title: Distinct clinical characteristics of atypical optic neuritis with seronegative aquaporin-4 antibody among Chinese patients.
abstract: To evaluate the clinical features and prognosis of atypical optic neuritis (ON) with seronegative aquaporin-4 (AQP4) antibody in Chinese patients.
  
  
  
  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.
  
  
  
  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.
  
  
  
  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.
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.|Department of Ophthalmology, The first Affiliated Hospital of Chinese PLA General Hospital, Beijing, China.
authors.forename: Huanfen
authors.initials: H
authors.lastname: Zhou
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Quangang
authors.initials: Q
authors.lastname: Xu
authors.affiliation: Department of Ophthalmology, Beijing Hospital, National Center of Gerontology, Beijing, China.
authors.forename: Shuo
authors.initials: S
authors.lastname: Zhao
authors.affiliation: State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou, China.
authors.forename: Wei
authors.initials: W
authors.lastname: Wang
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Junqing
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Radiodiagnosis, Chinese PLA General Hospital, Beijing, China.
authors.forename: Zhiye
authors.initials: Z
authors.lastname: Chen
authors.affiliation: Fujian Provincial Key Laboratory of Ecology-Toxicological Effects and Control Techniques of Emerging Contaminants, College of Environmental and Biological Engineering, Putian University, Fujian, China.
authors.forename: Dahe
authors.initials: D
authors.lastname: Lin
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Xiaoming
authors.initials: X
authors.lastname: Li
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Chunxia
authors.initials: C
authors.lastname: Peng
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Nanping
authors.initials: N
authors.lastname: Ai
authors.affiliation: Department of Ophthalmology, Chinese PLA General Hospital, Beijing, China.
authors.forename: Shihui
authors.initials: S
authors.lastname: Wei
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology* / Q000378:metabolism; D001323:Autoantibodies / Q000276:immunology*; D015415:Biomarkers / Q000097:blood / Q000134:cerebrospinal fluid; D002681:China / Q000453:epidemiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology; D009898:Optic Disk / Q000473:pathology; D012008:Recurrence; D012189:Retrospective Studies; D041623:Tomography, Optical Coherence; D055815:Young Adult
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D015415:Biomarkers
keywords: Optic neuritis; aetiology; multiple sclerosis; neuromyelitis optica; prevalence