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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.

Purpose: To observe the clinical and imaging characteristics of radiation-induced optic neuropathy (RION). Methods: We retrospectively reviewed the clinical data of 43 patients (69 eyes) who were diagnosed with RION at the Chinese PLA General Hospital from 2010 to 2021. Results: The latency from radiotherapy to onset of visual loss ranged from 1 to 132 (36.33 ​± ​30.48) months. Optic disc pallor and optic disc edema were found in 27.0% (10/37) and 8.1% (3/37) of the eyes, respectively, within 2 months. After treatment, the best corrected visual acuity (BCVA) was restored in 24.6% (17/69) of the eyes and the final BCVA improved in 13.0% (9/69) of the eyes. An 82.5% (33/40) of the eyes with magnetic resonance imaging (MRI) showed enhancement of the affected optic nerve, mostly (69.7%) in the intracranial segment, and 36.4% (12/33) of the eyes with expansion and T2-high signals also showed enhancement of the affected optic nerve. The superior retinal nerve fiber layer (RNFL) and the outer circle superior quadrant (OS) of the inner limiting membrane to retinal pigment epithelium (ILM-RPE) layer thinned significantly during the first month. The center of the ILM-RPE layer thickened significantly during the first two months and the inner circle temporal quadrant (IT) of the ILM-RPE layer thickened significantly from the third to sixth month. The RNFL thinned significantly after 6 months except for the temporal quadrant, and the average inner circle superior quadrant (IS) and outer circle of the ILM-RPE layer thinned significantly after 6 months. There was no significant difference between hyperbaric oxygen therapy (HBOT) and high-dose intravenous methylprednisolone (IVMP) therapy in improving BCVA recovery or final BCVA (P ​> ​0.05). Conclusions: The structural damage of the RNFL and ILM-RPE layer occurred during the first month, the RNFL showed progressive thinning during the follow-up period, while the ILM-RPE layer showed thinning during the first month, thickening from the third to sixth month, and thinning after 6 months. There was a discrete region of enhancement of the optic nerve, often with expansion and high-T2 signals on MRI. HBOT and high-dose IVMP therapy were hardly effective for treating RION in the non-acute stage.

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.

Background: Monoclonal antibodies such as rituximab (RTX), eculizumab, inebilizumab, satralizumab, and tocilizumab have been found to be effective therapies for neuromyelitis optica spectrum disease ​(NMOSD) in several clinical randomized controlled trials. Objective: The purpose of this meta-analysis of randomized controlled trials was to assess the efficacy and safety of monoclonal antibodies in the treatment of NMOSD. Methods: We searched the following databases for relevant English language literature from the establishment of the database to June 2021: PubMed, Embase, Cohorane Library, the Central Register of Controlled Trials (CENTRAL), and Web of Science. Randomized controlled trials of monoclonal antibodies were the targets of the review. Results: We included seven trials containing 775 patients (485 in the monoclonal antibody group and 290 in the control group). Patients in the monoclonal group (HR 0.24, 95% CI: 0.14 to 0.40, P ​

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 show the clinical characteristics, identify the magnetic resonance imaging (MRI) and optical coherence tomography (OCT) features, and observe the visual outcome of methanol-induced optic neuropathy. Methods. Clinical data were retrospectively collected from in-patients diagnosed with methanol-induced optic neuropathy in the Neuro-Ophthalmology Department of the Chinese People’s Liberation Army General Hospital from January 2016 to January 2021. Results. Eight patients were included in this study. The exposure time was 6–34 h for ingestion, 3-4 months for inhalation, and more than ten years for skin absorption. All patients demonstrated bilateral acute visual impairment. Seven of eight patients had other accompanying systemic symptoms. Seven of eight patients demonstrated optic nerve lesions in MRI, and five presented with a hyperintense T2 signal in a “central” type. OCT showed the macular ganglion cell layer and inner plexiform layer (mGCL-IPL) thinning before the peripapillary retinal nerve fiber layer (pRNFL) thinning. The visual improvement was achieved transiently for seven of eight patients after treatment. One patient with a mitochondrial DNA mutation maintained a bilateral no-light perception (NLP) from the onset to the last visit. All patients had poor visual prognoses, with either light perception or NLP. Conclusions. Methanol-induced optic neuropathy is a rare bilateral optic neuropathy with a poor visual outcome. A centrally hyperintense T2 signal of the optic nerve is common in methanol-induced optic neuropathy. The thinning of the mGCL-IPL is more sensitive than that of the pRNFL for early diagnosis. A mitochondrial genetic defect may be a predisposing factor for methanol-induced optic neuropathy.

Neuromyelitis optica spectrum disorder (NMOSD) is a central nervous system inflammatory demyelinating disease, the pathogenesis of which involves autoantibodies targeting the extracellular epitopes of aquaporin-4 on astrocytes. We neutralized the AQP4-IgG from NMOSD patient sera using synthesized AQP4 extracellular epitope peptides and found that the severe cytotoxicity produced by aquaporin-4 immunoglobin (AQP4-IgG) could be blocked by AQP4 extracellular mimotope peptides of Loop A and Loop C in astrocyte protection and animal models. ACT001, a natural compound derivative, has shown anti-tumor activity in various cancers. In our study, the central nervous system anti-inflammatory effect of ACT001 was investigated. The results demonstrated the superior astrocyte protection activity of ACT001 at 10 µM. Furthermore, ACT001 decreases the behavioral score in the mouse NMOSD model, which was not inferior to Methylprednisolone Sodium Succinate, the first-line therapy of NMOSD in clinical practice. In summary, our study showed that astrocytes are protected by specific peptides, or small molecular drugs, which is a new strategy for the treatment of NMOSD. It is possible for ACT001 to be a promising therapy for NMOSD.

Background: Magnetic resonance imaging (MRI) plays a significant role in assessing optic neuropathy and providing more detailed information about the lesion of the visual pathway to help differentiate optic neuritis from other visual disorders. This study aims to systematically review the literature and verify if there is a real difference in lesion location among different demyelinating optic neuritis (DON) subtypes. Methods: A systematic search was conducted including 8 electronic databases and related resources from the establishment of the database to August 25th, 2020. We classified DON into 5 subtypes and divided the visual pathways into five segments mainly comparing the differences in the involved visual pathway sites of different subtypes. Results: Fifty-five studies were included in the analysis, and the abnormal rate was as high as 92% during the acute phase (within 4 weeks of symptom onset). With respect to lesion location, the orbital segment of the optic nerve was the most frequently involved (87%), whereas optic tract involvement was very rare. Involvement of the orbital segment was more common in myelin oligodendrocyte glycoprotein antibody-related optic neuritis (MOG-ON) (78%) and chronic relapsing inflammatory optic neuropathy (CRION) (81%), while the lesion was found to be located more posteriorly in neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON). With respect to lesion length, approximately 77% of MOG-ON patients had lesions involving more than half of the optic nerve length. Conclusions: MRI examination is recommended for DON patients in the acute phase. In MOG-ON, anterior involvement is more common and the involved length is mostly more than 1/2 of the optic nerve length, whereas posterior involvement, intracranial segment, optic chiasm, or optic tract, is more common in NMOSD-ON. Prospero registration number: CRD42020222430 (25-11-2020);

Purpose: To investigate the application of critical flicker fusion frequency (CFF) in demyelinating optic neuritis (DON). Methods: A cross-sectional study. A total of 127 eyes in 69 DON patients and 63 eyes in 33 healthy control (HC) groups were included between January 2021 to September 2021 from Department of Ophthalmology, PLA General Hospital. Patients underwent best-corrected visual acuity (BCVA), visual field, optical coherence tomography (OCT), flash visual evoked potential (F-VEP), and CFF examinations. The affected eyes were divided into aquaporins 4 (AQP4-), myelin oligodendrocyte glycoprotein (MOG-), and double negative DON according to serum antibody; mild, moderate, severe degree visual impairment according to BCVA ≥ 0.5, 0.1-0.5, < 0.1; and 4 groups: < 1, 1 ∼< 3, 3 ∼ < 6 and > 6 months according to time interval from onset to CFF examination. One-way ANOVA was used to perform above subgroup analysis. The correlations between CFF and F-VEP peak time, peak value, BCVA and mean visual filed defect (MD) were analyzed in order via Pearson correlation analysis. Results: he trichromatic values of red, green, and yellow in DON affected eyes were 21.83 ± 9.03, 23.66 ± 10.21, 24.09 ± 10.77 Hz, respectively, which was significantly reduced compared with the HC group (t = -14.82, -14.22, -14.00; P < 0.001). The subgroup analysis showed no significant difference between different antibody subtypes (P = 0.914

Background: To evaluate the feature of different retinal layer segmentation in neuromyelitis optica spectrum disorders (NMOSD) with spectral-domain optical coherence tomography (SD-OCT) and to compare it with that in multiple sclerosis (MS), healthy controls (HC), and idiopathic optic neuritis (ION). Methods: We retrieved four electronic databases, including Pubmed, Embase, Cochrane Library, and Web of Science from inception to September 1st, 2021. A meta-analysis was performed to compare different retinal layer segmentation thicknesses between patients with or without a history of optic neuritis (ON) in NMOSD and the control group, including patients with MS, HC, and ION. Results: Forty-two studies were included and the interval between the last ON onset and examination was greater than 3 months. Compared with that in HC eyes, the loss of retinal nerve fiber layer (RNFL) and macular ganglion cell and inner plexiform layer (GC-IPL) was serious in NMOSD eye especially after ON. Moreover, compared with that in ION eyes or MS-related-ON eyes, the injury to the peripapillary retinal nerve fiber layer (pRNFL) was severe in NMOSD-related-ON eyes. In addition, the correlation coefficient between pRNFL and prognostic visual acuity was 0.43. However, the one-arm study revealed the inner nuclear layer (INL) was thickened in NMOSD-related-ON eyes compared with HC eyes. Conclusions: Inclusion of the RNFL and macular GC-IPL is recommended for monitoring disease progression and attention should be paid to changes in the INL.

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: Plasma exchange (PE) is often considered as an effective treatment for neuromyelitis optica spectrum disorder (NMOSD) and several inflammatory demyelinating disorders of the central nervous system. This study aimed to evaluate the visual outcomes of Chinese patients with severe acute isolated optic neuritis (ON) who received PE therapy after high-dose intravenous methylprednisolone (IVMP) treatment. Methods: Thirty-seven acute isolated ON patients experiencing their first attack of severe visual impairment without neurological disability were recruited. All subjects received five cycles of double-filtration plasmapheresis. Visual acuity (VA) was documented at onset, 1 day before PE treatment, at each cycle of PE therapy and at the 1-month follow-up visit. Results: This study included 26 female (70.3%) and 11 male (29.7%) subjects, and 18 subjects (48.6%) had bilateral involvement. The time window between onset and PE treatment was 27.3 ± 12.7 days (range: 6–53 days). Mean VA (logMAR) of the studied eyes at onset, 1-day before PE treatment/after IVMP and after the fifth PE treatment were 3.41 ± 1.50, 2.61 ± 1.64 and 1.66 ± 1.52, respectively ( p 20/800 after IVMP and PE treatments, among which five eyes (13.5%) recovered to Snellen VA 20/20 ( p

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.

Purpose. To describe different clinical characteristics and prognosis of optic neuritis (ON) in male patients with seropositive aquaporin-4 antibody (AQP4-Ab) or myelin oligodendrocyte glycoprotein antibody (MOG-Ab) in China. Method. Males with ON were recruited from the Neuro-ophthalmology Department of the Chinese People’s Liberation Army, General Hospital from January 2016 to February 2018. They were assigned to two groups based on antibodies status: MOG-Ab-seropositive ON (MOG-ON) and aquaporin-4 Ab-seropositive ON (AQP4-ON). Results. Seventy-six male patients were assessed, including 44 MOG-ON (57.9%) and 32 AQP4-ON (42.1%). The MOG-ON patients were significantly younger at onset compared to the AQP4-ON group (p

Objective. This study aims to describe the clinical manifestations and outcomes in a cohort of older Chinese patients. Method. A retrospective study of patients aged ≥ 45 years who had a first episode of optic neuritis (ON) between May 2008 and November 2012. Clinical features at onset and last follow-up were analyzed within subgroups (age 45–65 years and age ≥ 65 years). Results. 76 patients (99 eyes) were included, of which 58% were females. The mean age at presentation was 55.53 ± 8.29 years (range: 45–83 years). Vision loss was severe at presentation, with initial best corrected vision activity (BCVA)

This study aimed to analyse the structural injury of the peripapillary retinal nerve fibre layer (pRNFL) and segmented macular layers in optic neuritis (ON) in aquaporin4-antibody (AQP4-Ab) seropositivity(AQP4-Ab-positiveON) patients and in AQP4-Ab seronegativity (AQP4-Ab-negative ON) patients in order to evaluate their correlations with the best-corrected visual acuity (BCVA) and the value of the early diagnosis of neuromyelitis optica (NMO).This is a retrospective, cross-sectional and control observational study.In total, 213 ON patients (291 eyes) and 50 healthy controls (HC) (100 eyes) were recruited in this study. According to a serum AQP4-Ab assay, 98 ON patients (132 eyes) were grouped as AQP4-Ab-positive ON and 115 ON patients (159 eyes) were grouped as AQP4-Ab-negative ON cohorts. All subjects underwent scanning with spectralis optical coherence tomography (OCT) and BCVA tests. pRNFL and segmented macular layer measurements were analysed.The pRNFL thickness in AQP4-Ab-positive ON eyes showed a more serious loss during 0-2 months (-27.61μm versus -14.47 μm) and ≥6 months (-57.91μm versus -47.19μm) when compared with AQP4-Ab-negative ON eyes. AQP4-Ab-positive ON preferentially damaged the nasal lateral pRNFL. The alterations in the macular ganglion cell layer plus the inner plexiform layer (GCIP) in AQP4-Ab-positive ON eyes were similar to those in AQP4-Ab-negative ON eyes. AQP4-Ab-positive ON eyes had entirely different injury patterns in the inner nuclear layer (INL) compared with AQP4-Ab-negative ON eyes during the first 6 months after the initial ON attack. These differences were as follows: the INL volume of AQP4-Ab-positive ON eyes had a gradual growing trend compared with AQP4-Ab-negative ON eyes, and it increased rapidly during 0-2 months, reached its peak during 2-4 months, and then decreased gradually. The pRNFL and GCIP in AQP4-Ab-positive ON eyes had positive correlations with BCVA. When the pRNFL thickness decreased to 95%CI (50.77μmto 60.85μm) or when the GCIP volume decreased to 95%CI (1.288 mm3to 1.399 mm3), BCVA began to be irreversibly damaged.The structural alterations of pRNFL and GCIP could indicate the resulting visual damage. In addition, the injury pattern of INL could be a potential structural marker to predict the conversion of ON to NMO.

Purpose. Although optic neuritis (ON) in children is relatively common, visual outcomes and factors associated with the condition have not been well documented. The aim of this study was to evaluate the clinical features and visual outcomes of ON in Chinese children. Methods. Patients with a first episode of ON at a tertiary neuroophthalmic centre in China were assessed and followed up for at least three months. Visual outcomes and clinical, laboratory, and neuroimaging findings were reviewed. In patients with bilateral ON, only the eyes with worse visual acuity (VA) at presentation were used for statistical analysis. Results. Seventy-six children (76 eyes) with a first episode of ON were included. The mean age was 11.8 years, 60.5% were females, and 48.7% had bilateral involvement. The children were followed up for an average of 18.5 months (age range, 3–48 months). Vision loss at presentation was severe, with VA < 20/200 in 37 eyes (48.7%). At the final visit, 3 (3.9%) eyes had VA of at least 20/20, and 41 (53.9%) eyes had VA of at least 20/40. The final VA in 35 eyes (46.1%) was worse than 20/40. Children aged ≤ 10 years had better predicted visual outcomes when compared to children over 10 years (odds ratio = 2.73, 95% confidential interval: 1.05–7.07, and P=0.039). The other features of this cohort, such as sex, experienced bilateral attack, VA at presentation, presence of optic disc edema, systemic diseases, magnetic resonance imaging (MRI) findings, and aquaporin-4 (AQP-4) antibody status, were not significantly correlated with the final visual outcome. Conclusion. The data revealed the clinical characteristics and visual outcomes of ON in Chinese children. ON in children was associated with severe vision loss and relatively good visual recovery. The age at onset could predict the final visual function.

Purpose To describe the pattern of MRI changes in the pregeniculate visual pathway in Leber hereditary optic neuropathy (LHON). Method This retrospective observational study enrolled 60 patients with LHON between January 2015 and December 2021. The abnormal MRI features seen in the pregeniculate visual pathway were investigated, and then correlated with the causative mitochondrial DNA (mtDNA) mutation, the distribution of the MRI lesions and the duration of vision loss. Result The cohort included 48 (80%) males and 53 (88%) had bilateral vision loss. The median age of onset was 17.0 years (range 4.0-58.0). 28 (47%) patients had the m.11778G>A mutation. 34 (57%) patients had T2 hyperintensity (HS) in the pregeniculate visual pathway and 13 (22%) patients with chiasmal enlargement. 20 patients (71%) carrying the m.11778G>A mutation had T2 HS, significantly more than the 14 patients (44%) with T2 HS in the other LHON mutation groups (p=0.039). Furthermore, significantly more patients in the m.11778G>A group (16 patients (57%)) had T2 HS in optic chiasm (OCh)/optic tract (OTr) than the other LHON mutation groups (7 patients (22%), p=0.005). Optic chiasmal enlargement was more common in patients with vision loss duration <3 months compared with those =3 months (p=0.028). Conclusion T2 HS in the pregeniculate visual pathway is a frequent finding in LHON. Signal changes in the OCh/OTr and chiasmal enlargement, in particular within the first 3 months of visual loss, were more commonly seen in patients carrying the m.11778G>A mtDNA mutation, which may be of diagnostic significance. [ABSTRACT FROM AUTHOR]

Aims The optimal immunosuppressive therapy (IST) in patients with myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-On) remains uncertain. This study aimed to observe the disease course of MOG-ON and evaluate the therapeutic efficacy and tolerability of conventional immunosuppressants through Chinese cohort analysis. Methods This bidirectional cohort study included 121 patients with MOG-ON between January 2015 and December 2018. The clinical features and annualised relapse rate (ARR) of patients with and without IST were analysed. Results The median age at onset was 17.5 years, and the sex ratio (F:M) was 1.24. Of 121 patients, 77 patients relapsed and 61 patients were younger than 18 years at disease onset. The overall median ARR of 63 patients in the non-IST group was 0.5, with 46.0% patients showing relapse at a median follow-up of 33.5 months. In the IST group, the ARR decreased from 1.75 pre-IST to 0.00 post-IST in 53 patients who received IST exceeding 6 months, with 20.8% patients showing relapse at a median follow-up of 23.8 months. The relapse rates of patients treated with rituximab (RTX) and mycophenolate mofetil (MMF) were not statistically different, but the rate of discontinuation was significantly lower in the RTX-treated group (18.2% vs 57.7%, p=0.0017). Conclusion This study provides Class III evidence that both MMF and RTX may lower disease activity in patients with MOG-ON, and RTX showed better tolerability than MMF. However, observation after a single attack remains a good option because less than half of patients not on treatment suffered a relapse. [ABSTRACT FROM AUTHOR]

Background Different glial-autoantibodies-related paediatric optic neuritis (ON) are associated with different clinical characteristics and prognosis that require different treatments. Because glial autoantibody detection is not available in some parts of the world and there is often a delay in obtaining results, clinical factors that can be used to predict the subtype of paediatric ON are needed. Methods This was a single-centre retrospective cohort study. Children who presented with their first ON attack and with complete clinical data were included in the analysis. Single and multiple parameters for predicting paediatric myelin oligodendrocyte glycoprotein immunoglobin-associated ON (MOG-ON) and aquaporin4 immunoglobin-related ON (AQP4-ON) were calculated. Results 78 paediatric patients had their first ON attack from January 2016 to December 2019, of whom 69 were included in the final analysis, including 33 MOG-ON cases, 17 AQP4-ON cases and 19 Seronegative-ON cases. For predicting paediatric MOG-ON, the most sensitive predictors were ‘male or optic disc swelling (ODS) or bilateral’ (sensitivity 0.97 (95% CI 0.82 to 1.00)) and ‘follow-up visual acuity (VA) ≤0.1 logMAR or ODS’ (sensitivity 0.97 (95% CI 0.82 to 1.00)), and the most specific factor was ‘Age ≤11 y and simultaneous CNS involvement’ (specificity 0.97 (95% CI 0.84 to 1.00)). For predicting paediatric AQP4-ON, the most sensitive predictor was ‘Female or without ODS’ (sensitivity 1.00 (95% CI 0.77 to 1.00)), and the most specific factors were Neurological history (sensitivity 0.94 (95% CI 0.83 to 0.98)) and follow-up VA >1.0 logMAR (sensitivity 0.96 (95% CI 0.86 to 0.99)). Conclusion According to our data from a Chinese paediatric cohort, using multiple parameters increases the sensitivity and specificity of diagnosing paediatric MOGON and AQP4-ON. These can assist clinicians in diagnosing and treating paediatric ON when glial autoantibody status is not available. [ABSTRACT FROM AUTHOR]

Background: Several neuroimaging studies demonstrated that optic neuritis (ON) leads to functional and anatomical architecture changes in the brain. The alterations of interhemispheric functional connectivity (IFC) in patients with AQP4-ON and myelin oligodendrocyte glycoprotein (MOG)-ON are not well understood. Purpose: To investigate the differential patterns of VMHC in patients with AQP4-ON and MOG-ON. Material and Methods: Twenty-one patients with AQP4-ON, 11 patients with MOG-ON, and 34 healthy controls underwent resting-state MRI scans. One-way ANOVA was used to identify regions in which the zVMHC differed among the three groups. Post hoc two-sample t-tests were then conducted to compare zVMHC values between pairs of groups. Pearson correlation analysis was conducted to reveal relationships between mean zVMHC values and clinical variables in the AQP4-ON and MOG-ON groups. Results: The results revealed significant differences in zVMHC values in the PreCG among the three groups. Compared to the control group: the AQP4-ON group showed significantly lower VMHC values in the superior temporal gyrus, inferior frontal gyrus, and PreCG; and the MOG-ON group showed significantly higher zVMHC values in the PostCG. Compared to the AQP4-ON group, the MOG-ON group showed significantly lower zVMHC values in the PreCG/PostCG (voxel-level P<0.01, GRF correction, cluster-level P<0.05). Conclusion: Patients with AQP4-ON and those with MOG-ON showed abnormal VMHC in the motor cortices, sensorimotor cortices, and frontal lobe, possibly indicating impaired sensorimotor function in patients with ON. Moreover, differential patterns of VMHC in patients with AQP4-ON, compared to patients with MOG-ON, might serve as a clinical indicator for classification of ON. [ABSTRACT FROM AUTHOR]