MOG Antibodies Restricted to CSF in Children With Inflammatory CNS Disorders
Gemma Olivé-CireraArlette L. BruijstensElianet FonsecaLi-Wen ChenEva CaballeroEugenia Martínez‐HernándezMar GuaspMaría SepúlvedaLaura NaranjoRaquel Ruiz‐GarcíaYolanda BlancoAlbert SaizJosep DalmauThaís Armangué
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To assess the clinical significance of myelin oligodendrocyte glycoprotein antibodies (MOG-abs) restricted to CSF in children with inflammatory CNS disorders.Keywords:
Acute disseminated encephalomyelitis
Myelin oligodendrocyte glycoprotein
Encephalomyelitis
Abstract Background Myelin oligodendrocyte glycoprotein antibody‐associated disease myelin oligodendrocyte glycoprotein antibody‐associated disease is an emerging demyelinating condition distinct from neuromyelitis optica spectrum disorder and multiple sclerosis affecting both children and adults with a spectrum of clinical manifestations ranging from optic neuritis, myelitis and acute disseminated encephalomyelitis. Case report We describe the case of a 2 year‐old girl diagnosed with myelin oligodendrocyte glycoprotein antibody‐associated disease presenting as recurrent acute disseminated encephalomyelitis who showed suppression of disease activity after treatment with rituximab. This case represents the youngest Mexican patient with myelin oligodendrocyte glycoprotein antibody‐associated disease reported to date. Conclusions Information regarding the clinical presentation and evolution among different population could help to a better understanding of the presentation of this entity.
Myelin oligodendrocyte glycoprotein
Acute disseminated encephalomyelitis
Neuromyelitis Optica
Optic neuritis
Demyelinating disease
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Background: Serum myelin oligodendrocyte glycoprotein (MOG) immunoglobulin (IgG) antibodies have recently been detected in pediatric acute disseminating encephalomyelitis (ADEM).
Myelin oligodendrocyte glycoprotein
Acute disseminated encephalomyelitis
Encephalomyelitis
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We report the case of a 5-year-old Japanese girl who initially had acute disseminated encephalomyelitis (ADEM) and was positive for the myelin oligodendrocyte glycoprotein (MOG) antibodies and developed unilateral optic neuritis (ON) 71 days after ADEM onset. The patient's serum was positive for the anti-MOG antibodies from the onset of ADEM to the development of ON. This phenotype has been reported in only two previous articles, and the specific mechanism of action of the anti-MOG antibodies is not yet understood. Our case suggests that the anti-MOG antibody can be associated with the pathogenesis of ADEM followed by ON. Thus, patients with ADEM who test positive for the anti-MOG antibody may be at risk of developing subsequent ON.
Acute disseminated encephalomyelitis
Myelin oligodendrocyte glycoprotein
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Acute disseminated encephalomyelitis
Encephalomyelitis
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Encephalitis is a complex syndrome associated with significant morbidity and mortality. Despite biological and neuroimaging investigations, the cause of encephalitis remains undetermined in more than half of the cases. The aim of this review was to describe available data concerning diagnosis and treatment of postinfectious encephalitis, focusing on acute disseminated encephalomyelitis (ADEM) and acute hemorrhagic leukoencephalitis (AHLE).The increasing availability of brain MRI studies has allowed a better delineation of diagnosis and prognosis of postinfectious central nervous system disorders. Beneficial effects of steroids and plasma exchange have been described in the most severe forms of postinfectious encephalitis, including ADEM and AHLE, but randomized controlled studies are lacking. Intravenous immunoglobulins may be of value in ADEM with peripheral nerve involvement and for patients in whom corticosteroid therapy is contraindicated.Postinfectious encephalitis needs to be identified early in the management of patients with unexplained encephalitis as it represents a treatable disease. Randomized studies are needed in order to assess the potential benefit of early combined immunotherapy in ADEM.
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Acute disseminated encephalomyelitis (ADEM) followed by optic neuritis (ON) has been reported as a distinct phenotype associated with anti-myelin oligodendrocyte protein (MOG) antibody. We herein report the case of a 37-year-old woman who was diagnosed with ADEM at 4 years old of age and who subsequently developed ON followed by recurrent ADEM 33 years after the initial onset. A serum analysis showed anti-MOG antibody positivity. This phenotype has only previously been reported in pediatric cases. Neurologists thus need to be aware that the phenotype may occur in adult patients, in whom it may be assumed to be atypical multiple sclerosis.
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Encephalomyelitis
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Myelin oligodendrocyte glycoprotein (MOG) antibody disease is a rare autoimmune disorder with antibodies against the MOG predominantly involving the optic nerve and spinal cord leading to vision loss and paralysis. When MOG antibody disease involves the brain, the phenotype is similar to acute disseminated encephalomyelitis (ADEM). In this review, we discuss MOG-positive cases presenting with encephalitis, encephalopathy, or ADEM-like presentation based on recently published series.
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Antibodies to native myelin oligodendrocyte glycoprotein (nMOG) in 47 children during a first episode of CNS demyelination (acute disseminated encephalomyelitis [ADEM] in 19, and a clinical isolated syndrome [CIS] in 28) were investigated by a cell-based bioassay in a study at Children’s Hospital at Westmead, Sydney, Australia, and at the University of Munich, Germany.
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Correction to: Persistent Presence of the Anti-Myelin Oligodendrocyte Glycoprotein Autoantibody in a Pediatric Case of Acute Disseminated Encephalomyelitis Followed by Optic NeuritisNeuropediatrics 2014; 45(03): 196-199DOI: 10.1055/s-0034-1371179
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