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

Wingerchuk, Dean M.; Hogancamp, William F.; O’Brien, Peter C.; Weinshenker, Brian G.

doi:10.1212/wnl.53.5.1107

Cited by 1,654 publications

(1,946 citation statements)

References 26 publications

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“…Additional cases in which spinal cord MRI was performed typically showed spinal cord swelling with multisegmental T2-hyperintensity and corresponding patchy contrast enhancement (Glantz et al, 1994;Drach et al, 1996;Anderson and Borsaru, 2008;Mueller et al, 2008;Urai et al, 2009). These MRI changes are similar to the longitudinally extensive lesions characteristically observed during AM in NMO (Wingerchuk et al, 1999). When CSF results were reported, a mild to marked pleocytosis was sometimes, but not always, present, as was elevation in protein.…”

Section: Imagingsupporting

confidence: 75%

“…These criteria were 99% sensitive and 90% specific for differentiating NMO from MS with optic nerve and spinal cord presentations. The utility of these criteria was validated in an independent, prospectively gathered dataset that found the revised criteria to have greater specificity (83.3% versus 25%) but lower sensitivity (87.5% versus 93.7%) than earlier proposed criteria that did not include the anti-AQP4 antibody (Wingerchuk et al, 1999;Saiz et al, 2007). Furthermore, both positive (87.5% versus 62.5%) and negative (83.3% versus 75%) predictive values were improved with the newer criteria.…”

Section: Diagnostic Criteria and Utility Of The Anti-aqp4 Antibodymentioning

confidence: 98%

“…Thus, preventing attacks in NMO may prevent cumulative disability. Anecdotal experience found that NMO does not respond to immunomodulatory therapy (Wingerchuk et al, 1999). A multicenter, retrospective case series of 26 patients found that NMO patients (n ¼ 19) treated with immune suppression were less likely to relapse than NMO patients treated with interferon (n ¼ 7) (Papeix et al, 2007).…”

Section: Nmo Treatmentmentioning

confidence: 99%

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Acute inflammatory myelopathies

Cree

2014

Handbook of Clinical Neurology

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Section: Imagingsupporting

confidence: 75%

Section: Diagnostic Criteria and Utility Of The Anti-aqp4 Antibodymentioning

confidence: 98%

Section: Nmo Treatmentmentioning

confidence: 99%

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Acute inflammatory myelopathies

Cree

2014

Handbook of Clinical Neurology

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“…The clinical course of NMO is usually more severe than of MS. Within five years of onset, fifty percent of patients either lose functional vision in at least one eye or become unable to walk unassisted 6 . Detection of the autoantibody enables early diagnosis of NMO, before the presence of a full-blown clinical picture, allowing early initiation of appropriate immunosuppressive therapy.…”

mentioning

confidence: 99%

Seroprevalence of NMO-IgG antibody in Brazilian patients with neuromyelitis optica

Adoni

Lino

Marchiori

et al. 2008

Arq. Neuro-Psiquiatr.

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-Neuromyelitis optica (NMO; Devic's disease) and its spectrum disorders are idiopathic inflammatory demyelinating diseases of the central nervous system (CNS) that mainly affect the optic nerves and spinal cord 1 . The hallmark of NMO pathology is the presence of necrotic spinal cord lesions involving both gray and white matter, often resulting in cavitation, as well as the presence of vascular hyalinization 2 . NMO and multiple sclerosis (MS) are currently considered different diseases since the description of NMO-IgG, a serum gamma immunoglobulin autoantibody that is a specific marker for NMO 3,4 . NMO-IgG selectively binds to aquaporin-4 (AQP4) water channel, a component of the dystroglycan protein complex of astrocytic foot processes at the blood-brain barrier 5 . Its sensitivity and specificity for NMO diagnosis were confirmed in several studies and the presence of NMO-IgG is now one of the new diagnostic criteria for neuromyelitis optica 4 .NMO may represent the first example of a novel class of autoimmune channelopathy 5 . The clinical course of NMO is usually more severe than of MS. Within five years of onset, fifty percent of patients either lose functional vision in at least one eye or become unable to walk unassisted 6 . Detection of the autoantibody enables early diagnosis of NMO, before the presence of a full-blown clinical picture, allowing early initiation of appropriate immunosuppressive therapy. This is the first study of frequency of NMO-IgG in a series of Brazilian relapsing NMO patients. METHODTwenty-eight patients with NMO from the Center for Myelin Disorders of the Neurologic Clinic of São Paulo University School of Medicine, São Paulo, Brazil, were enrolled. All of them fulfilled the original criteria for diagnosis of NMO 6 . Demographic and clinical characteristics of patients included in our study are shown on Table 1. Briefly, median age at onset of disease was 27 years (range 7-51), median time of follow-up was 8 years (range 1-14). The mean time elapsed between optic neuritis and myelitis was 18 months (median 21; range 2-60), Kurtzke's Expanded Disease Severity Score on last visit was 6.0 (range 2-8).

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“…The prognosis appears to be particularly poor in patients who require mechanical ventilatory support [40]. The 5-year mortality in NMO is reported to be as high as 32%, with most deaths occurring due to respiratory failure [41,42]. Because NMO can present with tumefactive lesions, brain death due to herniation has also been described [20].…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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This chapter will review the spectrum of immunemediated diseases that affect the nervous system and may result in an admission to the neurological intensive care unit. Immunomodulatory strategies to treat acute exacerbations of neurological diseases caused by aberrant immune responses are discussed, but strategies for long-term immunosuppression are not presented. The recommendations for therapeutic intervention are based on a synthesis of the literature, and include recommendations by the Cochrane Collaborative, the American Academy of Neurology, and other key organizations. References from recent publications are provided for the disorders and therapies in which randomized clinical trials and large evidenced-based reviews do not exist. The chapter concludes with a brief review of the mechanisms of action, dosing, and side effects of commonly used immunosuppressive strategies in the neurocritical care unit.

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

Cited by 1,654 publications

References 26 publications

Acute inflammatory myelopathies

Acute inflammatory myelopathies

Seroprevalence of NMO-IgG antibody in Brazilian patients with neuromyelitis optica

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

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