Cited by 161Multiple Sclerosis Research StudiesPeripheral Neuropathies and DisordersAutoimmune Neurological Disorders and Treatments

Atypical inflammatory demyelinating syndromes of the CNS

Hardy, Todd A, Reddel, Stephen W, Barnett, Michael H, Palace, Jacqueline, Lucchinetti, Claudia F, Weinshenker, Brian G·2016·The Lancet Neurology

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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  1. [66]66. Reindl M, Di Pauli F, Rostásy K, Berger T. The spectrum of MOG autoantibodyassociated demyelinating diseases. Nat Rev Neurol. 2013 Aug;9(8):455-61.

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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 › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
  • Health Sciences › Medicine › Neurology › Peripheral Neuropathies and Disorders
  • Health Sciences › Medicine › 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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