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.
Infectious and infection-related optic neuritis is an important type of optic neuritis. Infectious optic neuritis is caused by direct spread of pathogenic organism to optic nerve from local infection or blood transmission. Infection-related optic neuritis is caused by pathogens-induced immune allergic reaction. They present with atypical clinical features of optic neuritis, including progressive vision loss, persistent eye pain or headache, ineffectiveness or even worse of glucocorticoid therapy. Fundus manifestations include optic disc swelling with peripapillary hemorrhage or neuro-retinitis, and the feature of concurrent uveitis. When these patients first visit ophthalmic clinics, they often lack signs of systemic infection, thus it is easy to misdiagnose them as other types of optic neuropathy and mistakenly treat them. In particular, high-dose glucocorticoid therapy can lead to very serious consequences. Therefore, how to correctly diagnose infectious and infection-related optic neuritis in the early stages are very important for ophthalmologists and need to be seriously kept in our mind.
Objective To explore underlying causes of presumptive optic neuritis (ON) in children. Methods Retrospective study of continuous cases with presumed diagnosis of optic neuritis in childhood. Results 104 cases(65.8%) met ON criteria in this cohort of children, among wh ich 80 cases (76.9%) were considered as idiopathic demyelinating optic neuritis (IDON). Infectious optic neuritis and inflammatory optic neuropathy were found on 3 cases respectively. The cause of 18 cases remains unknown. Leber hereditary optic neuropathy and non-organic visual acuity loss account most of the 54 case s misdiagnosed as optic neuritis. Conclusions As in adult patients, idiopathic demyelinating optic neuritis is the most common pathogeny of optic neuritis in children, while infectious events were more common in children. Leber hereditary optic neuropathy and nonorganic visual acuity loss were the most common disease confused with optic neuritis in childhood. Some rare disease in childhood which can cause optic nerve lesion should also be considered. (Chin J Ocul Fundus Dis, 2008,24：95-98)
Objective To construct and evaluate a screening and diagnostic system based on color fundus images and artificial intelligence (AI)-assisted screening for optic neuritis (ON) and non-arteritic anterior ischemic optic neuropathy (NAION). MethodsA diagnostic test study. From 2016 to 2020, 178 cases 267 eyes of NAION patients (NAION group) and 204 cases 346 eyes of ON patients (ON group) were examined and diagnosed in Zhongshan Ophthalmic Center of Sun Yat-sen University; 513 healthy individuals of 1 160 eyes (the normal control group) with normal fundus by visual acuity, intraocular pressure and optical coherence tomography examination were collected from 2018 to 2020. All 2 909 color fundus images were as the data set of the screening and diagnosis system, including 730, 805, and 1 374 images for the NAION group, ON group, and normal control group, respectively. The correctly labeled color fundus images were used as input data, and the EfficientNet-B0 algorithm was selected for model training and validation. Finally, three systems for screening abnormal optic discs, ON, and NAION were constructed. The subject operating characteristic (ROC) curve, area under the ROC (AUC), accuracy, sensitivity, specificity, and heat map were used as indicators of diagnostic efficacy. ResultsIn the test data set, the AUC for diagnosing the presence of an abnormal optic disc, the presence of ON, and the presence of NAION were 0.967 [95% confidence interval (CI) 0.947-0.980], 0.964 (95%CI 0.938-0.979), and 0.979 (95%CI 0.958-0.989), respectively. The activation area of the systems were mainly located in the optic disc area in the decision-making process. ConclusionAbnormal optic disc, ON and NAION, and screening diagnostic systems based on color fundus images have shown accurate and efficient diagnostic performance.
Objective To compare the differences of optic disc morphology and optic nerve head parameters between Vogt-Koyanagi-Harada (VKH) syndrome and optic neuritis (ON) with optic disc edema. Methods This is a retrospective study including 21 first-onset VKH patients (35 eyes) as VKH group and 22 first-onset ON patients with optic disc edema (27 eyes) as ON group. The differences of age (t=−1.11) and gender (χ2=0.20) between two groups were not significant (P＞0.05). Sixty-two eyes of 43 age and gender-matched healthy subjects were enrolled in this study as control group. All subjects underwent three dimensional optical coherence tomography (3D-OCT) examinations. The difference of optic disc morphology between two groups was observed. The parameters included average thickness of entire circumpapillary retinal nerve fiber layer (CP-RNFL), thickness of nasal, superior, temporal and inferior quadrant of CP-RNFL, disc area, disc cup area, rim area, cup/disc (C/D) area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio. The disc retinal pigment epithelium (RPE) angle was observed too. Results Among 35 eyes in VKH group, 31 eyes (88.57%) had retinal detachment next to the disc, 3 eyes (8.57%) had serrated inner limiting membranes of the disc. Twenty eyes (64.52%) had highly reflective points, lines, or membrane-like structures in the retinal detachment areas. No such signs appeared in ON patients. Compared with ON group, the optic cup area, C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio were bigger, the thickness of mean CP-RNFL and the superior, inferior quadrants of CP-RNFL were thinner, disc RPE angles was smaller in VKH group (P＜0.05). Compared with control group, the disc area, optic cup area, rim area were bigger, C/D vertical diameter ratio was smaller, the mean CP-RNFL and 4 quadrants CP-RNFL were thicker, disc RPE angles was smaller in VKH group (P＜0.05); the disc area, optic cup area, C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio were smaller, the mean CP-RNFL and 4 quadrants CP-RNFL were thicker, disc RPE angles was bigger in VKH group (P＜0.05). Conclusions VKH patients have smaller disc RPE angles and more chance to develop retinal detachment next to disc than ON patients. The C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio are bigger, the mean CP-RNFL and the superior, inferior quadrants of CP-RNFL are thinner in VKH eyes.
Neuromyelitis optica (NMO) is an autoimmune inflammatory diseases of the central nervous systems (CNS) mainly affecting the optic nerves and spinal cord. It has the characteristics of high recurrence rate and poor prognosis. NMO related optic neuritis is a common neuro-ophthalmic disease which often results in permanent visual loss or even blindness. Aquaporin 4 (AQP4) antibody is a specific and pathogenic autoantibody in NMO patients. Although AQP4 is expressed in multiple tissues, NMO pathology is remarkably limited to the CNS. Corticosteroids and other immunosuppressive drugs are the standard managements for NMO patients, in order to reduce the relapses and the severity of the acute attack. Multiple avenues of investigation in the laboratory have significantly advanced our understanding of NMO pathophysiology, which is helpful for our understanding of immunologic and nonimmunologic mechanisms. Many offer significant means for NMO therapy by selectively targeting pathways. In the future, moving these agents from the bench to the bedside offers the opportunity to identify safe and effective therapies that limit CNS injury and preserve visual function.
Objective To investigate the etiological distribution of the patients with optic neuritis in China and compare the results with those in western countries. Methods Ophthalmological and neurological detailed clinical and laboratorial examinations were performed on 204 patients with primarily diagnosed optic neuritis (ON). We determined the etiologies using international accepted diagnostic criteria. Results Among 113 patrents with ON, 83(73.5%) were considered as with idiopathic demyelinating optic neuritis ( IDON). Sinusitis was common in these patients but was considered to be the probable cause of ON only in 4. Tuberculo-meningitis caused ON was found in 2 cases and syphilitic ON in 1. The causes of 23 cases (20.4%) were unknown. Conclusions Idiopathic demyelinating ON is the most common pathogeny of ON. Despite of some minor differences of causes and prognosis, the etiology of presumed ON in our population is similar to that reported in western countries. (Chin J Ocul Fundus Dis,2006,22:367-369)
ObjectiveTo investigate the alteration of retinal perfusion in aquaporin-4 antibody (AQP4-ab) positive neuromyelitis optica spectrum disorders (NMOSD) patients by optical coherence tomography angiography (OCTA).MethodsA case-control study. Forty-eight AQP4-ab positive NMOSD patients (96 eyes) and 20 age and gender matched healthy controls (40 eyes) were recruited from September 2015 to August 2017 at the Eye & ENT Hospital of Fudan University. Patients of both eyes were included in the groups. The patients were further divided into 4 subgroups (0 ON, 1 ON, 2 ON, 3+ ON group) according to the number of episodes of ON (0, 1, 2, or 3+) with respect to 30、22、31、13 eyes. 0 ON group had no history of ON episodes; 1 ON group, 2 ON group, and 3+ ON group had ON episodes 1, 2, ≥3 times, respectively. All patients underwent complete ophthalmological examinations including BCVA, visual field and OCTA examination. The BCVA was recorded for each eye using metric notation from the Snellen chart, and then converted to the logarithm of the minimum angle of resolution. The central visual field was assessed using a Humphrey Field Analyzer 750 and the mean deviation (MD) was determined. OCTA scans of the optic disc (4.5 mm × 4.5 mm) and macula (6 mm × 6 mm) were acquired. Radial peripapillary capillary (RPC) vessel density, superficial capillary plexus vessel density (SVD), the thickness of ganglion cell and inner plexiform layer (GCIPL) and peripapillary retinal nerve fiber layer (pRNFL) were determined. The generalized estimating equations was performed to compare the difference of BCVA, MD, pRNFL thickness, GCIPL thickness, RPC vessel density and SVD among the groups and the correlations between retinal perfusion and retinal structure, visual function were analyzed. ResultsThe RPC vessel density and SVD were significant lower in the 0 ON group compared with healthy group (Wald χ2=7.190, 10.134; P＜0.01), however, the BCVA, pRNFL and GCIPL thickness were not significant difference between the two groups (Wald χ2=2.308, 1.020, 2.558; P＞0.05). The BCVA, visual field MD, RPC vessel density, SVD, pRNFL and GCIPL were significant lower in 1 ON, 2 ON and 3+ ON groups compared with healthy group and 0 ON group (Wald χ2=12.390, 11.346, 38.860, 18.040, 45.418, 26.608; P＜0.001 ), but the parameters had no significant difference among the three groups (P＞0.05). The RPC vessel density was significantly correlated with pRNFL thickness (β=0.372, standard error=0.018, P＜0.001), and the SVD was significantly correlated with GCIPL thickness (β=0.115, standard error=0.204, P＜0.001). The MD and BCVA was significantly correlated with peripapillary vessel density after adjustment for other variables (BCVA: β=0.025, standard error=0.005, P=0.000; visual field MD: β=0.737, standard error=0.185, P=0.000).ConclusionsSubclinical primary retinal vasculopathy may occur in NMOSD prior to ON attack, the ON attack may further impair visual function, retinal structure and perfusion, however, the extent of injure is not relevant with the increase of ON attack. The peripapillary vessel density might be a sensitive predictor of visual outcomes in NMOSD patients.
Neuromyelitis optica spectrum disorders (NMOSD) are a group of inflammatory disorders of the central nervous system characterized by episodes of immune-mediated demyelination and axonal damage mainly involving optic nerves and spinal cord. Neuromyelitis optica related optic neuritis (NMO-ON) is a common neuro-ophthalmic disease which often results in permanent blindness. The discovery of aquaporin 4 antibodies confirms that neuromyelitis optica is a distinct disease entity different from multiple sclerosis. In patients with NMO-ON, the correct therapeutic approach has to recognize two distinct clinical situations: treatment of the acute attacks and prevention of the relapses. With the in-depth study of the pathogenesis of NMOSD, new treatments are emerging in different targets of the disease. This review gives an update of latest treatment of NMO-ON, emphasizing both current situation and future immunotherapy strategies.
ObjectiveTo analyze the prognostic factors of vision of myelin oligodendrocyte glycoprotein (MOG) antibody positive associated optic neuritis (ON) after methylprednisolone pulse therapy. MethodsA clinical observational study. A total of 32 patients (47 eyes) with MOG antibody positive ON were observed and followed up in the ophthalmology department of Beijing Tongren Hospital Affiliated to Capital Medical University and Beijing Puren Hospital from March 2019 to January 2022. Clinical data including the best corrected visual acuity (BCVA) and orbital magnetic resonance imaging were recorded. The BCVA was examined by Snellen visual acuity chart, which was finally converted into the logarithm of the minimal angle of resolution (logMAR) for statistical analysis. There were 22 case (38 eyes) with complete image data. All patients were treated with intravenous methylprednisolone pulse (IVMP) for 3-5 days. According to the intervention time (from onset to glucocorticoid treatment), the patients were divided into three groups: ＜7 d group, 7-14 d and ＞14 d group, with 16, 13, 11 eyes, respectively. The median follow-up time was 28 months. After 1 week, 1, 3 and 6 months treatment, the same equipment and methods were used for relevant examinations to observe the changes of visual acuity and the factors influencing the prognosis of visual acuity after IVMP treatment. Logistic regression and linear regression were used to analyze the prognostic correlation factors. Receiver operating characteristic (ROC) curve was used to determine the critical cut-off point of intervention timing. ResultsAmong the patients, 16 were male and 16 were female. The median onset age was 26 years. The onset duration time was 5-60 days. There were 18 cases (56.3%, 18/32) with abnormal serum immune indexes. The initial symptom was decreased vision with unilateral or bilateral ON. Seventeen (53.1%, 17/32) cases had unilateral ON and 15 (46.9%, 15/32) cases with bilateral ON. Thirty-six eyes (76.6%, 36/47) got optic disc edema, 37 eyes (78.7%, 37/47) accompanied by pain of ocular movement. The nadir logMAR BCVA was mean 1.69±0.13. Long T2WI signals with segmental thickening in the orbital segment of the optic nerve were obtained in 27 eyes (71.1%, 27/38) and in 24 eyes (63.2%, 24/38) with optic nerve and sheath enhancement. During the follow-up period, there were 10 cases of relapse (31.3%, 10/32). The logMAR BCVA of attacked eyes were 0.52±0.09, 0.22±0.06, 0.12±0.06, 0.10±0.06 at 1 week and 1, 3 and 6 months after IVMP treatment, respectively. The rate of BCVA improvement was the fastest at 1 week after treatment, and BCVA returned to stability at 3 months. Logistic regression analysis showed that the timing of intervention was significantly correlated with the prognosis of vision in primary onset patients (odds ratio=12.17, P=0.006), with a negative linear regression relationship (r=－0.48, 95% confidence interval －0.71-－0.17, P=0.008). Comparing the logMAR BCVA between the intervention time ＞14 group with the ＜7 group and the 7-14 group, there were statistically significant difference (P=0.017, 0.037), respectively. The cut-off point of ROC curve to predict the optimal intervention time was 13.5 days. Other factors such as: gender, age, predisposing factor, pain of eye motion, edema of optic disc, bilateral ON, imaging changes, abnormal autoimmune indicators were not associated with the prognosis of visual acuity. ConclusionThe timing of hormone intervention in primary onset patients is an important factor affecting the prognosis of vision and the optimal intervention time window of IVMP is two weeks.