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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

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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.

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References

  1. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR (2005) IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 202:473–477

    Article  CAS  Google Scholar 

  2. Lennon VA, Wingerchuk DM, Kryzer TJ et al (2004) A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet 364:2106–2112

    Article  CAS  Google Scholar 

  3. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG (2007) The spectrum of neuromyelitis optica. Lancet Neurol 6:805–815

    Article  CAS  Google Scholar 

  4. Wingerchuk DM, Banwell B, Bennett JL et al (2015) International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology 85:177–189

    Article  Google Scholar 

  5. Bruscolini A, Sacchetti M, La Cava M et al (2018) Diagnosis and management of neuromyelitis optica spectrum disorders: an update. Autoimmun Rev 17:195–200

    Article  Google Scholar 

  6. Jiao Y, Cui L, Zhang W et al (2018) Dose effects of mycophenolate mofetil in Chinese patients with neuromyelitis optica spectrum disorders: a case series study. BMC Neurol 18:47

    Article  Google Scholar 

  7. Stellmann JP, Krumbholz M, Friede T et al (2017) Immunotherapies in neuromyelitis optica spectrum disorder: efficacy and predictors of response. J Neurol Neurosurg Psychiatry 88:639–647

    Article  Google Scholar 

  8. Houzen H, Kondo K, Niino M et al (2017) Prevalence and clinical features of neuromyelitis optica spectrum disorders in northern Japan. Neurology 89:1995–2001

    Article  Google Scholar 

  9. Kleiter I, Gahlen A, Borisow N et al (2016) Neuromyelitis optica: evaluation of 871 attacks and 1153 treatment courses. Ann Neurol 79:206–216

    Article  CAS  Google Scholar 

  10. Long Y, Liang J, Wu L et al (2017) Different phenotypes at onset in neuromyelitis optica spectrum disorder patients with aquaporin-4 autoimmunity. Front Neurol 8:62

    Article  Google Scholar 

  11. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG (2006) Revised diagnostic criteria for neuromyelitis optica. Neurology 66:1485–1489

    Article  CAS  Google Scholar 

  12. Kurtzke JF (1983) Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 33:1444–1452

    Article  CAS  Google Scholar 

  13. Palace J, Lin DY, Zeng D et al (2019) Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders. Brain 142:1310–1323

    Article  Google Scholar 

  14. Popescu BF, Lennon VA, Parisi JE et al (2011) Neuromyelitis optica unique area postrema lesions: nausea, vomiting, and pathogenic implications. Neurology 76:1229–1237

    Article  Google Scholar 

  15. Zhang W, Jiao Y, Cui L, Jiao J (2016) Differentiation of neuromyelitis optica spectrum disorders from ultra-longitudinally extensive transverse myelitis in a cohort of Chinese patients. J Neuroimmunol 291:96–100

    Article  CAS  Google Scholar 

  16. Shosha E, Dubey D, Palace J et al (2018) Area postrema syndrome: frequency, criteria, and severity in AQP4-IgG-positive NMOSD. Neurology 91:e1642–e1651

    Article  Google Scholar 

  17. Pirko I, Blauwet LA, Lesnick TG, Weinshenker BG (2004) The natural history of recurrent optic neuritis. Arch Neurol 61:1401–1405

    Article  Google Scholar 

  18. Sepulveda M, Blanco Y, Rovira A et al (2013) Analysis of prognostic factors associated with longitudinally extensive transverse myelitis. Mult Scler 19:742–748

    Article  Google Scholar 

  19. Weinshenker BG, Wingerchuk DM, Vukusic S et al (2006) Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 59:566–569

    Article  CAS  Google Scholar 

  20. Jonsson DI, Sveinsson O, Hakim R, Brundin L (2019) Epidemiology of NMOSD in Sweden from 1987 to 2013: a nationwide population-based study. Neurology 93:e181–e189

    Article  CAS  Google Scholar 

  21. Jarius S, Aboul-Enein F, Waters P et al (2008) Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. Brain 131:3072–3080

    Article  CAS  Google Scholar 

  22. Ketelslegers IA, Modderman PW, Vennegoor A, Killestein J, Hamann D, Hintzen RQ (2011) Antibodies against aquaporin-4 in neuromyelitis optica: distinction between recurrent and monophasic patients. Mult Scler 17:1527–1530

    Article  Google Scholar 

  23. Jarius S, Ruprecht K, Wildemann B et al (2012) Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflamm 9:14

    Article  CAS  Google Scholar 

  24. Petzold A, Woodhall M, Khaleeli Z et al (2019) Aquaporin-4 and myelin oligodendrocyte glycoprotein antibodies in immune-mediated optic neuritis at long-term follow-up. J Neurol Neurosurg Psychiatry 90:1021–1026

    Article  Google Scholar 

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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.

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Authors and Affiliations

  1. Department of Neurology, China-Japan Friendship Hospital, 2 Yinghua Dongjie, Hepingli, Beijing, 100029, China

    Weihe Zhang, Lei Cui, Yeqiong Zhang, Wei Wang, Renbin Wang, Zunjing Liu, Dantao Peng, Yujuan Jiao & Jinsong Jiao

Authors
  1. Weihe Zhang
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  2. Lei Cui
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  3. Yeqiong Zhang
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  4. Wei Wang
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  5. Renbin Wang
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  6. Zunjing Liu
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  7. Dantao Peng
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  8. Yujuan Jiao
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  9. Jinsong Jiao
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Corresponding authors

Correspondence to Yujuan Jiao or Jinsong Jiao.

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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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Zhang, W., Cui, L., Zhang, Y. et al. Questioning the existence of monophasic neuromyelitis optica spectrum disorder by defining a novel long-term relapse-free form from a large Chinese population. J Neurol 267, 1197–1205 (2020). https://doi.org/10.1007/s00415-019-09685-3

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  • DOI: https://doi.org/10.1007/s00415-019-09685-3

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