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MRI characteristics of neuromyelitis optica spectrum disorder: An international update
Open accessAbstract
Since its initial reports in the 19th century, neuromyelitis optica (NMO) had been thought to involve only the optic nerves and spinal cord. However, the discovery of highly specific anti-aquaporin-4 antibody diagnostic biomarker for NMO enabled recognition of more diverse clinical spectrum of manifestations. Brain MRI abnormalities in patients seropositive for anti-aquaporin-4 antibody are common and some may be relatively unique by virtue of localization and configuration. Some seropositive patients present with brain involvement during their first attack and/or continue to relapse in the same location without optic nerve and spinal cord involvement. Thus, characteristics of brain abnormalities in such patients have become of increased interest. In this regard, MRI has an increasingly important role in the differential diagnosis of NMO and its spectrum disorder (NMOSD), particularly from multiple sclerosis. Differentiating these conditions is of prime importance because early initiation of effective immunosuppressive therapy is the key to preventing attack-related disability in NMOSD, whereas some disease-modifying drugs for multiple sclerosis may exacerbate the disease. Therefore, identifying the MRI features suggestive of NMOSD has diagnostic and prognostic implications. We herein review the brain, optic nerve, and spinal cord MRI findings of NMOSD.
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“Attacks of myelitis are associated with longitudinally-extensive spinal cord lesions on MRI (≥3 vertebral bodies) with prominent involvement of the central cord 49 (Supplementary Figure 4), in contrast to MS where lesions are usually short (<1-2 vertebral bodies) and located peripherally.”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [49]
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“Lesions extending over more than half the length of the optic nerve, and sometimes into the optic chiasm are characteristic of NMOSD.49,50”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [49]
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“Brain involvement also occurs in NMOSD especially in areas rich in aquaporin-4 such as the dorsal medulla/area postrema (where lesions may cause intractable nausea, vomiting and hiccoughs), diencephalon, and periependymal regions of the corpus callosum, third and fourth ventricles (Supplementary Figure 1).49”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [49]
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Citations
Cites
Distinction between MOG antibody-positive and AQP4 antibody-positive NMO spectrum disorders
Sato, D. K., Callegaro, D., Lana-Peixoto, M. A., Waters, P. J., Jorge, F. M. D. H., Takahashi, T., Nakashima, I., Apostolos-Pereira, S. L., Talim, N., Simm, R. F., et al. (2014) · Neurology · doi:10.1212/wnl.0000000000000101 · Cited by 850
Neuromyelitis optica unique area postrema lesions: Nausea, vomiting, and pathogenic implications
Popescu, B. F. G., Lennon, V. A., Parisi, J. E., Howe, C. L., Weigand, S. D., Cabrera-Gómez, J. A., Newell, K., Mandler, R. N., Pittock, S. J., Weinshenker, B. G., et al. (2011) · Neurology · doi:10.1212/wnl.0b013e318214332c · Cited by 296
Distinction of seropositive NMO spectrum disorder and MS brain lesion distribution
Matthews, L., Marasco, R., Jenkinson, M., Küker, W., Luppe, S., Leite, M. I., Giorgio, A., De Stefano, N., Robertson, N., Johansen-Berg, H., et al. (2013) · Neurology · doi:10.1212/wnl.0b013e3182887957 · Cited by 225
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Hardy, T. A., Reddel, S. W., Barnett, M. H., Palace, J., Lucchinetti, C. F., & Weinshenker, B. G. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(16)30043-6 · Cited by 161
Self-citationWhite matter network disruption and cognitive dysfunction in neuromyelitis optica spectrum disorder.
Cho, E. B., Han, C. E., Seo, S. W., Chin, J., Shin, J.-H., Cho, H.-J., Seok, J. M., Kim, S. T., Kim, B. J., Na, D. L., et al. (2018) · Frontiers in Neurology · doi:10.3389/fneur.2018.01104 · Cited by 46
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Metadata
- Authors
- Kim, H. J., Paul, F., Lana-Peixoto, M. A., Tenembaum, S., Asgari, N., Palace, J., Klawiter, E. C., Sato, D. K., de Seze, J., Wuerfel, J., Banwell, B. L., Villoslada, P., Saiz, A., Fujihara, K., Kim, S.-H., With The Guthy-Jackson Charitable Foundation NMO International Clinical Consortium & Biorepository, Paul, F., Wuerfel, J., Cabre, P., Marignier, R., de Seze, J., Tedder, T., Garrahan, J. P., Tenembaum, S., van Pelt, D., Broadley, S., Saiz, A., Villoslada, P., Levy, M., Chitnis, T., Klawiter, E. C., Wingerchuk, D., Kim, H. J., Pandit, L., Kister, I., Leite, M. I., Palace, J., Apiwattanakul, M., Kleiter, I., Prayoonwiwat, N., Han, M., Hellwig, K., Banwell, B., van Herle, K., Behne, J., John, G., Hooper, D. C., Fujihara, K., Nakashima, I., Sato, D., Traboulsee, A., Yeaman, M. R., Waubant, E., Zamvil, S., Bennett, J., Lana-Peixoto, M., Greenberg, B., Stuve, O., Aktas, O., Wuerfel, J., Smith, T. J., Asgari, N., Jacob, A., & O'Connor, K.
- Affiliations
- From the Department of Neurology (H.J.K., S.-H.K.), Research Institute and Hospital of National Cancer Center, Goyang, Korea; NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center (F.P., J.W.), Department of Neurology, Charité University Medicine, Berlin, Germany; CIEM MS Research Center (M.A.L.-P.), Federal University of Minas Gerais Medical School, Belo Horizonte, Brazil; Department of Neurology (S.T.), National Paediatric Hospital Dr. Juan P. Garrahan...
- Universidade Federal de Minas Gerais (BR)
- ORCID
- Marignier, Romain
- Tedder, Thomas
- Garrahan, Juan P.
- Broadley, Simon
- Levy, Michael
- Chitnis, Tanuja
- Pandit, Lekha
- Kister, Ilya
- Leite, Maria Isabel
- Apiwattanakul, Metha
- Kleiter, Ingo
- Prayoonwiwat, Naraporn
- Han, May
- Hellwig, Kerstin
- Hooper, D. Craig
- Nakashima, Ichiro
- Traboulsee, Anthony
- Yeaman, Michael R.
- Waubant, Emmanuelle
- Zamvil, Scott
- Bennett, Jeffrey
- Greenberg, Benjamin
- Smith, Terry J.
- Jacob, Anu
- Venue
- Neurology
- Published
- 2015-03-17
- Volume
- 84
- Issue
- 11
- Pages
- 1165-1173
- ISSN
- 0028-3878 (ISSN-L)
- 1526-632X
- PMC
- PMC4371410
- OpenAlex
- W2151890979
- Cited by
- 664
- References
- 3
- Citations by year
- 2026: 39 · 2025: 49 · 2024: 59 · 2023: 47 · 2022: 60 · 2021: 73 · 2020: 68 · 2019: 67 · 2018: 60 · 2017: 52 · 2016: 69 · 2015: 17
- FWCI
- 9.67
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Neurology (publishedVersion)
- Europe PMC (PubMed Central) (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- HAL (submittedVersion)
- University of Southern Denmark Research Portal (University of Southern Denmark) (submittedVersion)
- Funders
- Guthy-Jackson Charitable Foundation
- Topics
- Health Sciences › Medicine › Clinical Neurology › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Clinical Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Ophthalmology › Ocular Diseases and Behçet’s Syndrome
- Keywords
- neuromyelitis optica spectrum disorder, neuromyelitis optica, aquaporin-4 antibodies, brain MRI abnormalities, optic nerve involvement, spinal cord involvement, differential diagnosis from multiple sclerosis, immunosuppressive therapy
- MeSH terms
- Animals, Brain, Diagnosis, Differential, Humans, Magnetic Resonance Imaging, Multiple Sclerosis, Neuromyelitis Optica, Spinal Cord, Internationality
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