Teaching NeuroImage: Imaging and Pathologic Findings in SARS-CoV-2–Related Acute Demyelinating Encephalomyelitis
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A 41-year-old woman with type 1 diabetes admitted with SARS-CoV-2 PCR confirmed respiratory failure developed altered mental status. EEG was unrevealing, and CSF showed an elevated protein (110 mg/dL) and normal glucose (159 mg/dL), without pleocytosis or oligoclonal bands and normal IgG index. MRI demonstrated FLAIR hyperintensities in the corpus callosum and periventricular white matter (Figure 1). She was treated with plasmapheresis for presumed SARS-CoV-2–related acute demyelinating encephalomyelitis (ADEM) but succumbed to cardiopulmonary arrest. Postmortem histology revealed irregular zones of demyelination with axonal sparing and perivascular inflammatory infiltrate, consistent with ADEM (Figure 2). There was no inflammation within the vessel walls as is seen in vasculitis. SARS-CoV-2 ADEM has variable clinical presentations. Involvement of deep white matter and the corpus callosum has been previously reported, as well as hemorrhagic leukoencephalopathy, although only minimal microhemorrhage was present for this patient.1 ADEM can be difficult to diagnose, and outcomes are often poor.2
Diffusion-weighted image (A) with Apparent Diffusion Coefficient (B) showing confluent areas of restricted diffusion with associated FLAIR hyperintensity (C) involving the corpus callosum and bilateral corona radiata. Microhemorrhages (D) in the corpus callosum. Postcontrast images (E) demonstrate heterogeneous enhancement.
(A) Areas of pallor representing demyelination (*) on Luxol Fast Blue stain. (B) Relative preservation of axons (blue arrows), shown by neurofilament immunohistochemistry. (C) Dense macrophage infiltration as evidenced by CD163 immunohistochemistry. (D) Scattered lymphocytic infiltration as demonstrated by leukocyte common antigen immunohistochemistry.
Author Contributions
R. Lalla: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. R. Narasimhan: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data. M. Abdalkader: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data. D. Virmani: study concept or design. K. Suchdev: drafting/revision of the manuscript for content, including medical writing for content. B. Moore: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data. A. Cervantes-Arslanian: drafting/revision of the manuscript for content, including medical writing for content; major role in the acquisition of data; study concept or design; analysis or interpretation of data.
Study Funding
The authors report no targeted funding.
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org/N for full disclosures.
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Submitted and externally peer reviewed. The handling editor was Resident and Fellow Deputy Editor Ariel Lyons-Warren, MD, PhD.
Teaching slides links.lww.com/WNL/C651
- Received July 6, 2022.
- Accepted in final form January 10, 2023.
- © 2023 American Academy of Neurology
References
Letters: Rapid online correspondence
- Reader Response: Teaching NeuroImage: Imaging and Pathologic Findings in SARS-CoV-2–Related Acute Demyelinating Encephalomyelitis
- Arayamparambil C Anilkumar, Professor, Neurology, SUNY Upstate Medical University
Submitted June 07, 2023
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