Neuromyelitis Spectrum Disorders
Brian G. Weinshenker,Dean M. Wingerchuk +1 more
- Vol. 92, Iss: 4, pp 663-679
Reads0
Chats0
TLDR
Current understanding of the clinical aspects, pathophysiology, and treatment of NMOSD is reviewed.Abstract:
The understanding of neuromyelitis optica spectrum disorder (NMOSD) has evolved substantially since its initial description over a century ago. The discovery in 2004 of a pathogenic autoantibody biomarker targeting aquaporin 4 IgG revolutionized diagnosis and therapeutic development. Although NMOSD resembles multiple sclerosis (MS), differences were identified and articulated in the late 1990s. New diagnostic criteria incorporating the biomarker as well as better understanding of the clinical and radiologic features of NMOSD now permit accurate diagnosis and differentiation from MS. Aquaporin 4 IgG-associated NMOSD is now regarded as an immune astrocytopathy with lytic and nonlytic effects on astrocytes. A second autoantibody, myelin oligodendrocyte glycoprotein IgG, which targets myelin rather than astrocytes, leads to an NMOSD syndrome with clinical and radiologic features that overlap but are distinct from those of aquaporin 4 IgG-associated NMOSD and MS. We review current understanding of the clinical aspects, pathophysiology, and treatment of NMOSD.read more
Citations
More filters
Journal ArticleDOI
Eculizumab in Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder
Sean J. Pittock,Achim Berthele,Kazuo Fujihara,Kazuo Fujihara,Ho Jin Kim,Michael J. Levy,Michael J. Levy,Jacqueline Palace,Ichiro Nakashima,Ichiro Nakashima,Murat Terzi,N. A. Totolyan,Shanthi Viswanathan,Kai-Chen Wang,A Pace,Kenji P Fujita,R Armstrong,Dean M. Wingerchuk +17 more
TL;DR: Patients with AQP4-IgG-positive NMOSD who received eculizumab had a significantly lower risk of relapse than those who received placebo, and there was no significant between-group difference in measures of disability progression.
Journal ArticleDOI
Myelin in the Central Nervous System: Structure, Function, and Pathology.
TL;DR: The biology of myelin, the expanded relationship of myelinating oligodendrocytes with its underlying axons and the neighboring cells, and its disturbances in various diseases such as multiple sclerosis, acute disseminated encephalomyelitis, and neuromyELitis optica spectrum disorders are reviewed.
Journal ArticleDOI
Association of MOG-IgG Serostatus With Relapse After Acute Disseminated Encephalomyelitis and Proposed Diagnostic Criteria for MOG-IgG–Associated Disorders
A. Sebastian Lopez-Chiriboga,Masoud Majed,James P. Fryer,Divyanshu Dubey,Andrew McKeon,Eoin P. Flanagan,Jiraporn Jitprapaikulsan,Naga Kothapalli,Jan Mendelt Tillema,John J. Chen,Brian G. Weinshenker,Dean M. Wingerchuk,Jessica Sagen,Avi Gadoth,Vanda A. Lennon,B. Mark Keegan,Claudia F. Lucchinetti,Sean J. Pittock +17 more
TL;DR: The data extend the clinical utility of Mog-IgG1 serological testing to adult patients and highlights that longitudinal serologic evaluation of MOG-IGG1 could help predict disease course and consideration of immunotherapy.
Journal ArticleDOI
Safety and efficacy of satralizumab monotherapy in neuromyelitis optica spectrum disorder: a randomised, double-blind, multicentre, placebo-controlled phase 3 trial
Anthony Traboulsee,Benjamin Greenberg,Jeffrey Bennett,Lech Szczechowski,Edward Fox,Svitlana Shkrobot,Takashi Yamamura,Y Terada,Yuichi Kawata,Padraig Wright,Athos Gianella-Borradori,Hideki Garren,Brian G. Weinshenker +12 more
TL;DR: Satralizumab monotherapy reduced the rate of NMOSD relapse compared with placebo in the overall trial population, with a favourable safety profile.
Journal ArticleDOI
Diagnosis and treatment of NMO spectrum disorder and MOG-encephalomyelitis
TL;DR: Recommendations include antibody and further laboratory testing, MR imaging and optical coherence tomography, as well as longterm immunosuppressive treatment, including azathioprine, rituximab, and immunoglobulins.
References
More filters
Journal ArticleDOI
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
TL;DR: 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.
Journal ArticleDOI
A serum autoantibody marker of neuromyelitis optica : distinction from multiple sclerosis
Vanda A. Lennon,Dean M. Wingerchuk,Thomas J. Kryzer,Sean J. Pittock,C. F. Lucchinetti,Kazuo Fujihara,Ichiro Nakashima,Brian G. Weinshenker +7 more
TL;DR: NMO-IgG is a specific marker autoantibody of neuromyelitis optica and binds at or near the blood-brain barrier that distinguishes neuromyleitis opticas from multiple sclerosis.
Journal ArticleDOI
Revised diagnostic criteria for neuromyelitis optica
TL;DR: Revised diagnostic criteria for definite neuromyelitis optica (NMO) that require optic neuritis, myelitis, and at least two of three supportive criteria: MRI evidence of a contiguous spinal cord lesion 3 or more segments in length, onset brain MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity.
Journal ArticleDOI
IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel.
TL;DR: It is shown that NMO-IgG binds selectively to the aquaporin-4 water channel, a component of the dystroglycan protein complex located in astrocytic foot processes at the blood-brain barrier, which may represent the first example of a novel class of autoimmune channelopathy.
Journal ArticleDOI
The spectrum of neuromyelitis optica
Dean M. Wingerchuk,Vanda A. Lennon,Claudia F. Lucchinetti,Sean J. Pittock,Brian G. Weinshenker +4 more
TL;DR: Data suggest that autoantibodies to aquaporin 4 derived from peripheral B cells cause the activation of complement, inflammatory demyelination, and necrosis that is seen in neuromyelitis optica.
Related Papers (5)
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