Abstract
We describe a woman with a history of relapsing acute optic neuritis and perineuritis. Testing failed to confirm a specific diagnosis; hence, she was diagnosed with seronegative neuromyelitis optica spectrum disorder and treated with the immunotherapy rituximab, later in conjunction with mycophenolate mofetil. She achieved a durable remission for 9 years until she presented with paresthesia affecting her left fifth digit, right proximal thigh, and left foot, while also reporting a 25-pound weight loss over the prior 3 months. New imaging demonstrated a longitudinally extensive and enhancing optic nerve, in conjunction with multifocal enhancing lesions within the spinal cord, in a skip-like distribution. The differential diagnosis is discussed.
Glossary
- ACE=
- angiotensin-converting enzyme;
- anti-AQP4=
- aquaporin-4 antibody;
- BNS=
- Bing-Neel syndrome;
- BTK=
- Bruton tyrosine kinase;
- CBA=
- cell-based assay;
- CLL=
- chronic lymphocytic lymphoma;
- ED=
- emergency department;
- MMF=
- mycophenolate mofetil;
- MOG=
- myelin oligodendrocyte glycoprotein;
- MOGAD=
- MOG antibody disease;
- NMOSD=
- neuromyelitis optica spectrum disorder;
- ON=
- optic neuritis;
- RBC=
- red blood cell;
- VA=
- visual acuity;
- WBC=
- white blood cell;
- WM=
- Waldenstrom macroglobulinemia
Footnotes
↵* These authors contributed equally to this work as co–senior authors.
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 National Multiple Sclerosis Society.
- Received May 12, 2022.
- Accepted in final form September 12, 2022.
- Copyright © 2022 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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