Advertisement

Myasthenia Gravis

Chapter

Abstract

Myasthenia gravis is a rare, auto-immune disease marked by fatigable weakness of striated muscles. Both ocular and systemic forms of myasthenia are seen, so that intermittent, variable ptosis, diplopia, proximal muscle weakness, poor deglutition, and dyspnea are all encountered; however, as some myasthenics present (at least initially) with ptosis alone, the disease remains a diagnostic consideration in nearly all patients with ptosis who present for oculoplastic assessment. Diagnosis is aided by a history of diurnal, fatigable symptoms; characteristic examination findings; serological assays for myasthenia-related auto-antibodies; electromyography; and response to trial treatment. Diagnostic imaging, both to look for thymoma and to rule out neurosurgical disease, is commonly performed. Usual treatment options are acetylcholine potentiators and immunosuppression. Some patients, however, may require thymectomy, plasmapheresis, or respiratory support.

Keywords

Myasthenic Crisis Eyelid Retraction Chronic Progressive External Ophthalmoplegia Myasthenic Patient Chronic Progressive External Ophthalmoplegia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 1.
    Juel VC, Massey JM. Myasthenia gravis. Orphanet J Rare Dis. 2007;2:44. http://www.ojrd.com/content/2/1/44
  2. 2.
    von Noorden GK, Campos EC. Binocular vision and ocular motility. 6th ed. St. Louis, MO: Mosby; 2002. p. 102. http://telemedicine.orbis.org/bins/content_page.asp?cid=1-2193
  3. 3.
    Tindall RS. Humoral immunity in myasthenia gravis: clinical correlations of anti-receptor antibody avidity and titer. Ann N Y Acad Sci. 1981;377:316–31.CrossRefPubMedGoogle Scholar
  4. 4.
    Kupersmith MJ, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol. 2003;60(2):243–8.CrossRefPubMedGoogle Scholar
  5. 5.
    Ortiz S, Borchert M. Long-term outcomes of pediatric ocular myasthenia gravis. Ophthalmology. 2008;115(7):1245–8.CrossRefPubMedGoogle Scholar
  6. 6.
    Weinberg DA, Lesser RL, Vollmer TL. Ocular myasthenia: a protean disorder. Surv Ophthalmol. 1994;39(3):169–210.CrossRefPubMedGoogle Scholar
  7. 7.
    Lepore FE, Sanborn GE, Slevin JT. Pupillary dysfunction in myasthenia gravis. Ann Neurol. 1979;6(1):29–33.CrossRefPubMedGoogle Scholar
  8. 8.
    Glass JP, Halpern J, Grossman RG. Uncovering ocular pseudomyasthenia. Ann Ophthalmol. 1993;25(11):418–21.PubMedGoogle Scholar
  9. 9.
    Cogan DG. Myasthenia gravis: a review of the disease and a description of lid twitch as a characteristic sign. Arch Ophthalmol. 1965;74:217–21.PubMedGoogle Scholar
  10. 10.
    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.CrossRefPubMedGoogle Scholar
  11. 11.
    Borenstein S, Desmedt JE. Local cooling in myasthenia. Improvement of neuromuscular failure. Arch Neurol. 1975;32(3):152–7.PubMedGoogle Scholar
  12. 12.
    Mitrani RD, Kloosterman EM, Huikuri H, Dylewski J, Atapattu S, Interian Jr A, et al. Muscarinic receptor stimulation with edrophonium hydrochloride does not elevate ventricular fibrillation thresholds in humans. J Cardiovasc Electrophysiol. 1999;10(6):809–16.CrossRefPubMedGoogle Scholar
  13. 13.
    Arsura EL, Brunner NG, Namba T, Grob D. Adverse cardiovascular effects of anticholinesterase medications. Am J Med Sci. 1987;293(1):18–23.CrossRefPubMedGoogle Scholar
  14. 14.
    Hsu SY, Tsai RK, Wang HZ, Su MY. A comparative study of ocular and generalized myasthenia gravis. Kaohsiung J Med Sci. 2002;18(2):62–9.PubMedGoogle Scholar
  15. 15.
    Soliven BC, Lange DJ, Penn AS, Younger D, Jaretzki III A, Lovelace RE, et al. Seronegative myasthenia gravis. Neurology. 1988;38(4):514–7.PubMedGoogle Scholar
  16. 16.
    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.CrossRefPubMedGoogle Scholar
  17. 17.
    Lanska DJ. Diagnosis of thymoma in myasthenics using anti-striated muscle antibodies: predictive value and gain in diagnostic certainty. Neurology. 1991;41(4):520–4.PubMedGoogle Scholar
  18. 18.
    Kuks JB, Limburg PC, Horst G, Oosterhuis HJ. Antibodies to skeletal muscle in myasthenia gravis. Part 2. Prevalence in non-thymoma patients. J Neurol Sci. 1993;120(1):78–81.CrossRefPubMedGoogle Scholar
  19. 19.
    Sanders DB, El-Salem K, Massey JM, McConville J, Vincent A. Clinical aspects of MuSK antibody ­positive seronegative MG. Neurology. 2003;60(12):1978–80.PubMedGoogle Scholar
  20. 20.
    Caress JB, Hunt CH, Batish SD. Anti-MuSK myasthenia gravis presenting with purely ocular findings. Arch Neurol. 2005;62(6):1002–3.CrossRefPubMedGoogle Scholar
  21. 21.
    Hanisch F, Hain B, Zierz S. [Ocular involvement in MuSK antibody-positive myasthenia gravis]. Klin Monatsbl Augenheilkd. 2006;223(1):81–3.CrossRefPubMedGoogle Scholar
  22. 22.
    Luchanok U, Kaminski HJ. Ocular myasthenia: diagnostic and treatment recommendations and the evidence base. Curr Opin Neurol. 2008;21(1):8–15.CrossRefPubMedGoogle Scholar
  23. 23.
    Schwartz MS, Stålberg E. Myasthenic syndrome studied with single fiber electromyography. Arch Neurol. 1975;32(12):815–7.PubMedGoogle Scholar
  24. 24.
    Batra P, Herrmann Jr C, Mulder D. Mediastinal imaging in myasthenia gravis: correlation of chest radiography, CT, MR, and surgical findings. Am J Roentgenol. 1987;148(3):515–9.Google Scholar
  25. 25.
    Chagnac Y, Hadani M, Goldhammer Y. Myasthenic crisis after intravenous administration of iodinated contrast agent. Neurology. 1985;35:1219–20.PubMedGoogle Scholar
  26. 26.
    Ferrer X, Ellie E, Deleplanque B, Lagueny A, Julien J. [Myasthenic crisis after intravenous injection of iodine contrast product.]. Presse Méd. 1992;21:1127–8.PubMedGoogle Scholar
  27. 27.
    Tackenberg B, Hemmer B, Oertel WH, Sommer N. Immunosuppressive treatment of ocular myasthenia gravis. BioDrugs. 2001;15(6):369–78.CrossRefPubMedGoogle Scholar
  28. 28.
    Díaz-Manera J, Rojas-García R, Illa I. Treatment strategies for myasthenia gravis. Expert Opin Pharmacother. 2009;10(8):1329–42.CrossRefPubMedGoogle Scholar
  29. 29.
    Chaudhuri A, Behan PO. Myasthenic crisis. QJM. 2009;102(2):97–107.CrossRefPubMedGoogle Scholar
  30. 30.
    Kupersmith MJ, Ying G. Ocular motor dysfunction and ptosis in ocular myasthenia gravis: effects of treatment. Br J Ophthalmol. 2005;89(10):1330–4.CrossRefPubMedGoogle Scholar
  31. 31.
    Kupersmith MJ. Ocular myasthenia gravis: treatment successes and failures in patients with long-term follow-up. J Neurol. 2009;256(8):1314–20.CrossRefPubMedGoogle Scholar
  32. 32.
    Juel VC, Massey JM. Autoimmune myasthenia gravis: recommendations for treatment and immunologic modulation. Curr Treat Options Neurol. 2005;7(1):3–14.CrossRefPubMedGoogle Scholar
  33. 33.
    Hart IK, Sharshar T, Sathasivam S. Immunosuppressant drugs for myasthenia gravis. J Neurol Neurosurg Psychiatry. 2009;80(1):5–6.CrossRefPubMedGoogle Scholar
  34. 34.
    Chiang LM, Darras BT, Kang PB. Juvenile myasthenia gravis. Muscle Nerve. 2009;39(4):423–31.CrossRefPubMedGoogle Scholar
  35. 35.
    Zebardast N, Patwa HS, Novella SP, Goldstein JM. Rituximab in the management of refractory myasthenia gravis. Muscle Nerve. 2010;41(3):375–8.CrossRefPubMedGoogle Scholar
  36. 36.
    Sanders DB, Hart IK, Mantegazza R, Shukla SS, Siddiqi ZA, De Baets MH, et al. An international, phase III, randomized trial of mycophenolate mofetil in myasthenia gravis. Neurology. 2008;71(6):400–6.CrossRefPubMedGoogle Scholar
  37. 37.
    Muscle Study Group. A trial of mycophenolate mofetil with prednisone as initial immunotherapy in myasthenia gravis. Neurology. 2008;71(6):394–9.CrossRefGoogle Scholar
  38. 38.
    Chan JW. Mycophenolate mofetil for ocular myasthenia. J Neurol. 2008;255(4):510–3.CrossRefPubMedGoogle Scholar
  39. 39.
    Gold R, Schneider-Gold C. Current and future standards in treatment of myasthenia gravis. Neurotherapeutics. 2008;5(4):535–41.CrossRefPubMedGoogle Scholar
  40. 40.
    US Food and Drug Administration. Communication about an ongoing safety review of CellCept (mycophenolate mofetil) and Myfortic (mycophenolic acid). April 10, 2008. http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformationforPatientsandProviders/DrugSafetyInformationforHeathcareProfessionals/ucm072438.htm
  41. 41.
    Ponseti JM, Gamez J, Azem J, López-Cano M, Vilallonga R, Armengol M. Tacrolimus for myasthenia­ gravis: a clinical study of 212 patients. Ann N Y Acad Sci. 2008;1132:254–63.CrossRefPubMedGoogle Scholar
  42. 42.
    Lavrnic D, Vujic A, Rakocevic-Stojanovic V, Stevic Z, Basta I, Pavlovic S, et al. Cyclosporine in the treatment of myasthenia gravis. Acta Neurol Scand. 2005;111(4):247–52.CrossRefPubMedGoogle Scholar
  43. 43.
    Drachman DB, Adams RN, Hu R, Jones RJ, Brodsky RA. Rebooting the immune system with high-dose cyclophosphamide for treatment of refractory myasthenia gravis. Ann N Y Acad Sci. 2008;1132:305–14.CrossRefPubMedGoogle Scholar
  44. 44.
    Meyer DM, Herbert MA, Sobhani NC, Tavakolian P, Duncan A, Bruns M, et al. Comparative clinical outcomes of thymectomy for myasthenia gravis performed by extended transsternal and minimally invasive approaches. Ann Thorac Surg. 2009;87(2):385–90.CrossRefPubMedGoogle Scholar
  45. 45.
    Bradley EA, Bartley GB, Chapman KL, Waller RR. Surgical correction of blepharoptosis in patients with myasthenia gravis. Ophthal Plast Reconstr Surg. 2001;17:103–10.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Neuro-opthalmologist, Comprehensive Surgical Ophthalmologist, Private PracticeSudburyUSA

Personalised recommendations