TY - JOUR
T1 - Atypical inflammatory demyelinating syndromes of the CNS
AU - Hardy, Todd A.
AU - Reddel, Stephen W.
AU - Barnett, Michael H.
AU - Palace, Jacqueline
AU - Lucchinetti, Claudia F.
AU - Weinshenker, Brian G.
N1 - Funding Information:
We searched PubMed with the search terms “multiple sclerosis”, “atypical demyelination”, “neuromyelitis optica spectrum disorder”, “neuromyelitis optica”, “NMO”, “NMOSD”, “aquaporin-4”, “myelin oligodendrocyte glycoprotein”, “MOG”, “tumefactive demyelination”, “Balo”, “Balo's”, “Baló”, “concentric”, “Schilder”, “Schilder's”, “Marburg”, and “Marburg's” for articles published between Jan 1, 2011, and March 31, 2016. We also searched the reference lists of retrieved articles, Google Scholar, and the authors' own files. We applied no language restrictions. The final reference list was generated on the basis of originality and relevance to the scope of this Review. Contributors TAH conceived the idea for the article and TAH and BGW drafted the Review. All authors read and revised the drafts for intellectual content and approved the final version. Declaration of interests TAH has received honoraria for speaking and participation on advisory boards, and support for attendance of scientific meetings from Alexion, Biogen Idec, Genzyme, and Merck Serono. SWR has received honoraria for speaking and participation on advisory boards, and support for attendance of scientific meetings from Bayer-Schering, Biogen Idec, Genzyme, Merck Serono, and Novartis, and is a director of Medical Safety Systems, which has provided IT services to Genzyme to monitor the safety of patients with multiple sclerosis treated with alemtuzumab; these services are not related to the content of this paper. MHB has received institutional support from Biogen, Genzyme, Novartis, and Teva, and travel support from Biogen and Novartis. JP receives part funding from NHS England highly specialised services to run a national congenital myasthenia service and a neuromyelitis service. She has received support for attendance of scientific meetings and has received honoraria for advisory work from Alexion, Bayer Schering, Biogen Idec, Chugai, MedImmune, Merck Serono, Novartis, and Teva, and has received unrestricted grants from Biogen Idec, Genzyme, Merck Serono, the MS Society, Novartis, and Teva. Her hospital trust receives funding for her role as clinical lead for the multiple sclerosis risk sharing scheme, and she has received grants from the Guthie Jackson Foundation and the MS Society for unrelated research studies. She is a board member for the charitable European Charcot Foundation, and is on the steering committee for the Magnetic Resonance Imaging in MS collaboration. CFL has a special project agreement to support some of her research with Biogen and Novartis. BGW receives royalties from RSR and Oxford University for technology license for aquaporin-4 autoantibodies used in the diagnosis of neuromyelitis optica spectrum disorders. He serves on data safety monitoring committees for Biogen Idec, Mitsubishi, and Novartis, and serves on an adjudication panel for Medimmune Pharmaceuticals. He has served as a consultant for Alexion, Chord, Chugai, Elan, GlaxoSmithKline, Novartis, and Ono.
Publisher Copyright:
© 2016 Elsevier Ltd
PY - 2016/8/1
Y1 - 2016/8/1
N2 - 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.
AB - 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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U2 - 10.1016/S1474-4422(16)30043-6
DO - 10.1016/S1474-4422(16)30043-6
M3 - Review article
C2 - 27478954
AN - SCOPUS:84992184044
VL - 15
SP - 967
EP - 981
JO - The Lancet Neurology
JF - The Lancet Neurology
SN - 1474-4422
IS - 9
ER -