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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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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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