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Late-onset generalized myasthenia gravis: clinical features, treatment, and outcome

Abstract

Late-onset myasthenia gravis (LOMG) is a unique MG subgroup. More information is needed on its subgroups such as non-thymomatous generalized LOMG. We evaluated the effect of demographic, clinical, and serological factors as well as different immunosuppressive modalities on outcome in generalized non-thymomatous LOMG with onset ≥ 50 years. Myasthenia Gravis Foundation of America (MGFA) Clinical Classification, MGFA postintervention score (MGFA PIS) and MG Composite scores were obtained to define the severity of disease and clinical outcome. In 95 patients with generalized non-thymomatous LOMG, 60 (63%) were men, 45 (47%) had mild disease, 80 (84%) were anti-AChR, and 56 (61%) were anti-titin positive. In those who received immunosuppressives and provided the clinical scores (84 patients), 50 (60%) had favorable outcome (MGFA PIS categories of complete stable remission, pharmacological remission and minimal manifestations) at the end of 3 years. Use of prednisone + azathioprine had significantly positive effect on outcome. The presence of anti-titin antibodies had no significant effect on severity and outcome. Five anti-MuSK-positive patients had favorable outcome. In conclusion, the presence of neither anti-titin nor anti-MuSK antibodies points to unfavorable outcome. Prednisone and azathioprine combination has beneficial effects in non-thymomatous generalized LOMG.

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References

  1. 1.

    Donaldson DH et al (1990) The relationship of age to outcome in myasthenia gravis. Neurology 40(5):786–790

    CAS  Article  Google Scholar 

  2. 2.

    Slesak G et al (1998) Late-onset myasthenia gravis. Follow-up of 113 patients diagnosed after age 60. Ann N Y Acad Sci 841:777–780

    CAS  Article  Google Scholar 

  3. 3.

    Evoli A et al (2000) Clinical characteristics and prognosis of myasthenia gravis in older people. J Am Geriatr Soc 48(11):1442–1448

    CAS  Article  Google Scholar 

  4. 4.

    Deymeer F et al (2000) Juvenile and late-onset myasthenia gravis. In: Deymeer F (ed) Neuromuscular diseases: from basic mechanisms to clinical management. Karger, Basel, pp 113–127

    Chapter  Google Scholar 

  5. 5.

    Aarli JA et al (2003) Myasthenia gravis in individuals over 40. Ann N Y Acad Sci 998:424–431

    Article  Google Scholar 

  6. 6.

    Suzuki S et al (2011) Clinical and immunological differences between early and late-onset myasthenia gravis in Japan. J Neuroimmunol 230(1–2):148–152

    CAS  Article  Google Scholar 

  7. 7.

    Zivkovic SA et al (2012) Characteristics of late-onset myasthenia gravis. J Neurol 259(10):2167–2171

    Article  Google Scholar 

  8. 8.

    Suzuki S et al (2011) Three types of striational antibodies in myasthenia gravis. Autoimmune Dis 2011:740583

    PubMed  PubMed Central  Google Scholar 

  9. 9.

    Compston DA et al (1980) Clinical, pathological, HLA antigen and immunological evidence for disease heterogeneity in myasthenia gravis. Brain 103(3):579–601

    CAS  Article  Google Scholar 

  10. 10.

    Seldin MF et al (2015) Genome-wide association study of late-onset myasthenia gravis: confirmation of TNFRSF11A, and identification of ZBTB10 and three distinct HLA associations. Mol Med

  11. 11.

    Saruhan-Direskeneli G et al (2016) Genetic heterogeneity within the HLA region in three distinct clinical subgroups of myasthenia gravis. Clin Immunol 166–167:81–88

    Article  Google Scholar 

  12. 12.

    Santos E et al (2017) HLA and age of onset in myasthenia gravis. Neuromuscul Disord 27(7):650–654

    Article  Google Scholar 

  13. 13.

    Szczudlik P et al (2014) Antititin antibody in early- and late-onset myasthenia gravis. Acta Neurol Scand 130(4):229–233

    CAS  Article  Google Scholar 

  14. 14.

    Meriggioli MN (2009) Myasthenia gravis with anti-acetylcholine receptor antibodies. Front Neurol Neurosci 26:94–108

    CAS  Article  Google Scholar 

  15. 15.

    Aarli JA (1999) Late-onset myasthenia gravis: a changing scene. Arch Neurol 56(1):25–27

    CAS  Article  Google Scholar 

  16. 16.

    Gilhus NE et al (2015) Myasthenia gravis: subgroup classification and therapeutic strategies. Lancet Neurol 14(10):1023–1036

    CAS  Article  Google Scholar 

  17. 17.

    Benatar M et al (2012) Design of the efficacy of prednisone in the treatment of ocular myasthenia (EPITOME) trial. Ann N Y Acad Sci 1275:17–22

    CAS  Article  Google Scholar 

  18. 18.

    Jaretzki A 3rd et al (2000) Myasthenia gravis: recommendations for clinical research standards. Task Force of the Medical Scientific Advisory Board of the Myasthenia Gravis Foundation of America. Neurology 55(1):16–23

    Article  Google Scholar 

  19. 19.

    Burns TM et al (2008) Construction of an efficient evaluative instrument for myasthenia gravis: the MG composite. Muscle Nerve 38(6):1553–1562

    Article  Google Scholar 

  20. 20.

    Murai H et al (2011) Characteristics of myasthenia gravis according to onset-age: Japanese nationwide survey. J Neurol Sci 305(1–2):97–102

    Article  Google Scholar 

  21. 21.

    Palace J et al (1998) A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Myasthenia Gravis Study Group. Neurology 50(6):1778–1783

    CAS  Article  Google Scholar 

  22. 22.

    Romi F et al (2000) Muscle autoantibodies in subgroups of myasthenia gravis patients. J Neurol 247(5):369–375

    CAS  Article  Google Scholar 

  23. 23.

    Grob D et al (2008) Lifetime course of myasthenia gravis. Muscle Nerve 37(2):141–149

    Article  Google Scholar 

  24. 24.

    Nishikawa N et al (2015) Treatment of myasthenia gravis in patients with elderly onset at advanced age. Jpn Clin Med 6:9–13

    Article  Google Scholar 

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Acknowledgements

We thank Dr. Murat Kurtuncu for his help in statistical analysis.

Funding

The study was supported by the Scientific Research Project, Istanbul University (#34256).

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Contributions

SYC, HD, VY and GSD collected and integrated the data. SYC and FD conceived and designed the study. SYC, YGP, PSO and FD analyzed the data, wrote and reviewed the paper. All authors approved the final version of this paper for publication.

Corresponding author

Correspondence to Senay Yildiz Celik.

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The authors declare that they have no conflict of interest.

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All procedures performed in this study were in accordance with the ethical standards of the Medical Ethical Committee at the Istanbul Medical Faculty, Istanbul University (no.: 2013/95) and the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

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Yildiz Celik, S., Durmus, H., Yilmaz, V. et al. Late-onset generalized myasthenia gravis: clinical features, treatment, and outcome. Acta Neurol Belg 120, 133–140 (2020). https://doi.org/10.1007/s13760-019-01252-x

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Keywords

  • Myasthenia gravis
  • Late-onset
  • Elderly
  • Treatment