ObjectiveTo observe the changes of serum cytokines in patients with neuromyelitis optic neuromyelitis optic spectrum disorder (NMOSD) associated optic neuritis (NMOSD-ON) before and after intravenous methylprednisolone pulse (IVMP) treatment. MethodsA prospective clinical study. From November 2020 to December 2021, 24 NMOSD-ON patients who visited the Neuro-Ophthalmology Clinic of Beijing Tongren Hospital Affiliated to Capital Medical University were included. Among them, 9 patients were male; 15 patients were female. According to the detection results of aquaporin 4 (AQP4) immunoglobulin G (IgG) antibody (AQP4-IgG) in peripheral blood, the patients were divided into AQP4-lgG positive group and AQP4-lgG negative group, which were 10 and 14 cases respectively. Twenty healthy volunteers were selected as control group. Age (F=0.639) and sex (χ2=2.373) composition ratio of the three groups were compared, the difference were not statistically significant (P=0.504, 0.333). All patients were treated with 500 mg/d or 1 000 mg/d IVMP. Peripheral venous blood of all subjects, and quantitatively analyze interferon-gamma (IFN-γ), interleukin (IL)- 4, IL-31, IL-33, IL-17A, IL-6, IL-21, IL-23, IL-10, tumor necrosis factor (TNF)-α level in serum with Luminex FLEX MAP 3D liquid-phase suspension chip detection system were collected. The differences among groups were analyzed by one-way ANOVA and Kruskal Wallis H test. ResultsBefore IVMP treatment, serum IL-17A concentrations in AQP4-lgG positive group, AQP4-lgG negative group and control group were 2.39, 2.17 and 1.97 pg/ml, respectively. TNF-α concentrations were 5.60, 4.17 and 5.89 pg/ml, respectively. Compared with control group, serum IL-17A concentration in AQP4-IgG positive group was increased, while TNF-α concentration in AQP4-IgG negative group was decreased, with statistical significance (H=12.720, 10.900; P=0.040, 0.039). The levels of IL-17A, IL-6 and other cytokines did not change significantly. After IVMP treatment, serum IL-6 in AQP4-lgG positive group and AQP4-lgG negative group were 0.72 pg/ml and 0.73 pg/ml, respectively. TNF-α concentrations were 4.17 pg/ml and 3.88 pg/ml, respectively. IFN-γ concentrations were 2.15 pg/ml and 2.55 pg/ml, respectively. Compared with before treatment, serum levels of IL-6, TNF-α and IFN-γ in AQP4-lgG positive patients were significantly decreased, with statistical significance (Z=－2.668, －2.547, －2.201; P=0.008, 0.011, 0.028). Serum levels of IL-6 and TNF-α were significantly decreased in AQP4-lgG negative patients, and the difference was statistically significant (Z=－2.501, －1.978; P=0.012, 0.048). ConclusionGlucocorticoid may play a therapeutic role by affecting the levels of serum IL-6, TNF-α, IFN-γ in patients with NMOSD-ON.
ObjectiveTo observe the clinical characteristics of the patients with positive anti-glial fibrillary acidic protein (GFAP) antibody. MethodsA retrospective study. From January 2017 through December 2021, 4 patients with positive anti-GFAP antibodies hospitalized in Departments of Ophthalmology and Neurology of Xijing Hospital, Air Force Medical University were included in this study. There were 3 patients with optic neuritis (ON) and 1 patient with the spinal and cerebral lesions. All patients were female, with an average age of 35 years. Three patients with ON received the examinations of best corrected visual acuity (BCVA), optical coherence tomography, visual evoked potential and magnetic resonance imaging (MRI) for the head and orbital. Another 1 patient with the spinal and cerebral lesions underwent MRI for the head, cervical and thoracic vertebras. All patients were tested for demyelinating ON-related antibodies in the serum, and the patient with the spinal and cerebral lesions for the antibodies in both serum and cerebrospinal fluid. Patients with ON received intravenous infusion of methylprednisolone sodium succinate in the acute stage, while the patients with spinal cord and brain lesions were given glucocorticoid and immunosuppressive therapy. ResultsThe initial symptoms of the patients with ON were sudden blurred vision in the right eye together with a pain when the eye rotated. BCVA were hand moving/in-front, 0.2 and 0.12, respectively. The serum anti-GFAP antibodies were positive. MRI showed a rough and thickened optic nerve in 1 patient. For patients with BCVA of hand moving/in-front, the BCVA was increased to counting fingers/30 cm on discharge; while the other 2 patients had no changes for BCVA. When followed up on phone 2-3 years after discharge, BCVA of the patients with ON increased to higher than 0.6. No ocular symptoms occurred in the patient with spinal and cerebral lesions and his initial symptoms were numbness, weakness and convulsions of limbs, accompanied by slurred speech. His anti-GFAP antibodies in the serum were negative but positive in the cerebrospinal fluid. MRI showed enhanced cerebellum and spinal dura mater. The initial symptoms were relieved on discharge, and vanished when followed up on phone after discharge. ConclusionsThe patients with positive anti-GFAP antibodies are more common in young and middle-aged women. Monocular optic neuritis is more often seen in the form of sudden blurred vision with an eye-rotating pain. Anti-GFAP antibodies in the serum are positive, and a few patient show a rough and thickened optic nerve. They are sensitive to glucocorticoid therapy with a satisfied prognosis.
ObjectiveTo investigate the changes in the nerve fiber layer of the cornea in patients with demyelinating optic neuritis (DON) and its correlation with visual acuity. MethodsA cross-sectional study. From March 2021 to July 2022, 27 cases (39 eyes) of DON patients diagnosed in the Department of Neurology and Ophthalmology of Beijing Tongren Hospital Affiliated to Capital Medical University were enrolled in this study. According to the serological test results, the patients were divided into aquaporin 4 antibody associated optic neuritis (AQP4-ON group) and myelin oligodendrocyte glycoprotein antibody associated optic neuritis (MOG-ON group), with 15 cases (19 eyes) and 12 cases (20 eyes) respectively. According to previous history of glucocorticoid treatment, the patients were divided into glucocorticoid treated group and non-glucocorticoid treated group, with 17 cases (27 eyes) and 10 cases (12 eyes) respectively. Twenty healthy volunteers (20 eyes) with age- and gender-matched were selected as the control group. All eyes underwent best corrected visual acuity (BCVA) and in vivo confocal microscopy (IVCM) examinations. BCVA was performed using Snellen's standard logarithmic visual acuity chart, which was converted into logarithmic minimum angle resolution (logMAR) visual acuity during statistics. The corneal nerve fiber length (CNFL), corneal nerve fiber density (CNFD), corneal nerve fiber branch length (CNBL), corneal nerve fiber branch density (CNBD) and the density of corneal dendritic cells (DC) were detected by IVCM examination. Parameter comparison between groups by t-test and Kruskal-Wallis rank sum test. The correlation between logMAR BCVA and pamameters of corneal nerve fibers were analyzed using Spearman analysis. ResultsThe CNFL, CNFD, and CNBL of the DON group and the control group were (10.67±2.55) mm/mm2, (57.78±12.35) root/mm2, (3.27±1.34) mm/mm2, and (13.74±3.05) mm/mm2, (70.95±13.14) root/mm2, and (4.22±1.03) mm/mm2, respectively; the difference in CNFL, CNFD, and CNBL between the two groups were statistically significant (t=4.089, 3.795, 2.773; P＜0.05). The CNFL, CNBL, and CNBD of the affected eyes in the MOG-ON group and AQP4-ON group were (12.02±2.13) mm/mm2, (3.80±1.19) mm/mm2, (47.97±8.86) fibers/mm2, and (9.25±2.19) mm/mm2, (2.72±1.19) mm/mm2, (39.43±13.86) fibers/mm2, respectively; the differences in CNFL, CNBL, and CNBD between the two groups were statistically significant (t=－4.002, －2.706, －2.306; P＜0.05). The corneal DC density of the patients in the hormone treated group and the non-hormone treated group was (24.43±8.32) and (41.22±9.86) cells/mm2, respectively. The difference in corneal DC density between the two subgroups was statistically significant (P＜0.001). Correlation analysis showed that there was a significant negative correlation between logMAR BCVA and CNBL and CNFL in patients with DON (r=－0.422, －0.456; P＜0.05). ConclusionsThere are different degrees of corneal nerve fiber damage in patients with different types of DON. There was a negative correlation between BCVA and the length of corneal nerve fibers.
ObjectiveTo evaluate the visual improvement of therapeutic plasma exchange (TPE) for refractory optic neuritis (ON) patients in acute phase.MethodsSeventy-five affected eyes from 44 refractory ON patients with severe visual defect or resistance to high-dose intravenous methylprednisolone (IVMP) therapy, who were admitted to The Chinese PLA General Hospital between January 2015 and August 2016, were recruited and received TPE therapy. Among these patients, 11 were male and 33 were female; the average age was 39.1±13.9; 31 patients had two affected eyes, 13 patients had one affected eye. The course of the disease on the group of patients were more than 2 weeks, and the visual acuity worsened for more than 10 days and continued to deteriorate. TPE treatment was performed on all of the patients. BCVA was recorded before and 24 h after treatment, and the visual function was scored using visual outcome scale (VOS). At the same time, the adverse reactions of TPE treatment were observed. The paired t-test was used to compare the VOS before and after treatment. The correlation between VOS before and after treatment was analyzed by Linear-by-Linear correlation analysis.ResultsAmong 75 affected eyes, the post-therapy VOS 3.89±2.13 was significantly improved from pre-therapy VOS 5.56±1.69 (t=6.77, P＜0.001). Forty-eight of 75 eyes were improved at lease 1 score of VOS, the overall rate of visual improvement was 64.0%. Especially among the eyes with initial vision of light perception, an improved rate of 82.4% was presented. 75.0% in those eyes with initial vision of count fingers and 67.7% in no light perception. Linear-by-Linear correlation analysis showed a significant linear correlation between the scores of VOS before and after TPE treatment (r=0.398, P=0.01). During the course of TPE treatment, 5 patients had mild adverse reactions such as low calcium reaction and allergic reaction and were well controlled after treatment.ConclusionUsing TPE to treat refractory ON in acute phased can improve the visual function of patients.
Optic neuritis (ON) is one of the symptom of a central nervous system demyelinating, systemic or infectious disease. The pathogenetic mechanism of ON is still not completely clear, and its core is inflammation and immune that occurred in the optic nerve axons, and apoptosis of RGC was induced. Few patients experience recurrent episodes after treatment, presenting a remission - recurrence course of polyphasic disease, named recurrent ON (RON). Two forms of RON have been assigned: recurrent isolated optic neuritis, which is a chronic corticosteroid-dependent optic neuropathy with intermittent acute relapses, and recurrent isolated optic neuritis, which is a non-progressive relapsing ON without steroid dependence. Recurrence of ON causes cumulative damage to the optic nerve lesions and impaired visual signal transmission, thus causing irreversible damage to vision. Therefore, it is very important to have a deep understanding of the pathogenesis of ON and the clinical characteristics of RON, so as to better conduct clinical treatment.
Plasma exchange (PE) is a therapeutic blood component replacement method. The blood of patients is first separated into plasma and blood cell components using a blood cell separator in vitro, the plasma containing harmful pathogenic substances is then discarded and replaced with the same volume of exchange solution. Finally the separated blood cells together with the exchange solution are returned back to the blood circulation of patients. By reducing the circulating antibodies, abnormal plasma proteins or cytokines and other pathogenic molecules, PE can block the disease process. PE has a good therapeutic effect on neuromyelitis optica-related optic neuritis (NMO-ON), which shows resistant to glucocorticoid therapy for the first onset. The American Society for Apheresis guideline evaluates PE for acute optic neuritis as a recommended grade 1B, type II indication. In the implementation of PE treatment for NMO-ON and other diseases, indications and contraindications should be strictly adhered to the guideline, treatment procedures and protocols should be optimized, common adverse events and its prevention and management should be known and alerted. It is important to conduct multi-center clinical cooperation and a high standard clinical randomized controlled study, to find out the optimal time window, the best protocol, and the associated factors for the efficacy and prognosis of PE in NMO-ON.