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.
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.
Objective To observed and analyze the clinical features of patients with nonarteritic anterior ischemic optic neuropathy (NAION) causes of misdiagnosis. MethodsA retrospective case study. From November 2014 to July 2022, 49 NAION patients with 49 eyes diagnosed in Department of Ophthalmology, The First People’s Hospital of Lanzhou were included in the study. All patients were misdiagnosed with other eye diseases at first diagnosis. All eyes were examined by best corrected visual acuity (BCVA), relative afferent pupil defect (RAPD), orbital magnetic resonance imaging (MRI), visual field, optical coherence tomography (OCT), and graphic visual evoked potential (P-VEP). Fluorescein fundus angiography (FFA) was performed in 32 eyes. Clinical and MRI, visual field, P-VEP、FFA features of the patients were retrospectively analyzed. ResultsThere were 31 males and 18 females among the 49 patients. All cases were monocular. Age was (59.3±7.8) years. All of them complained of painless visual acuity loss or occlusion sensation in one eye. There were 12 (24.5%, 12/49) and 37 (75.6%, 37/49) cases with disease duration ＞2 months and ≤2 months, respectively. In 49 eyes, misdiagnosed as optic neuritis, normal tension glaucoma (NTG) or suspected glaucoma, optic disc vasculitis, cataract, diabetic retinopathy, traumatic optic neuropathy and toxic optic neuropathy were 28 (57.1%, 28/49), 11 (22.4%, 11/49), 5 (10.2%, 5/49), 2 (4.1%, 2/49), 1 (2.0%, 1/49), 1 (2.0%, 1/49), 1 (2.0%, 1/49) eyes. 24 (49.0%, 24/49), 16 (32.7%, 16/49) and 9 (18.4%, 9/49) eyes had BCVA＜0.1, 0.1-0.5 and＞0.5, respectively. RAPD was positive in 45 eyes (91.8%, 45/49). There were 37 (75.6%, 37/49) and 12 (24.5%, 12/49) eyes with and without optic disc edema, respectively. Bleeding was observed on and around the optic disc in 15 eyes (30.6%, 15/49). MRI examination showed no obvious abnormality in the optic nerve segments of all affected eyes. OCT showed an increase in retinal nerve fiber layer thickness (307.1±62.1) μm in 37 patients with optic disc edema. The visual field examination showed that 24 eyes (49.0%, 24/49) had typical lower visual field defect connected with the physiological blind spot and circumvented the central fixation point, 6 eyes (12.2%, 6/49) had limited visual field defect connected with the physiological blind spot, and 19 eyes (38.8%, 19/49) had diffuse visual field defect. By P-VEP examination, the amplitude of P100 wave decreased moderately to severely in all affected eyes. There were 24 eyes (49.0%, 24/49) with mild peak delay and 11 eyes (22.4%, 11/49) with moderate peak delay. In 32 eyes examined by FFA, the arteries had early peridisk limitation or diffuse delayed filling, and mid-course fluorescein leakage in the corresponding area. ConclusionsThe main symptoms of NAION patients are painless visual acuity loss in one eye or occlusion of vision. The main clinical features of NAION patients are visual field defect, retinal nerve fiber layer thickening and visual electrophysiological abnormalities. NAION patients with acute or subacute visual loss accompanied by optic disc edema and/or bleeding are often misdiagnosed as optic neuritis, optic neurovasculitis and other types of optic neuropathy. NAION patients with a disease course of ＞2 months are easily misdiagnosed as NTG.
Objective To observe serum uric acid (UA) level of patients with optic neuritis (ON). Methods Thirty-nine patients with ON (ON group), 53 healthy control subjects (control group), 69 patients with multiple sclerosis (MS group) and 51 patients with neuromyelitis optica (NMO group) matched in age and sex were enrolled in the study. In ON group, there were 25 patients with papillitis and 14 patients with retrobulbar type ON. Twenty-eight patients were first time onset while 11 patients were recurrent. The disease duration was less than a year for 28 patients, and over a year for the remainder. Venous blood samples were collected from all individuals in the morning after an overnight fast. UA concentration was measured by the urate oxidaseindirect peroxidase couple assay. Differences of UA concentration were comparatively analyzed among all the groups. UA levels between different genders, different groups, different lesion sites, recurrence and duration of ON were comparatively analyzed. Results Serum UA level in ON group was significantly lower than that in control group (t=3.16,P＜0.05). However, no significant differences were found between ON and MS, ON and NMO, MS and NMO group (t=0.26, 0.94, 1.36;P＞0.05). Serum UA level was significantly lower in female than in male in all groups (F=6.27, 16.20, 21.09, 11.96;P＜0.05). In male and female patients of ON group, UA levels were significantly lower when compared with same gender in control group(t=2.13, 3.04;P＜0.05). However, no differences (P＞0.05) were found between ON and MS of same gender (t=0.25, 0.59), ON and NMO of same gender (t=0.33, 0.63), MS and NMO of same gender (t=0.63, 1.41). Patients with recurrent ON had lower serum UA level than that with first episodes (F=2.73). Patients with duration of over a year had lower serum UA level than that with duration of less than a year (F=0.23). Patients with retrobulbar neuritis also had lower serum UA level than that with papillitis (F=0.76). But the differences were not significant (P＞0.05). Conclusions A reduced serum UA level is found in patients with ON compared with healthy control. But serum UA level is not correlated with recurrence, lesion site or duration of disease.
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)
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.
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.
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)
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.