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.
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 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.
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 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.
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.
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 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.
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 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.