Objective To detect the color damage in patients with idiopathic optical neuritis (ION) after the treatment.Methods A total of 26 ION patients (44 eyes) with ION whose visual acuity were above 1.0 were collected. All the patients had undergone the treatment of incretion and had the visual acuity more than 1.0 after the treatment.The results of MRI and blood examination were normal. Another 24 healthy persons were selected as the normal control. Total error scores (TES) and each error score of red, green and blue were measured via Farnsworth Munsell100 hue tester. The TES origin scores and their square roots were used for a statistical analysis. The results of the two groups were compared.Results There weresignificant differences in TES and its square roots between ION group and the normal control group (t=3.079，3.133；P=0.0033，0.0026).The differences in the level of error scores of each color between the tow groups were not significant (t=1.91,1.15,1.62; P=0.061,0.26,0.11);but the differences in the square roots of red color between the two groups were statistically(t=2.21,P=0.031).Conclusion After the treatment,the visual acuity of ION patients increases,but the color damage still exist; red color damage happens first.
Purpose To investigate mitochondrial DNA (mt-DNA) mutations in optic neuritis of unknow reason (ONUR) and to assess the pathogenic and differential diagnostic values of screening for mt-DNA mutations in ONUR. Method Thirty patients with ONUR were screened for mt-DNA mutations by using SSCP,mutation-specific primer PCR and sequencing. Results mt-DNA mutations were found in 12 out of the thirty patients.All of the mutations were at 11778 position,but no one at 3460 and 15257. Conclusions Quite a number of patients (12/30,40%) with ONUR were caused actually by mt-DNA mutation．Screening for mt-DNA mutation in these patients has a pathogenic and differential diagnostic significance. (Chin J Ocul Fundus Dis,2000,16:78-79)
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.
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.
ObjectiveTo observe the thickness of per-papillary retinal fiber layer (pRNFL) and structural changes of inner macular segmented layers in optic neuritis (ON) patients with positive aquaporin-4 antibody[AQP4-Ab(+)]. Methods60 ON patients (84 eyes) including 30 of AQP4-Ab(+) ON patients (42 eyes) and AQP4-Ab(-) ON patients (42 eyes), and 40 age-gender matched health controls(80 eyes) were recruited in present study. There was no statistical significance in gender (χ2=0.568) and age (χ2=1.472) between the three groups (P > 0.05). There was no statistical significance in the percentage of different course (χ2=0.000) and logMAR best corrected visual acuity (Z=-1.492) between AQP4-Ab(+)ON and AQP4-Ab(-)ON group (P=1.000, 0.136). All subjects were examined by Spectralis-OCT. The thickness of per-papillary, nasal, nasal lower, temporal lower, temporal, temporal upper, nasal upper and papillomacular bundle (PMB) were analyzed as well as nasal pRNFL/temporal pRNFL (N/T). The macular area was divided into three concentric circles which including central region with 1 mm diameter, inner area with > 1 mm but≤3 mm diameter, and outer ring area with > 3 mm but≤6 mm diameter. The macular volume in each partition and volume in macular RNFL (mRNFL), macular ganglion cell layer (mRGCL), macular inner plexiform layer (mIPL) and macular inner nuclear layer (mINL) were analyzed. ResultsCompared to HC group, the thickness of pRNFL, every quadrants and PMB were decreased significantly in ON group (P=0.000); the macular volume and the volume of mRNFL, mRGCL, mIPL were also decreased significantly in ON group (P=0.000); but there was no statistical difference in mINL volume between two groups (P=0.700). Compared to AQP4-Ab(-)ON group, the thickness of nasal and nasal lower were decreased significantly in AQP4-Ab(+)ON group (P=0.010, 0.000); the macular and mIPL volume were also decreased significantly in AQP4-Ab(+)ON group (P=0.038, 0.033); the thickness of inferior, superior and inferior mIPL in outer ring area and nasal mRNFL in inner area were decreased significantly in AQP4-Ab(+)ON group (P < 0.05). ConclusionsCompared to AQP4-Ab(-)ON patients, the pRNFL thickness and mIPL volume decreased in AQP4-Ab(+)ON patients. The thinner pRNFL area is mainly located in nasal, nasal lower quadrants, and inferior, superior mIPL.
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)
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.
ObjectiveTo analyze the clinical features and prognosis of adult optic neuritis patients with positive serum myelin oligodendrocyte glycoprotein antibody (MOG-ON) or aquaporin 4 antibody (AQP4-ON).MethodsA retrospective study. From December 2015 to February 2018, in the Beijing Chaoyang Hospital of Capital Medical University and Chinese PLA General Hospital, 162 eyes of 132 patients with positive serum MOG antibody and AQP4 were included in the study. There were 42 MOG-ON patients (49 eyes, 31.8%), 90 AQP4-ON patients (113 eyes, 68.2%). The clinical features of optic neuritis (annual recurrence frequency, incidence of optic disc edema), brain and optic nerve enhanced MRI, serum autoimmune antibodies and cerebrospinal fluid test results were compared between MOG-ON and AQP4-ON patients. All patients were treated with intravenous methylprednisolone sodium succinate in the acute phase and then switched to oral prednisone acetate tablets. The average follow-up time was 15 months. The glucocorticoid dependence, visual prognosis, spinal cord symptoms, and myelitis at the last follow-up were comparatively analyzed between MOG-ON and AQP4-ON patients. The comparison of the count data was performed by χ2 test, and the measurement data were compared by t test.ResultsCompared with AQP4-ON patients, MOG-ON patients had higher annual recurrence frequency (t=3.760, P=0.005), higher incidence of optic disc edema (χ2=14.777, P＜0.001), higher incidence of hormone dependence (χ2=25.496, P＜0.001), and better visual prognosis (χ2=28.759, P＜0.001). MOG-ON patients were more likely to involve the optic nerve, AQP4-ON patients were more likely to involve the optic chiasm and the optic tract. There was a significant difference in the location of lesions between MOG-ON and AQP4-ON patients (χ2= 5.447, P= 0.015). The proportion of AQP4-ON patients with autoimmune antibodies was significantly higher than that of MOG-ON patients (χ2 = 20.453, P＜0.001). The results of cerebrospinal fluid test showed that the white blood cell count of patients with MOG-ON and AQP4-ON were within the normal range, but the IgG level of AQP4-ON patients was significantly higher than that of MOG-ON patients (t=8.669, P＜0.001). At the last follow-up, there were 7 and 29 patients of myelitis in MOG-ON and AQP4-ON patients respectively (χ2=3.494, P=0.046).ConclusionsThe clinical characteristics of MOG-ON were different from AQP4-ON. The incidence of optic disc edema and recurrence rate were higher, but the proportion of autoimmune antibodies was lower. MOG-ON was more likely to show hormone dependence, but the visual prognosis was better. AQP4-ON was easily involved in optic chiasm and optic tract, and the incidence of myelitis was higher.
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.
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.