“FLAMES: A novel burning entity in MOG IgG associated disease”

Jain, Kshiteeja; Cherian, Ajith; Kajaria, Divya; Rajalakshmi, P; Nandana, J

doi:10.1016/j.msard.2021.102759

Cited by 27 publications

(16 citation statements)

References 5 publications

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“…Therefore, we speculated that the lesions of the cortex are involved in vascular distribution. Reviewing the cases in our study, we observed that in the 11 patients who underwent cerebrovascular perfusion tests, including MRI, arterial spin labelling imaging, and single-photon emission computed tomography, 10 patients displayed vasodilation of the cerebrovascular branch, indicating high cerebrovascular perfusion ( Ogawa et al, 2017 ; Ikeda et al, 2018 ; Fujimori et al, 2020b ; Katsuse et al, 2020 ; Matoba et al, 2020 ; Otani et al, 2020 ; Jain et al, 2021 ; Nie et al, 2021 ), which usually can be completely restored to normal after steroid therapy. Although there is no clear conclusion about the relationship between vascular distribution and lesions, we speculate that peripheral vessels of the middle cerebral artery or anterior cerebral artery may also be involved in the pathogenesis of the disease after cortical inflammation occurs.…”

Section: Discussionmentioning

confidence: 78%

“…We finally obtained 33 cases from 25 articles and combined with our two cases, a total of 35 cases were reviewed systematically ( Ogawa et al, 2017 ; Hamid et al, 2018 ; Ikeda et al, 2018 ; Budhram et al, 2019 , 2020 ; Haddad et al, 2019 ; Patterson et al, 2019 ; Fujimori et al, 2020b ; Hochmeister et al, 2020 ; Katsuse et al, 2020 ; Matoba et al, 2020 ; Otani et al, 2020 ; Russ et al, 2020 ; Takamatsu et al, 2020 ; Tao et al, 2020 ; Ahsan et al, 2021 ; Chang et al, 2021 ; Doig et al, 2021 ; Jain et al, 2021 ; Kim et al, 2021 ; Maniscalco et al, 2021 ; Nie et al, 2021 ; Stamenova et al, 2021 ; Tian et al, 2021 ; Wang et al, 2021 ; Table 1 ). The main demographic data, clinical characteristics, laboratory results, and imaging results are presented in Table 2 .…”

Section: Resultsmentioning

confidence: 99%

“…Myelin oligodendrocyte glycoprotein is a membrane protein uniquely expressed on the surface of oligodendrocytes and myelin in the central nervous system of humans and other mammals ( Ramanathan et al, 2016 ; Jain et al, 2021 ). Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) overlaps with multiple sclerosis, acute disseminated encephalomyelitis, and aquaporin 4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorders (NMOSD) in terms of clinical phenotype, and is now considered to be a new and independent entity in inflammatory demyelinating diseases of the central nervous system ( Ramanathan et al, 2016 ; Cobo-Calvo et al, 2019 ).…”

Section: Introductionmentioning

confidence: 99%

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

Shu

Ding

Shang

et al. 2022

Front. Hum. Neurosci.

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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.

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Section: Discussionmentioning

confidence: 78%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Shu

Ding

Shang

et al. 2022

Front. Hum. Neurosci.

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“…This case is an example of a young pediatric patient with new-onset seizures, secondary to a subset of MOGAD, called FLAIR, with imaging showing cortical hyperintense lesions in MOG-associated encephalitis with seizures, otherwise referred to as “FLAMES”. This is a rare but reported entity, characterized typically by seizures, headache, fever, and the classic imaging findings of cortical hyperintensities, which are typically uni-lateral, but in a few cases, as demonstrated in our patient, can be bilateral [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 94%

“…Anti-MOG antibodies can be associated with several pediatric demyelinating syndromes, including acute disseminated encephalomyelitis (ADEM), optic neuritis, neuromyelitis optica-like disease, and multiple sclerosis [ 4 , 5 ]. Rarely, this autoimmune condition can present as a form of encephalitis that clinically and radiologically can resemble that of an infectious or neoplastic etiology [ 6 , 7 ]. European studies show an overall prevalence of this disease to be 1.6 per million, with a predominance in White patients [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

et al. 2022

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Patient: Male, 17-month-old Final Diagnosis: Fluid attenuated inversion recovery (FLAIR) imaging showing cortical hyperintense lesions in myelin-oligodendrocyte-glycoprotein (MOG) associated encephalitis with seizures (FLAMES Symptoms: Encephalopathy • fever • left sided weakness • seizures Medication: — Clinical Procedure: Plasmapharesis Specialty: Neurology Objective: Rare disease Background: The relatively new autoimmune disorder, anti-myelin oligodendrocyte glycoprotein (MOG) disease is particularly interesting because of its broad range of presentations. This entity’s appearance on magnetic resonance imaging (MRI) of the brain often makes identifying this disease a challenging process. Younger patients tend to present with an acute disseminated encephalomyelitis picture, with encephalopathy and multifocal neurological signs, while older patients are more likely to present with optic neuritis. We, however, report an atypical case of a patient who presented with encephalopathy, seizures, and significant cortical and subcortical gray matter involvement and was found to have anti-MOG positivity in serum. Case Report: A 17-month-old previously healthy boy presented to Emergency Department with fever, lethargy, vomiting, and left-sided weakness. Eventually, he required intubation due to a prolonged seizure. Continuous electroencephalogram captured several focal seizures, and MRI of the brain showed cortical and subcortical T2 hyperintensities. After extensive laboratory evaluation, he tested positive for anti-MOG antibody. He was empirically started on high-dose intravenous pulse methylprednisolone, followed by plasma exchange, given the poor response to the intravenous steroids. At the 5-month follow-up, the results of the neurological examination had dramatically improved, and MRI findings had largely resolved. Conclusions: This case highlights the importance of suspecting anti-MOG antibody-mediated encephalitis, even while ruling out infectious etiologies, in children presenting with encephalopathy, seizures and MRI abnormalities. Prompt recognition would allow for less delay in treatment and hopefully improve prognosis.

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MOG Antibody Disease: Nuances in Presentation, Diagnosis, and Management

Stefan,

Ciotti

2024

Curr Neurol Neurosci Rep

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“FLAMES: A novel burning entity in MOG IgG associated disease”

Cited by 27 publications

References 5 publications

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

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

Encephalitis with Extensive Cortical Brain Magnetic Resonance Imaging Changes Secondary to Myelin Oligodendrocyte Glycoprotein Antibody Disease

MOG Antibody Disease: Nuances in Presentation, Diagnosis, and Management

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