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Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy.
Neuropediatrics. 2016 Aug; 47(4):245-52.N

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+Show Affiliations

Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.Department of Pediatrics, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

27128728

Citation

Thulasirajah, Salini, et al. "Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy." Neuropediatrics, vol. 47, no. 4, 2016, pp. 245-52.
Thulasirajah S, Pohl D, Davila-Acosta J, et al. Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy. Neuropediatrics. 2016;47(4):245-52.
Thulasirajah, S., Pohl, D., Davila-Acosta, J., & Venkateswaran, S. (2016). Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy. Neuropediatrics, 47(4), 245-52. https://doi.org/10.1055/s-0036-1583184
Thulasirajah S, et al. Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy. Neuropediatrics. 2016;47(4):245-52. PubMed PMID: 27128728.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Myelin Oligodendrocyte Glycoprotein-Associated Pediatric Central Nervous System Demyelination: Clinical Course, Neuroimaging Findings, and Response to Therapy. AU - Thulasirajah,Salini, AU - Pohl,Daniela, AU - Davila-Acosta,Jorge, AU - Venkateswaran,Sunita, Y1 - 2016/04/29/ PY - 2016/4/30/entrez PY - 2016/4/30/pubmed PY - 2017/2/22/medline SP - 245 EP - 52 JF - Neuropediatrics JO - Neuropediatrics VL - 47 IS - 4 N2 - 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. SN - 1439-1899 UR - https://www.unboundmedicine.com/medline/citation/27128728/Myelin_Oligodendrocyte_Glycoprotein_Associated_Pediatric_Central_Nervous_System_Demyelination:_Clinical_Course_Neuroimaging_Findings_and_Response_to_Therapy_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-0036-1583184 DB - PRIME DP - Unbound Medicine ER -