Autoimmune cortical encephalitis in two children with anti-myelin oligodendrocyte glycoprotein (MOG) antibody
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Autoimmune cortical encephalitis in two children with anti‑myelin oligodendrocyte glycoprotein (MOG) antibody D. Doig1 · C. McNamara2 · L. Mewasingh3 · S. Beri3 · B. Jones2 · C. Kachramanoglou2 · W. Jan2 Received: 18 June 2020 / Revised: 27 September 2020 / Accepted: 7 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Purpose Anti-myelin oligodendrocyte glycoprotein antibodies (anti-MOG), directed against a component of the myelin sheath, are sometimes detected in the blood or cerebrospinal fluid (CSF) of patients with acute demyelinating conditions. Cortical encephalitic presentations in anti-MOG-antibody-positive patients are recognized but rare, and few pediatric cases have been described. Methods We describe clinical, biochemical, and MRI findings in two children presenting with generalized seizures due to cortical encephalitis, and review potential underlying immunological processes. Results In both patients, anti-MOG antibodies were detected. Both underwent MRI scans which demonstrated bilateral cortical swelling and T2/fluid-attenuated inversion recovery (FLAIR) hyperintensity with corresponding regions of reduced diffusion. Conclusion Early detection of anti-MOG antibodies in patients with a similar presentation and imaging features would enable rapid institution of appropriate treatment, and potentially reduce the need for invasive diagnostic procedures such as brain biopsy. Keywords Encephalitis · Demyelinating diseases · Pediatrics · Anti-myelin oligodendrocyte glycoprotein · Magnetic resonance imaging · Radiology
Introduction Myelin oligodendrocyte glycoprotein (MOG), located on the surface of myelin and encoded by the MOG gene, is involved in normal myelination in the central nervous system. Immunoglobulin G (IgG) antibodies to MOG can be found in the sera of pediatric patients presenting with a variety of inflammatory demyelinating neurological syndromes, including acute disseminated encephalomyelitis (ADEM), optic neuritis, aquaporin-negative neuromyelitis optica spectrum disorders, and transverse myelitis [1]. Cortical encephalitis * D. Doig [email protected] 1
Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London WC1N 3BG, UK
2
Imaging Department, Imperial College Healthcare NHS Trust, London, UK
3
Paediatric Neurology Department, Imperial College Healthcare NHS Trust, London, UK
associated with anti-MOG antibodies is uncommon [2], and is rarely described in children. We review the clinical, laboratory, and imaging features of two children presenting with cortical encephalitis causing generalized seizures, in whom anti-MOG-IgG was the only significant underlying finding.
Case series Patient 1 Clinical presentation and investigations A previously healthy 11-year-old boy presented acutely with a 30-min tonic–clonic seizure requiring intubation and requiring the administration of diazepam and phenytoin to terminate the se
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