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November 2021; 8 (6) ArticleOpen Access

Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody

Mai Yamakawa, Keenan O. Hogan, John Leever, Yasir N. Jassam
First published October 12, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001081
Mai Yamakawa
From the Department of Neurology (M.Y., J.L., Y.N.J.); Department of Pathology and Laboratory Medicine (K.O.H.); Department of Radiology (J.L.), University of Kansas Medical Center; and Hoag Memorial Hospital Presbyterian (Y.N.J.), Pickup Family Neuroscience Institute, Newport Beach, CA.
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Keenan O. Hogan
From the Department of Neurology (M.Y., J.L., Y.N.J.); Department of Pathology and Laboratory Medicine (K.O.H.); Department of Radiology (J.L.), University of Kansas Medical Center; and Hoag Memorial Hospital Presbyterian (Y.N.J.), Pickup Family Neuroscience Institute, Newport Beach, CA.
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John Leever
From the Department of Neurology (M.Y., J.L., Y.N.J.); Department of Pathology and Laboratory Medicine (K.O.H.); Department of Radiology (J.L.), University of Kansas Medical Center; and Hoag Memorial Hospital Presbyterian (Y.N.J.), Pickup Family Neuroscience Institute, Newport Beach, CA.
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Yasir N. Jassam
From the Department of Neurology (M.Y., J.L., Y.N.J.); Department of Pathology and Laboratory Medicine (K.O.H.); Department of Radiology (J.L.), University of Kansas Medical Center; and Hoag Memorial Hospital Presbyterian (Y.N.J.), Pickup Family Neuroscience Institute, Newport Beach, CA.
MBChB, MRCP (UK)
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Citation
Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody
Mai Yamakawa, Keenan O. Hogan, John Leever, Yasir N. Jassam
Neurol Neuroimmunol Neuroinflamm Nov 2021, 8 (6) e1081; DOI: 10.1212/NXI.0000000000001081

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Abstract

Background and Objectives To describe the autopsy findings and neuropathologic evaluation of autoimmune meningoencephalomyelitis associated with glial fibrillary acidic protein (GFAP) antibody.

Methods We reviewed the clinical course, imaging, laboratory, and autopsy findings of a patient with autoimmune meningoencephalomyelitis associated with GFAP antibody who had a refractory course to multiple immunosuppressive therapies.

Results The patient was a 70-year-old man who was diagnosed as GFAP antibody-associated autoimmune meningoencephalomyelitis. MRI of the head showed linear perivascular enhancement in the midbrain and the basal ganglia. Despite treatment with high-dose corticosteroids, plasma exchange, IV immunoglobulins, and cyclophosphamide, he died with devastating neurologic complications. Autopsy revealed a coexistent neuroendocrine tumor in the small intestine and diffuse inflammation in the brain parenchyma, perivascular spaces, and leptomeninges, with predominant T-cells, macrophages, and activated microglia. B-cells and plasma cells were absent. There was no astrocyte involvement with change in GFAP immunostaining.

Discussion This case illustrates autoimmune meningoencephalomyelitis associated with GFAP antibody in the CSF and coexistent neuroendocrine tumor. The autopsy findings were nonspecific and did not demonstrate astrocyte involvement. Further accumulation of cases is warranted to delineate the utility and pathogenic significance of the GFAP autoantibody.

Glossary

AQP-4=
aquaporin 4;
CLIPPERS=
chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids;
GFAP=
glial fibrillary acidic protein;
WDNET=
well-differentiated neuroendocrine tumor

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 the authors.

  • Received January 4, 2021.
  • Accepted in final form July 16, 2021.
  • Copyright © 2021 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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Letters: Rapid online correspondence

  • Reader Response: Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody
    • Youming Long, Neurology, the Second Affiliated Hospital of GuangZhou Medical University
    • Jie Yang, Department of Neurology, the Second Affiliated Hospital of GuangZhou Medical University
    Submitted April 14, 2022
  • Author Response: Autopsy Case of Meningoencephalomyelitis Associated With Glial Fibrillary Acidic Protein Antibody
    • Mai Yamakawa, Neurologist, University of Kansas Medical Center
    • Keenan Hogan, Pathologist, University of Kansas Medical Center
    • John Leever, Radiologist, University of Kansas Medical Center
    • Yasir Jassam, Neurologist, Pickup family neuroscience institute
    Submitted April 01, 2022
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