A population-based study of neuromyelitis optica in Caucasians

Asgari, Nasrin; Lillevang, S T; Skejoe, Hanne Pernille Bro; Falah, Masoud; Stenager, Egon; Kyvik, Kirsten Ohm

doi:10.1212/wnl.0b013e3182190f74

Cited by 241 publications

(277 citation statements)

References 24 publications

(36 reference statements)

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“…NMO is rare and accounts for only 1% of all demyelinating diseases [26][27][28]. The incidence of NMO is estimated to be approximately 4 per million of the population and the prevalence is somewhere between 3 and 44 per million, depending on ethnicity and geographic location [26][27][28][29]. Disease onset is usually in the fourth decade of life; women are affected more commonly than men (approximately 3:1); and non-Caucasians (especially Japanese) are affected more commonly than Caucasians [29][30][31][32].…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

“…The incidence of NMO is estimated to be approximately 4 per million of the population and the prevalence is somewhere between 3 and 44 per million, depending on ethnicity and geographic location [26][27][28][29]. Disease onset is usually in the fourth decade of life; women are affected more commonly than men (approximately 3:1); and non-Caucasians (especially Japanese) are affected more commonly than Caucasians [29][30][31][32]. Most patients with NMO (60-90%) have autoantibodies directed toward the aquaporin 4 (AQP4) channel, and these antibodies are thought to be pathogenic [29,33].…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

“…Disease onset is usually in the fourth decade of life; women are affected more commonly than men (approximately 3:1); and non-Caucasians (especially Japanese) are affected more commonly than Caucasians [29][30][31][32]. Most patients with NMO (60-90%) have autoantibodies directed toward the aquaporin 4 (AQP4) channel, and these antibodies are thought to be pathogenic [29,33]. AQP4 is the most common water channel in the brain and is expressed on astrocytes of the glia limitans.…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

“…Spinal cord involvement is longitudinally extensive (≥3 vertebral segments) in patients with NMO in contrast to the spinal lesions seen in patients with MS, which are much more limited in extent [39]. Respiratory function can be affected by spinal lesions that extend into the brainstem, but isolated brainstem lesions leading to respiratory compromise have also been described [29,40]. The prognosis appears to be particularly poor in patients who require mechanical ventilatory support [40].…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

“…The AQP4 antibodies are fairly specific for NMO but have limited sensitivity, and thus seronegative patients can be diagnosed with NMO [44]. CSF analysis may show pleocytosis and elevated protein, and oligoclonal bands (OCBs) may be seen, but are much less common than in MS [29,38,45].…”

Section: Neuromyelitis Opticamentioning

confidence: 99%

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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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Section: Neuromyelitis Opticamentioning

confidence: 99%

Section: Neuromyelitis Opticamentioning

confidence: 99%

Section: Neuromyelitis Opticamentioning

confidence: 99%

Section: Neuromyelitis Opticamentioning

confidence: 99%

Section: Neuromyelitis Opticamentioning

confidence: 99%

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Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Geldern¹,

McPharlin

Becker

2012

Neurotherapeutics

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Epidemiology of Neuromyelitis Optica in the United States

Mealy¹,

Wingerchuk²,

Greenberg³

et al. 2012

Arch Neurol

264

200

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Background: Rare diseases require integrated multicenter clinical networks to facilitate clinical research. Neuromyelitis optica (NMO) and NMO spectrum disorders (NMOSDs) are uncommon neuroinflammatory syndromes that are distinct from multiple sclerosis and associated with NMO-IgG, a serologic antibody against aquaporin 4.Objective: To develop a national multicenter NMO clinical consortium and report initial demographic, clinical, and radiographic features of a cohort of patients with NMO/NMOSD in the United States.Design: Review of medical records from patients undergoing evaluation during a 5-year period. We used uniform diagnostic criteria and clinical, laboratory, and neuroimaging definitions to describe the cohort.Setting: Three academic medical centers.Patients: One hundred eighty-seven patients with NMO/ NMOSD.Results: Of the 187 patients included in the analysis, 86 had NMO-IgG-seropositive NMO; 40, NMO-IgGseronegative NMO; and 61, NMO-IgG-seropositive NMOSD. Altogether, 29.4% of our patients were initially misdiagnosed with multiple sclerosis. The average age at onset of NMO/NMOSD was 41.1 years with a strong female predilection, similar to other autoimmune disorders. Nonwhite patients constituted 52.4% of the cohort. The hallmark of NMO and NMOSD is recurrent longitudinally extensive transverse myelitis, but patients with NMO tend to initially present with optic neuritis. Conclusions:A national multicenter consortium to study NMO/NMOSD is feasible and facilitates accurate clinical diagnosis. This network establishes a foundation for determining disease prevalence, translational research, and clinical trials.

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Mortality of neuromyelitis optica spectrum disorders in a Chinese population

Shi

Chen

et al. 2021

Ann Clin Transl Neurol

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Objective: Neuromyelitis optica spectrum disorder (NMOSD) is a rapidly disabling disease. Epidemiologic studies have suggested varying NMOSD mortality across ethnic groups. However, NMOSD mortality data in China are scarce. This study's objectives were to explore mortality and causes of death among Chinese NMOSD patients and to identify independent predictors of death. Methods: We performed a retrospective study with a 10-year follow-up of Chinese NMOSD patients. A Cox proportional hazards model was used to identify independent predictors of death. Results: Five hundred and sixty-nine patients were included; 24 patients died during follow-up, for overall mortality of 4.2%. In these patients, the median disease duration at the time of death was 3.4 years. The most common cause of death was secondary infection (62.5%), especially respiratory infection (45.8%). The second most common cause of death was extensive cervical myelitis with respiratory failure (16.7%). Other causes included suicide (8.3%), cancer (4.2%), cerebral embolism (4.2%), and unknown causes (4.2%). The multivariate Cox analyses indicated that a short first interattack interval (HR = 0.93, 95% CI 0.89-0.98, p = 0.003), lack of regular immunotherapy (HR = 10.34, 95% CI 4.05-26.37, p < 0.001), and older age at onset were independent predictors of death. Every increasing decade of onset age increased the risk of death 2.59 times (95% CI 1.74-3.86, p < 0.001). Interpretation: Infections were more common in patients not treated with any immunotherapy, indicating that early and consequent immunotherapy might prevent death by infections, which is of great importance for further treatment of NMOSD patients to avoid undertreatment due to fear of treatmentassociated infections.

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A population-based study of neuromyelitis optica in Caucasians

Abstract: Background: Epidemiologic studies have suggested different prevalence of neuromyelitis optica

Cited by 241 publications

References 24 publications

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

Epidemiology of Neuromyelitis Optica in the United States

Mortality of neuromyelitis optica spectrum disorders in a Chinese population

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