scholarly journals Intractable Hiccup in Demyelinating Disease with Anti-Myelin Oligodendrocyte Glycoprotein (MOG) Antibody

2016 ◽  
Vol 55 (19) ◽  
pp. 2905-2906 ◽  
Author(s):  
Tetsuya Akaishi ◽  
Masatoshi Konno ◽  
Ichiro Nakashima ◽  
Masashi Aoki
Keyword(s):  
Demyelinating Disease ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Intractable Hiccup

scholarly journals Anti-Myelin Oligodendrocyte Glycoprotein and Human Leukocyte Antigens as Markers in Pediatric and Adolescent Multiple Sclerosis: on Diagnosis, Clinical Phenotypes, and Therapeutic Responses

2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Maria P. Gontika ◽  
Maria C. Anagnostouli
Keyword(s):  
Multiple Sclerosis ◽  
Early Onset ◽  
Demyelinating Disease ◽  
Age Of Onset ◽  
Genetic Locus ◽  
Human Leukocyte ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Disease Course ◽  
Clinical Phenotypes

Early-onset (pediatric and adolescent) multiple sclerosis (MS) is a well-established demyelinating disease that accounts for approximately 3-5% of all MS cases. Thus, identifying potential biomarkers that can reflect the pathogenic mechanisms, disease course and prognosis, and therapeutic response in such patients is of paramount importance. Myelin oligodendrocyte glycoprotein (MOG) has been regarded as a putative autoantigen and autoantibody target in patients with demyelinating diseases for almost three decades. However, recent studies have suggested that antibodies against MOG represent a distinct clinical entity of dominantly humoral profile, with a range of clinical phenotypes closely related to the age of onset, specific patterns of disease course, and responses to treatment. Furthermore, the major histocompatibility complex (MHC)—which has been regarded as the “gold standard” for attributing genetic burden in adult MS since the early 1970s—has also emerged as the primary genetic locus in early-onset MS, particularly with regard to the human leukocyte antigen (HLA) alleles DRB1⁎1501 and DRB1⁎0401. Recent studies have investigated the potential interactions among HLA, MOG, and environmental factors, demonstrating that early-onset MS is characterized by genetic, immunogenetic, immunological, and familial trait correlations. In this paper, we review recent evidence regarding HLA-genotyping and MOG antibodies—the two most important candidate biomarkers for early-onset MS—as well as their potential application in the diagnosis and treatment of MS.

scholarly journals Myelin oligodendrocyte glycoprotein antibodies in pathologically proven multiple sclerosis: frequency, stability and clinicopathologic correlations

2007 ◽  
Vol 13 (1) ◽  
pp. 7-16 ◽  
Author(s):  
S J Pittock ◽  
M Reindl ◽  
S Achenbach ◽  
T Berger ◽  
W Bruck ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Western Blot ◽  
Demyelinating Disease ◽  
Clinical Course ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Poor Agreement ◽  
Predictive Role ◽  
Mog Antibodies ◽  
Serial Measurements

Controversy exists regarding the pathogenic or predictive role of anti-myelin oligodendrocyte glycoprotein (MOG) antibodies in patients with multiple sclerosis (MS). Four immunopathological patterns (IP) have been recognized in early active MS lesions, suggesting heterogeneous pathogenic mechanisms. Whether MOG antibodies contribute to this pathological heterogeneity and potentially serve as biomarkers to identify specific pathological patterns is unknown. Here we report the frequencies of antibodies to human recombinant MOG (identified by Western blot and enzymelinked immunoabsorbent assay (ELISA)) in patients with pathologically proven demyelinating disease, and investigate whether antibody status is associated with clinical course, HLA-DR2 genotype, IP or treatment response to plasmapheresis. The biopsy cohort consisted of 72 patients: 12 pattern I, 43 pattern II and 17 pattern III. No association was found between MOG antibody status and conversion to clinically definite MS, DR-2 status, IP or response to plasmapheresis. There was poor agreement between Western blot and ELISA (kappa=0.07 for MOG IgM). Fluctuations in antibody seropositivity were seen for 3/4 patients tested serially by Western blot. This study does not support a pathologic pattern-specific role for MOG-antibodies. Variable MOG-antibody status on serial measurements, coupled with the lack of Western blot and ELISA correlations, raises concern regarding the use of MOG-antibody as an MS biomarker and underscores the need for methodological consensus.

scholarly journals Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G in the CSF

2021 ◽  
Vol 9 (1) ◽  
pp. e1095
Author(s):  
Young Nam Kwon ◽  
Boram Kim ◽  
Jun-Soon Kim ◽  
Heejung Mo ◽  
Kyomin Choi ◽  
...  
Keyword(s):  
Immunoglobulin G ◽  
Demyelinating Disease ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Multicenter Cohort Study ◽  
Prognostic Features ◽  
Multicenter Cohort ◽  
Igg Synthesis ◽  
Referral Hospitals ◽  
Paired Serum ◽  
Severity Of The Disease

Background and ObjectiveTo investigate the clinical relevance of CSF myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) testing in a large multicenter cohort.MethodsIn this multicenter cohort study, paired serum-CSF samples from 474 patients with suspected inflammatory demyelinating disease (IDD) from 11 referral hospitals were included. After serum screening, patients were grouped into seropositive myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD, 31), aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG + NMOSD, 60), other IDDs (217), multiple sclerosis (MS, 45), and non-IDDs (121). We then screened CSF for MOG-IgG and compared the clinical and serologic characteristics of patients uniquely positive for MOG-IgG in the CSF to seropositive patients with MOGAD.ResultsNineteen patients with seropositive MOGAD (61.3%), 9 with other IDDs (CSF MOG + IDD, 4.1%), 4 with MS (8.9%), but none with AQP4-IgG + NMOSD nor with non-IDDs tested positive in the CSF for MOG-IgG. The clinical, pathologic, and prognostic features of patients uniquely positive for CSF MOG-IgG, with a non-MS phenotype, were comparable with those of seropositive MOGAD. Intrathecal MOG-IgG synthesis, observed from the onset of disease, was shown in 12 patients: 4 of 28 who were seropositive and 8 who were uniquely CSF positive, all of whom had involvement of either brain or spinal cord. Both CSF MOG-IgG titer and corrected CSF/serum MOG-IgG index, but not serum MOG-IgG titer, were associated with disability, CSF pleocytosis, and level of CSF proteins.DiscussionCSF MOG-IgG is found in IDD other than MS and also in MS. In IDD other than MS, the CSF MOG-IgG positivity can support the diagnosis of MOGAD. The synthesis of MOG-IgG in the CNS of patients with MOGAD can be detected from the onset of the disease and is associated with the severity of the disease.Classification of EvidenceThis study provides Class II evidence that the presence of CSF MOG-IgG can improve the diagnosis of MOGAD in the absence of an MS phenotype, and intrathecal synthesis of MOG-IgG was associated with increased disability.

Antibodies to myelin oligodendrocyte glycoprotein in chronic relapsing inflammatory optic neuropathy

2018 ◽  
Vol 103 (10) ◽  
pp. 1423-1428 ◽  
Author(s):  
Hongjuan Liu ◽  
Huanfen Zhou ◽  
Junqing Wang ◽  
Quangang Xu ◽  
Shihui Wei
Keyword(s):  
Optic Neuropathy ◽  
Demyelinating Disease ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Onset Age ◽  
Bilateral Involvement ◽  
Severe Visual Loss ◽  
The Status ◽  
The Mean ◽  
Relapse Rates

Background/aimsTo evaluate the status of myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) in chronic relapsing inflammatory optic neuropathy (CRION) and investigate its different clinical characteristics and prognosis.MethodsPatients diagnosed with CRION were recruited by the Neuro-ophthalmology Department of the Chinese People's Liberation Army General Hospital from December 2015 to April 2017. Based on antibody status, they were assigned to either the MOG-CRION or seronegative-CRION groups.ResultsA total of 33 patients (38 eyes) were assessed and divided into the following groups: 22 (66.7%) MOG-CRION and 11 (33.3%) seronegative-CRION. The ratio of female to male was 1:1, and 81.8% of total CRION patients were adults (≥18 years). A total of 29 eyes (76.3%) showed severe visual loss (<20/200) during the first optic neuritis episode, and 37 eyes (72.5%) demonstrated good visual recovery (>20/40) during the final follow-up. The mean onset age of MOG-CRION patients was 28 ± 16 years (range 6–62), which was significantly younger than that of seronegative-CRION (45 ± 12 years, range 22–59) (p=0.029). The intraorbital and canalicular segments were highly involved in the orbital MRI of CRION patients. During the final follow-up, MOG-CRION patients had more bilateral involvement (p=0.008) and higher annualised relapse rates compared with the seronegative-CRION patients (p=0.019).ConclusionCRION was predominantly found in adults with unilateral ON and exhibited a higher rate of seropositive MOG-IgG. MOG-CRION, which may be a disparate subtype of MOG-IgG-induced demyelinating disease that needs further investigation, was found in younger patients at onset, with more bilateral involvement and more relapse tendency.

scholarly journals Myelin oligodendrocyte glycoprotein antibody-associated disease: an immunopathological study

Brain ◽  
2020 ◽  
Vol 143 (5) ◽  
pp. 1431-1446 ◽  
Author(s):  
Yoshiki Takai ◽  
Tatsuro Misu ◽  
Kimihiko Kaneko ◽  
Norio Chihara ◽  
Koichi Narikawa ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Humoral Immunity ◽  
Demyelinating Disease ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Demyelinating Diseases ◽  
Perivascular Spaces ◽  
Clinical Diagnoses ◽  
Demyelinating Lesions ◽  
Oligoclonal Igg ◽  
Aqp4 Antibody

Abstract Conformation-sensitive antibodies against myelin oligodendrocyte glycoprotein (MOG) are detectable in patients with optic neuritis, myelitis, opticomyelitis, acute or multiphasic disseminated encephalomyelitis (ADEM/MDEM) and brainstem/cerebral cortical encephalitis, but are rarely detected in patients with prototypic multiple sclerosis. So far, there has been no systematic study on the pathological relationship between demyelinating lesions and cellular/humoral immunity in MOG antibody-associated disease. Furthermore, it is unclear whether the pathomechanisms of MOG antibody-mediated demyelination are similar to the demyelination patterns of multiple sclerosis, neuromyelitis optica spectrum disorders (NMOSD) with AQP4 antibody, or ADEM. In this study, we immunohistochemically analysed biopsied brain tissues from 11 patients with MOG antibody-associated disease and other inflammatory demyelinating diseases. Patient median onset age was 29 years (range 9–64), and the median interval from attack to biopsy was 1 month (range 0.5–96). The clinical diagnoses were ADEM (n = 2), MDEM (n = 1), multiple brain lesions without encephalopathy (n = 3), leukoencephalopathy (n = 3) and cortical encephalitis (n = 2). All these cases had multiple/extensive lesions on MRI and were oligoclonal IgG band-negative. Most demyelinating lesions in 10 of 11 cases showed a perivenous demyelinating pattern previously reported in ADEM (153/167 lesions) and a fusion pattern (11/167 lesions) mainly in the cortico-medullary junctions and white matter, and only three lesions in two cases showed confluent demyelinated plaques. In addition, 60 of 167 demyelinating lesions (mainly in the early phase) showed MOG-dominant myelin loss, but relatively preserved oligodendrocytes, which were distinct from those of AQP4 antibody-positive NMOSD exhibiting myelin-associated glycoprotein-dominant oligodendrogliopathy. In MOG antibody-associated diseases, MOG-laden macrophages were found in the perivascular spaces and demyelinating lesions, and infiltrated cells were abundant surrounding multiple blood vessels in and around the demyelinating lesions, mainly consisting of macrophages (CD68; 1814 ± 1188 cells/mm2), B cells (CD20; 468 ± 817 cells/mm2), and T cells (CD3; 2286 ± 1951 cells/mm2), with CD4-dominance (CD4+ versus CD8+; 1281 ± 1196 cells/mm2 versus 851 ± 762 cells/mm2, P &lt; 0.01). Humoral immunity, evidenced by perivascular deposits of activated complements and immunoglobulins, was occasionally observed in some MOG antibody-associated demyelinating lesions, and the frequency was much lower than that in AQP4 antibody-positive NMOSD. Subpial lesions with perivenous demyelination were observed in both ADEM and cortical encephalitis. Our study suggests that ADEM-like perivenous inflammatory demyelination with MOG-dominant myelin loss is a characteristic finding of MOG antibody-associated disease regardless of whether the diagnostic criteria of ADEM are met. These pathological features are clearly different from those of multiple sclerosis and AQP4 antibody-positive NMOSD, suggesting an independent autoimmune demyelinating disease entity.

Fatal underhanded chronic enterovirus infection associated with anti-CD20 monotherapy for central nervous system demyelinating disease

2020 ◽  
pp. 135245852092397
Author(s):  
Léa Luciani ◽  
Laetitia Ninove ◽  
Christine Zandotti ◽  
Virginie Chalvignac ◽  
David Lagier ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Chronic Infection ◽  
Demyelinating Disease ◽  
Fatal Case ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Sudden Onset ◽  
Fulminant Myocarditis ◽  
Enterovirus Infection ◽  
Coxsackievirus B4 ◽  
Anti Cd20

We report a fatal case of coxsackievirus B4 chronic infection in a 30-year-old woman with a diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disorder controlled by rituximab monotherapy for 3 years. Initially presenting as self-limited meningitis, the infection remained silent for 8 months before the sudden onset of fulminant myocarditis. Analysis of the complete genome showed that the same virus was responsible for both episodes.

scholarly journals Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

2022 ◽  
Vol 15 ◽  
Author(s):  
Hang Shu ◽  
Manqiu Ding ◽  
Pei Shang ◽  
Jia Song ◽  
Yue Lang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Demyelinating Disease ◽  
Age Of Onset ◽  
Clinical Phenotype ◽  
Brain Mri ◽  
Clinical Manifestations ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Lessons Learned ◽  
Population Characteristics ◽  
High Dose

Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

scholarly journals Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Life ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 19
Author(s):  
Michela Ada Noris Ferilli ◽  
Roberto Paparella ◽  
Ilaria Morandini ◽  
Laura Papetti ◽  
Lorenzo Figà Talamanca ◽  
...  
Keyword(s):  
Neuromyelitis Optica ◽  
Pediatric Patients ◽  
Demyelinating Disease ◽  
Area Postrema ◽  
Case Series ◽  
Myelin Oligodendrocyte Glycoprotein ◽  
Spectrum Disorder ◽  
Neuromyelitis Optica Spectrum Disorder ◽  
Periaqueductal Gray Matter ◽  
Aquaporin 4 Antibody

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.

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