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BackgroundBilateral optic neuritis associated with optic disc swelling is a common feature of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, extensive deep retinal haemorrhages have not been described in the context of MOG-associated optic neuritis. Here, we report a case of infectious mononucleosis with marked binocular peripapillary and perivascular haemorrhages as well as extensive deep retinal haemorrhages in the presence of MOGAD.Case reportA 39-year-old Chinese woman presenting with subacute binocular vision reduction with no light perception was diagnosed with MOGAD. Fundus examination revealed the presence of binocular peripapillary and perivascular haemorrhages as well as extensive deep retinal haemorrhages with severe optic disc swelling greater in the right eye than in the left and dilated and tortuous retinal venules. The patient tested positive for the Epstein–Barr virus (EBV) antigen (595 U/mL) and the EBV capsid antigen (>750 U/mL). She had a fever and right upper quadrant abdominal pain, and a doctor determined splenomegaly 1 week before the onset of orbital pain and decreased vision acuity. Medical history and laboratory tests indicated the presence of concurrent infectious mononucleosis. Other investigational indicators of retinal haemorrhages, including hypertension, diabetes mellitus, vascular disease, systemic lupus erythematosus, metabolic disease, and renal or liver dysfunction, were absent.DiscussionThis case suggests that retinal haemorrhage is a possible complication of infectious mononucleosis in the presence of MOGAD.

PurposeAs glial autoantibody testing is not yet available in some areas of the world, an alternative approach is to use clinical indicators to predict which subtypes of middle-aged and elderly-onset optic neuritis (ON) have manifested.MethodThis study was a single-center hospital-based retrospective cohort study. Middle-aged and elderly-onset ON patients (age > 45 years) who had experienced the first episode of ON were included in this cohort. Single- and multi-parametric diagnostic factors for middle-aged and elderly-onset myelin oligodendrocyte glycoprotein immunoglobulin-associated ON (MOG-ON) and aquaporin-4 immunoglobulin-related ON (AQP4-ON) were calculated.ResultsFrom January 2016 to January 2020, there were 81 patients with middle-aged and elderly-onset ON, including 32 (39.5%) AQP4-ON cases, 19 (23.5%) MOG-ON cases, and 30 (37.0%) Seronegative-ON cases. Bilateral involvement (47.4%, P = 0.025) was most common in the MOG-ON group. The presence of other concomitant autoimmune antibodies (65.6%, P = 0.014) and prior neurological history (37.5%, P = 0.001) were more common in the AQP4-ON group. The MOG-ON group had the best follow-up best-corrected visual acuity (BCVA) (89.5% ≤ 1.0 LogMAR, P = 0.001). The most sensitive diagnostic factors for middle-aged and elderly-onset MOG-ON were ‘follow-up VA ≤ 0.1 logMAR’ (sensitivity 0.89), ‘bilateral involvement or follow-up VA ≤ 0.1 logMAR’ (sensitivity 0.95), ‘bilateral involvement or without neurological history’ (sensitivity 1.00), and ‘follow-up VA ≤ 0.1 logMAR or without neurological history’ (sensitivity 1.00), and the most specific factor was ‘bilateral involvement’ (specificity 0.81). The most sensitive diagnostic factors for middle-aged and elderly-onset AQP4-ON were ‘unilateral involvement’ (sensitivity 0.88), ‘unilateral involvement or neurological history’ (sensitivity 0.91), and ‘unilateral involvement or other autoimmune antibodies’ (sensitivity 1.00), and the most specific factor was neurological history (specificity 0.98).ConclusionBased on our cohort study of middle-aged and elderly-onset ON, MOG-ON is less prevalent than AQP4-ON and Seronegative-ON. Using multiple combined parameters improves the sensitivity and negative predictive value for diagnosing middle-aged and elderly-onset MOG-ON and AQP4-ON. These combined parameters can help physicians identify and treat middle-aged and elderly-onset ON early when glial autoantibody status is not available.

BackgroundC-X-C motif chemokine 12 (CXCL12) is a chemokine that performs many functions. Studies have shown that CXCL12 can aggravate inflammatory symptoms in the central nervous system (CNS). Evidence also indicates that CXCL12 can promote the repair of myelin sheaths in the CNS in experimental autoimmune encephalomyelitis (EAE). Here, we investigated the function of CXCL12 in CNS inflammation by upregulating CXCL12 in the spinal cord and subsequently inducing EAE.Materials and methodsCXCL12 upregulation in the spinal cords of Lewis rats was induced by the injection of adeno-associated virus 9 (AAV9)/eGFP-P2A-CXCL12 after intrathecal catheter implantation. Twenty-one days after AAV injection, EAE was induced and clinical score was collected; Immunofluorescence staining, WB and LFB-PAS staining were used to evaluate the effect of CXCL12 upregulation. In the in vitro study, oligodendrocyte precursor cells (OPCs) were harvested, cultured with CXCL12 and AMD3100, and subjected to immunofluorescence staining for functional assessment.ResultsCXCL12 was upregulated in the lumbar enlargement of the spinal cord by AAV injection. In each stage of EAE, upregulation of CXCL12 significantly alleviated clinical scores by inhibiting leukocyte infiltration and promoting remyelination. In contrast, the addition of AMD3100, which is a CXCR4 antagonist, inhibited the effect of CXCL12. In vitro, 10 ng/ml CXCL12 promoted the differentiation of OPCs into oligodendrocytes.ConclusionAAV-mediated upregulation of CXCL12 in the CNS can alleviate the clinical signs and symptoms of EAE and significantly decrease the infiltration of leukocytes in the peak stage of EAE. CXCL12 can promote the maturation and differentiation of OPCs into oligodendrocytes in vitro. These data indicate that CXCL12 effectively promotes remyelination in the spinal cord and decreases the signs and symptoms of EAE.

Neuromyelitis optica spectrum disorder (NMOSD), a severe demyelinating disease, is rare among children. Plasma exchange (PE) is widely used as a salvage therapy for severe and corticosteroid-unresponsive patients with NMOSD. Presently, there are limited studies on the safety and efficacy of PE in children with NMOSD. Herein, we report the case of six children with NMOSD who received PE along with the outcomes and adverse events. All six children (female, age at onset 4 years 9 months–13 years 2 months) were AQP4-IgG positive and received standard PE using the COM.TEC Cell Separator. The interval between NMOSD onset and PE was 29 days (range 10–98). Only one patient (P3) who received PE 10 days after acute exacerbations exhibited clinical improvement. Her left visual acuity increased from 0.06 to 0.6 (spectacle-corrected visual acuity was 1.0) and her EDSS score decreased from 4 to 3 points. The other five patients had no clinical improvement and no EDSS scores changes after PE. Adverse events included rashes (P1, P3), acute non-occlusive thrombosis of the internal jugular vein (P1), and thrombocytopenia (P2). In conclusion, the timing of PE initiation as a rescue therapy for severe and corticosteroid-unresponsive pediatric AQP4-IgG positive NMOSD may be crucial to treatment efficacy, and early initiation of PE may be associated with a better outcome. Furthermore, PE has the potential risk for clinically significant adverse effects that should be considered before initiating the therapy and should be weighed against potential benefits.

ObjectiveThis study aimed to investigate the clinical spectra and outcomes in pregnancy-related optic neuritis (ON).MethodsWe analyzed the clinical subtype and prognosis of women with pregnancy-related ON in the neuro-ophthalmology department of the First Medical Center at the Chinese PLA General Hospital from January 2014 to December 2019.ResultsA total of 54 patients, including 21 (38.9%) with idiopathic ON (ION), 27 (50.0%) with aquaporin-4 (AQP4)-ON, and 6 (11.6%) with myelin oligodendrocyte glycoprotein (MOG)-ON, who experienced 58 informative pregnancies and 67 episodes of pregnancy-related ON were assessed. Among the ON attacks, there were 11 (16.4%) during pregnancy and 56 (83.6%) within 1 year postpartum (PP1) or after abortion, including 33 (49.3%) in the first trimester. In total, 14 (25.9%) patients with ON onset before pregnancy had a higher relapse rate during PP1 than within 1 year before pregnancy (p = 0.021), and 24 (85.7%) eyes with ION and nine (100%) with MOG-ON had significantly better visual outcomes (p ≥ 0.5) than those with AQP4-ON (14, 35%) (p < 0.001 and p < 0.001, respectively). Two AQP4-ON patients had premature birth and low baby weight, respectively. There were no birth defects or stillbirths.ConclusionThe significantly increased relapse rate and numerous cases of ON after pregnancy suggest that delivery adversely affects the course of ON.

PurposeWe aimed to investigate the refractive status and prevalence of refractive error, as well as its characteristics in Chinese preschool children aged 1–6 years old.MethodsA population-based cross-sectional study—Changsha Children Eye Study (CCES) was conducted. The prevalence of refractive errors among children aged 1–6 years old from 18 community health service centers was surveyed. A handheld child vision screener, Suowei, was used for examination.ResultsA total of 43,105 preschool children were included. The mean spherical equivalent (SE) was 0.42 ± 1.05 D for the right eyes. The mean astigmatism (diopter of cylinder, DC) was −0.83 ± 1.02 D for the right eyes. The magnitude of refractive error was lower in older children, indicating the ongoing of the emmetropization during the 1–6-year-old children. The prevalence of myopia (SE ≤ −1.00 D), hyperopia (SE ≥ +2.00 D) and astigmatism (DC ≥1.50 D) was 2.94, 13.8 and 17.6%, respectively. The prevalence of myopia decreased with the increase of age between the six age groups (P < 0.001). The prevalence of hyperopia was lower in 5–6 years old, whereas, the prevalence of myopia was slightly higher at this period of time. With-the-rule (WTR) astigmatism (+ cylinder axis 90° ± 15°) was the most prevalent type of astigmatism than against-the-rule (ATR) astigmatism (+ cylinder axis 180° ± 15°) and oblique (OBL) astigmatism (X2 = 209.5, P < 0.001). The binary logistic regression model showed that older age and suffering astigmatism were independently associated with the development of myopia. In addition, there was no significant gender difference in the prevalence of myopia, emmetropia, and hyperopia.ConclusionsOur population-based cross-sectional study investigated the prevalence of myopia, hyperopia, and astigmatism in preschool children aged 1–6 years old. The distribution of the refractive error was disperse in the younger group and gradually turned more centralized in older group. Similar to hyperopia, with age increased, the prevalence of myopia was lower in preschool children younger than 5 years old and then slightly increased at 5–6 years, which may indicate an early sign of myopia in school-age children. Therefore, we emphasize that more attention should be given to the children at this age.

PurposeTo investigate the longitudinal changes in refractive error of preschool children and explore the factors related to these changes and the timing of intervention.MethodsThe refractive data of preschool children aged 1–6 years were collected from 16 community Health Service Centers in Changsha during April 2016 to July 2019 for the retrospective cohort study. The refractive data of each participant was measured with a hand-held vision screener without cycloplegia. A follow-up for all the included participants was performed. The spherical equivalent change was calculated, subsequently, an analysis of risk factors related to the change was performed.ResultsFour thousand nine hundred twenty-one cases were included in the study with the follow-up for 1–2 years. The refractive status was found smoothly changed in 67.8% of children. The overall initial SE was 0.62 ± 1.13 D, and the average SE change was −0.20 ± 1.23 D per year. However, profound myopic shift was observed in 32.2% of children. The change of SE in 3-year-old group is most overt. The proportions of 1–6 years old who showed moderate and severe myopic shift (SE change ≥–1.00 D) were 21.6, 18.9, 28.2, 25.5, 13.4, and 10%, respectively. At the first visit, the younger children with greater hyperopic state exhibited more noticeable myopic shift, no significant difference was found in gender.ConclusionThe shift from hyperopia to myopia in preschool children is smooth, with −0.20D change on average per year. We suggest that an optometry screening should start at 3-year-old to track children's refractive status. We recommend that preschool children whose SE changes more than −1.00 D per year go to the ophthalmology department for further examination. Our study also found that at the first visit, the younger the child is and the more positive initial SE is, the degree of shift of myopia is greater.

Purpose: To prospectively investigate the efficacy and tolerance of low-dose rituximab (RTX) for the treatment of neuromyelitis optica-associated optic neuritis (NMO-ON).Methods: Optic Neuritis patients with seropositive aquaporin 4-antibody (AQP4-Ab) were diagnosed with NMO-ON and recruited for treatment with low-dose RTX (100 mg * 4 infusions) and were then followed monthly for a minimum of 3 months. Reinfusion of 100 mg RTX was given when the CD19+ B lymphocyte frequency was elevated to above 1%. The serum AQP4-Ab level was tested by an enzyme-linked immunosorbent assay (ELISA).Results: A total of 43 NMO-ON patients (1 male/42 female, 75 involved eyes) were included in this study. CD19+ B cell clearance in the peripheral blood was induced in 97.7% of patients after induction treatment. A significant decrease in serum AQP4-Ab concentration was observed after induction treatment (P = 0.0123). The maintenance time of B cell clearance was 5.2 ± 2.25 months. The relapse-free rate was 92.3% in patients followed-up for over 12 months, and patients with non-organ-specific autoimmune antibodies tended to relapse within 6 months. A total of 96.2% of patients had stable or improved vision, and a decrease in the average expanded disability status scale (EDSS) score was found. Structural alterations revealed by optic coherence tomography were observed in both ON and unaffected eyes. The rates of infusion-related reactions and long-term adverse events (AEs) were 18.6 and 23.1%, respectively. No severe AEs was observed.Conclusions: Low-dose rituximab is efficient and well-tolerated in treating NMO-ON.

Background: Optic nerve lymphoma can present a diagnostic challenge because of its confusing clinical features and the difficulty of obtaining lesion tissue for biopsy. The objective of this study was to find some flags of lymphomatous infiltration of optic nerves.Methods: We report two cases of optic nerve lymphoma and conduct a literature review to determine whether a common diagnostic characteristic can be identified.Results: We examined 22 optic nerve lymphoma cases. Thirteen cases were systemic lymphoma infiltration of the optic nerve, five were primary central nervous system lymphoma (PCNSL), and four were primary isolated optic nerve lymphoma. Twenty patients manifested significant enlargement of the lesions in orbital/brain MRI. Seventeen contrast-enhanced MRIs showed abnormal enhancement of the optic nerve. All PCNSL and isolated optic nerve lymphoma patients in the series showed marked enhancement. Moderate and subtle enhancement was found in systemic lymphoma patients only. At the enhancement site, six isolated optic nerve lymphoma and PCNSL patients presented intrinsic enhancement, ten systemic patients showed both optic nerve and sheath enhancement, and one demonstrated sheath enhancement. Cerebrospinal fluid (CSF) tests showed elevated protein levels in six patients, and a neoplasm in one patient. We found abnormality of CSF immunity in both of our patients.Conclusion: Combined characteristics of orbital MRI and CSF tests may facilitate expeditious suspicion establishment of optic nerve lymphoma.