scispace - formally typeset

Journal ArticleDOI

International consensus diagnostic criteria for neuromyelitis optica spectrum disorders

14 Jul 2015-Neurology (Lippincott Williams and Wilkins)-Vol. 85, Iss: 2, pp 177-189

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.
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.
Citations
More filters

Journal ArticleDOI
21 Dec 2017-Lancet Neurology
TL;DR: The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical clinically isolated syndrome, define what is needed to fulfil dissemination in time and space of lesions in the CNS, and stress the need for no better explanation for the presentation.
Abstract: The 2010 McDonald criteria for the diagnosis of multiple sclerosis are widely used in research and clinical practice. Scientific advances in the past 7 years suggest that they might no longer provide the most up-to-date guidance for clinicians and researchers. The International Panel on Diagnosis of Multiple Sclerosis reviewed the 2010 McDonald criteria and recommended revisions. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical clinically isolated syndrome, define what is needed to fulfil dissemination in time and space of lesions in the CNS, and stress the need for no better explanation for the presentation. The following changes were made: in patients with a typical clinically isolated syndrome and clinical or MRI demonstration of dissemination in space, the presence of CSF-specific oligoclonal bands allows a diagnosis of multiple sclerosis; symptomatic lesions can be used to demonstrate dissemination in space or time in patients with supratentorial, infratentorial, or spinal cord syndrome; and cortical lesions can be used to demonstrate dissemination in space. Research to further refine the criteria should focus on optic nerve involvement, validation in diverse populations, and incorporation of advanced imaging, neurophysiological, and body fluid markers.

2,258 citations


Journal ArticleDOI
TL;DR: The Eighth Edition of the JCA Special Issue seeks to continue to serve as a key resource that guides the utilization of TA in the treatment of human disease.
Abstract: The American Society for Apheresis (ASFA) Journal of Clinical Apheresis (JCA) Special Issue Writing Committee is charged with reviewing, updating, and categorizing indications for the evidence-based use of therapeutic apheresis in human disease. Since the 2007 JCA Special Issue (Fourth Edition), the Committee has incorporated systematic review and evidence-based approaches in the grading and categorization of apheresis indications. This Seventh Edition of the JCA Special Issue continues to maintain this methodology and rigor to make recommendations on the use of apheresis in a wide variety of diseases/conditions. The JCA Seventh Edition, like its predecessor, has consistently applied the category and grading system definitions in the fact sheets. The general layout and concept of a fact sheet that was used since the fourth edition has largely been maintained in this edition. Each fact sheet succinctly summarizes the evidence for the use of therapeutic apheresis in a specific disease entity. The Seventh Edition discusses 87 fact sheets (14 new fact sheets since the Sixth Edition) for therapeutic apheresis diseases and medical conditions, with 179 indications, which are separately graded and categorized within the listed fact sheets. Several diseases that are Category IV which have been described in detail in previous editions and do not have significant new evidence since the last publication are summarized in a separate table. The Seventh Edition of the JCA Special Issue serves as a key resource that guides the utilization of therapeutic apheresis in the treatment of human disease. J. Clin. Apheresis 31:149-162, 2016. © 2016 Wiley Periodicals, Inc.

1,261 citations


Journal ArticleDOI
Massimo Filippi1, Maria A. Rocca1, Olga Ciccarelli2, Olga Ciccarelli3  +13 moreInstitutions (12)
01 Mar 2016-Lancet Neurology
TL;DR: State-of-the-art MRI findings in patients presenting with a clinically isolated syndrome were discussed in a MAGNIMS workshop, the goal of which was to provide an evidence-based and expert-opinion consensus on diagnostic MRI criteria modifications.
Abstract: In patients presenting with a clinically isolated syndrome, MRI can support and substitute clinical information in the diagnosis of multiple sclerosis by showing disease dissemination in space and time and by helping to exclude disorders that can mimic multiple sclerosis. MRI criteria were first included in the diagnostic work-up for multiple sclerosis in 2001, and since then several modifications to the criteria have been proposed in an attempt to simplify lesion-count models for showing disease dissemination in space, change the timing of MRI scanning to show dissemination in time, and increase the value of spinal cord imaging. Since the last update of these criteria, new data on the use of MRI to establish dissemination in space and time have become available, and MRI technology has improved. State-of-the-art MRI findings in these patients were discussed in a MAGNIMS workshop, the goal of which was to provide an evidence-based and expert-opinion consensus on proposed modifications to MRI criteria for the diagnosis of multiple sclerosis.

518 citations


Journal ArticleDOI
Sven Jarius1, Klemens Ruprecht2, Ingo Kleiter3, Nadja Borisow2  +26 moreInstitutions (9)
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.
Abstract: A subset of patients with neuromyelitis optica spectrum disorders (NMOSD) has been shown to be seropositive for myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) To describe the epidemiological, clinical, radiological, cerebrospinal fluid (CSF), and electrophysiological features of a large cohort of MOG-IgG-positive patients with optic neuritis (ON) and/or myelitis (n = 50) as well as attack and long-term treatment outcomes Retrospective multicenter study The sex ratio was 1:28 (m:f) Median age at onset was 31 years (range 6-70) The disease followed a multiphasic course in 80% (median time-to-first-relapse 5 months; annualized relapse rate 092) and resulted in significant disability in 40% (mean follow-up 75 ± 465 months), with severe visual impairment or functional blindness (36%) and markedly impaired ambulation due to paresis or ataxia (25%) as the most common long-term sequelae Functional blindness in one or both eyes was noted during at least one ON attack in around 70% Perioptic enhancement was present in several patients Besides acute tetra-/paraparesis, dysesthesia and pain were common in acute myelitis (70%) Longitudinally extensive spinal cord lesions were frequent, but short lesions occurred at least once in 44% Fourty-one percent had a history of simultaneous ON and myelitis Clinical or radiological involvement of the brain, brainstem, or cerebellum was present in 50%; extra-opticospinal symptoms included intractable nausea and vomiting and respiratory insufficiency (fatal in one) CSF pleocytosis (partly neutrophilic) was present in 70%, oligoclonal bands in only 13%, and blood-CSF-barrier dysfunction in 32% Intravenous methylprednisolone (IVMP) and long-term immunosuppression were often effective; however, treatment failure leading to rapid accumulation of disability was noted in many patients as well as flare-ups after steroid withdrawal Full recovery was achieved by plasma exchange in some cases, including after IVMP failure Breakthrough attacks under azathioprine were linked to the drug-specific latency period and a lack of cotreatment with oral steroids Methotrexate was effective in 5/6 patients Interferon-beta was associated with ongoing or increasing disease activity Rituximab and ofatumumab were effective in some patients However, treatment with rituximab was followed by early relapses in several cases; end-of-dose relapses occurred 9-12 months after the first infusion Coexisting autoimmunity was rare (9%) Wingerchuk’s 2006 and 2015 criteria for NMO(SD) and Barkhof and McDonald criteria for multiple sclerosis (MS) were met by 28%, 32%, 15%, 33%, respectively; MS had been suspected in 36% Disease onset or relapses were preceded by infection, vaccination, or pregnancy/delivery in several cases Our findings from a predominantly Caucasian cohort strongly argue against the concept of MOG-IgG denoting a mild and usually monophasic variant of NMOSD 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

494 citations


Cites background or methods from "International consensus diagnostic ..."

  • ...longitudinally extensive (more than half the length of the corpus callosum), as considered typical for AQP4IgG-positive NMOSD [29], in 1/8 (12....

    [...]

  • ...%) patients met the 2015 international consensus criteria for NMOSD [29] (Table 1)....

    [...]

  • ...Both LEON lesions and chiasmatic lesions were previously thought to be indicative of (AQP4-IgG-positive) NMOSD [29]....

    [...]

  • ...Other suspected diagnoses included acute disseminated EM (ADEM), multiphasic disseminated EM, AQP4-IgG-negative NMO according to Wingerchuk’s 2006 criteria [28], AQP4-IgGnegative NMOSD according to the 2015 international diagnostic consensus criteria [29], viral encephalitis, bacterial encephalitis, paraneoplastic encephalitis, isolated vasculitis of the CNS, chronic relapsing inflammatory optic neuropathy (CRION), CNS lymphoma, sarcoidosis, spinal stenosis, “spinal tumor of unknown dignity”, suspected spinal ischemia, para- or postinfectious ON, and myelitis; some patients were diagnosed with ON, rON, (longitudinally extensive transverse) myelitis, brainstem encephalitis or EM “of unknown origin”....

    [...]

  • ...Keywords: Myelin oligodendrocyte glycoprotein antibodies (MOG-IgG), Autoantibodies, Neuromyelitis optica spectrum disorders (NMOSD), Aquaporin-4 antibodies (AQP4-IgG, NMO-IgG), Optic neuritis, Transverse myelitis, Longitudinally extensive transverse myelitis, Magnetic resonance imaging, Cerebrospinal fluid, Oligoclonal bands, Electrophysiology, Evoked potentials, Treatment, Therapy, Methotrexate, Azathioprine, Rituximab, Ofatumumab, Interferon beta, Glatiramer acetate, Natalizumab, Outcome, Pregnancy, Infections, Vaccination, Multiple sclerosis, Barkhof criteria, McDonald criteria, Wingerchuk criteria 2006 and 2015, IPND criteria, International consensus diagnostic criteria for neuromyelitis optica spectrum disorders...

    [...]


Journal ArticleDOI
Chong Tin Tan, Zhifeng Mao, Dean M. Wingerchuk, Wei Qiu  +2 moreInstitutions (1)
02 Feb 2016-Neurology
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 …
Abstract: Editors' Note: The international consensus diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD) article prompted reactions from our readers. Tan is concerned that the new criteria may include patients without true neuromyelitis optica and may affect treatment funding. Recounting their own experience, Mao et al. caution about overinterpretation of MRI results and suggest that aquaporin-4 antibody (AQP4-Ab) testing be considered in late-onset demyelinating disease. In response, Wingerchuk et al., authors of the guidelines, stress that the diagnostic category of NMOSD without AQP4–immunoglobulin G requires exclusion of alternative diagnoses, believe that the new criteria will facilitate—rather than hinder—insurance coverage for appropriate immunosuppressive treatments, and agree with the observations raised by Mao et al. —Chafic Karam, MD, and Robert C. Griggs, MD Wingerchuk et al.1 proposed criteria for neuromyelitis optica (NMO) spectrum disorders (NMOSD) that incorporated unifying the terms NMO and NMOSD, including patients with aquaporin-4 (AQP4)–immunoglobulin G (IgG) negative/unknown, thus giving these patients …

476 citations


References
More filters

Journal ArticleDOI
11 Dec 2004-The Lancet
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.
Abstract: Methods Indirect immunofluorescence with a composite substrate of mouse tissues identified a distinctive NMO-IgG staining pattern, which we characterised further by dual immunostaining. We tested masked serum samples from 102 North American patients with neuromyelitis optica or with syndromes that suggest high risk of the disorder, and 12 Japanese patients with optic-spinal multiple sclerosis. Control patients had multiple sclerosis, other myelopathies, optic neuropathies, and miscellaneous disorders. We also established clinical diagnoses for 14 patients incidentally shown to have NMO-IgG among 85 000 tested for suspected paraneoplastic autoimmunity. Findings NMO-IgG outlines CNS microvessels, pia, subpia, and Virchow-Robin space. It partly colocalises with laminin. Sensitivity and specificity were 73% (95% CI 60–86) and 91% (79–100) for neuromyelitis optica and 58% (30–86) and 100% (66–100) for optic-spinal multiple sclerosis. NMO-IgG was detected in half of patients with high-risk syndromes. Of 14 seropositive cases identified incidentally, 12 had neuromyelitis optica or a high-risk syndrome for the disease.

2,519 citations


Journal ArticleDOI
23 May 2006-Neurology
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.
Abstract: Background: The authors previously proposed diagnostic criteria for neuromyelitis optica (NMO) that facilitate its distinction from prototypic multiple sclerosis (MS) However, some patients with otherwise typical NMO have additional symptoms not attributable to optic nerve or spinal cord inflammation or have MS-like brain MRI lesions Furthermore, some patients are misclassified as NMO by the authors’ earlier proposed criteria despite having a subsequent course indistinguishable from prototypic MS A serum autoantibody marker, NMO-IgG, is highly specific for NMO The authors propose revised NMO diagnostic criteria that incorporate NMO-IgG status Methods: Using final clinical diagnosis (NMO or MS) as the reference standard, the authors calculated sensitivity and specificity for each criterion and various combinations using a sample of 96 patients with NMO and 33 with MS The authors used likelihood ratios and logistic regression analysis to develop the most practical and informative diagnostic model Results: Fourteen patients with NMO (146%) had extra-optic-spinal CNS symptoms NMO-IgG seropositivity was 76% sensitive and 94% specific for NMO The best diagnostic combination was 99% sensitive and 90% specific for NMO and consisted of at least two of three elements: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity Conclusions: The authors propose 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 CNS involvement beyond the optic nerves and spinal cord is compatible with NMO

2,327 citations


Journal ArticleDOI
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.
Abstract: Neuromyelitis optica (NMO) is an inflammatory demyelinating disease that selectively affects optic nerves and spinal cord. It is considered a severe variant of multiple sclerosis (MS), and frequently is misdiagnosed as MS, but prognosis and optimal treatments differ. A serum immunoglobulin G autoantibody (NMO-IgG) serves as a specific marker for NMO. Here we show 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. NMO may represent the first example of a novel class of autoimmune channelopathy.

1,868 citations


Journal ArticleDOI
01 Sep 2007-Lancet Neurology
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.
Abstract: Summary Neuromyelitis optica (also known as Devic's disease) is an idiopathic, severe, demyelinating disease of the central nervous system that preferentially affects the optic nerve and spinal cord. Neuromyelitis optica has a worldwide distribution, poor prognosis, and has long been thought of as a variant of multiple sclerosis; however, clinical, laboratory, immunological, and pathological characteristics that distinguish it from multiple sclerosis are now recognised. The presence of a highly specific serum autoantibody marker (NMO-IgG) further differentiates neuromyelitis optica from multiple sclerosis and has helped to define a neuromyelitis optica spectrum of disorders. NMO-IgG reacts with the water channel aquaporin 4. 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. The knowledge gained from further assessment of the exact role of NMO-IgG in the pathogenesis of neuromyelitis optica will provide a foundation for rational therapeutic trials for this rapidly disabling disease.

1,756 citations


"International consensus diagnostic ..." refers background in this paper

  • ...Neuromyelitis optica (NMO) is an inflammatory CNS disorder distinct from multiple sclerosis (MS).(1,2) It became known as Devic disease following a seminal 1894 report....

    [...]

  • ..., first-attack LETM or recurrent or bilateral optic neuritis) who were at high risk for future attacks.(1) The NMOSD term also encompassed the cerebral, diencephalic, and brainstem lesions that occur in a minority of patients with otherwise typical NMO....

    [...]


Journal ArticleDOI
01 Sep 1999-Neurology
TL;DR: Clinical, laboratory, and imaging features generally distinguish neuromyelitis optica from MS, and patients with relapsing optic neuritis and myelitis may have neuromyeliitis opticas rather than MS.
Abstract: Objectives: To evaluate the spectrum of neuromyelitis optica (NMO), including characteristics of the index events (optic neuritis [ON]) and myelitis), neuroimaging, CSF, and serologic studies, and to evaluate the long-term course. Methods: Review of 71 patients with NMO evaluated at the Mayo Clinic between 1950 and 1997. Results: NMO was either monophasic or relapsing. Patients with a monophasic course (n = 23) usually presented with rapidly sequential index events (median 5 days) with moderate recovery. Most with a relapsing course (n = 48) had an extended interval between index events (median 166 days) followed within 3 years by clusters of severe relapses isolated to the optic nerves and spinal cord. Most relapsing patients developed severe disability in a stepwise manner, and one-third died because of respiratory failure. Features of NMO distinct from “typical” MS included >50 cells/mm 3 in CSF (often polymorphonuclear), normal initial brain MRI, and lesions extending over three or more vertebral segments on spinal cord MRI. Conclusions: Clinical, laboratory, and imaging features generally distinguish neuromyelitis optica from MS. Patients with relapsing optic neuritis and myelitis may have neuromyelitis optica rather than MS. Patients with a relapsing course of neuromyelitis optica have a poor prognosis and frequently develop respiratory failure during attacks of cervical myelitis.

1,532 citations


"International consensus diagnostic ..." refers background in this paper

  • ...The Panel does not recommend CNS biopsy but recognizes that in atypical cases, expert pathologic review of biopsy tissue of brain or spinal cord might help establish NMOSD and exclude competing diagnoses.60 Opticospinal MS....

    [...]

  • ...The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS. Neurology® 2015;85:177–189...

    [...]

  • ...The Panel considered absence of CSF oligoclonal bands as supportive evidence for NMOSD (although they are sometimes transiently detectable at the time of an attack) and presence of bands a red flag, but sensitivity and specificity are modest.(4,53) CSF pleocytosis ....

    [...]

  • ...The criteria should also provide greater specificity for distinguishing both AQP4-IgG-seropositive and AQP4-IgG-seronegative NMOSD from MS. Early-stage diagnostic specificity is critical because recent observational data suggest that interferon-b, natalizumab, and fingolimod may worsen NMO.15–18,e24–e26 The IPND criteria are expected to facilitate more comprehensive and comparable epidemiologic studies by supplying a uniform case definition and a glossary of defined terms....

    [...]

  • ...For example, altitudinal visual field defects may result from ischemic optic neuropathy and bilateral simultaneous optic neuritis may occur in MS.e53 Diagnostic requirements are more stringent for patients in whom AQP4-IgG is not detected or for whom testing is unavailable....

    [...]


Network Information
Related Papers (5)
01 Sep 2007, Lancet Neurology

Dean M. Wingerchuk, Vanda A. Lennon +3 more

23 May 2006, Neurology

Dean M. Wingerchuk, Vanda A. Lennon +3 more

Performance
Metrics
No. of citations received by the Paper in previous years
YearCitations
20225
2021511
2020457
2019339
2018312
2017311