Multiple Sclerosis Followed by Neuromyelitis Optica Spectrum Disorder: From the National Multiple Sclerosis Society Case Conference Proceedings

A woman presented at age 18 years with partial myelitis and diplopia and experienced multiple subsequent relapses. Her MRI demonstrated T2 abnormalities characteristic of multiple sclerosis (MS) (white matter ovoid lesions and Dawson fingers), and CSF demonstrated an elevated IgG index and oligoclon...

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Published in:Neurology : neuroimmunology & neuroinflammation Vol. 10; no. 1
Main Authors: Goldschmidt, Carolyn, Galetta, Steven L., Lisak, Robert P., Balcer, Laura J., Hellman, Andrew, Racke, Michael K., Lovett-Racke, Amy E., Cruz, Roberto, Parsons, Matthew S., Sattarnezhad, Neda, Steinman, Lawrence, Zamvil, Scott S., Frohman, Elliot M., Frohman, Teresa C.
Format: Journal Article
Language:English
Published: Hagerstown, MD Lippincott Williams & Wilkins 01.01.2023
Subjects:
Diagnostic & Treatment Challenges
ISSN:2332-7812, 2332-7812
Online Access:Get full text
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Summary:A woman presented at age 18 years with partial myelitis and diplopia and experienced multiple subsequent relapses. Her MRI demonstrated T2 abnormalities characteristic of multiple sclerosis (MS) (white matter ovoid lesions and Dawson fingers), and CSF demonstrated an elevated IgG index and oligoclonal bands restricted to the CSF. Diagnosed with clinically definite relapsing-remitting MS, she was treated with various MS disease-modifying therapies and eventually began experiencing secondary progression. At age 57 years, she developed an acute longitudinally extensive transverse myelitis and was found to have AQP4 antibodies by cell-based assay. Our analysis of the clinical course, radiographic findings, molecular diagnostic methods, and treatment response characteristics support the hypothesis that our patient most likely had 2 CNS inflammatory disordersMS, which manifested as a teenager, and neuromyelitis optica spectrum disorder, which evolved in her sixth decade of life. This case emphasizes a key principle in neurology practice, which is to reconsider whether the original working diagnosis remains tenable, especially when confronted with evidence (clinical and/or paraclinical) that raises the possibility of a distinctively different disorder.
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Submitted and externally peer reviewed. The handling editor was Josep O. Dalmau, MD, PhD, FAAN.
The Article Processing Charge was funded by the National Multiple Sclerosis Society.
Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.
ISSN:2332-7812
2332-7812
DOI:10.1212/NXI.0000000000200037