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Generalization after ocular onset in myasthenia gravis: a case series in Germany

Abstract

Background and purpose

Approximately, 50% of myasthenia gravis (MG) patients initially present with purely ocular symptoms. Of these, about 60% will develop secondary generalized MG, typically within 2 years. Risk factors for secondary generalization are still controversial. In this study, we reviewed clinical parameters, thymic pathologies and medical treatments of MG patients with purely ocular symptoms at onset to investigate risk factors for secondary generalization.

Methods

In this monocentric retrospective study, we reviewed consecutive patients who underwent robotic thymectomy between January 2003 and October 2017 in Charite Universitaetsmedizin Berlin. We used univariate and multivariate Cox proportional hazards regression models to identify factors associated with secondary generalization. Survival curves were plotted using Kaplan–Meier method and log-rank tests were performed to analyze the association between corticosteroids use and secondary generalization in subgroups defined by anti-AChR antibody status and thymic pathology.

Results

One hundred and eighty of 572 MG patients who underwent robotic thymectomy were eligible for inclusion, of whom 110 (61.1%) developed a secondary generalized MG over a mean follow-up time of 23.6 months. The presence of a thymoma (HR 1.659, 95% CI (1.52–2.617), P = 0.029) was the only risk factor for secondary generalization in our series. Treating with corticosteroids was associated with a lower conversion rate in ocular myasthenia patients with thymic hyperplasia (n = 55, P = 0.028), but not with other thymic pathologies including thymoma and normal or atrophic thymus.

Conclusions

The conversion rate in ocular myasthenia was high in our series, predicted by the presence of a thymoma. Our findings suggest that corticosteroids can prevent secondary generalization in ocular myasthenia patients with thymic hyperplasia, which requires further research.

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Acknowledgements

We are thankful to Dr. Alexandra McAleenan, Senior Research Associate in Research Synthesis, University of Bristol for her assistance in editing this manuscript.

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Correspondence to Jens-C. Rückert.

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The authors declare no financial or other conflicts of interest.

Ethical standards

This research was approved by Ethics Committee of Charite Universitaetsmedizin Berlin (Berlin, Germany).

Informed consent

All patients gave their informed consent prior to their inclusion in the study.

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Li, F., Hotter, B., Swierzy, M. et al. Generalization after ocular onset in myasthenia gravis: a case series in Germany. J Neurol 265, 2773–2782 (2018). https://doi.org/10.1007/s00415-018-9056-8

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Keywords

  • Ocular myasthenia
  • Secondary generalization
  • Risk factor
  • Corticosteroids
  • Thymectomy