Anti-Myelin Oligodendrocyte Glycoprotein Antibody Associated With Gray Matter Predominant Transverse Myelitis Mimicking Acute Flaccid Myelitis: A Presentation of Two Cases

2018 ◽  
Vol 86 ◽  
pp. 42-45 ◽  
Author(s):  
Cynthia Wang ◽  
Ram Narayan ◽  
Benjamin Greenberg
Author(s):  
Deirdre O'Sullivan ◽  
Michael Moore ◽  
Susan Byrne ◽  
Andreas O. Reiff ◽  
Susanna Felsenstein

AbstractAcute disseminated encephalomyelitis in association with extensive longitudinal transverse myelitis is reported in a young child with positive anti-myelin oligodendrocyte glycoprotein (MOG) antibody with heterozygous NLRP3 missense mutations; p.(Arg488Lys) and p.(Ser159Ile). This case may well present an exceptional coincidence, but may describe a yet unrecognized feature of the spectrum of childhood onset cryopyrinopathies that contribute to the understanding of the genetic basis for anti-MOG antibody positive encephalomyelitis. Based on this observation, a larger scale study investigating the role of NLRP3 and other inflammasomes in this entity would provide important pathophysiological insights and potentially novel avenues for treatment.


2018 ◽  
Vol 19 (3) ◽  
pp. 187-195 ◽  
Author(s):  
Maciej Juryńczyk ◽  
Anu Jacob ◽  
Kazuo Fujihara ◽  
Jacqueline Palace

The field of central nervous system (CNS) inflammatory diseases has recently broadened to include a new condition associated with pathogenic serum antibodies against myelin oligodendrocyte glycoprotein (MOG). This is distinct from multiple sclerosis (MS) and aquaporin-4 (AQP4) antibody neuromyelitis optica spectrum disorders (NMOSD). MOG antibody-associated disease phenotypes are varied and range from classical neuromyelitis optica to acute demyelinating encephalomyelitis and cortical encephalitis. The diagnosis depends on using a reliable, specific and sensitive assay of the antibody. Clinical and imaging features of MOG-associated syndromes overlap with AQP4 antibody NMOSD but can be usually distinguished from MS: in particular, the silent lesions typical of MS that progressively increase lesion volume are rare in MOG antibody disease. The disease can relapse but medium-term immunosuppression appears to be protective. Permanent disability, particularly severe ambulatory and visual disability, is less frequent than in AQP4 antibody NMOSD and usually results from the onset attack. However, sphincter and sexual dysfunction after a transverse myelitis is common. Here we review the practical aspects of diagnosing and managing a patient with MOG antibody-associated disease.


2019 ◽  
Vol 2 (10) ◽  
pp. e1912732 ◽  
Author(s):  
Romina Mariano ◽  
Silvia Messina ◽  
Kurun Kumar ◽  
Wilhelm Kuker ◽  
Maria Isabel Leite ◽  
...  

Biomedicines ◽  
2019 ◽  
Vol 7 (2) ◽  
pp. 42 ◽  
Author(s):  
Marco A. Lana-Peixoto ◽  
Natália Talim

Neuromyelitis optica spectrum disorder (NMOSD) and anti-myelin oligodendrocyte glycoprotein (anti-MOG) syndromes are immune-mediated inflammatory conditions of the central nervous system that frequently involve the optic nerves and the spinal cord. Because of their similar clinical manifestations and habitual relapsing course they are frequently confounded with multiple sclerosis (MS). Early and accurate diagnosis of these distinct conditions is relevant as they have different treatments. Some agents used for MS treatment may be deleterious to NMOSD. NMOSD is frequently associated with antibodies which target aquaporin-4 (AQP4), the most abundant water channel in the CNS, located in the astrocytic processes at the blood-brain barrier (BBB). On the other hand, anti-MOG syndromes result from damage to myelin oligodendrocyte glycoprotein (MOG), expressed on surfaces of oligodendrocytes and myelin sheaths. Acute transverse myelitis with longitudinally extensive lesion on spinal MRI is the most frequent inaugural manifestation of NMOSD, usually followed by optic neuritis. Other core clinical characteristics include area postrema syndrome, brainstem, diencephalic and cerebral symptoms that may be associated with typical MRI abnormalities. Acute disseminated encephalomyelitis and bilateral or recurrent optic neuritis are the most frequent anti-MOG syndromes in children and adults, respectively. Attacks are usually treated with steroids, and relapses prevention with immunosuppressive drugs. Promising emerging therapies for NMOSD include monoclonal antibodies and tolerization.


2019 ◽  
Vol 10 (5) ◽  
pp. 435-443
Author(s):  
Lydia Marcus ◽  
Sumit Singh ◽  
Jayne Ness

ObjectiveTo observe whether cases of acute flaccid myelitis (AFM) before and since August 1, 2014, had important differences and to further characterize patients with AFM regarding clinical, laboratory, imaging, and treatment findings.MethodsAll pediatric patients with AFM at our institution were reviewed. Demographic, clinical, and diagnostic data were collected through medical record review. Patients with onset before August 1, 2014, and after that date were compared and when applicable compared with Centers for Disease Control and Prevention data.ResultsSixteen patients were included, 6 in the pre-2014 and 10 in the post-2014 group. The mean age in the pre-2014 group was 7.4 years and in the post-2014 group was 6.4 years. Initial symptoms were similar in both groups, as were functional and motor abilities at disease nadir and the most recent follow-up. Post-2014 patients had a higher mean CSF white blood cell count (57) and neutrophil count (30%) compared with pre-2014 patients (3.2 and 0.5%, respectively). Eighty percent of post-2014 patients had positive enterovirus/rhinovirus testing, with 57% of specimens positive for enterovirus D68 (EV-D68). On acute imaging, a triad of brainstem, cervical cord gray matter involvement, and ventral nerve root/cauda equina (CE) thickening/enhancement was found in 5 patients.ConclusionThe groups had more similarities than differences but with a more inflammatory picture in the post-2014 patients. The constellation of cervical cord gray matter, brainstem, and nerve root/CE thickening should raise suspicion for AFM in the appropriate clinical setting. Most post-2014 patients had associated enterovirus infections, and over half tested for EV-D68 were positive. There was minimal clinical improvement in both groups despite various immunotherapies.


2020 ◽  
Vol 22 (2) ◽  
pp. 85-90 ◽  
Author(s):  
Neha Kumar ◽  
Kelsey Graven ◽  
Nancy I. Joseph ◽  
John Johnson ◽  
Scott Fulton ◽  
...  

Abstract Stimulation of the immune response after vaccination can occasionally result in adverse effects, including demyelination of the central nervous system. The most common presentation of postvaccination demyelination is acute disseminated encephalomyelitis, but cases of optic neuritis, transverse myelitis, and multiple sclerosis relapses have been reported. More recently, an increasing number of postvaccination neuromyelitis optica spectrum disorder (NMOSD) cases have surfaced in the literature, especially in patients with aquaporin-4 antibodies. In this article, we report an unusual case of myelin oligodendrocyte glycoprotein antibody–related NMOSD after the receipt of multiple vaccines in a first-trimester pregnant woman from Africa. We review the reported cases of postvaccination demyelination in the past decade, with a focus on the relationship between NMOSD and vaccination in patients with aquaporin-4 or myelin oligodendrocyte glycoprotein antibodies. Finally, we discuss the clinical relevance of the present case and similar reported cases as it relates to patient care in the neuroimmunology clinic and identify potential areas for future research.


2020 ◽  
pp. 213-234
Author(s):  
Molly Hartrich ◽  
Emily Rose

Neurologic emergencies may occur in children and generate a significant differential diagnosis of potentially life-threatening etiologies. This chapter reviews the most common and/or important neurological emergencies, including febrile seizures, first presentation of afebrile seizures, seizure variants, breakthrough seizures in epilepsy, seizures due to toxic ingestion, status epilepticus, altered mental status, headache, migraine syndromes, encephalitis, acute demyelinating encephalomyelitis, acute cerebellar ataxia, anti-NMDA receptor encephalitis, motor dysfunction/weakness syndromes such as Guillain-Barre syndrome, polyneuropathies, transverse myelitis, acute periodic paralysis, acute flaccid myelitis, and neuromuscular disorders such as botulism, myasthenia gravis. The common presentations, atypical presentations, evaluation, disposition, clinical pearls, and pitfalls of these important pediatric neurologic emergencies are discussed.


2021 ◽  
Vol 335 ◽  
pp. 113488
Author(s):  
Suyog Pol ◽  
Serena Liang ◽  
Ferdinand Schweser ◽  
Ravendra Dhanraj ◽  
Anna Schubart ◽  
...  

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