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Long-term outcomes and prognostic factors in generalized myasthenia gravis

  • Original Communication
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Abstract

Objective

This study aimed to investigate the timing of meeting the criteria for a status of “minimal manifestation (MM) or better” and the factors that influenced whether “MM or better status” or “MM or better status with an oral prednisolone (PSL) dose of 5 mg/day or less (5-mg MM)” was met in patients with acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (MG).

Methods

We performed a retrospective study in 93 patients with AChR antibody-positive generalized MG who were followed for 3 years after the start of immunotherapy. We reviewed clinical data, such as MG-related symptoms, the MG activities of daily living profile (MGADL) score, immunotherapy including the dose of PSL, and achievement of the status of MM or better at baseline and 3, 6, 12, 24, and 36 months after treatment.

Results

An MM or better status was achieved in 60% of the patients 3 months and in 90% of the patients 2 years after initiating immunotherapy. At 2 years, 60% of the patients had achieved the treatment goal, which was an “5-mg MM”. More frequent plasmapheresis and higher dose of PSL within 3 months after immunotherapy initiation were associated with difficulty in achieving the 5-mg MM status at 2 years.

Conclusion

Approximately 60% of the MG patients achieved the treatment goal within 2 years after treatment. PSL dose and the cumulative number of plasmapheresis procedures at 3 months after immunotherapy initiation may help identify treatment-resistant patients with MG.

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Data availability

Any data not published within the article will be shared anonymized upon request from any qualified investigator.

Abbreviations

MG:

Myasthenia gravis

AChR:

Acetylcholine receptor

MGADL score:

MG activities of daily living score

PSL:

Prednisolone

IVIg:

Intravenous immunoglobulin

PP:

Plasmapheresis

CNIs:

Calcineurin inhibitors

IVMP:

High-dose intravenous methyl-prednisolone

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Acknowledgements

The author would like to thank Enago (www.enago.jp) for the English language review.

Funding

This work was supported in part by the Health and Labour Sciences Research Grant on Intractable Diseases (Neuroimmunological Diseases) from the Ministry of Health, Labour and Welfare of Japan (20FC1030).

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Authors and Affiliations

  1. Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670, Japan

    Yukiko Ozawa, Akiyuki Uzawa, Manato Yasuda, Yuta Kojima, Yosuke Onishi, Fumiko Oda, Tetsuya Kanai, Keiichi Himuro, Naoki Kawaguchi & Satoshi Kuwabara

  2. Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan

    Yuta Kojima

  3. Department of Neurology, National Hospital Organization Chiba Medical Center, Chiba, Japan

    Fumiko Oda

  4. Kanai Medical Clinic, Chiba, Japan

    Tetsuya Kanai

  5. Department of Neurology, Matsudo Neurology Clinic, Chiba, Japan

    Keiichi Himuro

  6. Department of Neurology, Neurology Clinic Chiba, Dowa Institute of Clinical Neuroscience, Chiba, Japan

    Naoki Kawaguchi

Authors
  1. Yukiko Ozawa
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  2. Akiyuki Uzawa
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  3. Manato Yasuda
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Contributions

YO, AU, and SK made substantial contribution to the conception and design of study. YO, AU, MY, YK, YOn, FO, TK, KH and NK collected the data. YO and TK conducted the statistical analysis. YO drafted the manuscript; All authors were involved in drafting the article or critically revising its important intellectual content. All authors have read and approved the final version of the manuscript..

Corresponding author

Correspondence to Akiyuki Uzawa.

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Conflicts of interest

The authors declare no financial or other conflicts of interest. All authors report no disclosures.

Ethical statement

The ethics committee of our institute approved this study (IRB No. 2266). The investigation conforms to the principles outlined in the Declaration of Helsinki in relation to the ethical principles for medical research involving human subjects.

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Ozawa, Y., Uzawa, A., Yasuda, M. et al. Long-term outcomes and prognostic factors in generalized myasthenia gravis. J Neurol 268, 3781–3788 (2021). https://doi.org/10.1007/s00415-021-10520-x

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  • DOI: https://doi.org/10.1007/s00415-021-10520-x

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