubMed  Google Scholar 

  • Nagappa M, Netravathi M, Taly AB, Sinha S, Bindu PS, Mahadevan A. Long-term efficacy and limitations of cyclophosphamide in myasthenia gravis. J Clin Neurosci. 2014;21(11):1909–14.

    Article  CAS  PubMed  Google Scholar 

  • Nastuk WL, Strauss AJL, Osserman KE. Search for a neuromuscular blocking agent in the blood of patients with myasthenia gravis. Am J Med. 1959;26(3):394–409.

    Article  CAS  PubMed  Google Scholar 

  • Nikolić AV, Bačić GG, Daković MŽ, Lavrnić SĐ, Rakočević Stojanović VM, Basta IZ, Lavrnić DV. Myopathy, muscle atrophy and tongue lipid composition in MuSK myasthenia gravis. Acta Neurol Belg. 2015;115(3):361–5.

    Article  PubMed  Google Scholar 

  • Oger J, Frykman H. An update on laboratory diagnosis in myasthenia gravis. Clin Chim Acta. 2015;449:43–8.

    Article  CAS  PubMed  Google Scholar 

  • Pascuzzi RM. The history of myasthenia gravis. Neurol Clin. 1994;12(2):231–42.

    CAS  PubMed  Google Scholar 

  • Pascuzzi RM. The edrophonium test. Semin Neurol. 2003;23(1):83–8.

    Article  PubMed  Google Scholar 

  • Pascuzzi RM, Coslett HB, Johns TR. Long-term corticosteroid treatment of myasthenia gravis: report of 116 patients. Ann Neurol. 1984;15(3):291–8.

    Article  CAS  PubMed  Google Scholar 

  • Pasnoor M, He J, Herbelin L, Burns TM, Nations S, Bril V, Wang AK, Elsheikh BH, Kissel JT, Saperstein D, Shaibani JA, Jackson C, Swenson A, Howard JF Jr, Goyal N, David W, Wicklund M, Pulley M, Becker M, Mozaffar T, Benatar M, Pazcuzzi R, Simpson E, Rosenfeld J, Dimachkie MM, Statland JM, Barohn RJ, Methotrexate in MG Investigators of the Muscle Study Group. A randomized controlled trial of methotrexate for patients with generalized myasthenia gravis. Neurology. 2016;87(1):57–64.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Patil SA, Bokoliya SC, Nagappa M, Taly AB. Diagnosis of myasthenia gravis: comparison of anti-nicotinic acetyl choline receptor antibodies, repetitive nerve stimulation and neostigmine tests at a tertiary neuro care centre in India, a tenyear study. J Neuroimmunol. 2016a;292:81–4.

    Article  CAS  PubMed  Google Scholar 

  • Patil SA, Bokoliya SC, Nagappa M, Taly AB. Diagnosis of myasthenia gravis: comparison of anti-nicotinic acetyl choline receptor antibodies, repetitive nerve stimulation and neostigmine tests at a tertiary neuro care centre in India, a ten year study. J Neuroimmunol. 2016b;292:81–4.

    Article  CAS  PubMed  Google Scholar 

  • Patrick J, Lindstrom J. Autoimmune response to acetylcholine receptor. Science. 1973;180(4088):871–2.

    Article  CAS  PubMed  Google Scholar 

  • Romi F. Thymoma in myasthenia gravis: from diagnosis to treatment. Autoimmune Dis. 2011;2011:474512.

    PubMed  PubMed Central  Google Scholar 

  • Sanders DB, Siddiqi ZA. Lessons from two trials of mycophenolate mofetil in myasthenia gravis. Ann N Y Acad Sci. 2008;1132:249–53.

    Article  CAS  PubMed  Google Scholar 

  • Sanders DB, Wolfe GI, Benatar M, Evoli A, Gilhus NE, Illa I, Kuntz N, Massey JM, Melms A, Murai H, Nicolle M, Palace J, Richman DP, Verschuuren J, Narayanaswami P. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016;87(4):419–25.

    Article  PubMed  PubMed Central  Google Scholar 

  • Shah PA, Wadia PM. Reversible man-in-the-barrel syndrome in myasthenia gravis. Ann Indian Acad Neurol. 2016;19(1):99–101.

    Article  PubMed  PubMed Central  Google Scholar 

  • Sharma S, Lal V, Prabhakar S, Agarwal R. Clinical profile and outcome of myasthenic crisis in a tertiary care hospital: a prospective study. Ann Indian Acad Neurol. 2013;16(2):203–7.

    Article  PubMed  PubMed Central  Google Scholar 

  • Silvestri NJ, Wolfe GI. Myasthenia gravis. Semin Neurol. 2012;32(3):215–26.

    Article  PubMed  Google Scholar 

  • Silvestri NJ, Wolfe GI. Treatment-refractory myasthenia gravis. J Clin Neuromuscul Dis. 2014a;15(4):167–78.

    Article  PubMed  Google Scholar 

  • Silvestri NJ, Wolfe GI. Treatment-refractory myasthenia gravis. J Clin Neuromuscul Dis. 2014b;15(4):167–78.

    Article  PubMed  Google Scholar 

  • Simpson JA. Myasthenia gravis, a new hypothesis. Scott Medical. 1960;5:419–36.

    Article  Google Scholar 

  • Singhal BS, Bhatia NS, Umesh T, Menon S. Myasthenia gravis: a study from India. Neurol India. 2008;56(3):352–5.

    Article  CAS  PubMed  Google Scholar 

  • Skeie GO, Apostolski S, Evoli A, Gilhus NE, Illa I, Harms L, Hilton-Jones D, Melms A, Verschuuren J, Horge HW, European Federation of Neurological Societies. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010;17(7):893–902.

    Article  CAS  PubMed  Google Scholar 

  • Soliven B, Rezania K, Gundogdu B, Harding-Clay B, Oger J, Arnason BG. Terbutaline in myasthenia gravis: a pilot study. J Neurol Sci. 2009;277(1–2):150–4.

    Article  CAS  PubMed  Google Scholar 

  • Sudulagunta SR, Sepehrar M, Sodalagunta MB, Settikere Nataraju A, Bangalore Raja SK, Sathyanarayana D, Gummadi S, Burra HK. Refractory myasthenia gravis - clinical profile, comorbidities and response to rituximab. Ger Med Sci. 2016;14:Doc12.

    PubMed  PubMed Central  Google Scholar 

  • Tindall RS, Phillips JT, Rollins JA, Wells L, Hall K. A clinical therapeutic trial of cyclosporine in myasthenia gravis. Ann N Y Acad Sci. 1993;681:539–51.

    Article  CAS  PubMed  Google Scholar 

  • Vaphiades MS, Bhatti MT, Lesser RL. Ocular myasthenia gravis. Curr Opin Ophthalmol. 2012;23(6):537–42.

    Article  PubMed  Google Scholar 

  • Vincent A. Unravelling the pathogenesis of myasthenia gravis. Nat Rev Immunol. 2002;2(10):797–804.

    Article  CAS  PubMed  Google Scholar 

  • Vincent A, Leite MI. Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravis. Curr Opin Neurol. 2005;18(5):519–25.

    Article  CAS  PubMed  Google Scholar 

  • Vincent A, Palace J, Hilton-Jones D. Myasthenia gravis. Lancet. 2001;357(9274):2122–8.

    Article  CAS  PubMed  Google Scholar 

  • Walker MB. Case showing the effect of prostigmin on myasthenia gravis. J R Soc Med. 1935;28:759–61.

    CAS  Google Scholar 

  • Werner P, Kiechl S, Löscher W, Poewe W, Willeit J. Distal myasthenia gravis frequency and clinical course in a large prospective series. Acta Neurol Scand. 2003;108(3):209–11.

    Article  CAS  PubMed  Google Scholar 

  • Witoonpanich R, Dejthevaporn C, Sriphrapradang A, Pulkes T. Electrophysiological and immunological study in myasthenia gravis: diagnostic sensitivity and correlation. Clin Neurophysiol. 2011;122:1873–7.

    Article  PubMed  Google Scholar 

  • Witte AS, Cornblath DR, Parry GJ, Lisak RP, Schatz NJ. Azathioprine in the treatment of myasthenia gravis. Ann Neurol. 1984;15(6):602–5.

    Article  CAS  PubMed  Google Scholar 

  • Wolfe GI, Kaminski HJ, Aban IB, Minisman G, Kuo HC, Marx A, Ströbel P, Mazia C, Oger J, Cea JG, Heckmann JM, Evoli A, Nix W, Ciafaloni E, Antonini G, Witoonpanich R, King JO, Beydoun SR, Chalk CH, Barboi AC, Amato AA, Shaibani AI, Katirji B, Lecky BR, Buckley C, Vincent A, Dias-Tosta E, Yoshikawa H, Waddington-Cruz M, Pulley MT, Rivner MH, Kostera-Pruszczyk A, Pascuzzi RM, Jackson CE, Garcia Ramos GS, Verschuuren JJ, Massey JM, Kissel JT, Werneck LC, Benatar M, Barohn RJ, Tandan R, Mozaffar T, Conwit R, Odenkirchen J, Sonett JR, Jaretzki A 3rd, Newsom-Davis J, Cutter GR, MGTX Study Group. Randomized trial of thymectomy in myasthenia gravis. N Engl J Med. 2016;375(6):511–22.

    Article  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Bombay Hospital Institute of Medical Sciences, Mumbai, Maharashtra, India

    Satish V. Khadilkar

  2. Consultant Neurologist, Suyog Neurology Clinic, NH Multispeciality Hospital, Ahmedabad, Gujarat, India

    Rakhil S. Yadav

  3. Consultant Neurologist, TN Medical College and Nair Hospital, Mumbai, Maharashtra, India

    Bhagyadhan A. Patel

Authors
  1. Satish V. Khadilkar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Rakhil S. Yadav
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Bhagyadhan A. Patel
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer Nature Singapore Pte Ltd.

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Khadilkar, S.V., Yadav, R.S., Patel, B.A. (2018). Myasthenia Gravis. In: Neuromuscular Disorders. Springer, Singapore. https://doi.org/10.1007/978-981-10-5361-0_22

Download citation

  • DOI: https://doi.org/10.1007/978-981-10-5361-0_22

  • Published:

  • Publisher Name: Springer, Singapore

  • Print ISBN: 978-981-10-5360-3

  • Online ISBN: 978-981-10-5361-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation