Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies
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Abstract
Objective To describe the presentations, radiologic features, and outcomes of children with autoimmune encephalitis associated with myelin oligodendrocyte glycoprotein antibodies (MOG abs).
Methods Identification of children fulfilling the diagnostic criteria for possible autoimmune encephalitis (AE) and testing positive for serum MOG abs. Chart review and comprehensive analysis of serum MOG abs using live cell assays and rat brain immunohistochemistry.
Results Ten children (4 girls, 6 boys) with AE and serum MOG abs were identified. The median age at onset was 8.0 years (range: 4–16 years). Children presented with a combination of encephalopathy (10/10), headache (7/10), focal neurologic signs (7/10), or seizures (6/10). CSF pleocytosis was common (9/10, median 80 white cell count/μL, range: 21–256). Imaging showed cortical and deep gray matter involvement in all in addition to juxtacortical signal alterations in 6/10 children. No involvement of other white matter structures or contrast enhancement was noted. MOG abs were detected in all children (median titer 1:640; range: 1:320–1:10,540). Nine children had a favorable outcome at discharge (modified Rankin scale of < 2). Five of 10 children had up to 3 additional demyelinating relapses associated with persisting MOG abs. One child had NMDA receptor (NMDAR) abs at initial presentation. A second child had a third demyelinating episode with MOG abs with overlapping NMDAR encephalitis.
Discussion AE associated with serum MOG abs represents a distinct form of autoantibody-mediated encephalitis in children. We therefore recommend including MOG abs testing in the workup of children with suspected AE.
Glossary
- ab=
- antibody;
- ADEM=
- acute disseminated encephalomyelitis;
- ADS=
- acquired demyelinating syndrome;
- AE=
- autoimmune encephalitis;
- AQP4=
- aquaporin 4;
- CBA=
- cell-based assay;
- FLAIR=
- fluid-attenuated inversion recovery;
- FLAMES=
- unilateral FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with seizures;
- HSV=
- herpes simplex virus;
- IVIG=
- IV immunoglobulins;
- IVMP=
- IV methylprednisolone;
- MOG=
- myelin oligodendrocyte glycoprotein;
- MOG-SD=
- MOG spectrum disease;
- mRS=
- modified Rankin scale;
- NMDAR=
- NMDA receptor;
- NMOSD=
- neuromyelitis optica spectrum disorders;
- ON=
- optic neuritis;
- TBA=
- tissue-based screening;
- WCC=
- white cell count
Footnotes
Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.
The Article Processing Charge was funded by authors.
- Received February 17, 2020.
- Accepted in final form March 25, 2020.
- Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
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