Song Honglu 1,2 , Yang Mo 1 , Sun Mingming 1 , Zhou Huanfen 1 , Xu Quangang 1 , Wei Shihui 1
  • 1. Department of Ophthalmology, The First Medical Center, Chinese PLA General Hospital, Beijing 100853, China;
  • 2. Department of Ophthalmology, The 980th Hospital of the Chinese PLA Joint Logistics Support Force, Shijiazhuang 050082, China;
Wei Shihui, Email: weishihui706@hotmail.com
Aquaporin 4; Optic neuritis; Neuromyelitis optica; Child
10.3760/cma.j.cn511434-20200207-00043
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Objective To observe the clinical, radiographic features and prognosis of aquaporin-4 antibody positive pediatric optic neuritis (AQP4-PON).Methods A retrospective case series. Twenty-three eyes of 14 children with AQP4-PON who were clinically confirmed in the Department of Ophthalmology of the First Medical Center of the Chinese PLA General Hospital from January 2015 to December 2018 were included in the study. All patients underwent BCVA, fundus color photography, and magnetic resonance imaging (MRI). OCT was performed on 15 eyes of 10 patients, and the peripapillary retinal nerve fiber layers (pRNFL), macular ganglion cell-inner plexiform layers (mGCIPL) thickness of the affected eyes were measured. Cell-based indirect fluorescent immunoassay was used to detect serum AQP4 antibodies and myelin oligodendrocyte glycoprotein antibodies. The follow-up time ranged from 28 to 59 months. The clinical, neuroimaging characteristics and prognosis of the children were analyzed.Results Among 14 children, 2 were male (14.3%) and 12 were female (85.7%). The mean age of onset was 13.3 ± 3.0 years. On the first visit, there were 10 unilateral patients and 4 bilateral patients. The first manifestations were 11 patients of optic neuritis (78.6%), 2 patients of posterior pole syndrome (14.3%), and 1 patient of myelitis (7.1%). There were 10 patients (71.4%) with eye pain, and 5 patients (35.7%) combined with autoantibodies positive. When the first onset time was less than 2 weeks, fundus examination revealed disc edema in 7 eyes (38.9%). After 3 months, the average pRNFL and mGCIPL thickness of 15 eyes underwent OCT examination were 62.33 ± 11.07 and 54.17 ± 5.42 μm, respectively. Orbital MRI showed that the optic nerve showed a long T2 signal in 14 patients (100.0%) and 11 patients (78.6%) with T1 intensive lesions. When the first onset was less than 2 weeks, 16 eyes (88.9%) had BCVA≤0.1, and 7 eyes (38.9%) had BCVA≤0.1 and 9 eyes (50.0%) with BCVA≥0.5 after glucocorticoid treatment. Recurrence occurred in 11 patients during follow-up and was treated with immunosuppressive agents. At the last visit, in 14 patients, 9 eyes (64.3%) were involved in both eyes, and 5 patients (35.7%) progressed to neuromyelitis optica; in 23 eyes, 8 eyes (34.8%) had BCVA≥0.5.Conclusions AQP4-PON patients are more common in women, severely impaired visual function, easy to relapse, and some patients will progress to neuromyelitis optica.

Citation： Song Honglu, Yang Mo, Sun Mingming, Zhou Huanfen, Xu Quangang, Wei Shihui. Clinical features and prognosis of aquaporin-4 antibody positive pediatric optic neuritis. Chinese Journal of Ocular Fundus Diseases, 2020, 36(4): 257-261. doi: 10.3760/cma.j.cn511434-20200207-00043 Copy

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