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Distinct clinical characteristics of paraneoplastic optic neuropathy
  1. Quangang Xu1,
  2. Wenjuan Du2,
  3. Huanfen Zhou1,
  4. Xin Zhang3,
  5. Hongjuan Liu1,
  6. Honglu Song1,
  7. Xueqiong Wang4,
  8. Shihui Wei1
  1. 1 Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China
  2. 2 Medical Administration Division, Chinese PLA General Hospital, Beijing, China
  3. 3 Department of Ophthalmology, The First Affiliated Hospital of Zhejiang University of Traditional Chinese Medicine, Hangzhou, China
  4. 4 Department of Ophthalmology, The First People's Hospital of Lanzhou, Lanzhou, China
  1. Correspondence to Dr Quangang Xu, Department of Ophthalmology, Chinese PLA General Hospital, Beijing 28, China; xuquangang{at}


Objective Paraneoplastic optic neuropathy (PON) is relatively uncommon, and the visual outcomes and prognosis of this disease have not been well documented. The aim of this study was to investigate the clinical features and prognosis of antibody-mediated PON.

Methods Clinical data were retrospectively collected from hospitalised patients diagnosed with PON at the Neuro-Ophthalmology Department at the Chinese People’s Liberation Army General Hospital from January 2015 to June 2017.

Results A total of seven patients (four females and three males, 13 involved eyes) were included with a mean age of 56.28±11.32 years (36–70 years). Simultaneous or early sequential bilateral eye involvement (5/7, 71.4%) was common in the patients with PON. Severe vision loss (≤0.1) was seen in 76.9% (10/13) of the eyes. There were 13 eyes in the acute phase of the disease, and six eyes presented with optic disc oedema. All patients had definite evidence of paraneoplastic-associated antibodies (three with serum positive for antiamphilphysin, one for anti-PNMA2 (Ma2/Ta), one for anti-Yo, one for anti-Ma2 and one for anti-CV2). All of the serum samples were negative for myelin oligodendrocyte glycoprotein antibody and two patients companied with seropositive for the aquaporin-4 antibody. Five patients had history of primary malignancy, including thyroid cancer, type B thymoma, testicular seminoma, cervical cancer and lung carcinoma. Two patients had positive paraneoplastic syndrome antibodies (anti-Yo and antiamphiphysin), but the solid tumour had not been found through a PET scan. Visual acuity in 9/13 (69.2%) eyes was below 0.1, and all of the patients survived to the follow-up with no metastatic lesions.

Conclusions PON is relative rare, with a predominance of bilateral involvement and more with a poor visual prognosis. Paraneoplastic antibody testing can contribute to the diagnosis of PON, distinct from other types of optic neuropathies, which can help doctors to find the primary cancer earlier to guide further treatment.

  • paraneoplastic optic neuropathy
  • paraneoplastic syndrome (PNS)
  • PNS antibodies

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  • Contributors QX and WD shared the first authorship and contributed equally. Design and conduct of the study by QX and SW, who shared the co-corresponding authorship. Collection, analysis, management and interpretation of the data by WD, HZ, XZ, HL, HS and XW. Preparation of the manuscript by QX, HZ and XZ. Critical revision of the manuscript was performed by XQ and WD. Review and final approval of the manuscript by all the authors.

  • Funding This study was supported by the 863 Plan Biological and Medical Technology project 'Development of equipments in diagnosis and visual function evaluation for optic neuritis', 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 in its 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 Professor Quangang Xu (

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