Treatment of neuromyelitis optica: review and recommendations
Open accessAbstract
Neuromyelitis optica (NMO) is an autoimmune demyelinating disease preferentially targeting the optic nerves and spinal cord. Once regarded as a variant of multiple sclerosis (MS), NMO is now recognized to be a different disease with unique pathology and immunopathogenesis that does not respond to traditional MS immunomodulators such as interferons. Preventive therapy in NMO has focused on a range of immunosuppressive medications, none of which have been validated in a rigorous randomized trial. However, multiple retrospective and a few recent prospective studies have provided evidence for the use of six medications for the prevention of NMO exacerbations: azathioprine, rituximab, mycophenolate mofetil, prednisone, methotrexate and mitoxantrone. This review provides a comprehensive analysis of each of these medications in NMO and concludes with a set of recommended consensus practices.
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An accurate diagnosis of NMOSD is essential since prompt treatment of acute attacks and long-term immunosuppression appear to reduce disability, while conventional MS treatments may aggravate NMOSD.
“An accurate diagnosis of NMOSD is essential since prompt treatment of acute attacks and long-term immunosuppression appear to reduce disability, while conventional MS treatments may aggravate NMOSD.44,48”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [48]
The qualitative demonstration of two or more CSF-specific oligoclonal bands (OCBs) more reliably indicates intrathecal antibody synthesis than other tests, such as IgG Index.
“The qualitative demonstration of two or more CSF-specific oligoclonal bands (OCBs) more reliably indicates intrathecal antibody synthesis than other tests, such as IgG Index. 58-60”
From Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria (2018) · cites this paper as [58]
The Introduction discusses NMO-IgG as a diagnostic biomarker, not CSF-specific oligoclonal bands. Searches for oligoclonal bands, intrathecal antibody synthesis, and IgG Index found no passage in our copy comparing these tests or stating the two-band threshold. The cited paper therefore does not verify the claim. (LLM, conf=0.76)
Experimental — accuracy not yet measuredIn the appropriate clinical setting, evidence of intrathecal antibody synthesis, though not specific for MS, supports the diagnosis.
“In the appropriate clinical setting, evidence of intrathecal antibody synthesis, though not specific for MS, supports the diagnosis. 58”
From Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria (2018) · cites this paper as [58]
The Introduction (page unavailable) discusses aquaporin-4 antibodies as a biomarker distinguishing neuromyelitis optica from MS, but does not address whether intrathecal antibody synthesis supports an MS diagnosis. Searches for intrathecal antibody synthesis and CSF oligoclonal bands found no passage making that point. (LLM, conf=0.72)
Experimental — accuracy not yet measuredThe sensitivity of OCB testing depends on the method used; agarose gel electrophoresis with isoelectric focusing and immunoblotting or immunofixation for IgG is the most sensitive at present.
“The sensitivity of OCB testing depends on the method used; agarose gel electrophoresis with isoelectric focusing and immunoblotting or immunofixation for IgG is the most sensitive at present. 58-60”
From Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria (2018) · cites this paper as [58]
The Introduction discusses the NMO-IgG biomarker, and the Summary and Current Recommendations addresses treatment, but neither discusses oligoclonal-band (OCB) testing. Searches for OCB sensitivity and for agarose gel electrophoresis, isoelectric focusing, immunoblotting, and immunofixation found no passage addressing the claimed comparison of testing methods. No page numbers were available in our copy. (LLM, conf=0.75)
Experimental — accuracy not yet measuredNegative CSF OCBs does not rule out MS, particularly early in the condition and in children.
“While negative CSF OCBs does not rule out MS, particularly early in the condition and in children, 58,59 caution should be exercised in diagnosing MS when CSF OCBs are not detected and, certainly, in the presence of atypical clinical, imaging, or CSF findings.”
From Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria (2018) · cites this paper as [58]
The Introduction discusses distinguishing neuromyelitis optica from MS using an aquaporin-4 antibody biomarker, not whether negative CSF oligoclonal bands exclude MS. Searches for OCBs, oligoclonal bands, and their absence in early or pediatric MS found no passage in our copy addressing the claim. Page numbers were not available. (LLM, conf=0.76)
Experimental — accuracy not yet measured
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- Authors
- Kimbrough, D. J., Fujihara, K., Jacob, A., Lana-Peixoto, M. A., Isabel Leite, M., Levy, M., Marignier, R., Nakashima, I., Palace, J., de Seze, J., Stuve, O., Tenembaum, S. N., Traboulsee, A., Waubant, E., Weinshenker, B. G., & Wingerchuk, D. M.
- Affiliations
- Johns Hopkins University (US)
- Tohoku University (JP)
- Walton Centre (GB)
- Université de Lyon (FR)
- John Radcliffe Hospital (GB)
- Garrahan Hospital (AR)
- University of British Columbia (CA)
- University of California, San Francisco (US)
- Mayo Clinic (US)
- Mayo Clinic in Arizona (US)
- Corresponding author
- Levy, Michael
- ORCID
- Venue
- Multiple Sclerosis and Related Disorders
- Publisher
- Elsevier BV
- Published
- 2012-10
- Volume
- 1
- Issue
- 4
- Pages
- 180-187
- ISSN
- 2211-0348 (ISSN-L)
- PMC
- PMC3926208
- OpenAlex
- W2150598981
- Cited by
- 260
- References
- 1
- Citations by year
- 2026: 7 · 2025: 16 · 2024: 13 · 2023: 9 · 2022: 16 · 2021: 23 · 2020: 29 · 2019: 17 · 2018: 28 · 2017: 24 · 2016: 24 · 2015: 29 · 2014: 15 · 2013: 9 · 2012: 1
- FWCI
- 11.78
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Multiple Sclerosis and Related Disorders (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- HAL (submittedVersion)
- Funders
- Japan Society for the Promotion of Science (22229008)
- National Institute of Neurological Disorders and Stroke (K08NS078555)
- Topics
- Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
- Keywords
- neuromyelitis optica, immunosuppressive therapy, azathioprine, rituximab, mycophenolate mofetil, prednisone, methotrexate, mitoxantrone, autoimmune demyelinating disease
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