MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis.
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ABSTRACT:
SUBMITTER: Wildemann B
PROVIDER: S-EPMC7785547 | biostudies-literature | 2021 Jan
REPOSITORIES: biostudies-literature
Publications
MOG-expressing teratoma followed by MOG-IgG-positive optic neuritis.
Wildemann Brigitte B Jarius Sven S Franz Jonas J Ruprecht Klemens K Reindl Markus M Stadelmann Christine C
Acta neuropathologica 20201019 1
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Project description:Optic neuritis (ON) in patients with anti-myelin oligodendrocyte glycoprotein (MOG)-IgG antibodies has been associated with a better clinical outcome than anti-aquaporin 4 (AQP4)- IgG ON. Average retinal nerve fiber layer thickness (RNFL) correlates with visual outcome after ON.The aim of this study was to examine whether anti-MOG-IgG ON is associated with better average RNFL compared to anti-AQP4-IgG ON, and whether this corresponds with a better visual outcome.A retrospective study was done in a consecutive cohort of patients following anti-AQP4-IgG and anti-MOG-IgG ON. A generalized estimating equation (GEE) models analysis was used to compare average RNFL outcomes in ON eyes of patients with MOG-IgG to AQP4-IgG-positive patients, after adjusting for the number of ON events. The final mean visual field defect and visual acuity were compared between ON eyes of MOG-IgG and AQP4-IgG-positive patients. A correlation between average RNFL and visual function was performed in all study eyes.Sixteen patients were analyzed; ten AQP4-IgG-positive and six MOG-IgG-positive. The six patients with MOG-IgG had ten ON events with disc edema, five of which were bilateral. In the AQP4-IgG-positive ON events, 1/10 patients had disc edema. Final average RNFL was significantly better in eyes following MOG-IgG-ON (75.33μm), compared to 63.63μm in AQP4-IgG-ON, after adjusting for the number of ON attacks (GEE, p = 0.023). Mean visual field defects were significantly smaller (GEE, p = 0.046) among MOG-IgG positive ON eyes compared to AQP-IgG positive ON eyes, but last visual acuity did not differ between the groups (GEE, p = 0.153). Among all eyes, average RNFL positively correlated with mean visual field defect (GEE, p = 0.00015) and negatively correlated with final visual acuity (GEE, p = 0.00005).Following ON, RNFL is better preserved in eyes of patients with MOG-IgG antibodies compared to those with AQP4-IgG antibodies, correlating with better visual outcomes.
| S-EPMC5268377 | biostudies-other
Project description:BackgroundRecurrent optic neuritis (ON) was previously thought to be associated with multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD). Meningoencephalitis has recently been suggested to be a clinical finding typical of myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis. We report a Chinese patient with recurrent ON at disease initiation, who had a delayed diagnosis of MOG-IgG syndrome, until recurrent meningoencephalitis appeared and serum MOG-IgG was detected.Case presentationFrom the age of 7 years, an AQP4-IgG negative female patient had 10 disease recurrences, including 4 episodes of recurrent ON, 4 episodes of fever and meningoencephalitis, and 2 episodes of ON as well as meningoencephalitis. She was initially diagnosed as recurrent ON and treated with glucocorticoids followed by gradual tapering when ON reoccurred. Later, she was diagnosed as central nervous system infection when fever and meningoencephalitis appeared, and antiviral drugs and glucocorticoids were used. However, when she returned to our department for follow-up on July 2017, the results of serum demyelinating autoimmune antibody revealed positive MOG-IgG (titer 1:320 by an in-house, cell-based assay using live cells transfected with full-length human MOG). A diagnosis of MOG-IgG syndrome was established.ConclusionsTesting for MOG-IgG in atypical MS and NMOSD patients, and patients with meningoencephalitis with a history of relapsing demyelinating symptoms is warranted.
| S-EPMC6506947 | biostudies-literature
Project description:BackgroundNeurosyphilis refers to an infection of the central nervous system by Treponema pallidum. The clinical manifestations of neurosyphilis are diverse, making it easy to miss or misdiagnose. Anti-myelin oligodendrocyte glycoprotein antibody-associated disease is a recently defined immune-mediated inflammatory demyelinating central nervous system disease. Few studies have reported the coexistence of the two diseases.Case presentationThis case report presents a 37 years-old male patient with neurosyphilis manifested as optic neuritis with a positive myelin oligodendrocyte glycoprotein (MOG) antibody. This patient received intravenous administration of 3.2 million units of procaine penicillin every 4 h for 2 weeks, followed by a two-week intramuscular injection of benzathine penicillin. Additionally, methylprednisolone sodium succinate was administered intravenously at 1,000 mg/day, gradually reduced to 500 mg/day and 240 mg/day every 3 days. Subsequently, prednisone tablets at a dosage of 60 mg/day were orally administered, with a gradual reduction of 5 mg/day every 3 days until reaching a dosage of 30 mg/day. The patient's visual acuity was improved after 26 days of hospitalization. However, the visual field and color vision did not. At 3 months of follow-up, the symptoms remained unchanged despite the patient continued taking oral prednisone tablets at a dosage of 30 mg/day.ConclusionNeurosyphilis could be a potential triggering factor for MOGAD. In patients with neurosyphilis, it is strongly recommended to perform testing for MOG antibody along with other brain disease antibodies.
| S-EPMC10583717 | biostudies-literature
Project description:Background: Optic neuritis (ON) is a cardinal manifestation of multiple sclerosis (MS), aquaporin-4 (AQP4)-IgG-, and myelin oligodendrocyte glycoprotein (MOG)-IgG-associated disease. However, the prevalence of AQP4-IgG seropositivity and MOG-IgG seropositivity in isolated ON is unclear, and studies comparing visual outcomes and optical coherence tomography (OCT)-derived structural retinal measures between MS-ON, AQP4-ON, and MOG-ON eyes are limited by small sample sizes. Objectives: (1) To assess the prevalence of AQP4-IgG and MOG-IgG seropositivity among patients presenting with isolated ON; (2) to compare visual outcomes and OCT measures between AQP4-ON, MOG-ON, and MS-ON eyes. Methods: In this systematic review and meta-analysis, a total of 65 eligible studies were identified by PubMed search. Statistical analyses were performed with random effects models. Results: In adults with isolated ON, AQP4-IgG seroprevalence was 4% in non-Asian and 27% in Asian populations, whereas MOG-IgG seroprevalence was 8 and 20%, respectively. In children, AQP4-IgG seroprevalence was 0.4% in non-Asian and 15% in Asian populations, whereas MOG-IgG seroprevalence was 47 and 31%, respectively. AQP4-ON eyes had lower peri-papillary retinal nerve fiber layer (pRNFL; -11.7 μm, 95% CI: -15.2 to -8.3 μm) and macular ganglion cell + inner plexiform layer (GCIPL; -9.0 μm, 95% CI: -12.5 to -5.4 μm) thicknesses compared with MS-ON eyes. Similarly, pRNFL (-11.2 μm, 95% CI: -21.5 to -0.9 μm) and GCIPL (-6.1 μm, 95% CI: -10.8 to -1.3 μm) thicknesses were lower in MOG-ON compared to MS-ON eyes, but did not differ between AQP4-ON and MOG-ON eyes (pRNFL: -1.9 μm, 95% CI: -9.1 to 5.4 μm; GCIPL: -2.6 μm, 95% CI: -8.9 to 3.8 μm). Visual outcomes were worse in AQP4-ON compared to both MOG-ON (mean logMAR difference: 0.60, 95% CI: 0.39 to 0.81) and MS-ON eyes (mean logMAR difference: 0.68, 95% CI: 0.40 to 0.96) but were similar in MOG-ON and MS-ON eyes (mean logMAR difference: 0.04, 95% CI: -0.05 to 0.14). Conclusions: AQP4-IgG- and MOG-IgG-associated disease are important diagnostic considerations in adults presenting with isolated ON, especially in Asian populations. Furthermore, MOG-IgG seroprevalence is especially high in pediatric isolated ON, in both non-Asian and Asian populations. Despite a similar severity of GCIPL and pRNFL thinning in AQP4-ON and MOG-ON, AQP4-ON is associated with markedly worse visual outcomes.
| S-EPMC7578376 | biostudies-literature
Project description:PurposeTo compare the different immunological mechanisms between aquaporin 4 antibody-associated optic neuritis (AQP4-ON) and myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) based on RNA sequencing (RNA-seq) of whole blood.MethodsWhole blood was collected from seven healthy volunteers, 6 patients with AQP4-ON and 8 patients with MOG-ON, and used for RNA-seq analysis. An examination of immune cell infiltration was performed using the CIBERSORTx algorithm to identify infiltrated immune cells.ResultsRNA-seq analysis showed that the inflammatory signaling was mainly activated by TLR2, TLR5, TLR8 and TLR10 in AQP4-ON patients, while which was mainly activated by TLR1, TLR2, TLR4, TLR5 and TLR8 in MOG-ON patients. Biological function identification of differentially expressed genes (DEGs) based on Gene Ontology (GO) term and Kyoto encyclopedia of genes and genomes (KEGG) pathway enrichment analysis, as well as Disease Ontology (DO) analysis, showed that the inflammation in AQP4-ON was likely mediated by damage-associated molecular pattern (DAMP), while which in MOG-ON was likely mediated by pathogen-associated molecular pattern (PAMP). Analysis of immune cell infiltration showed that the proportion of immune cell infiltration was related to patients' vision. The infiltration ratios of monocytes (rs=0.69, P=0.006) and M0 macrophages (rs=0.66, P=0.01) were positively correlated with the BCVA (LogMAR), and the infiltration ratio of neutrophils was negatively correlated with the BCVA (LogMAR) (rs=0.65, P=0.01).ConclusionThis study reveals different immunological mechanisms between AQP4-ON and MOG-ON based on transcriptomics analysis of patients' whole blood, which may expand the current knowledge regarding optic neuritis.
| S-EPMC10036921 | biostudies-literature
Project description:IntroductionOptic neuritis (ON) is a common neuro-ophthalmological condition and remains a significant indicator of inflammatory conditions affecting the central nervous system. Varying etiologies exist for ON including multiple sclerosis, NMOSD, and MOGAD. Differential diagnosis is achieved using both radiological and serological testing. MRI characteristics of MOG-ON include T2 hypersensitive lesions, nerve swelling, and gadolinium enhancement of the affected optic nerve. While acute MOG-ON usually presents with optic nerve enhancement, recognizing atypical presentations is critical in accurate diagnosis and effective management.Case presentationWe herein present a case of a 67-year-old woman presenting with sudden decrease in vision in the right eye. The patient underwent a 3T MRI of the orbits and brain 5 days post vision loss which returned normal right optic nerve appearance at presentation (no edema, enhancement or increased T2 signal). Further serological testing of the MOG antibody returned positive (1:100) while APQ4 antibodies were negative. This yielded a diagnosis of MOG-IgG-ON. Subsequently, the patient was treated with IV methylprednisolone 1 g daily for 5 days followed by prednisone 1 mg/kg, resulting in marked improvement in vision.ConclusionThis case highlights the complexity involved in diagnosing ON, especially in the context of MOGAD. Absence of optic nerve enhancement in this patient calls attention to the possibility of subclinical inflammation and/or detectable enhancement later in clinical course. Our findings, along with existing literature, highlight the need for clinicians to consider atypical MRI presentations in MOG-ON cases. Recognizing that normal MRI does not exclude MOG-ON is important for optimizing diagnostic accuracy and effective treatment interventions.
| S-EPMC11703545 | biostudies-literature
Project description: Not available
| S-EPMC4496630 | biostudies-literature
Project description:To compare the different immunological mechanisms between aquaporin 4 antibody-associated optic neuritis (AQP4-ON) and myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) based on RNA sequencing (RNA-seq) of whole blood.
2023-03-07 | GSE226808 | GEO
Project description:BackgroundPain is a frequent symptom in aquaporin-4-immunoglobulin-G-positive neuromyelitis optica spectrum disorders (AQP4-IgG-pos. NMOSD). Data on pain in myelin-oligodendrocyte-glycoprotein-immunoglobulin-G autoimmunity with a clinical NMOSD phenotype (MOG-IgG-pos. NMOSD) are scarce.ObjectiveThe objective of this paper is to investigate pain in MOG-IgG-pos. NMOSD, AQP4-IgG-pos. NMOSD and NMOSD without AQP4/MOG-IgG detection (AQP4/MOG-IgG-neg. NMOSD).MethodsForty-nine MOG-IgG-pos. (n = 14), AQP4-IgG-pos. (n = 29) and AQP4/MOG-IgG-neg. (n = 6) NMOSD patients were included in this cross-sectional baseline analysis from an ongoing observational study. We identified spinal cord lesions on magnetic resonance imaging, assessed pain by the painDETECT and McGill Pain questionnaires, quality of life by Short Form Health Survey, and depression by Beck Depression Inventory.ResultsTwelve MOG-IgG-pos. NMOSD patients (86%), 24 AQP4-IgG-pos. NMOSD patients (83%), and all AQP4/MOG-IgG-neg. NMOSD patients (100%) suffered from pain. MOG-IgG-pos. NMOSD patients had mostly neuropathic pain and headache; AQP4-IgG-pos. and AQP4/MOG-IgG-neg. NMOSD patients had mostly neuropathic pain. A history of myelitis was less frequent in MOG-IgG-pos. NMOSD than in AQP4-IgG-pos. NMOSD patients. Pain influenced quality of life in all patients. Thirty-six percent of patients with pain received pain medication; none of them were free of pain.ConclusionsPain is a frequent symptom of patients with MOG-IgG-pos. NMOSD and is as important as in AQP4-IgG-pos. and AQP4/MOG-IgG-neg. NMOSD. Despite its impact on quality of life, pain is insufficiently alleviated by medication.
| S-EPMC6117869 | biostudies-literature
Project description:ObjectiveTo investigate whether visual disability which is known to accumulate by poor recovery from optic neuritis (ON) attacks can be lessened by early treatment, we investigated whether the time from symptom onset to high-dose IV methylprednisolone (IVMP) affected visual recovery.MethodsA retrospective study was performed in a consecutive cohort of patients following their first aquaporin-4 (AQP4)-IgG or myelin oligodendrocyte glycoprotein (MOG)-IgG-ON. Best-corrected visual acuity (BCVA) in ON eyes at 3 months (BCVA3mo) was correlated with time to IVMP (days). In cases of bilateral ON, 1 eye was randomly selected.ResultsA total of 29 of 37 patients had ON (27 AQP4-seropositive neuromyelitis optica spectrum disorder [NMOSD] and 9 MOG-IgG-ON), 2 of whom refused treatment. Of the 27 patients included, 10 presented later than 7 days from onset. The median BCVA3mo of patients treated >7 days was 20/100 (interquartile range 20/100-20/200). Patients treated >7 days had an OR of 5.50 (95% CI 0.88-34.46, p = 0.051) of failure to regain 0.0 logMAR vision (20/20) and an OR of 10.0 (95% CI 1.39-71.9) of failure to regain 0.2 logMAR vision (20/30) (p = 0.01) compared with patients treated within 7 days. ROC analysis revealed that the optimal criterion of delay in IVMP initiation was ≤4 days, with a sensitivity and specificity of 71.4% and 76.9%, respectively.ConclusionsIn this retrospective study of ON with AQP4 and MOG-IgG, even a 7-day delay in IVMP initiation was detrimental to vision. These results highlight the importance of early treatment for the long-term visual recovery in this group of patients. A prospective, multicenter study of the effects of timing of IVMP is currently underway.Classification of evidenceThis study provides Class IV evidence that hyperacute treatment of AQP4 and MOG-ON with IVMP increases the chance for good visual recovery (20/20 vision) and that even a greater than 7-day delay in treatment is associated with a higher risk for poor visual recovery.
| S-EPMC6624092 | biostudies-literature