Dataset Information

Pattern Visually Evoked Potentials in Japanese Girl With Optic Neuritis and Seropositive to Anti-myelin Oligodendrocyte Glycoprotein (MOG) Antibody.

ABSTRACT: Purpose: To describe a Japanese girl with unilateral optic neuritis who was seropositive for the anti-myelin-oligodendrocyte glycoprotein (MOG). Serial recordings of the pattern visual evoked potentials (pVEPs) were made to follow the dynamic changes of the disease activity. Observations: A 5-year-old girl developed a sudden reduction of vision and deep ocular pain in her right eye. On examination at our university hospital, the best-corrected visual acuity (BCVA) was light perception, and a swelling of the optic disc and tortuous vessels at the posterior pole of the right eye were observed. MRI demonstrated that her right optic nerve was hyperintense on short TI inversion recovery (STIR) sequence. A diagnosis of right papillitis was made, and she was treated with steroid pulse therapy followed by a gradual tapering of oral prednisolone. The visual acuity decreased to no light perception and plasmapheresis combined with high-dose intravenous immunoglobulin therapy was performed. The decimal visual acuity rapidly improved and recovered to 1.2, and no recurrence was observed for at least 1 year. On day 19, she was found to be anti-MOG antibody positive and anti-Aquaporin 4 antibody negative. pVEPs were recorded during the course of the disease process which showed the dynamic changes of the physiology of the visual pathways. The implicit times of the N75 and P100 components were prolonged in the right eye in the acute phase. The right visual acuity remained at 1.2 for at least 1 year, but the implicit times of the N75 and P100 components of the pVEPs of the right eye were still prolonged compared to left eye. Conclusion: Our findings indicate a positive relationship between the anti-MOG antibodies-positivity and the prolonged pVEPs. Further analyses of the pVEPs and other clinical findings of the optic neuritis are needed to establish the clinical significance of the anti-MOG antibodies positivity and optic neuritis for the diagnosis, treatment, and prognosis for this disease.

SUBMITTER: Takano S

PROVIDER: S-EPMC6931555 | biostudies-literature | 2019

REPOSITORIES: biostudies-literature

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Pattern Visually Evoked Potentials in Japanese Girl With Optic Neuritis and Seropositive to Anti-myelin Oligodendrocyte Glycoprotein (MOG) Antibody.

Takano Shunichiro S Hanabusa Aya A Yoshikawa Yuji Y Sassa Kaori K Shimura Airi A Shoji Takuhei T Ohde Hisao H Shinoda Kei K Yamanouchi Hideo H

Frontiers in neurology 20191219

<b>Purpose:</b> To describe a Japanese girl with unilateral optic neuritis who was seropositive for the anti-myelin-oligodendrocyte glycoprotein (MOG). Serial recordings of the pattern visual evoked potentials (pVEPs) were made to follow the dynamic changes of the disease activity. <b>Observations:</b> A 5-year-old girl developed a sudden reduction of vision and deep ocular pain in her right eye. On examination at our university hospital, the best-corrected visual acuity (BCVA) was light percept ...[more]

PMID: 31920953

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Project description:ObjectiveWe examined a cohort of adults with aquaporin-4 (AQP4) antibody-negative neuromyelitis optica/neuromyelitis optica spectrum disorder (NMO/NMOSD) for antibodies to myelin oligodendrocyte glycoprotein (MOG).MethodsWe performed a flow cytometry cell-based assay using live human lentivirus-transduced cells expressing full-length surface MOG. Serum was tested in 23 AQP4 antibody-negative NMO/NMOSD patients with bilateral and/or recurrent optic neuritis (BON, n = 11), longitudinally extensive transverse myelitis (LETM, n = 10), and sequential BON and LETM (n = 2), as well as in patients with multiple sclerosis (MS, n = 76) and controls (n = 52).ResultsMOG antibodies were detected in 9/23 AQP4 antibody-negative patients with NMO/NMOSD, compared to 1/76 patients with MS and 0/52 controls (p < 0.001). MOG antibodies were detected in 8/11 patients with BON, 0/10 patients with LETM, and 1/2 patients with sequential BON and LETM. Six of 9 MOG antibody-positive patients had a relapsing course. MOG antibody-positive patients had prominent optic disc swelling and were more likely to have a rapid response to steroid therapy and relapse on steroid cessation than MOG antibody-negative patients (p = 0.034 and p = 0.029, respectively). While 8/9 MOG antibody-positive patients had good follow-up visual acuity, one experienced sustained visual impairment, 3 had retinal nerve fiber layer thinning, and one had residual spinal disability.ConclusionsMOG antibodies have a strong association with BON and may be a useful clinical biomarker. MOG antibody-associated BON is a relapsing disorder that is frequently steroid responsive and often steroid dependent. Failure to recognize the disorder early and institute immunotherapy promptly may be associated with sustained impairment.Classification of evidenceThis study provides Class II evidence that MOG antibodies are associated with AQP4 antibody-negative BON (sensitivity 69%, 95% confidence interval [CI] 42%-87%; specificity 99%, 95% CI 93.7%-99.8%).

Project description:Background: Optic neuritis (ON) is an important clinical manifestation of neuromyelitis optic spectrum disease (NMOSD). Myelin oligodendrocyte glycoprotein (MOG) antibody-related and aquaporin 4 (AQP4) antibody-related ON show different disease patterns. The aim of this study was to explore the differences in structure and function of the visual pathway in patients with ON associated with MOG and AQP4 antibodies. Methods: In this prospective study, we recruited 52 subjects at Beijing Tiantan Hospital, including 11 with MOG Ig+ ON (MOG-ON), 13 with AQP4 Ig+ ON (AQP4-ON), and 28 healthy controls (HCs). Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) of optic radiation (OR), primary visual cortex volume (V1), brain volume, and visual acuity (VA) were compared among groups. A multiple linear regression was used to explore associations between VA and predicted factors. In addition, we used optical coherence tomography (OCT) to examine thickness of the peripapillary retinal nerve fiber layer (pRNFL) and retinal ganglion cell complex (GCC) in a separate cohort consisting of 15 patients with ON (8 MOG-ON and 7 AQP4-ON) and 28 HCs. Results: Diffusion tensor imaging showed that the FA of OR was lower than controls in patients with AQP4-ON (p = 0.001) but not those with MOG-ON (p = 0.329) and was significantly different between the latter two groups (p = 0.005), while V1 was similar in patients with MOG-ON and AQP4-ON (p = 0.122), but was lower than controls in AQP4-ON (p = 0.002) but not those with MOG-ON (p = 0.210). The VA outcomes were better in MOG-ON than AQP4-ON, and linear regression analysis revealed that VA in MOG-ON and AQP4-ON was both predicted by the FA of OR (standard β = -0.467 and -0.521, p = 0.036 and 0.034). Both patients of MOG-ON and AQP4-ON showed neuroaxonal damage in the form of pRNFL and GCC thinning but showed no statistically significant difference (p = 0.556, 0.817). Conclusion: The structural integrity of OR in patients with MOG-ON, which is different from the imaging manifestations of AQP4-ON, may be a reason for the better visual outcomes of patients with MOG-ON.

Project description:Introduction: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a recently described central nervous system (CNS) inflammatory disorder with phenotypic overlap with Neuromyelitis Optica Spectrum Disorder (NMOSD). NMOSD seronegative patients, and those with limited forms of the disorder, become suspects for MOG antibody-associated disease. We describe a multi-ethnic population with MOG antibody seropositivity from the University of British Columbia MS/NMO clinic. Methods: AQP4-antibody seronegative patients presenting 2005-2016 with CNS inflammatory disease suspicious for NMOSD, as well as 20 MS controls, were retrospectively tested for MOG-IgG1 antibodies by live cell-based assay at Oxford Autoimmune Neurology Diagnostic Laboratory (UK) and by a commercial fixed cell-based assay at MitogenDx (Calgary, Canada). Additional MOG seropositive cases were identified through routine clinical interaction (2016-2018) using one of these laboratories. Clinical data was reviewed retrospectively. Results: Retrospective testing identified 21 MOG seropositives (14 by live assay only, 3 by fixed assay only and 4 by both) representing 14% of the "NMOSD suspects" cohort. One multiple sclerosis (MS) control serum was MOG seropositive. Twenty additional MOG positive cases were identified prospectively. Of 42 patients (27 female), median disease onset age was 29 years (range 3-62; 9 pediatric cases), 20 (47%) were non-Caucasian, and 3 (7%) had comorbid autoimmune disease. Most common onset phenotypes were optic neuritis (23, 55%; 8 bilateral) and myelitis (9, 21%; 6 longitudinally extensive) Three of the patients in our cohort experienced cortical encephalitis; two presented with seizures. Onset was moderate-severe in 64%, but 74% had good response to initial steroid therapy. Cumulative relapse probability for the MOG positive group at 1 year was 0.428 and at 4 years was 0.628. Most had abnormal brain imaging, including cortical encephalitis and poorly demarcated subcortical and infratentorial lesions. Few "classic MS" lesions were seen. Optic nerve lesions (frequently bilateral) were long and predominantly anterior, but 5 extended to the chiasm. Spinal cord lesions were long and short, with involvement of multiple spinal regions simultaneously, including the conus medullaris. Conclusions: Our MOG seropositive patients display phenotypes similar to previous descriptions, including cortical lesions with seizures and conus medullaris involvement. Many patients relapsed, predominantly in a different CNS location from onset. Serologic data from two different cell-based antibody assays highlight the discrepancies between live and fixed testing for MOG antibodies.

Dataset Information

Pattern Visually Evoked Potentials in Japanese Girl With Optic Neuritis and Seropositive to Anti-myelin Oligodendrocyte Glycoprotein (MOG) Antibody.

Publications

Pattern Visually Evoked Potentials in Japanese Girl With Optic Neuritis and Seropositive to Anti-myelin Oligodendrocyte Glycoprotein (MOG) Antibody.

Similar Datasets

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