Neurosarcoidosis
Pathophysiology, Diagnosis, and Treatment
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Abstract
Although often regarded as a protean illness with myriad clinical and imaging manifestations, neurosarcoidosis typically presents as recognizable syndromes that can be approached in a rational, systematic fashion. Understanding of neurosarcoidosis has progressed significantly in recent years, including updated diagnostic criteria and advances in treatment. The diagnosis of neurosarcoidosis is established by the clinical syndrome, imaging and histopathological findings, and exclusion of other causes. Mounting evidence supports the use of tumor necrosis factor inhibitors as an important addition to the therapeutic armamentarium, along with glucocorticoids and steroid-sparing cytotoxic immunosuppressants. In this narrative review, we summarize recent advances in the diagnosis and treatment of neurosarcoidosis.
Glossary
- ACE=
- angiotensin-converting enzyme;
- FDG=
- fluorodeoxyglucose;
- IgG=
- immunoglobulin G;
- LETM=
- longitudinally extensive myelitis;
- PNS=
- peripheral nervous system;
- TNF=
- tumor necrosis factor
Footnotes
Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.
↵* Co–senior authors and co–corresponding authors.
The Article Processing Charge was funded by the authors.
- Received March 20, 2021.
- Accepted in final form July 12, 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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