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 observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16.
Methods
A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis.
Results
23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage.
Conclusions
Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.
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.
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 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)
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 observe and analyze the subtype-specific prognostic factors for visual recovery in patients with demyelinating optic neuritis (DON) after glucocorticoid pulse therapy. MethodsA retrospective cohort study. A total of 195 patients (249 eyes) with DON diagnosed by ophthalmology examination at Department of Ophthalmology, Xi'an People's Hospital (Xi'an Fourth Hospital) from January 2021 to December 2024 were included in the study. According to the results of serum antibody detection and clinical diagnostic criteria, the patients were divided into the neuromyelitis optica spectrum disorder (NMOSD)-associated optic neuritis (ON) (NMOSD-ON) group, the myelin oligodendrocyte glycoprotein antitide-associated ON (MOG-ON) group, and the double antibody negative ON group. They were 51 cases (58 eyes), 72 cases (103 eyes), and 72 cases (88 eyes) respectively. Baseline clinical data, imaging characteristics, and treatment protocols were collected. The primary endpoints were complete visual recovery [best-corrected visual acuity (BCVA) ≥1.0] and moderate recovery (BCVA ≥0.5) at 3 months post-onset. Multivariate logistic regression was used to identify independent prognostic factors for visual outcomes within each subtype. ResultsAt 3 months post-onset, complete recovery rates were 9 (15.5%, 9/58) in the NMOSD-ON group, 64 (62.1%, 64/103) in the MOG-ON group, and 31 (35.2%, 31/88) in the double-seronegative ON group. The results of multivariate regression analysis showed that age [odds ratio (OR) =0.901, 95% confidence interval (CI) 0.854-0.950, P＜0.001] and peak visual acuity (OR=0.311, 95%CI 0.147-0.660, P=0.002) and the involvement of optic nerve length ≥1/2 (OR=3.849, 95%CI 1.083-13.682, P=0.037) were the influencing factors for the complete recovery of visual acuity in the affected eyes of the double antibody negative ON group. Age (OR=0.958, 95%CI 0.933-0.983, P=0.001) was the only influencing factor for the complete recovery of visual acuity in the affected eyes of the MOG-ON group. Peak visual acuity (OR=0.288, 95%CI 0.090-0.927, P=0.037) and optic nerve involvement length ≥1/2 (OR=19.974, 95%CI 1.905-209.559, P=0.013) were the influencing factors for the complete recovery of visual acuity in the affected eyes of the NMOSD-ON group. Age (OR=0.936, 95%CI 0.890-0.983, P=0.009), time from onset to intravenous infusion of methylprednisolone sodium succinate intervention (OR=0.854, 95%CI 0.759-0.961, P=0.009), optic disc edema (OR=4.405, 95%CI 1.108-17.512, P=0.035) and peak visual acuity (OR=0.13, 95%CI 0.046-0.365, P＜0.001) were the influencing factors for the moderate recovery of visual acuity in the affected eyes of the double antibody negative ON group. Peak visual acuity was the only influencing factor for the moderate recovery of visual acuity in the MOG-ON group (OR=0.060, 95%CI 0.010-0.352, P=0.002) and the NMOSD-ON group (OR=0.163, 95%CI 0.053-0.500, P=0.001). ConclusionsThe prognostic factors for visual recovery in patients with DON after glucocorticoid pulse therapy are subtype-specific. Peak visual acuity is a common predictor for all subtypes. For NMOSD-ON and double antibody-negative ON, attention should be paid to the length of optic nerve lesions. MOG-ON is age-related. Early intravenous infusion of methylprednisolone sodium succinate for double antiantibody negative ON is more likely to achieve moderate vision recovery.
ObjectiveTo analyze the clinical features and prognosis factors of aquaporin 4 (AQP4) antibody-positive neuromyelitis optica spectrum disorders related optic neuritis (NMOSD-ON). MethodsAn ambidirectional cohort study. From June 1, 2015 to June 1, 2019, 103 patients with AQP4 antibody-positive NMOSD-ON in Department of Neuro-ophthalmology, The First Medical Center of PLA General Hospital were included. All patients of followed-up period were ≥24 months. According to the best corrected visual acuity (BCVA) at the last follow-up, the affected eyes were divided into the low vision group [log of minimum resolution angle (logMAR) BCVA≥1.0] and the non-low vision group (logMAR BCVA＜1.0), 66 and 37 cases, respectively. The two groups of patients were compared the genernal clinical characteristics, and the logistic regression model and COX proportional hazard model were used to analyze the relevant factors affecting the patient's visual prognosis and recurrence. ResultsAmong the 103 cases, 96 cases (93.2%, 96/103) were female; 94 cases (91.3%, 94/103) had unilateral disease; 48 cases (46.6%, 48/103) were the first onset; 85 cases (82.5%, 85/103) were effected by eye pain or orbital pain; 21 cases (20.4%, 21/103) had optic disc edema; 51 cases (49.5%, 51/103) serologically autoimmune antibody test were positive. Orbital magnetic resonance imaging (MRI) was performed in 101 cases. There was no obvious abnormal signal in visual pathways except for 5 cases (5.0%, 5/101); 96 cases (95.0%, 96/101) had abnormal signal in the visual path, and the optic nerve was found in the orbit; 52 cases had abnormal optic nerve in orbital segment (51.5%, 52/101); 37 cases (35.9%, 37/103) recurred within 24 months. The recovery of logMAR BCVA after the first onset and the logMAR BCVA at the first onset, at 6 months of follow-up in two groups were 1.4±1.0, 0.3±0.4, 1.9±0.7 and 0.4±0.5, 2.1±0.6, 0.3±0.4, respectively; and there were statistically significant differences between the two groups of patients at different times（Z=－4.967,－7.603,－8.027; P＜0.001). Logistic regression multivariate analysis showed that recovery of BCVA≥1.0 logMAR after the first onset [odds ratio (OR)=226.276, P＜0.001] and the number of attacks (OR=8.554, P=0.003) were independent risk factors for low vision. Multivariate analysis of the Cox proportional hazards model showed the higher the MRI score [hazard ratio (HR)=0.588, P=0.007] and plasma exchange (HR=0.124, P=0.049) in the acute phase were protective factors for recurrence. ConclusionsVision loss accompanied by eye pain or orbital pain is the main symptom of onset AQP4 antibody-positive NMOSD-ON, a small number of patients have disc edema, 49.5% patients serologically autoimmune antibody test are positive. Abnormal optic nerve signals can be seen in 95.0% of patients in orbital MRI, and 51.5% patients have abnormalities in the orbital optic nerve. The worse the recovery of BCVA after the first onset and the greater the number of attacks are unfavorable factors affecting the prognosis of vision. High MRI scores and plasma exchange in the acute phase are favorable factors to prevent the recurrence of the disease.
Objective To observe the effect of intravenous methylprednisolone (IVMP) pulse therapy on the best corrected visual acuity (BCVA) and the number of relapses in patients with neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) after total IVMP dose. MethodsA retrospective clinical study. From March 2020 to February 2023, 23 patients of 27 eyes with NMOSD-ON in Shanxi Eye Hospital were included in the study. BCVA examinations were performed on all affected eyes using the international standard visual acuity chart, which was statistically converted into logMAR visual acuity. Serum aquaporin-4 antibody (AQP4-IgG) was detected by indirect immunofluorescence assay based on cell detection technology in all patients. According to Guideline for the diagnosis and treatment of NMOSD spectrum disorders in China (2021 edition), patients were given IVMP impact therapy. Among them, 18 and 5 patients received 1 000 and 500 mg/d IVMP pulse therapy respectively for 3-5 consecutive days, followed by a reduction to 500 or 250 mg/d for 2-3 consecutive days. The average total IVMP dose during the treatment was 4 500 mg (1 500-5 250 mg). The changes in BCVA at 1 week, 1 month, 3 months, and 6 months after treatment were observed for the initial and post-treatment BCVA of ≤0.1, ＞0.1-<0.5, and ≥0.5. The changes of BCVA at 1 week and 1, 3 and 6 months after treatment were observed. The comparison of BCVA between different age, disease duration, and IVMP total dose conditions was performed using the Mann-Whitney U test. The comparison of BCVA between different relapse times was performed using the Kruskal-Wallis test. The influence of IVMP total dose on the number of relapses during the 6-month follow-up was analyzed using χ2 test. The factors affecting BCVA ≥0.5 after 6 months of IVMP treatment were analyzed by logistic regression, and the correlation between ΔlogMAR BCVA and IVMP pulse total dose was analyzed by Spearman correlation. ResultsIn 23 cases with 27 eyes, there were 3 males and 20 females. The median age was 35 years. The median duration of illness was 5 days. There were 21 (91.30%, 21/23) positive and 2 (8.70%, 2/23) negative cases of AQP4-IgG, respectively. There were 3 cases (13.04%, 3/23) with the first course of disease and 4 eyes (14.81%, 4/27). There were 20 cases (86.96%, 20/23) with recurrence course and 23 eyes (85.19%, 23/27). The median time from initial onset to the initiation of corticosteroid treatment was 7 days. During the 6-month follow-up after treatment, 5 patients (21.74%, 5/23) relapsed in 6 eyes (22.22%, 6/27), all of which were patients with initial relapse course. Among them, recurred 1 or ≥2 times in 4 (66.67%, 4/6) and 2 (33.33%, 2/6) eyes respectively. BCVA≤0.1, ＞0.1-＜0.5, ≥0.5 in 20, 4, 3 eyes and 3, 13, 11 eyes at the beginning and 6 months after treatment, respectively. There was significant difference in the number of eyes with BCVA≤0.1, ＞0.1-＜0.5 and ≥0.5 at different time after treatment (χ2=40.772, P＜0.001). The treatment effect of female patients was better than that of male patients. The patients with initial BCVA≥0.1 had more increased eye number of BCVA than those with BCVA＜0.1, the patients with first course of disease had more increased eye number of BCVA than those with recurrent course of disease, and the patients with total dose of IVMP ＞4 500 mg had less increased eye number of BCVA than those with total dose ≤4 500 mg. The differences were statistically significant (Z=?2.449, ?2.904, ?2.485, ?2.286; P=0.014, 0.004, 0.013, 0.022). Logistic regression analysis showed that the higher the initial BCVA≤0.1 and the total impact dose of IVMP, the lower the possibility of obtaining BCVA≥0.5 after treatment (odds ratio=0.069, 0.899; 95% confidence interval 0.010-0.463, 0.798-0.998; P=0.006, 0.020). Spearman correlation analysis showed that ΔlogMAR BCVA was negatively correlated with total impact dose of IVMP (rs=?0.472, P=0.013). There was no significant difference in the number of recurrence after different total doses of IVMP (P＞0.05). ConclusionsIVMP total dose ≤4 500 mg can achieve better BCVA prognosis compared with IVMP total dose ＞4 500 mg. IVMP total dose has no effect on the number of recurrences after treatment.
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.
