ObjectiveTo summarize the clinical features and visual outcome of posterior scleritis presented with symptoms involving affected optic nerve.MethodsRetrospective case series study. Twelve eyes of 12 female patients with posterior scleritis were included in this study. The average age was 35.2±14.31 years old. The patients got diagnosed with an average of 24.75±22.91 days. Ocular pain was complained in all patients, and blurred vision in 11 patients. The best corrected visual acuity (BCVA), intraocular pressure (IOP), slit lamp microscope examination, B-scan ultrasound, optical coherence tomography (OCT), fundus photography, fundus fluorescein angiography (FFA) and ocular wall thickness measurement were performed in all patients. Nine eyes received visual field examination. All patients received systemic corticosteroid and steroidal eye drops for 3 months. Clinical features and outcome were retrospectively studied.ResultsBefore treatment, the BCVA was from ＜0.1 to ＞0.8. There were 3 eyes with scleral hyperemia, 3 eyes with anterior chamber flares, 12 eyes with papilledema and different degrees of retinal vein dilatation, 3 eyes with star-shaped macular exudates and 2 eyes with macular retinal pigment epithelium detachment. B-scan ultrasound demonstrated that the ocular walls were thickening in all eyes with typical T-sign, and the average thickness was 2.76±0.68 mm. OCT demonstrated optic disc swelling, and the macular retinal detachment in 2 eyes. In the FFA examination, the fluorescein leakage of the disc was enhanced with time. In the Humphrey test, the value of mean deviation (MD) was 12.56±5.73 dB and pattern standard deviation (PSD) was 8.15±4.23 dB in 9 eyes before the treatment. After treatment for 3 months, the symptoms were attenuated and the visual acuity was obviously improved with BCVA>0.1 in all eyes. Scleral hyperemia and anterior chamber flares were only found in 1 eye. The optic disc edema gradually faded away. The ocular wall thickness in the poster part of the eyeball decreased, and the T-sign disappeared in all eyes, the average thickness was 1.53±0.41 mm. Compared with parameters before the treatment, the difference was statistically significant (t=0.003 5, P＜0.05). OCT demonstrated the recovery of the macular retinal detachment. There was no abnormal leakage evidenced in FFA in the optic disc and macular. After treatment, the value of MD and PSD was 5.19±4.82 dB and (4.33±3.76) dB, respectively. The difference of MD value between before and after the treatment was significant (t=0.026, P＜0.05).ConclusionsPosterior scleritis with an initial symptom of optic nerve was tend to affect middle-aged patients, with clinical manifestations of anterior segment signs in some patients and optic disc swelling with retinal vein dilatation in all patients. B ultrasound examination showed typical T sign. Systemic corticosteroid treatment always obtained remission of the ocular inflammatory activity, and could achieve favorable visual outcome.
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.
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.
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 observe the results of function MRI and perimetry in patients with visual pathway diseases.Methods Three patients (6 eyes) with pituitary adenoma and craniopharyngioma diagnosed via pathological examination and three healthy volunteers aged from 24 to 30 were collected. The best corrected visual acuity was nonlight perception1.0 in the 6 sick eyes and 1.0 in the healthy eyes; all the involved individuals had no other ocular diseases except myopia and without any contraindications of MRI. Common tests including the best visual acuity, fundus test by direct or indirect ophthalmoscope, center static visual field tested by Octopus 101 perimeter, program 32, tendency oriented perimetry were performed. The visual stimulation subtended a field of view of about 12 degrees，consisted of high contrast and drifting checkerboards. MRI parameters: GE signa VH/i 30T scanner. Functional data: GRE-EPI sequence，20 slices lying perpendicular to the calcarine sulcus. Anatomical data was obtained using 3DSPGR sequence to acquire high resolution. The cortical surface was unfolded and then cut and inflated. Functional data was presented to the inflated surface and subsequently analyzed by AFNI software.Results In six eyes, three had temporal defects, two had upper temporal visual field defects, and the other one did not finish the visual field test. The retinotopic representations of health adults were obtained by using the phaseencoded visual stimulation. The Eccentricity coordinate maps showed that foveal representations lay in the occipital poles and the representations appeared further anterior as eccentricity increased. The polar angle coordinate maps showed that early retinotopically organized areas had a representation of visual field. The visual cortex beneath the calcarine sulcus matched with the upper visual field of the opposite side and which upon the calcarine sulcus matched with the under visual field of the opposite side. Less or no visual cortex response was revealed in the patients′ function MRI or the response in injury side was vanished. The visual cortex response related with the visual field defects could not be induced in function MRI.Conclusion There is a good correlation between function MRI data and the results of perimetric evaluation. The function MRI can show the visual cortex response correlated with the visual field defects of the patients with visual pathway diseases.
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.
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.
Neuromyelitis optica-related optic neuritis (NMO-ON) is a kind of severe optic nerve disease, which always leads to replase, poor prognosis, and even blindness. Aquaporin 4 antibody (AQP4-IgG) is the main diagnostic biomarker for neuromyelitis optica with high specificity. Serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is helpful for the diagnosis of AQP4-IgG negative patients. The study of biomarkers is helpful to deeply understand the pathogenesis of NMO-ON, help the diagnosis of the disease, and finally make precise treatment. Orbital MRI can help to differentiate MOG-IgG positive from AQP4-IgG positive neuromyelitis optica and optic neuritis, which is very important for the diagnosis of NMO-ON. At present, the standardized treatment of NMO-ON can be divided into two clinical stages: acute stage and remission stage. Corticosteroids and plasma exchange are the main treatments in acute stage, aiming at alleviating acute inflammatory reaction and improving prognosis. Immunosuppressive agents and biological agents are the main treatments in remission stage, aiming at preventing or reducing recurrence. With the development of the diagnosis and treatment of NMO-ON, we find that it is more and more important to strengthen the construction of neuro-ophthalmology team in China, establish clinical epidemiological database of NMO-ON, and carry out multi-centre, large-sample, prospective clinical control studies in China to provide evidence-based medicine for Chinese people. In addition, we need to strengthen efforts to establish and improve the diagnostic criteria for NMO-ON and the promotion of diagnostic and therapeutic criteria, and strive to improve the clinical diagnosis and treatment level of NMO-ON in China.
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)