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Distinct clinical characteristics of atypical optic neuritis with seronegative aquaporin-4 antibody among Chinese patients
  1. Huanfen Zhou1,2,
  2. Quangang Xu1,
  3. Shuo Zhao3,
  4. Wei Wang4,
  5. Junqing Wang1,
  6. Zhiye Chen5,
  7. Dahe Lin6,
  8. Xiaoming Li1,
  9. Chunxia Peng1,
  10. Nanping Ai1,
  11. Shihui Wei1
  1. 1 Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China
  2. 2 Department of Ophthalmology, The first Affiliated Hospital of Chinese PLA General Hospital, Beijing, China
  3. 3 Department of Ophthalmology, Beijing Hospital, National Center of Gerontology, Beijing, China
  4. 4 State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou, China
  5. 5 Department of Radiodiagnosis, Chinese PLA General Hospital, Beijing, China
  6. 6 Fujian Provincial Key Laboratory of Ecology-Toxicological Effects and Control Techniques of Emerging Contaminants, College of Environmental and Biological Engineering, Putian University, Fujian, China
  1. Correspondence to Dr Shihui Wei, Department of Ophthalmology, Chinese PLA General Hospital, Fuxing Road No.28, Haidian district, Beijing, China; docwsh301{at}


Objective To evaluate the clinical features and prognosis of atypical optic neuritis (ON) with seronegative aquaporin-4 (AQP4) antibody in Chinese patients.

Methods All patients with first or relapsing ON were recruited from the Neuro-ophthalmology Department of the Chinese People’s Liberation Army General Hospital from January 2013 to December 2014 and assigned to one of three groups based on diagnosis: atypical ON, typical ON and neuromyelitis optica spectrum disorder (NMOSD)-ON.

Results A total of 173 patients were included in the cohort. Fifty patients (28.9%) were AQP4-Ab-positive and diagnosed with NMOSD-ON. Of 123 patients with seronegative AQP4-Ab, 37 (30.1%) patients had atypical ON, with male predominance (25, 67.6%). The atypical ON group (compared with the typical ON and NMOSD-ON groups) had a significantly lower female:male ratio (1:2.1 vs 1.8:1 and 9:1, respectively, p=0.001 and p<0.001), an older mean age of onset (44.8, 13–71 years vs 36.9, 13–73 years and 36.2, 13–66 years, p=0.003 and p=0.004), a lower rate of good (≥0.5) visual recovery (6.7% vs 79.8% and 30.9%, p<0.001 and p<0.001) and (compared with the NMOSD-ON group) a lower recurrence rate during a 2-year follow-up (29.3% vs 60%, p=0.009). However, none developed to multiple sclerosis or neuromyelitis optica in the atypical ON group.

Conclusions Atypical ON with seronegative AQP4-Ab had unique clinical features in this Chinese cohort, including male predominance, an older age of onset, worse visual acuity recovery and resistance to corticosteroid therapy. This condition may be a distinct nosological entity with an unusual clinical and therapeutic profile.

  • Optic neuritis
  • neuromyelitis optica
  • multiple sclerosis
  • prevalence
  • aetiology

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  • Contributors HZ, QX and SZ are co-first authors and contributed equally. Design and conduct of the study by HZ, SZ, QX and SW.Collection, analysis, management and interpretation of the data by HZ, WW, JW,ZC, DL, XL, CP and NA. Preparation of the manuscript by HZ, SZ and QX. Critical revision of the manuscript was performed by HZ and WW. Review and final approval of the manuscript by all the authors.

  • Funding This study was supported by the 863 Plan Biological andMedical Technology project of China (NO: 2015AA020511).

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval This study was approved by the Chinese PLAGH Ethics Committee and was conducted following the Declaration of Helsinki inits currently applicable version and applicable Chinese laws.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement The data involved in this study are owned by the Department of Ophthalmology of Chinese PLA General Hospital. If you want to share the data, please contact to Prof Shihui Wei (