Aquaporin-4 antibody–negative neuromyelitis optica

Marignier, Romain; Bernard‐Valnet, Raphaël; Giraudon, Pascale; Collongues, Nicolas; Papeix, Caroline; Zephir, Hélène; Cavillon, Gaëlle; Rogemond, Véronique; Casey, Romain; Frangoulis, Bernard; Sèze, Jérôme De; Vukusic, Sandra; Honnorat, Jérôme; Confavreux, Christian

doi:10.1212/wnl.0b013e318296e917

Cited by 159 publications

(152 citation statements)

References 35 publications

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“…We believe that this fact may be due to a selection bias, overestimating those cases where CNS inflammatory disease was suspected but did not match MS criteria, such as recurrent optic neuritis or idiopathic LETM. Taking into account the comparison between AQP4-IgG seropositive and seronegative patients, we found a higher proportion of females among the positive ones, similarly to what have been previously reported [24]. Analysing the group of AQP4-IgG seropositive patients, we found similar clinical characteristics to what have been reported in the UK and Japan cohorts [25], including mean age at onset, percentage of female patients, disease course and form of presentation.…”

Section: Discussionsupporting

confidence: 88%

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Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

Domingos

Isidoro

Figueiredo

et al. 2015

Clinical Neurology and Neurosurgery

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Results: Sixty-seven patients fulfilled the inclusion criteria. They were mainly Caucasian, 55 female. Median age at onset was 32.0 years and mean follow-up 7.4 ± 6.0 years. Twenty-one patients were definite NMO and optic neuritis (ON) the most frequent initial presentation. Forty-six were classified as NMO spectrum disorders. The main subtypes were recurrent ON and single longitudinally extensive transverse myelitis. Twenty-four patients had positive AQP4-IgG. Twenty-three had other circulating autoantibodies. Fifteen out of 67 patients had concomitant autoimmune disease. There was a significant correlation between the presence of autoimmune disease and the positivity for AQP4-IgG. Five patients died, all definite NMO. Conclusion: This is the first study about this rare disease in Portugal. Demographic features were similar to other studies. The existence of concomitant autoimmune disease was significantly associated with seropositivity for AQP4-IgG.

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Section: Discussionsupporting

confidence: 88%

“…The mortality rate was 8.3% in our cohort, higher than the Japanese but lower than the UK cohorts [25]. In fact, recent studies comparing seropositive and seronegative patients brought new concepts, including switching the NMO definition from a clinical phenotype to a biological one [24].…”

Section: Discussionmentioning

confidence: 58%

Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

Domingos

Isidoro

Figueiredo

et al. 2015

Clinical Neurology and Neurosurgery

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“…This is in keeping with recent findings and contrasts with AQP4 antibody-positive patients, who are more likely to be non-Caucasian. 7,8 A recent report of 16 MOG antibody-positive patients of a similar median age found that most patients were male. 24 We did not find a statistically significant association with sex, age at onset, and MOG antibody status.…”

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confidence: 98%

Antibodies to myelin oligodendrocyte glycoprotein in bilateral and recurrent optic neuritis

Ramanathan

Reddel

Henderson

et al. 2014

Journal of Neuroimmunology

110

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“…Also, cases of sero-negative NMO are quite different from those of sero-positive NMO as they present with concurrent acute myelitis and optic nerve involvement with mono-phasic presentation and lesser female preponderance [5]. Nevertheless it's important to have an early diagnosis and differentiation from MS, more so because of the high incidence of relapse in NMO and even deterioration in NMO in response to MS modifying treatment [6].…”

Section: Introductionmentioning

confidence: 99%

Sero-Negative Neuromyelitis Optica

Bhargava¹,

Upadhayaya²,

Dixit³

et al. 2017

JCR

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Background: Neuromyelitis optica (NMO, Devic's syndrome) is an immune mediated inflammatory demyelinating disease of the central nervous system that predominantly affects the spinal cord and optic nerves. Often confused with multiple sclerosis (MS), early diagnosis and differentiation from MS is important because of different treatment regimens. Case Report: We hereby report a sero-negative mono-phasic manifestation of this rare disease in a nine year old Indian boy. NMO was diagnosed in our case despite being sero-negative because of typical clinical findings and evidence of myelitis and optic neuritis seen in MRI. Patient was treated with pulse methyl-prednisolone followed by oral prednisolone and patient improved with some residual deformity. Conclusion: High index of suspicion is required to diagnose and treat patients at an early stage to prevent co-morbidities and complications.

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Aquaporin-4 antibody–negative neuromyelitis optica

Abstract: This raises the question of a distinct physiopathology for patients with AQP4-Ab-negative NMO and of their place in the spectrum of the disease.

Cited by 159 publications

References 35 publications

Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

Neuromyelitis optica in Portugal (NEMIPORT) – A multicentre study

Antibodies to myelin oligodendrocyte glycoprotein in bilateral and recurrent optic neuritis

Sero-Negative Neuromyelitis Optica

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