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AimsThe optimal immunosuppressive therapy (IST) in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) remains uncertain. This study aimed to observe the disease course of MOG-ON and evaluate the therapeutic efficacy and tolerability of conventional immunosuppressants through Chinese cohort analysis.MethodsThis bidirectional cohort study included 121 patients with MOG-ON between January 2015 and December 2018. The clinical features and annualised relapse rate (ARR) of patients with and without IST were analysed.ResultsThe median age at onset was 17.5 years, and the sex ratio (F:M) was 1.24. Of 121 patients, 77 patients relapsed and 61 patients were younger than 18 years at disease onset. The overall median ARR of 63 patients in the non-IST group was 0.5, with 46.0% patients showing relapse at a median follow-up of 33.5 months. In the IST group, the ARR decreased from 1.75 pre-IST to 0.00 post-IST in 53 patients who received IST exceeding 6 months, with 20.8% patients showing relapse at a median follow-up of 23.8 months. The relapse rates of patients treated with rituximab (RTX) and mycophenolate mofetil (MMF) were not statistically different, but the rate of discontinuation was significantly lower in the RTX-treated group (18.2% vs 57.7%, p=0.0017).ConclusionThis study provides Class III evidence that both MMF and RTX may lower disease activity in patients with MOG-ON, and RTX showed better tolerability than MMF. However, observation after a single attack remains a good option because less than half of patients not on treatment suffered a relapse.

Background Several neuroimaging studies demonstrated that optic neuritis (ON) leads to functional and anatomical architecture changes in the brain. The alterations of interhemispheric functional connectivity (IFC) in patients with AQP4-ON and myelin oligodendrocyte glycoprotein (MOG)-ON are not well understood. Purpose To investigate the differential patterns of VMHC in patients with AQP4-ON and MOG-ON. Material and Methods Twenty-one patients with AQP4-ON, 11 patients with MOG-ON, and 34 healthy controls underwent resting-state MRI scans. One-way ANOVA was used to identify regions in which the zVMHC differed among the three groups. Post hoc two-sample t-tests were then conducted to compare zVMHC values between pairs of groups. Pearson correlation analysis was conducted to reveal relationships between mean zVMHC values and clinical variables in the AQP4-ON and MOG-ON groups. Results The results revealed significant differences in zVMHC values in the PreCG among the three groups. Compared to the control group: the AQP4-ON group showed significantly lower VMHC values in the superior temporal gyrus, inferior frontal gyrus, and PreCG; and the MOG-ON group showed significantly higher zVMHC values in the PostCG. Compared to the AQP4-ON group, the MOG-ON group showed significantly lower zVMHC values in the PreCG/PostCG (voxel-level PConclusion Patients with AQP4-ON and those with MOG-ON showed abnormal VMHC in the motor cortices, sensorimotor cortices, and frontal lobe, possibly indicating impaired sensorimotor function in patients with ON. Moreover, differential patterns of VMHC in patients with AQP4-ON, compared to patients with MOG-ON, might serve as a clinical indicator for classification of ON.

To investigate the clinical characteristics and outcomes of myelin oligodendrocyte glycoprotein antibody-seropositive optic neuritis (MOG-ON) in patients with varying ages of onset in China.Patients displaying symptoms of MOG-ON were recruited from the Neuro-ophthalmology Department in the Chinese People's Liberation Army General Hospital from January 2016 to May 2018. They were assigned to one of three subgroups based on age of onset: pediatric (18 years), young (18-46 years), and middle-aged (46 years) MOG-ON.110 patients (188 eyes) were assessed, including 58 pediatric (52.7%), 34 young (30.9%), and 18 middle-aged (16.4%) patients. Of the pediatric patients, 93.9% had good recovery of visual acuity (≥0.5) compared with 79.7% of young patients and 66.7% of middle-aged patients (p .001). The annual relapse rate was lower in the pediatric group than young and middle-aged groups (0.32 ± 0.50 vs 0.73 ± 0.87 vs 0.49 ± 1.08, p = .036). Six children (10.3%) were diagnosed with acute disseminated encephalomyelitis, while seven young patients (20.6%) were diagnosed with aquaporin-4 antibody seronegative neuromyelitis optica spectrum disorder upon follow-up. The average peripapillary RNFL and macular GCIPL thicknesses were not statistically different between subgroups (p = .996, p = .608). Overall, MRIs of the optic nerve showed perineural enhancement in 52.0% of patients and longitudinal extensive involvement in 87.7%. MRIs also revealed a greater proportion of pediatric patients with intracranial optic nerve involvement than in the other two subgroups (45.4% vs. 21.2% vs. 36.7%, p = .014).Pediatric ON was the most common MOG-ON subgroup. Pediatric patients had different clinical features, including better recovery of visual acuity, lower annual relapse rate, and more intracranial optic nerve involvement than young and middle-aged patients. Additionally, age of onset may be a potential predictor for determining visual prognosis with MOG-ON.

Objective To study the differences in immunopathogenesis based on chemokine profile in neuromyelitis optica patients positive for AQP4 antibodies or MOG antibodies. Patients and methods We measured 52 cytokines/chemokines using ELISA in 59 serum samples, which were divided into three groups according to CBA results: HCs (n=16), AQP4+ (n=20) and MOG+ (n=23). The regression equation (R 2>0.98) of the standard curve was calculated according to the standard concentration and the corresponding A value. And then the corresponding sample concentration was calculated according to the A value of the sample. Results Eleven of 52 measured serum cytokine/chemokines (CCL22/MDC, CCL13/MCP-4, CCL21/6Ckine, CCL27/CTACK, CCL8/MCP-2, CXCL14/BRAK, Contactin-1, Kallilrein 6/Neurosin, Midkine, VCAM-1 and Fas) were significantly different between MOG+ group and controls. Ten of 52 measured serum cytokine/chemokines (CCL1/I-309, CCL22/MDC, CCL28, CCL17/TARC, CCL27/CTACK, CXCL2/GRO beta, Contactin-1, Midkine, Chemerin and Synuclein-alpha) were significantly different between AQP4+ group and controls. There was no difference between serum AQP4+ and MOG+ groups for CC chemokines. All measured chemokines CXC except CXCL6/GCP-2 showed no significant differences in serum AQP4+ group compared to MOG+ group. However, there was significant difference between serum AQP4+ and MOG+ groups for C5/C5a and Midkine. C5/C5a and Midkine were significantly higher in AQP4+ group compared to MOG+ group (P Conclusion Our findings suggest that the differences of mean concentration in CXCL6/GCP-2, Midkine and C5/C5a probably reveal different immunologic mechanism between AQP4+ NMO and MOG+ NMO. This cytokine/chemokine profiling provides new insight into NMO pathogenesis associated with MOG antibody seropositivity and provides guidance to monitor inflammation and response to treatment in a way.

Purpose: To prospectively investigate the efficacy and tolerance of low-dose rituximab (RTX) for the treatment of neuromyelitis optica-associated optic neuritis (NMO-ON).Methods: Optic Neuritis patients with seropositive aquaporin 4-antibody (AQP4-Ab) were diagnosed with NMO-ON and recruited for treatment with low-dose RTX (100 mg * 4 infusions) and were then followed monthly for a minimum of 3 months. Reinfusion of 100 mg RTX was given when the CD19+ B lymphocyte frequency was elevated to above 1%. The serum AQP4-Ab level was tested by an enzyme-linked immunosorbent assay (ELISA).Results: A total of 43 NMO-ON patients (1 male/42 female, 75 involved eyes) were included in this study. CD19+ B cell clearance in the peripheral blood was induced in 97.7% of patients after induction treatment. A significant decrease in serum AQP4-Ab concentration was observed after induction treatment (P = 0.0123). The maintenance time of B cell clearance was 5.2 ± 2.25 months. The relapse-free rate was 92.3% in patients followed-up for over 12 months, and patients with non-organ-specific autoimmune antibodies tended to relapse within 6 months. A total of 96.2% of patients had stable or improved vision, and a decrease in the average expanded disability status scale (EDSS) score was found. Structural alterations revealed by optic coherence tomography were observed in both ON and unaffected eyes. The rates of infusion-related reactions and long-term adverse events (AEs) were 18.6 and 23.1%, respectively. No severe AEs was observed.Conclusions: Low-dose rituximab is efficient and well-tolerated in treating NMO-ON.

Background: Optic nerve lymphoma can present a diagnostic challenge because of its confusing clinical features and the difficulty of obtaining lesion tissue for biopsy. The objective of this study was to find some flags of lymphomatous infiltration of optic nerves. Methods: We report two cases of optic nerve lymphoma and conduct a literature review to determine whether a common diagnostic characteristic can be identified. Results: We examined 22 optic nerve lymphoma cases. Thirteen cases were systemic lymphoma infiltration of the optic nerve, five were primary central nervous system lymphoma (PCNSL), and four were primary isolated optic nerve lymphoma. Twenty patients manifested significant enlargement of the lesions in orbital/brain MRI. Seventeen contrast-enhanced MRIs showed abnormal enhancement of the optic nerve. All PCNSL and isolated optic nerve lymphoma patients in the series showed marked enhancement. Moderate and subtle enhancement was found in systemic lymphoma patients only. At the enhancement site, six isolated optic nerve lymphoma and PCNSL patients presented intrinsic enhancement, ten systemic patients showed both optic nerve and sheath enhancement, and one demonstrated sheath enhancement. Cerebrospinal fluid (CSF) tests showed elevated protein levels in six patients, and a neoplasm in one patient. We found abnormality of CSF immunity in both of our patients. Conclusion: Combined characteristics of orbital MRI and CSF tests may facilitate expeditious suspicion establishment of optic nerve lymphoma.

Background/aimsTo evaluate the status of myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) in chronic relapsing inflammatory optic neuropathy (CRION) and investigate its different clinical characteristics and prognosis.MethodsPatients diagnosed with CRION were recruited by the Neuro-ophthalmology Department of the Chinese People's Liberation Army General Hospital from December 2015 to April 2017. Based on antibody status, they were assigned to either the MOG-CRION or seronegative-CRION groups.ResultsA total of 33 patients (38 eyes) were assessed and divided into the following groups: 22 (66.7%) MOG-CRION and 11 (33.3%) seronegative-CRION. The ratio of female to male was 1:1, and 81.8% of total CRION patients were adults (≥18 years). A total of 29 eyes (76.3%) showed severe visual loss (20/40) during the final follow-up. The mean onset age of MOG-CRION patients was 28 ± 16 years (range 6–62), which was significantly younger than that of seronegative-CRION (45 ± 12 years, range 22–59) (p=0.029). The intraorbital and canalicular segments were highly involved in the orbital MRI of CRION patients. During the final follow-up, MOG-CRION patients had more bilateral involvement (p=0.008) and higher annualised relapse rates compared with the seronegative-CRION patients (p=0.019).ConclusionCRION was predominantly found in adults with unilateral ON and exhibited a higher rate of seropositive MOG-IgG. MOG-CRION, which may be a disparate subtype of MOG-IgG-induced demyelinating disease that needs further investigation, was found in younger patients at onset, with more bilateral involvement and more relapse tendency.

Background/AimsTo investigate clinical characteristics and prognosis of paediatric optic neuritis (PON) in patients seropositive for myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China.MethodsChildren displaying initial onset of optic neuritis (ON) were recruited from the Neuro-ophthalmology Department in the Chinese People’s Liberation Army General Hospital from January 2016 to August 2017. They were assigned into three groups based on antibody status: MOG-Ab-seropositive ON (MOG-ON), aquaporin-4 antibody-seropositive ON (AQP4-ON) and double seronegative ON (seronegative-ON).ResultsTotally 48 patients were assessed, including 25 MOG-ON (52.1%), 7 AQP4-ON (14.6%) and 16 seronegative-ON (33.3%). The MOG-ON and seronegative-ON cohorts had equal ratios of female/male, but the AQP4-ON cohort was predominantly females (100%). The patients with MOG-ON were significantly younger at onset compared with the AQP4-ON group. Of the MOG-ON eyes, 97.6% had good recovery of visual acuity (VA) (≥0.5) compared with33.3% of AQP4-ON eyes (p=0.052). Two children in the MOG-ON group ended up being diagnosed with acute disseminated encephalomyelitis, while only one patient in the AQP4-ON group developed neuromyelitis optica during follow-up. Patients with MOG-ON had thicker peripapillary retinal nerve fibre layers overall and in the superior and inferior quadrants than in patients with AQP4-ON (p=0.005, p=0.002 and p=0.024, respectively). In addition, the macular ganglion cell-inner plexiform in MOG-ON eyes became significantly thicker than in AQP4-ON eyes (p=0.029). Orbital MRI revealed a larger proportion of patients with MOG-ON had intracranial optic nerve involvement than patients with seronegative-ON (51.2% vs 17.4%, p=0.009).ConclusionMOG-ON was the most common PON subtype in China. MOG-ON had different clinical features including earlier age of onset, equal female/male ratio, better recovery of VA and thicker peripapillary retinal nerve fibre and macular ganglion cell-inner plexiform layers. MOG-Abs may be a potential biomarker for determining visual prognosis with PON.

ObjectiveParaneoplastic 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.MethodsClinical 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.ResultsA 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.ConclusionsPON 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.

Background To investigate the application of critical flicker fusion frequency (CFF) in demyelinating optic neuritis (DON). Methods A total of 127 eyes in 69 DON patients and 63 eyes in 33 healthy control (HC) groups were included between January 2021 and September 2021. Patients underwent best-corrected visual acuity (BCVA), visual field, optical coherence tomography (OCT), flash visual evoked potential (F-VEP), and CFF examinations. The patients were divided into different subgroups according to antibody subtypes, different degrees of visual acuity impairment, the different time intervals from onset to CFF examination, and performed subgroup analysis. The correlations between CFF and other ophthalmological examination: BCVA, visual field, flash visual evoked potential (F-VEP), in DON patients were analyzed in order. Results The trichromatic values of red, green, and yellow in DON affected eyes were 21.83 ± 9.03, 23.66 ± 10.21, 24.09 ± 10.77 Hz, respectively, which was significantly reduced compared with the HC group (t = −14.82, −14.22, −14.00; P 0.05), whereas, a significant decrease of CFF trichromatic value was found in severe visual acuity impairment (P Conclusions A significant decrease of CFF value was found in DON-affected patients, and it has a good correlation with visual function and latency of F-VEP, and it can provide more valuable information about the impairment of visual function.

Objective To evaluate the optic nerve impairment using MRI histogram texture analysis in the patients with optic neuritis.Methods The study included 60 patients with optic neuritis and 20 normal controls. The coronal T2 weighted imaging (T2WI) with fat saturation and enhanced T1 weighted imaging (T1WI) were performed to evaluate the optic nerve. MRI histogram texture features of the involved optic nerve were measured on the corresponding coronal T2WI images. The normal optic nerve (NON) was measured in the posterior 1/3 parts of the optic nerve. Kruskal-Wallis one-way ANOVA was used to compare the difference of texture features and receiver operating characteristic (ROC) curve were performed to evaluate the diagnostic value of texture features for the optic nerve impairment among the affected optic nerve with enhancement (ONwEN), affected optic nerve without enhancement (ONwoEN), contralateral normal appearing optic nerve (NAON) and NON.Results The histogram texture Energy and Entropy presented significant differences for ONwEN vs. ONwoEN (both P=0.000), ONwEN vs. NON (both P=0.000) and NAON vs. NON (both P

Purpose. To describe different clinical characteristics and prognosis of optic neuritis (ON) in male patients with seropositive aquaporin-4 antibody (AQP4-Ab) or myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China. Method. Males with ON were recruited from the Neuro-ophthalmology Department of the Chinese People’s Liberation Army, General Hospital from January 2016 to February 2018. They were assigned to two groups based on antibodies status: MOG-Ab-seropositive ON (MOG-ON) and aquaporin-4 Ab-seropositive ON (AQP4-ON). Results. Seventy-six male patients were assessed, including 44 MOG-ON (57.9%) and 32 AQP4-ON (42.1%). The MOG-ON patients were significantly younger at onset compared to the AQP4-ON group (p<0.001). Frequencies of optic disc swelling, presence of abnormal autoimmune antibodies, and elevated levels of CSF IgG were significantly higher in the AQP4-ON group than the MOG-ON group (p=0.040, p=0.016, and p=0.10, respectively). At the final visit, 85.3% of MOG-ON eyes had increased visual acuity (≥0.5) compared to 35.1% of AQP4-ON eyes (p<0.001). The ratio of this steroid-dependent condition is higher in MOG-ON patients than the AQP4-ON group (p<0.001). The ratio of conversion to NMO is higher in the AQP4-ON group than the MOG-ON group, with more AQP4-ON patients developing NMO by the follow-up (p=0.012). MOG-ON patients had thicker average peripapillary retinal nerve fiber layers and macular ganglion cell-inner plexiform than AQP4-ON patients (p=0.008 and p=0.012, respectively). Orbital MRI revealed more AQP4-ON patients had chiasmal involvement than MOG-ON patients (p<0.001). Conclusion. Male MOG-ON patients had different clinical features including earlier age of onset, higher optic disc swelling ratio, better visual acuity recovery, thicker peripapillary retinal nerve fiber and macular ganglion cell-inner plexiform layers, and less chiasmal involvement than male AQP4-ON patients. Serum antibody may be a potential biomarker for determining visual prognosis in male ON.

Little work has been performed on the long-term outcome of optic neuritis (ON) according to the status of aquaporin-4 antibody (AQP4-Ab) and long-term prognosis in older patients in China. This study retrospectively analyzed medical records in a cohort of Chinese patients with 5-year follow-up according to AQP4-Ab status and ages from January 2009 to December 2010. The clinical features, laboratory findings and risk factors for prognosis were analyzed. A total of 128 ON patients were included, 66.4 % of whom were female. The median age at onset was 36.8 years (range 18-73). Serum AQP4-Ab was positive in 45 (35.2 %) patients, with greater frequency in the female, bilateral, and recurrent ON groups (48.2, 42.5 and 53.6 %, respectively). Seropositive AQP4-Ab ON patients had worse visual recovery compared to seronegative patients (p = 0.033). The average and four quadrants of retinal nerve fiber layer (RNFL) thickness were significantly thinner in the seropositive group than in the seronegative group (p

Background: Plasma exchange (PE) is often considered as an effective treatment for neuromyelitis optica spectrum disorder (NMOSD) and several inflammatory demyelinating disorders of the central nervous system. This study aimed to evaluate the visual outcomes of Chinese patients with severe acute isolated optic neuritis (ON) who received PE therapy after high-dose intravenous methylprednisolone (IVMP) treatment. Methods: Thirty-seven acute isolated ON patients experiencing their first attack of severe visual impairment without neurological disability were recruited. All subjects received five cycles of double-filtration plasmapheresis. Visual acuity (VA) was documented at onset, 1 day before PE treatment, at each cycle of PE therapy and at the 1-month follow-up visit. Results: This study included 26 female (70.3%) and 11 male (29.7%) subjects, and 18 subjects (48.6%) had bilateral involvement. The time window between onset and PE treatment was 27.3 ± 12.7 days (range: 6–53 days). Mean VA (logMAR) of the studied eyes at onset, 1-day before PE treatment/after IVMP and after the fifth PE treatment were 3.41 ± 1.50, 2.61 ± 1.64 and 1.66 ± 1.52, respectively ( p 20/800 after IVMP and PE treatments, among which five eyes (13.5%) recovered to Snellen VA 20/20 ( p Conclusion: This study revealed that PE treatment effectively improves the visual outcomes of patients experiencing their first attack of severe acute isolated ON after high-dose IVMP treatment. Better visual outcomes can be achieved with early PE treatment.

Purpose The ocular choroid is a sensitive biomarker of vascular perfusion in optic neuritis (ON) patients due to its vascular structures. The purpose of this study was to evaluate alterations in sub-macular choroidal thicknesses (sub-MCT) in aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) sero-positive neuromyelitis optica spectrum disease (AQP4-IgG+/NMOSD) and isolated ON (ION) patients using optical coherence tomography (OCT). Methods A total of 208 ON patients (275 eyes) and healthy controls (HCs) who underwent sub-MCT and retinal microstructure detection with OCT were enrolled in this study. Results Among all the ON patients, 102 (49.0%) cases were identified as serum AQP4-IgG-positive, with 106 (51.0%) cases being negative, excluding multiple sclerosis as the ION cohort. The sub-MCT in the AQP4-IgG+/NMOSD patients decreased in 0-6 months after ON attacks. However, for the ION cohort, the sub-MCT decreased in 0-2 months and then stayed normal or slightly increased in 2-4 months after the first ON attack, finally sharply decreasing after 6 months. For unilateral AQP4-IgG+/NMOSD patients, eyes without ON also presented retinal layer thinning and sub-MCT slight reduction independent of ON attacks. Conclusions The sub-MCT in AQP4-IgG+/NMOSD patients were reduced at all stages of ON, which distinguished the ION patients as decreasing only at chronic stage of ON. It implied that ocular vascular hypoperfusion plays a potential role in ON pathogenesis and the different patterns could be caused by the distinct pathogenesis of AQP4-IgG+/NMOSD and ION.

Adult demyelinating optic neuritis (ON) with positive myelin-oligodendrocyte glycoprotein antibody (MOG-Ab) has distinct clinical features. This study aimed to investigate the point prevalence, relationship with steroid dependency and prognosis value of MOG-Ab in adult ON.Clinical data analysis was undertaken in adults with ON admitted between December 2014 and January 2016. Patients were classified into three groups based on aquaporin-4 antibody (AQP4-Ab) and MOG-Ab status: AQP4-ON, MOG-ON and seronegative-ON.A total of 158 adults with ON (190 eyes) were assessed, including 31 MOG-ON (19.6%), 67 AQP4-ON (42.4%) and 60 seronegative-ON (38.0%) cases. The female-to-male ratio was significantly lower in MOG-ON (1.8:1) than that in AQP4-ON (8.6:1) groups (p = .005). The median age, percentage of bilateral ON and visual loss at the nadir at onset was similar among the three groups. Thirty-eight eyes (76%) in the MOG-ON group showed good visual recovery (20/40) in the final visit, which is statistically better than that in the AQP4-ON and seronegative-ON groups (p .001 and p = .006, resoectively). Fifteen adults with ON (9.5%) showed dependency on steroid, which was particularly prominent in the MOG-ON group (11/31, 35.5%) and rarely presented in the AQP4-ON (2, 3.0%) and seronegative-ON (2, 3.3%) groups. Results suggested less loss of pRNFL in MOG-ON than that in AQP4-ON group (p .001), and a larger proportion of canalicular segment involved in MOG-ON adults (p = .007 and p .001).MOG-ON had the smallest proportion of acute demyelinating ON in Chinese adults. One third of adults with MOG-ON predominantly showed a substantial dependency on steroids and relapse on steroid reduction or cessation, which rarely presented in AQP4-ON and seronegative-ON adults.

To review the clinical features of optic neuritis (ON) combined with chronic hepatitis B (CHB) retrospectively.Clinical data were reviewed for hospitalized patients diagnosed with isolated ON combined with chronic hepatitis B (CHB-ON) in the Chinese People's Liberation Army General Hospital. The ON diagnosis was confirmed following the criteria of the Optic Neuritis Treatment Trial (ONTT) group. The diagnostic criteria for CHB was serological positivity for hepatitis B surface antigen (HBsAg) for more than 6 months. Other infectious conditions that might lead to bias were excluded.A total of 13 patients (6 female and 7 male, 23 involved eyes) diagnosed with CHB-ON were selected. A total of 12/13 patients presented as atypical ON: 10/13 of the patients exhibited simultaneous or early sequential bilateral eye involvement; 11/13 involved eyes in the acute phase presented with pronounced optic disc edema; and 11/13 patients exhibited corticosteroid resistant. A total of 12/23 affected eyes suffered severe vision loss (20/200) at the end of the follow-up period, which averaged 13.7 (4-31) months. None of the patients progressed to multiple sclerosis (MS) or neuromyelitis optica (NMO). All blood samples were negative for serological aquaporin 4-antibody using the cell-based assay.CHB-ON usually presented as the atypical form. Chronic hepatitis B virus infection may lead to the tendency for ON exacerbation.

Objective. This study aims to describe the clinical manifestations and outcomes in a cohort of older Chinese patients.Method. A retrospective study of patients aged ≥ 45 years who had a first episode of optic neuritis (ON) between May 2008 and November 2012. Clinical features at onset and last follow-up were analyzed within subgroups (age 45–65 years and age ≥ 65 years).Results. 76 patients (99 eyes) were included, of which 58% were females. The mean age at presentation was 55.53 ± 8.29 years (range: 45–83 years). Vision loss was severe at presentation, with initial best corrected vision activity (BCVA) Conclusion. Chinese patients in the age group ≥ 65 years with ON are less likely to develop NMOSD or MS. Notwithstanding, they had more severe visual loss at onset and poor recovery.

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 p0.001), 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%, p0.001 and p0.001) 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.

This study retrospectively analyzed the frequency of anti-thyroid antibodies (ATAs) and thyroid disease in patients with optic neuritis (ON).Tests of serum thyroglobulin (TG) and thyroid peroxidase (TPO) antibodies and thyroid function were performed in 97 ON patients. Blood also was drawn to test for AQP4-Ab using cell-based and enzyme-linked immunosorbent assays. Comparisons of the frequencies of ATAs, thyroid diseases and thyroid function were performed based on AQP4-Ab status.Seropositive AQP4-Ab was found in 47/97 (48.5%) patients. ATA was considered positive in 34/97 (35.1%) patients. The prevalence of ATA was two times higher (P = 0.019) in the AQP4-Ab+ group compared to the AQP4-Ab- group. AQP4-Ab+ ON patients exhibited lower FT3 (P = 0.006) and FT4 (P = 0.025) levels and a higher prevalence of definite Hashimoto thyroiditis (HT) (P = 0.005). Among AQP4-Ab+ patients, those with HT had a worse visual outcome than non-HT patients.A high prevalence of ATAs and HT was found in AQP4-Ab+ ON patients, and AQP4-Ab+ patients with HT exhibited worse visual outcomes than non-HT patients.

Objectives This study aimed to analyse the structural injury of the peripapillary retinal nerve fibre layer (pRNFL) and segmented macular layers in optic neuritis (ON) in aquaporin4-antibody (AQP4-Ab) seropositivity(AQP4-Ab-positiveON) patients and in AQP4-Ab seronegativity (AQP4-Ab-negative ON) patients in order to evaluate their correlations with the best-corrected visual acuity (BCVA) and the value of the early diagnosis of neuromyelitis optica (NMO). Design This is a retrospective, cross-sectional and control observational study. Methods In total, 213 ON patients (291 eyes) and 50 healthy controls (HC) (100 eyes) were recruited in this study. According to a serum AQP4-Ab assay, 98 ON patients (132 eyes) were grouped as AQP4-Ab-positive ON and 115 ON patients (159 eyes) were grouped as AQP4-Ab-negative ON cohorts. All subjects underwent scanning with spectralis optical coherence tomography (OCT) and BCVA tests. pRNFL and segmented macular layer measurements were analysed. Results The pRNFL thickness in AQP4-Ab-positive ON eyes showed a more serious loss during 0–2 months (-27.61μm versus -14.47 μm) and ≥6 months (-57.91μm versus -47.19μm) when compared with AQP4-Ab-negative ON eyes. AQP4-Ab-positive ON preferentially damaged the nasal lateral pRNFL. The alterations in the macular ganglion cell layer plus the inner plexiform layer (GCIP) in AQP4-Ab-positive ON eyes were similar to those in AQP4-Ab-negative ON eyes. AQP4-Ab-positive ON eyes had entirely different injury patterns in the inner nuclear layer (INL) compared with AQP4-Ab-negative ON eyes during the first 6 months after the initial ON attack. These differences were as follows: the INL volume of AQP4-Ab-positive ON eyes had a gradual growing trend compared with AQP4-Ab-negative ON eyes, and it increased rapidly during 0–2 months, reached its peak during 2–4 months, and then decreased gradually. The pRNFL and GCIP in AQP4-Ab-positive ON eyes had positive correlations with BCVA. When the pRNFL thickness decreased to 95%CI (50.77μmto 60.85μm) or when the GCIP volume decreased to 95%CI (1.288 mm3to 1.399 mm3), BCVA began to be irreversibly damaged. Conclusion The structural alterations of pRNFL and GCIP could indicate the resulting visual damage. In addition, the injury pattern of INL could be a potential structural marker to predict the conversion of ON to NMO.

Purpose. Although optic neuritis (ON) in children is relatively common, visual outcomes and factors associated with the condition have not been well documented. The aim of this study was to evaluate the clinical features and visual outcomes of ON in Chinese children.Methods. Patients with a first episode of ON at a tertiary neuroophthalmic centre in China were assessed and followed up for at least three months. Visual outcomes and clinical, laboratory, and neuroimaging findings were reviewed. In patients with bilateral ON, only the eyes with worse visual acuity (VA) at presentation were used for statistical analysis.Results. Seventy-six children (76 eyes) with a first episode of ON were included. The mean age was 11.8 years, 60.5% were females, and 48.7% had bilateral involvement. The children were followed up for an average of 18.5 months (age range, 3–48 months). Vision loss at presentation was severe, with VA < 20/200 in 37 eyes (48.7%). At the final visit, 3 (3.9%) eyes had VA of at least 20/20, and 41 (53.9%) eyes had VA of at least 20/40. The final VA in 35 eyes (46.1%) was worse than 20/40. Children aged ≤ 10 years had better predicted visual outcomes when compared to children over 10 years (odds ratio = 2.73, 95% confidential interval: 1.05–7.07, andP=0.039). The other features of this cohort, such as sex, experienced bilateral attack, VA at presentation, presence of optic disc edema, systemic diseases, magnetic resonance imaging (MRI) findings, and aquaporin-4 (AQP-4) antibody status, were not significantly correlated with the final visual outcome.Conclusion. The data revealed the clinical characteristics and visual outcomes of ON in Chinese children. ON in children was associated with severe vision loss and relatively good visual recovery. The age at onset could predict the final visual function.

The detection of anti-aquaporin-4 autoantibody (AQP-4 Ab) is crucial to detect patients who will develop neuromyelitis optica (NMO); however, there are few studies on the AQP-4 Ab serostatus of patients with neuromyelitis optica spectrum ON. We analyzed the clinical and paraclinical features of neuromyelitis optica spectrum ON patients in China according to the patients' AQP4-Ab serostatus. 125 patients with recurrent and bilateral ON with simultaneous attacks were divided into AQP-4 Ab-seropositive and -seronegative groups. Demographic, clinical, serum autoantibody data, connective tissue disorders (CTDs), visual performance were compared. A Visual Acuity (VA) of less than 0.1 during acute ON attacks occurred more frequently in the seropositive group (p = 0.023); however, there was not a significant difference between groups on VA recovery after the first attack. The seropositive group experienced the worst outcome during the last attack (p = 0.017). Other co-existing autoimmunity antibodies (p < 0.001) and CTDs (p < 0.001) were more prevalent in seropositive patients. There were no significant differences on VA recovery and RNFLT combined with other autoantibodies or CTDs. The two groups did not differ significantly with regard to time to relapse, annualized relapse rates, time of diagnosis NMO, or RNFL. There were no significant differences on VA recovery and RNFLT combined with other autoantibodies or CTDs. RNFLT was thinner in NMO seropositive patients. Although AQP-4 Ab expression predicted poor visual outcome, positive patients were usually associated with mild symptoms at first onset. Anti-SSA/SSB antibody or Sjogren syndrome may be associated with AQP-4 Ab in neuromyelitis optica spectrum ON.

This paper aims to explore the relationship of retinal neuron apoptosis and manganese superoxidase dismutase (MnSOD) at early phase of diabetic retinopathy. Sprague-Dawley rats were grouped into normal controls and diabetics. Data were collected after 4, 8, and 12 weeks (n=12). The pathological changes and ultrastructure of the retina, the apoptosis rate of retinal neurons by TdT-mediated dUTP nick end label (TUNEL), mRNA expressions of MnSOD and copper-zinc superoxide dismutase (Cu–Zn SOD), and the activities of total SOD (T-SOD) and subtypes of SOD were tested. For the controls, there was no abnormal structure or apoptosis of retinal neurons at any time. There was no change of structure for rats with diabetes at 4 or 8 weeks, but there was a decrease of retinal ganglion cells (RGCs) number and thinner inner nuclear layer (INL) at 12 weeks. The apoptosis ratio of RGCs was higher than that of the controls at 8 and 12 weeks (P<0.001). The activity and mRNA levels of MnSOD were lower in diabetics at 4, 8, and 12 weeks (P<0.05). In summary, the apoptosis of the retinal neurons occurred at 8 weeks after the onset of diabetes. Retinal neuron apoptosis in early diabetic rats may be associated with the decreased activity and mRNA of MnSOD.