Optic neuromyelitis syndrome in Brazilian patients

Papais-Alvarenga, Regina María; Miranda-Santos, C.; Puccioni-Sohler, Márzia; Almeida, A.M.V; Oliveira, Sayonara Rangel; Oliveira, Carlos; Alvarenga, Hélcio; Poser, Charles M.

doi:10.1136/jnnp.73.4.429

Cited by 135 publications

(123 citation statements)

References 22 publications

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“…The prevalence of NMO and MS varies significantly among different regions of the world 15,16 . Such differences are due to variability in genetic susceptibility and environmental factors 17 .…”

Section: Discussionmentioning

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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Section: Discussionmentioning

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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“…[2][3][4] We describe the first European case of the syndrome in a Caucasian female unrelated to the patients described to date.…”

Section: Introductionmentioning

confidence: 99%

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?

et al. 2006

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“…[7][8][9][10] However, the criteria have limitations. They fail to capture patients with a disease course otherwise highly compatible with NMO but whose neurologic symptoms or signs implicate CNS regions outside the optic nerves and spinal cord or whose brain MRI reveals lesions that may meet MS imaging criteria.…”

mentioning

confidence: 99%

Revised diagnostic criteria for neuromyelitis optica

Wingerchuk¹,

Lennon²,

Pittock³

et al. 2006

Neurology

2,408

1,273

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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 (14.6%) 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.

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Optic neuromyelitis syndrome in Brazilian patients

Cited by 135 publications

References 22 publications

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

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

Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?

Revised diagnostic criteria for neuromyelitis optica

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