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.
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.
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 observe the clinical and imaging features of infiltrative optic neuropathy (ION) secondary to extraocular malignant tumors. MethodsA retrospective case study. From January 2017 to October 2022, 26 eyes of 20 patients with ION secondary to extraocular malignancies and 32 eyes of 16 patients with early papilloedema (EP) secondary to intracranial metastatic carcinoma were included in the study. All eyes underwent best corrected visual acuity (BCVA), fundus color photography, orbital and/or craniocerebral magnetic resonance imaging (MRI). A total of 54 eyes were examined by visual field examination, among which ION and EP were 22 and 32 eyes, respectively. Clinical and imaging features of the affected eye were retrospectively analyzed. ResultsAmong 26 eyes of 20 ION patients, there were 13 males and 7 females, with the mean age of (52.8±16.9) years. There were 10 patients of hematologic malignancy, 7 patients of periorbital malignancy, 2 patients of lung cancer, 1 patient of gastric cancer, 1 patient of breast cancer and 1 patient of prostate cancer. Two patients of nasal lymphoma were recorded as hematologic malignancies and periorbital malignancies. Sixteen patients had a history of systemic or periorbital malignancy, among which 4 patients reported that they had been "clinically cured". Optic neuritis was diagnosed in 15 patients. Among the 16 patients with EP, 5 were males and 11 were females, with the mean age of (47.9±12.3) years. The primary malignant tumors were lung cancer, breast cancer, leukemia, gastric cancer, ovarian cancer, colon cancer and rectal cancer in 7, 2, 2, 2, 1, 1, 1, respectively. In 26 eyes of ION, 20 eyes complained of blurred vision or peripheral vision occlusion and progressive aggravation; no obvious visual symptoms in 6 eyes. BCVA was light sensing to 1.0 with a median of 0.3, including light sensing and light sensing in 4 eyes. Optic disc edema was observed in 19 eyes; no obvious abnormality in 7 eyes. Visual field examination showed that in 22 eyes, normal or mild enlargement of blind spot in 3 eyes, arcuate scotoma in 4 eyes, annular scotoma in 6 eyes, tubular visual field or concentric contraction of visual field in 6 eyes, and diffuse depression in 3 eyes. MRI showed optic nerve enlargement with sheath enhancement in all ION eyes. Among 32 eyes of EP, 28 eyes showed recurrent transient amaurosis, and the other 4 eyes showed horizontal diplopia. BCVA ranged from 0.8 to 1.5, with a median of 1.0. All EP patients showed different degrees of optic disc hyperemia and edema by fundus examination. The visual field examination showed normal or mild enlargement of the physiological blind spot. MRI showed thickening of the optic nerve and widening of the intrathecal space, but no obvious enhancement of the optic nerve and its intrathecal membrane, and obviously enhanced space-occupying lesions in the brain parenchyma, accompanied by compression and edema of the surrounding brain tissue and midline displacement. ConclusionsION secondary to extrocular malignant tumors mainly manifested as mild visual symptoms and obvious optic disc edema. MRI showed thickened optic nerve and strengthened sheath, and no obvious abnormality in optic nerve parenchyma.
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)
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 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.
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.
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.
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)