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March 2019; 6 (2) ArticleOpen Access

Anti-MOG encephalitis mimicking small vessel CNS vasculitis

Kristina Patterson, Estibaliz Iglesias, Maclean Nasrallah, Verónica González-Álvarez, Mariona Suñol, Jordi Anton, Albert Saiz, Eric Lancaster, Thaís Armangue
First published February 1, 2019, DOI: https://doi.org/10.1212/NXI.0000000000000538
Kristina Patterson
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Estibaliz Iglesias
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Maclean Nasrallah
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Verónica González-Álvarez
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Mariona Suñol
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Jordi Anton
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Albert Saiz
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Eric Lancaster
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Thaís Armangue
From the Neurology Department (K.P., E.L.), University of Pennsylvania, Philadelphia; Rheumatology Department, Sant Joan de Deu Children's Hospital (E.I., J.A.), University of Barcelona, Spain; Department of Pathology (M.N.), University of Pennsylvania, Philadelphia; Pediatric Neuroimmunology Unit (V.G.-Á., T.A.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; Department of Pathology (M.S.), Sant Joan de Déu Children's Hospital, University of Barcelona, Spain; and Neuroimmunology Program (A.S., T.A.), IDIBAPS-Hospital Clinic, University of Barcelona, Spain.
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Citation
Anti-MOG encephalitis mimicking small vessel CNS vasculitis
Kristina Patterson, Estibaliz Iglesias, Maclean Nasrallah, Verónica González-Álvarez, Mariona Suñol, Jordi Anton, Albert Saiz, Eric Lancaster, Thaís Armangue
Neurol Neuroimmunol Neuroinflamm Mar 2019, 6 (2) e538; DOI: 10.1212/NXI.0000000000000538

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Abstract

Objective To report 2 patients with anti–myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis.

Methods Review of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay.

Results Symptoms 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).

Conclusions Anti–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.

Glossary

MMF=
mycophenolate mofetil;
MOG=
myelin oligodendrocyte glycoprotein

Footnotes

  • Funding information and disclosures are provided at the end of the article. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/NN.

  • The Article Processing Charge was funded by the Clinic Foundation.

  • See page e544

  • Received August 16, 2018.
  • Accepted in final form October 19, 2018.
  • Copyright © 2019 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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