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Atypical inflammatory demyelinating syndromes of the CNS
Abstract
Atypical inflammatory demyelinating syndromes are rare disorders that differ from multiple sclerosis owing to unusual clinical or MRI findings or poor response to treatments used for multiple sclerosis. These syndromes include neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Baló's concentric sclerosis, Schilder's disease, and Marburg's multiple sclerosis. The overlapping features of these syndromes with multiple sclerosis and with each other complicate diagnosis and their categorisation as distinct or related conditions. Recognition of these syndromes is crucial because they differ from multiple sclerosis and other demyelinating and non-demyelinating conditions in their prognosis and treatment. Advances in MRI, pathology, and immunobiology are needed to increase understanding of these syndromes, including the extent to which some of them represent distinct entities, and to assist with improvements in their diagnosis and management.
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“Atypical demyelinating lesions are also seen in NMOSD and ADEM.41”
From Diagnosis of multiple sclerosis: progress and challenges (2017) · cites this paper as [41]
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Cites
International consensus diagnostic criteria for neuromyelitis optica spectrum disorders
Wingerchuk, D. M., Banwell, B., Bennett, J. L., Cabre, P., Carroll, W., Chitnis, T., de Seze, J., Fujihara, K., Greenberg, B., Jacob, A., et al. (2015) · Neurology · doi:10.1212/wnl.0000000000001729 · Cited by 4,649
Self-citationA serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis
Lennon, V. A., Wingerchuk, D. M., Kryzer, T. J., Pittock, S. J., Lucchinetti, C. F., Fujihara, K., Nakashima, I., & Weinshenker, B. G. (2004) · The Lancet · doi:10.1016/s0140-6736(04)17551-x · Cited by 3,239
Self-citationRevised diagnostic criteria for neuromyelitis optica
Wingerchuk, D. M., Lennon, V. A., Pittock, S. J., Lucchinetti, C. F., & Weinshenker, B. G. (2006) · Neurology · doi:10.1212/01.wnl.0000216139.44259.74 · Cited by 2,568
Self-citationThe spectrum of neuromyelitis optica
Wingerchuk, D. M., Lennon, V. A., Lucchinetti, C. F., Pittock, S. J., & Weinshenker, B. G. (2007) · The Lancet Neurology · doi:10.1016/s1474-4422(07)70216-8 · Cited by 2,182
Self-citationA role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica.
Lucchinetti, C., Mandler, R., McGavern, D., Brück, W., Gleich, G., Ransohoff, R., Trebst, C., Weinshenker, B., Wingerchuk, D., Parisi, J., et al. (2002) · Brain : a journal of neurology · doi:10.1093/brain/awf151 · Cited by 1,228
Self-citationKrupp, L. B., Tardieu, M., Amato, M. P., Banwell, B., Chitnis, T., Dale, R. C., Ghezzi, A., Hintzen, R., Kornberg, A., Pohl, D., et al. (2013) · Multiple Sclerosis Journal · doi:10.1177/1352458513484547 · Cited by 1,130
MRI criteria for the diagnosis of multiple sclerosis: MAGNIMS consensus guidelines
Filippi, M., Rocca, M. A., Ciccarelli, O., De Stefano, N., Evangelou, N., Kappos, L., Rovira, A., Sastre-Garriga, J., Tintorè, M., Frederiksen, J. L., et al. (2016) · The Lancet Neurology · doi:10.1016/s1474-4422(15)00393-2 · Cited by 880
Self-citationDistinction 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
Consensus definitions proposed for pediatric multiple sclerosis and related disorders
Krupp, L., Banwell, B., & Tenembaum, S. N. (2007) · Neurology · doi:10.1212/01.wnl.0000259422.44235.a8 · Cited by 757
Roemer, S. F., Parisi, J. E., Lennon, V. A., Benarroch, E. E., Lassmann, H., Brück, W., Mandler, R. N., Weinshenker, B. G., Pittock, S. J., Wingerchuk, D. M., et al. (2007) · Brain · doi:10.1093/brain/awl371 · Cited by 716
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Research works printed but not linked: 1
- Marburg, 1906, Die sogenannte “akute Multiple Sklerose.”, J Psychiatr Neurol, 27, 211
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Myelin in the Central Nervous System: Structure, Function, and Pathology
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Diagnosis of multiple sclerosis: progress and challenges
Brownlee, W. J., Hardy, T. A., Fazekas, F., & Miller, D. H. (2017) · The Lancet · doi:10.1016/s0140-6736(16)30959-x · Cited by 701
Self-citationMyelin oligodendrocyte glycoprotein antibodies in neurological disease
Reindl, M., & Waters, P. J. (2018) · Nature Reviews Neurology · doi:10.1038/s41582-018-0112-x · Cited by 646
Microglia in multiple sclerosis: Friend or foe
Guerrero, B. L., & Sicotte, N. L. (2020) · Frontiers in Immunology · doi:10.3389/fimmu.2020.00374 · Cited by 215
Myelin oligodendrocyte glycoprotein antibodies: How clinically useful are they?
Reindl, M., Jarius, S., Rostasy, K. M., & Berger, T. (2017) · Current Opinion in Neurology · doi:10.1097/wco.0000000000000446 · Cited by 100
Metadata
- Authors
- Hardy, T. A., Reddel, S. W., Barnett, M. H., Palace, J., Lucchinetti, C. F., & Weinshenker, B. G.
- Affiliations
- The University of Sydney (AU)
- John Radcliffe Hospital (GB)
- Mayo Clinic (US)
- Corresponding author
- Hardy, Todd A
- Venue
- The Lancet Neurology
- Publisher
- Elsevier BV
- Published
- 2016-08
- Volume
- 15
- Issue
- 9
- Pages
- 967-981
- ISSN
- 1474-4422 (ISSN-L)
- OpenAlex
- W2467089182
- Cited by
- 161
- References
- 130
- Citations by year
- 2026: 10 · 2025: 10 · 2024: 19 · 2023: 9 · 2022: 19 · 2021: 27 · 2020: 17 · 2019: 19 · 2018: 15 · 2017: 12 · 2016: 4
- FWCI
- 2.62
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Lancet Neurology (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Multiple Sclerosis Research Studies
- Health Sciences › Medicine › Clinical Neurology › Peripheral Neuropathies and Disorders
- Health Sciences › Medicine › Clinical Neurology › Autoimmune Neurological Disorders and Treatments
- Keywords
- neuromyelitis optica spectrum disorder, acute disseminated encephalomyelitis, tumefactive demyelination, Balo's concentric sclerosis, Schilder's disease, multiple sclerosis differential diagnosis, demyelination, MRI findings, immunobiology
- MeSH terms
- Brain, Central Nervous System Diseases, Demyelinating Diseases, Humans, Immunoglobulin G, Magnetic Resonance Imaging, Aquaporin 4
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