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Questioning the existence of monophasic neuromyelitis optica spectrum disorder by defining a novel long-term relapse-free form from a large Chinese population

  • Weihe Zhang
  • Lei Cui
  • Yeqiong Zhang
  • Wei Wang
  • Renbin Wang
  • Zunjing Liu
  • Dantao Peng
  • Yujuan JiaoEmail author
  • Jinsong JiaoEmail author
Original Communication
  • 22 Downloads

Abstract

Objective

To clarify the existence of monophasic neuromyelitis optica spectrum disorders (NMOSD) and to identify predictive factors of long-term relapse-free form.

Methods

We retrospectively analyzed 289 Chinese patients with NMOSD. Selected subjects were divided into three groups based on the time interval between disease onset and the first relapse, if any. Clinical and imaging data were acquired from each patient’s medical record and evaluated as predictive factors for NMOSD.

Results

In total, none of the participating patients exhibited a monophasic form of NMOSD. Rather, 241 patients were selected for relapse tendency analysis; 143 (59.3%) patients relapsed within the first year, 66 (27.4%) during 1–5 years, and 32 (13.3%) beyond 5 years. Such onset symptoms as optic neuritis (ON) and non-longitudinally extensive transverse myelitis (LETM) were independent prognostic factors for a prolonged remission interval (P < 0.05). The relapse rate was bi-modal for ON patients in the first year (47.9%) and beyond 5 years (24.0%) after disease onset, respectively. However, most TM and area postrema syndrome (APS) patients experienced an attack within the first year (61.3% for TM and 76.9% for APS). A survival analysis showed that attacks with APS (P < 0.0001) and TM (P < 0.05) have a significantly higher risk of early relapse than with ON and that seropositive aquaporin-4 antibody may shorten the relapse interval for all onset symptoms (P < 0.0001).

Conclusions

Our study indicated that the monophasic form of NMOSD may not exist when a sufficient follow-up period is considered. Onset phenotypes with ON, non-APS, or non-LETM attacks had a lower risk of early relapse.

Keywords

Neuromyelitis optica spectrum disorders Monophasic Relapse Predictive factors Follow-up Chinese 

Notes

Acknowledgements

The authors would like to thank the physicians from the referring hospitals for providing the valuable case and image information.

Funding

This study had no targeted funding.

Compliance with ethical standards

Conflicts of interest

The authors declare that there are no potential competing interests among the authors and/or other organizations. The authors declare no financial or other conflicts of interest.

Ethical standards

This study was approved by the Institutional Review Board of China-Japan Friendship Hospital, and the need for informed consent was waived by the committee because of the study’s retrospective nature.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2020

Authors and Affiliations

  1. 1.Department of NeurologyChina-Japan Friendship HospitalBeijingChina

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