International consensus diagnostic criteria for neuromyelitis optica spectrum disorders
Dean M. Wingerchuk,Brenda Banwell,Jeffrey Bennett,Philippe Cabre,William M. Carroll,Tanuja Chitnis,Jérôme De Seze,Kazuo Fujihara,Benjamin Greenberg,Anu Jacob,Sven Jarius,Marco Aurélio Lana-Peixoto,Michael J. Levy,Jack H. Simon,Silvia Tenembaum,Anthony Traboulsee,Patrick Waters,Kay E. Wellik,Brian G. Weinshenker +18 more
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TLDR
The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasicNMOSD and opticospinal MS.Abstract:
Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.read more
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MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome
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TL;DR: The predominantly relapsing and often severe disease course and the short median time to second attack support the use of prophylactic long-term treatments in patients with MOG-IgG-positive ON and/or myelitis; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal.
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International consensus diagnostic criteria for neuromyelitis optica spectrum disordersAuthor Response
TL;DR: Criteria for neuromyelitis optica (NMO) spectrum disorders (NMOSD) was proposed that incorporated unifying the terms NMO and NMOSD, including patients with aquaporin-4 (AQP4)–immunoglobulin G (IgG) negative/unknown, thus giving these patients …
References
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Journal ArticleDOI
Myelin-oligodendrocyte glycoprotein antibodies in adults with a neuromyelitis optica phenotype
J Kitley,M Woodhall,Patrick Waters,Maria Isabel Leite,E Devenney,J Craig,Jacqueline Palace,Angela Vincent +7 more
TL;DR: 4 patients with an NMO/NMOSD phenotype who had antibodies to MOG appear to have more favorable clinical outcomes than those with typical AQP4 antibody–mediated disease, and MOG antibody titers fell in all 4 patients.
Journal ArticleDOI
Antibody to aquaporin-4 in the Long-Term Course of Neuromyelitis Optica
Sven Jarius,F Aboul-Enein,Patrick Waters,Bettina Kuenz,A Hauser,Thomas Berger,Wilfried Lang,Markus Reindl,Angela Vincent,W Kristoferitsch +9 more
TL;DR: A strong relationship between AQP4-Abs and clinical state is demonstrated, and the hypothesis that these antibodies are involved in the pathogenesis of NMO is supported.
Journal ArticleDOI
NMO-IgG predicts the outcome of recurrent optic neuritis
Marcelo Matiello,Vanda A. Lennon,Anu Jacob,Sean J. Pittock,Claudia F. Lucchinetti,Dean M. Wingerchuk,Brian G. Weinshenker +6 more
TL;DR: Neuromyelitis optica (NMO)–immunoglobulin G seropositivity predicts poor visual outcome and development of NMO and seropositive recurrent optic neuritis is a limited form of N MO.
Journal ArticleDOI
CNS aquaporin-4 autoimmunity in children
Andrew McKeon,Vanda A. Lennon,Timothy Lotze,S. Tenenbaum,Jayne Ness,Mary Rensel,Nancy L. Kuntz,J. P. Fryer,Henry A. Homburger,Jill V. Hunter,Brian G. Weinshenker,Karl N. Krecke,Claudia F. Lucchinetti,Sean J. Pittock +13 more
TL;DR: The neurologic, serologic, and radiographic findings associated with CNS AQP4 autoimmunity in childhood are described, indicating a distinctive recurrent and widespread inflammatory CNS disease in children.
Journal ArticleDOI
The pathology of an autoimmune astrocytopathy: lessons learned from neuromyelitis optica.
Claudia F. Lucchinetti,Yong Guo,Bogdan F. Gh. Popescu,Bogdan F. Gh. Popescu,Kazuo Fujihara,Yasuto Itoyama,Tatsuro Misu +6 more
TL;DR: Neuromyelitis optica is a disabling autoimmune astrocytopathy characterized by typically severe and recurrent attacks of optic neuritis and longitudinally extensive myelitis and recent neuropathological studies provide novel insights into the pathogenic mechanisms, cellular targets, as well as the spectrum of tissue damage in NMO.
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