The clinical course of neuromyelitis optica (Devic's syndrome)

doi:10.1212/WNL.53.5.1107

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The clinical course of neuromyelitis optica (Devic's syndrome)

Journal Article•DOI•

The clinical course of neuromyelitis optica (Devic's syndrome)

Dean M. Wingerchuk¹, William F. Hogancamp¹, Peter C. O'Brien¹, Brian G. Weinshenker•Institutions (1)

Mayo Clinic¹

01 Sep 1999-Neurology (Lippincott Williams & Wilkins)-Vol. 53, Iss: 5, pp 1107-1114

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.

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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.

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Journal Article•DOI•

Diagnostic criteria for multiple sclerosis: 2010 Revisions to the McDonald criteria

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Chris H. Polman¹, Stephen C. Reingold, Brenda Banwell², Michel Clanet³, Jeffrey A. Cohen⁴, Massimo Filippi, Kazuo Fujihara⁵, Eva Havrdova⁶, Michael Hutchinson⁷, Ludwig Kappos⁸, Fred D. Lublin⁹, Xavier Montalban, Paul L. O’Connor², Magnhild Sandberg-Wollheim¹⁰, Alan J. Thompson¹¹, Emmanuelle Waubant¹², Brian G. Weinshenker¹³, Jerry S. Wolinsky¹⁴ - Show less +14 more•Institutions (14)

University of Amsterdam¹, University of Toronto², Centre Hospitalier Universitaire de Toulouse³, Cleveland Clinic⁴, Tohoku University⁵, Charles University in Prague⁶, University College Dublin⁷, University of Basel⁸, Icahn School of Medicine at Mount Sinai⁹, Lund University¹⁰, University College London¹¹, University of California, San Francisco¹², Mayo Clinic¹³, University of Texas Health Science Center at Houston¹⁴

01 Feb 2011-Annals of Neurology

TL;DR: These revisions simplify the McDonald Criteria, preserve their diagnostic sensitivity and specificity, address their applicability across populations, and may allow earlier diagnosis and more uniform and widespread use.

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Abstract: New evidence and consensus has led to further revision of the McDonald Criteria for diagnosis of multiple sclerosis. The use of imaging for demonstration of dissemination of central nervous system lesions in space and time has been simplified, and in some circumstances dissemination in space and time can be established by a single scan. These revisions simplify the Criteria, preserve their diagnostic sensitivity and specificity, address their applicability across populations, and may allow earlier diagnosis and more uniform and widespread use.

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8,883 citations

Cites background from "The clinical course of neuromyeliti..."

...Among Asian patients with CNS inflammatory demyelinating disease, a phenotype characterized by NMO, longitudinally extensive spinal cord lesions, and positive AQP4 autoantibody seropositivity19 has been relatively more common than in Western populations.55–57 The Panel solicited input on use of the McDonald Criteria in Asia and Latin America, where there is evidence of a similar phenotype distinction.41 Although the McDonald Criteria are widely used in these parts of the world, there is some uncertainty, especially in Asia, about whether MS and NMO are distinct and if so, how they should be distinguished.39 As currently applied, the term opticospinal MS appears to be an admixture of conventional MS and NMO....
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...NMO sometimes is mistaken for MS, and recurrent optic neuritis and transverse myelitis might be limited forms of NMO.(17,18) Recent advances in the development of serum antibody markers specific to NMO(19) may aid in such differential diagnoses....
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...Scans at 7.0T showed lesions in the white and gray matter with enhanced in vivo detection of pathological hallmarks of MS lesions.80–83 Finally, MRI techniques such as magnetic transfer imaging allow the detection of damage outside focal lesions (for instance, in normal-appearing brain tissues) not present in conditions such as ADEM and NMO.15,84,85 The utility of these scanning technologies for MS diagnosis in patients with CIS remains a matter for future research and validation....
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Journal Article•DOI•

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W. Ian McDonald¹, A Compston², Gilles Edan, Donald E. Goodkin³, Hans-Peter Hartung⁴, Fred D. Lublin⁵, Henry F. McFarland⁶, Donald W. Paty⁷, Chris H. Polman⁸, Stephen C. Reingold⁹, Magnhild Sandberg-Wollheim¹⁰, William A. Sibley¹¹, Alan J. Thompson¹², Stanley van den Noort¹³, Brian Y. Weinshenker¹⁴, Jerry S. Wolinsky¹⁵ - Show less +12 more•Institutions (15)

Royal College of Physicians¹, University of Cambridge², University of California, San Francisco³, University of Graz⁴, Icahn School of Medicine at Mount Sinai⁵, National Institutes of Health⁶, University of British Columbia⁷, VU University Amsterdam⁸, National Multiple Sclerosis Society⁹, Lund University¹⁰, University of Arizona¹¹, University College London¹², University of California, Irvine¹³, Mayo Clinic¹⁴, University of Texas Health Science Center at Houston¹⁵

01 Jul 2001-Annals of Neurology

TL;DR: The revised criteria facilitate the diagnosis of MS in patients with a variety of presentations, including “monosymptomatic” disease suggestive of MS, disease with a typical relapsing‐remitting course, and disease with insidious progression, without clear attacks and remissions.

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Abstract: The International Panel on MS Diagnosis presents revised diagnostic criteria for multiple sclerosis (MS). The focus remains on the objective demonstration of dissemination of lesions in both time and space. Magnetic resonance imaging is integrated with dinical and other paraclinical diagnostic methods. The revised criteria facilitate the diagnosis of MS in patients with a variety of presentations, including "monosymptomatic" disease suggestive of MS, disease with a typical relapsing-remitting course, and disease with insidious progression, without clear attacks and remissions. Previously used terms such as "clinically definite" and "probable MS" are no longer recommended. The outcome of a diagnostic evaluation is either MS, "possible MS" (for those at risk for MS, but for whom diagnostic evaluation is equivocal), or "not MS."

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6,720 citations

Journal Article•DOI•

Diagnostic criteria for multiple sclerosis: 2005 revisions to the "McDonald Criteria".

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Chris H. Polman¹, Stephen C. Reingold, Gilles Edan², Massimo Filippi³, Hans-Peter Hartung⁴, Ludwig Kappos⁵, Fred D. Lublin⁶, Luanne M. Metz⁷, Henry F. McFarland⁸, Paul O'Connor⁹, Magnhild Sandberg-Wollheim¹⁰, Alan J. Thompson, Brian G. Weinshenker¹¹, Jerry S. Wolinsky¹² - Show less +10 more•Institutions (12)

VU University Amsterdam¹, University of Rennes², Vita-Salute San Raffaele University³, University of Düsseldorf⁴, University of Basel⁵, Icahn School of Medicine at Mount Sinai⁶, Foothills Medical Centre⁷, National Institutes of Health⁸, University of Toronto⁹, Lund University¹⁰, Mayo Clinic¹¹, University of Texas Health Science Center at Houston¹²

01 Dec 2005-Annals of Neurology

TL;DR: New evidence and consensus now strengthen the role of these criteria in the multiple sclerosis diagnostic workup to demonstrate dissemination of lesions in time, to clarify the use of spinal cord lesions, and to simplify diagnosis of primary progressive disease.

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Abstract: New diagnostic criteria for multiple sclerosis integrating magnetic resonance image assessment with clinical and other paraclinical methods were introduced in 2001. The "McDonald Criteria" have been extensively assessed and used since 2001. New evidence and consensus now strengthen the role of these criteria in the multiple sclerosis diagnostic workup to demonstrate dissemination of lesions in time, to clarify the use of spinal cord lesions, and to simplify diagnosis of primary progressive disease. The 2005 Revisions to the McDonald Diagnostic Criteria for MS should simplify and speed diagnosis, whereas maintaining adequate sensitivity and specificity.

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4,862 citations

Cites background from "The clinical course of neuromyeliti..."

...NMO sometimes is mistaken for MS, and recurrent optic neuritis and transverse myelitis might be limited forms of NMO.(17,18) Recent advances in the development of serum antibody markers specific to NMO(19) may aid in such differential diagnoses....
[...]
...NMO sometimes is mistaken for MS, and recurrent optic neuritis and transverse myelitis might be limited forms of NMO.17,18 Recent advances in the development of serum antibody markers specific to NMO19 may aid in such differential diagnoses....
[...]
...Priority should be given to visualization of intracortical lesions, use of higher field strength, and analysis of “normal appearing brain tissue,”23 because preliminary evidence suggests that “occult” damage in normal-appearing white and gray matter seen with magnetization transfer, diffusion tensor imaging, or spectroscopy is an early feature of MS, whereas it may not occur in other demyelinating conditions such as acute disseminated encephalomyelitis and NMO.24 However, the Panel does recommend some changes in the use and interpretation of imaging criteria for dissemination in time in its 2005 revisions and provides clearer guidance on incorporating spinal cord lesions into imaging criteria (see later)....
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Journal Article•DOI•

International consensus diagnostic criteria for neuromyelitis optica spectrum disorders

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Dean M. Wingerchuk¹, Brenda Banwell², Jeffrey Bennett³, Philippe Cabre, William M. Carroll⁴, Tanuja Chitnis⁵, Jérôme De Seze⁶, Kazuo Fujihara⁷, Benjamin Greenberg⁸, Anu Jacob⁹, Sven Jarius¹⁰, Marco Aurélio Lana-Peixoto¹¹, Michael J. Levy¹², Jack H. Simon¹³, Silvia Tenembaum, Anthony Traboulsee¹⁴, Patrick Waters¹⁵, Kay E. Wellik¹, Brian G. Weinshenker¹ - Show less +15 more•Institutions (15)

Mayo Clinic¹, University of Pennsylvania², Anschutz Medical Campus³, Sir Charles Gairdner Hospital⁴, Harvard University⁵, University of Strasbourg⁶, Tohoku University⁷, University of Texas Southwestern Medical Center⁸, Walton Centre⁹, Heidelberg University¹⁰, Universidade Federal de Minas Gerais¹¹, Johns Hopkins University¹², Oregon Health & Science University¹³, University of British Columbia¹⁴, University of Oxford¹⁵

14 Jul 2015-Neurology

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.

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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.

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2,945 citations

Cites background from "The clinical course of neuromyeliti..."

...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....
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...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...
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...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 ....
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...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....
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...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....
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Journal Article•DOI•

A serum autoantibody marker of neuromyelitis optica : distinction from multiple sclerosis

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Vanda A. Lennon¹, Dean M. Wingerchuk¹, Thomas J. Kryzer¹, Sean J. Pittock¹, C. F. Lucchinetti¹, Kazuo Fujihara², Ichiro Nakashima², Brian G. Weinshenker¹ - Show less +4 more•Institutions (2)

Mayo Clinic¹, Tohoku University²

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.

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2,793 citations

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References

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Journal Article•DOI•

Epidemiology of multiple sclerosis

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Brian G. Weinshenker¹•Institutions (1)

Mayo Clinic¹

01 May 1996-Neurologic Clinics

TL;DR: Analysis of highly informative populations, such as discordant identical twins and adoptive siblings of MS patients, likely will improve the specificity of case-control studies by minimizing the vast number of potential differences between cases and controls.

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Journal Article•DOI•

MRI in the diagnosis of MS A prospective study with comparison of clinical evaluation, evoked potentials, oligoclonal banding, and CT

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Donald W. Paty¹, Joel Oger, Lorne F. Kastrukoff, S. A. Hashimoto, John P. Hooge, Andrew Eisen, K. A. Eisen, S. J. Purves, M. D. Low, V. Brandejs, W. D. Robertson, David Kb Li - Show less +8 more•Institutions (1)

University of British Columbia¹

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TL;DR: MRI was the best method for demonstrating dissemination in space and laboratory-supported definite MS (LSDMS) could be diagnosed in 85 patients of the total 200, and MRI predicted that diagnosis in 18/19 (95%).

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Abstract: We compared the diagnostic capabilities of MRI to CT, evoked potentials (EP), and CSF oligoclonal banding analysis in a prospective evaluation of 200 patients with suspected multiple sclerosis (MS). MRI was the best method for demonstrating dissemination in space. An abnormal appropriate EP in monosymptomatic disease was usually supported by MRI and CSF anaylsis as being predictive of MS as a clinical diagnosis. A normal appropriate EP study was not satisfactory because MRI and CSF analysis often did not support a diagnosis of non-MS . When there is agreement between three of these paraclinical studies, the diagnosis of MS is probably unequivocal. For use in research studies, laboratory-supported definite MS (LSDMS) could be diagnosed in 85 patients of the total 200 (42.5%), in 19/38 (50%) of optic neuritis (ON) patients, and in 24/52 (46%) of chronic progressive myelopathy (CPM) patients. MRI was 100% successful in identifying patients who qualified for LSDMS in the ON and CPM groups. In a short follow-up (less than 1 year), 19/200 (10%) went on to develop clinically definite MS (CDMS), and MRI predicted that diagnosis in 18/19 (95%). Only long-term follow-up will show how well these studies and the category of LSDMS predict the development of CDMS. The clinical diagnosis of MS (CDMS), even though only 95% accurate, must remain the gold standard.

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Idiopathic intracranial hypertension. A prospective study of 50 patients.

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Michael Wall¹, Donna George•Institutions (1)

Tulane University¹

01 Feb 1991-Brain

TL;DR: Monitoring of newly-diagnosed patients with idiopathic intracranial hypertension over a period of 2 to 39 months found visual loss in patients with IIH is common and is often reversible, and improvement of visual field grade was significantly associated only with weight gain during the year before diagnosis.

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Abstract: Management of patients with idiopathic intracranial hypertension (IIH) should be based on the presence and progression of visual loss. To characterize the clinical course of IIH more completely, we monitored the clinical status, especially visual function, in 50 consecutive newly-diagnosed patients over a period of 2 to 39 months (average follow-up 12.4 months). The mean age at onset of symptoms was 31 (range 11-58) yrs; 46 (92%) were women and 47 (94%) were obese (mean weight 90 kg). Common symptoms were headache (92%), transient visual obscurations (72%) and intracranial noises (60%); 13 of the patients (26%) initially had complaints of sustained visual loss. There was visual loss as determined by Goldmann perimetry in 96% and by automated perimetry in 92%. Contrast sensitivity testing was abnormal in 50% and Snellen acuity in 22%. Two patients (4%) became blind in both eyes. The Goldmann visual field grade improved in 60% of patients but visual function deteriorated in 5 (10%). Deterioration of visual field grade was significantly associated only with weight gain during the year before diagnosis. Visual loss in patients with IIH is common and is often reversible. Patients should be evaluated by perimetry using an appropriate strategy and contrast sensitivity testing, along with careful examination of the optic discs.

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606 citations

Journal Article•DOI•

Transport and storage of vitamin A

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Rune Blomhoff¹, Michael H. Green¹, Trond Berg¹, Kaare R. Norum¹•Institutions (1)

University of Oslo¹

19 Oct 1990-Science

TL;DR: A group of ligand-dependent transcription factors, the retinoic acid receptors, that apparently mediate many of the extravisual effects of retinoids are presented.

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Abstract: The requirement of vitamin A (retinoids) for vision has been recognized for decades. In addition, vitamin A is involved in fetal development and in the regulation of proliferation and differentiation of cells throughout life. This fat-soluble organic compound cannot be synthesized endogenously by humans and thus is an essential nutrient; a well-regulated transport and storage system provides tissues with the correct amounts of retinoids in spite of normal fluctuations in daily vitamin A intake. An overview is presented here of current knowledge and hypotheses about the absorption, transport, storage, and metabolism of vitamin A. Some information is also presented about a group of ligand-dependent transcription factors, the retinoic acid receptors, that apparently mediate many of the extravisual effects of retinoids.

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585 citations

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Natural history of multiple sclerosis

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Orhun H. Kantarci¹, Brian G. Weinshenker¹•Institutions (1)

Mayo Clinic¹

01 Feb 2005-Neurologic Clinics

TL;DR: Primary progressive MS may differ from relapsing-remitting MS in MRI lesion frequency, immunogenetic profile, responsiveness to immunosuppressive treatment, and histology.

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569 citations