Anti-MOG encephalitis mimicking small vessel CNS vasculitis

OBJECTIVETo report 2 patients with anti–myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis. METHODSReview of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined i...

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Bibliographic Details
Published in:Neurology : neuroimmunology & neuroinflammation 2019-03, Vol.6 (2), p.e538
Main Authors: Patterson, Kristina, Iglesias, Estibaliz, Nasrallah, Maclean, González-Álvarez, Verónica, Suñol, Mariona, Anton, Jordi, Saiz, Albert, Lancaster, Eric, Armangue, Thaís
Format: Article
Language:English
Subjects:
Adult
Brain
Child, Preschool
Diagnosis, Differential
Encephalitis - complications
Encephalitis - diagnosis
Encephalitis - drug therapy
Encephalitis - pathology
Female
Humans
Magnetic Resonance Imaging
Male
Myelin-Associated Glycoprotein
Myelin-Oligodendrocyte Glycoprotein - immunology
Retrospective Studies
Vasculitis, Central Nervous System - diagnosis
Vasculitis, Central Nervous System - pathology
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Summary:OBJECTIVETo report 2 patients with anti–myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis. METHODSReview of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay. RESULTSSymptoms included fever, headache, and progressive mental status changes and focal neurologic deficits. CSF studies revealed lymphocytic pleocytosis, and both patients had abnormal brain MRIs. Brain biopsy samples showed prominent lymphocytic infiltration of the wall of small vessels; these findings initially suggested small vessel CNS vasculitis, and both patients were treated accordingly. Although 1 patient had a relapsing-remitting course not responsive to cyclophosphamide, the other one (also treated with cyclophosphamide) did not relapse. Retrospective assessment of serum and CSF demonstrated MOG antibodies in both cases, and review of biopsy specimens showed absence of fibrinoid necrosis (a pathologic requirement for small vessel CNS vasculitis). CONCLUSIONSAnti–MOG-associated encephalitis can be mistaken for small vessel CNS vasculitis. This is important because the diagnosis of anti–MOG-associated encephalitis does not require brain biopsy and can be established with a serologic test.
ISSN:2332-7812
2332-7812
DOI:10.1212/NXI.0000000000000538