title: Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy.
abstract: Under the umbrella of pediatric-acquired demyelinating syndromes, there is a multitude of disorders, including optic neuritis, transverse myelitis, acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), and neuromyelitis optica spectrum disorders (NMOSD). Due to overlapping clinical and magnetic resonance imaging (MRI) features, it can be challenging to provide an accurate diagnosis. In view of therapeutic and prognostic implications, an early and reliable diagnosis is however of utmost importance. Recent studies of myelin oligodendrocyte glycoprotein (MOG) identify MOG, as a promising target for antibody-mediated demyelination and a biomarker for a relatively benign and non-MS disease course. We describe the clinical and MRI presentation of five children presenting with an acute, severe central nervous system inflammatory disease involving the brain and spinal cord, all of whom were positive for MOG-IgG antibody. Encephalopathy was uncommon at presentation and all had quick resolution of symptoms with intravenous steroid and intravenous immunoglobulin (IVIG) treatment. All patients recovered well, and have been treated with IVIG to potentially prevent relapses.
authors.affiliation: Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors.forename: Salini
authors.initials: S
authors.lastname: Thulasirajah
authors.affiliation: Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors.forename: Daniela
authors.initials: D
authors.lastname: Pohl
authors.affiliation: Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors.forename: Jorge
authors.initials: J
authors.lastname: Davila-Acosta
authors.affiliation: Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.
authors.forename: Sunita
authors.initials: S
authors.lastname: Venkateswaran
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D001921:Brain / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D003711:Demyelinating Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology*; D004673:Encephalomyelitis, Acute Disseminated / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D009188:Myelitis, Transverse / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D009471:Neuromyelitis Optica / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D012189:Retrospective Studies; D013116:Spinal Cord / Q000000981:diagnostic imaging*; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D063308:Myelin-Oligodendrocyte Glycoprotein; D008775:Methylprednisolone