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Espectro clínico de neuromielite óptica
ORPHA:71211CID-10 · G36.0CID-11 · 8A43DOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
neuroInício infantilHerança Complex
Também conhecida comoDoença de DevicSíndrome de DevicNeuromielite ópticaNMOSDON
Sinônimos clínicos: Neuromielite óptica · Doença de Devic

Doença inflamatória rara do sistema nervoso central caracterizada principalmente por crises de neurite óptica (ON) uni ou bilateral e mielite aguda.

Em construçãohistóricoSugerir correção
Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

💬
EM LINGUAGEM SIMPLES

Neuromielite óptica é um distúrbio autoimune que afeta os nervos dos olhos e o sistema nervoso central, que inclui o cérebro e a medula espinhal. Distúrbios autoimunes ocorrem quando o sistema imunológico funciona de forma inadequada e ataca os próprios tecidos e órgãos do corpo. Na neuromielite óptica, o ataque autoimune causa inflamação dos nervos, e o dano resultante leva aos sinais e sintomas da condição.

Neuromielite óptica é caracterizada por neurite óptica, que é a inflamação do nervo que transporta informações do olho para o cérebro (nervo óptico). A neurite óptica causa dor ocular e perda de visão, que podem ocorrer em um ou ambos os olhos.

Neuromielite óptica também é caracterizada por mielite transversa, que é a inflamação da medula espinhal. A inflamação associada à mielite transversa danifica a medula espinhal, causando uma lesão que frequentemente se estende ao longo de três ou mais ossos da coluna (vértebras). Além disso, a mielina, que é a cobertura que protege os nervos e promove a transmissão eficiente dos impulsos nervosos, pode ser danificada. A mielite transversa causa fraqueza, dormência e paralisia dos braços e das pernas. Outros efeitos do dano à medula espinhal podem incluir distúrbios nas sensações, perda de controle da bexiga e dos intestinos, soluços incontroláveis e náuseas. Além disso, a fraqueza muscular pode dificultar a respiração e pode causar insuficiência respiratória potencialmente fatal em pessoas com neuromielite óptica.

Existem duas formas de neuromielite óptica, a forma recorrente e a forma monofásica. A forma recorrente é a mais comum. Essa forma é caracterizada por episódios recorrentes de neurite óptica e mielite transversa. Esses episódios podem ocorrer a meses ou anos de intervalo, e geralmente há recuperação parcial entre os episódios. No entanto, a maioria das pessoas afetadas eventualmente desenvolve fraqueza muscular permanente e comprometimento visual que persistem mesmo entre os episódios. Por razões desconhecidas, aproximadamente nove vezes mais mulheres do que homens apresentam a forma recorrente. A forma monofásica, que é menos comum, causa um único episódio de neuromielite óptica que pode durar vários meses. Pessoas com essa forma da condição também podem ter fraqueza muscular ou paralisia duradoura e perda de visão. Essa forma afeta homens e mulheres igualmente. O início de qualquer forma de neuromielite óptica pode ocorrer a qualquer momento, da infância à idade adulta, embora a condição com maior frequência comece nos quarenta anos de idade de uma pessoa.

Aproximadamente um quarto dos indivíduos com neuromielite óptica apresentam sinais ou sintomas de outro distúrbio autoimune, como miastenia gravis, lúpus eritematoso sistêmico ou síndrome de Sjögren. Alguns cientistas acreditam que uma condição descrita em pacientes japoneses como esclerose múltipla óptico‑espinhal (ou esclerose múltipla ópticoespinhal) que afeta os nervos dos olhos e do sistema nervoso central seja a mesma que a neuromielite óptica.

Fonte: MedlinePlus Genetics (NLM/NIH), traduzido
📋
Informacoes curadas por IA — podem conter imprecisoes

Doença inflamatória rara do sistema nervoso central caracterizada principalmente por crises de neurite óptica (ON) uni ou bilateral e mielite aguda.

Pesquisas ativas
86 ensaios
183 total registrados no ClinicalTrials.gov
Publicações científicas
3.152 artigos
Último publicado: 2026
Medicamentos
4 com registro
Enspryng (SATRALIZUMAB), UPLIZNA (INEBILIZUMAB), Ultomiris (RAVULIZUMAB)

Tem tratamento?

✓ 4 medicamentos específicos desta doença (registro ANVISA, FDA ou SUS/CEAF)
Ver detalhes, fases e interações →
ENSPRYNG (SATRALIZUMAB)UPLIZNA (INEBILIZUMAB)ULTOMIRIS (RAVULIZUMAB)SOLIRIS (ECULIZUMAB)

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
2.071
Worldwide
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Cobertura mínimaScore: 65%
2 procedimentos SIGTAPCID-10: G36.0
🇧🇷Dados SUS / DATASUS
EXAMES E PROCEDIMENTOS RELACIONADOS (2)
•
Sequenciamento completo do exoma (WES)
•
Atendimento em reabilitação — doenças raras
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🧠
Neurológico
2 sintomas
👁️
Olhos
2 sintomas
🫘
Rins
1 sintomas
🫁
Pulmão
1 sintomas
🛡️
Imunológico
1 sintomas
🫃
Digestivo
1 sintomas

+ 9 sintomas em outras categorias

Características mais comuns

90%prev.
Perda neuronal no sistema nervoso central
Muito frequente (99-80%)
90%prev.
Mielite
Muito frequente (99-80%)
90%prev.
Anormalidade funcional da bexiga
Muito frequente (99-80%)
90%prev.
Positividade de autoanticorpos
Muito frequente (99-80%)
90%prev.
Disfunção somatossensorial
Muito frequente (99-80%)
90%prev.
Perda visual
Muito frequente (99-80%)
17sintomas
Muito frequente (10)
Frequente (2)
Ocasional (5)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 17 características clínicas mais associadas, ordenadas por frequência.

Perda neuronal no sistema nervoso centralNeuronal loss in central nervous system
Muito frequente (99-80%)90%
MieliteMyelitis
Muito frequente (99-80%)90%
Anormalidade funcional da bexigaFunctional abnormality of the bladder
Muito frequente (99-80%)90%
Positividade de autoanticorposAutoimmune antibody positivity
Muito frequente (99-80%)90%
Disfunção somatossensorialSomatic sensory dysfunction
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico3.152PubMed
Últimos 10 anos200publicações
Pico2026140 papers
Linha do tempo
2026Hoje · 2026🧪 1995Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
✓Aprovado5
3Fase 312
2Fase 216
1Fase 118
·Pré-clínico49
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 100 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Espectro clínico de neuromielite óptica

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Publicações mais relevantes

Timeline de publicações
3.055 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 3.055

#1

Paraneoplastic neuromyelitis optica spectrum disorder with dual AQP4-IgG and CRMP5 antibodies following thymectomy: a case report.

Frontiers in neuroscience2026

We present a rare case of paraneoplastic neuromyelitis optica spectrum disorder (NMOSD) in a 50-year-old woman with a history of B3 thymoma, marked by dual positivity for AQP4-IgG and CV2/CRMP5 antibodies. The patient developed sequential bilateral optic neuritis, with orbital MRI revealing enhancement of the left optic nerve. Serological analysis showed a high AQP4-IgG titer (1:320) and positive CV2/CRMP5 antibodies, while other paraneoplastic markers were absent. Treatment with glucocorticoids and mycophenolate mofetil led to substantial visual recovery. This case underscores the association between thymoma and paraneoplastic NMOSD and suggests a potential interaction between AQP4- and CRMP5-related autoimmune responses. Although CV2/CRMP5 antibodies are typically linked to lung cancer and thymoma, their coexistence with AQP4-IgG in NMOSD points to a distinct paraneoplastic mechanism. These findings support the need for thorough tumor screening in NMOSD patients with atypical features, particularly those with CV2/CRMP5 seropositivity.

#2

Human umbilical cord mesenchymal stromal cells therapy for neuromyelitis optica spectrum disorder: a phase 1/2a trial.

Cell death and differentiation2026 Mar 17

Current treatments for neuromyelitis optica spectrum disorder (NMOSD) highlight recurrence management, while little attention is paid to the relief of residual neurological dysfunction. Here we aimed to evaluate the safety and efficacy of human umbilical cord-derived mesenchymal stromal cells (hUC-MSCs) in reducing relapses and mitigating neurological impairments. This trial, hUC-MSC-NMOSD (ChiCTR-INR-16008037), a single-arm, dose-escalation, open-label study, included 31 NMOSD patients of three dose groups received four infusions every three months, with 15-month follow-up. Primary outcome was time to first recurrence; secondary outcomes focused on clinical scores, MRI lesions and exploratory findings. HUC-MSC infusion was well tolerated in all groups of patients. Adverse events were mostly mild, with urinary tract infections being the most common. Severe adverse events were rare and unrelated to the treatment. The median relapse-free interval increased significantly post-treatment from 305 (95%CI 226-382·5) to 760 (589-1016·5) (p < 0·001), especially in the medium- and high-dose groups. During the two years before and after therapy, the mean Annualized Relapse Rate (ARR) dropped considerably from 1 (0·75-1) to 0 (0-0·5) (p < 0·001). Clinical scores improved in the low and medium-dose groups. The total volume of high-signal white matter lesions in brain significantly decreased after therapy from 4144·5 (2857·2-5508·6) to 2914·4 (2453-3684·11) (p = 0·016). Exploratory single-cell RNA sequencing and metabolomics detection revealed a potential participation of thioredoxin and oxidative phosphorylation (OXPHOS)-mediated boosting of Treg differentiation and suppressive capacity. This trial indicates that intravenous hUC-MSC administration is safe and shows potential efficacy in treating NMOSD. Medium dose might be the best possible compromise between safety and effectiveness.

#3

Frequency of AQP4 and MOG Antibodies in Patients With Optic Neuritis Fulfilling Minimal New Multiple Sclerosis MRI Criteria.

Neurology2026 Apr 14

Recent revisions to multiple sclerosis (MS) diagnostic criteria include the optic nerve as a site of dissemination in space, enabling this diagnosis in patients with acute optic neuritis (ON) and a single additional MS-typical location on MRI if dissemination in time (DIT) is demonstrated. We aimed to assess the frequency of non-MS diagnoses in this context. We retrospectively analyzed consecutive patients with inaugural acute ON and at least 1 MS-typical lesion in a single brain location on baseline MRI across 3 French centers. All patients met DIT criteria and underwent aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing. Final diagnoses were based on clinical, radiologic, and follow-up data. Among 96 patients (mean age 35.8 years; 70.8% female), 73 (76.0%) were diagnosed with MS and 23 (24.0%) with MOG antibody-associated disease (n = 18) or neuromyelitis optica spectrum disorder. Longitudinally extensive lesions, bilateral involvement, chiasmal enhancement, or optic perineuritis were observed in 100% of non-MS patients and 24.6% of patients with MS (difference 75.4%, 95% CI: 64.3-86.6; p < 0.001). All patients without these patterns were ultimately diagnosed with MS. AQP4 and MOG antibody testing, along with careful orbital MRI interpretation, is recommended in patients with ON meeting minimal new MS criteria.

#4

Real-world safety of satralizumab in neuromyelitis optica spectrum disorder: a FAERS-based risk stratification study.

Journal of neurology2026 Mar 17

Satralizumab, an interleukin-6 receptor monoclonal antibody, is an established therapy for neuromyelitis optica spectrum disorder (NMOSD). While randomized trials have demonstrated their efficacy and short-term safety, real-world data characterizing report-level associations and severe adverse outcomes remain limited. We performed a retrospective pharmacovigilance study using the U.S. FDA Adverse Event Reporting System (FAERS) from Q1 2004 to Q3 2025. Disproportionality analyses (reporting odds ratios [RORs], information components [ICs]), subgroup analyses, co-medication analysis, time-to-onset evaluation, and multivariable logistic regression for most severe outcomes (death/life-threatening events) were conducted. Among 1174 satralizumab-related reports, infections were the most prominent signals, with key signals including atypical mycobacterial infection (ROR 109.24, 95% CI 51.92-229.82; IC 6.76, IC025 1.88), pneumonia pneumococcal (ROR 41.51, 95% CI 13.36-128.94; IC 5.37, IC025 0.45), and pyelonephritis (ROR 40.80, 95% CI 25.32-65.75; IC 5.34, IC025 2.98). Forty unlabeled events (e.g., lymphocyte count decrease [ROR 18.52, 95% CI 11.33-30.27]) were identified. Concomitant corticosteroid use was associated with increased reporting of infectious and hepatic adverse events. Among 827 reports with complete demographic data, 72 were classified as most severe. Factors associated with severe outcome reports included age ≥ 65 years (adjusted odds ratio [OR] 3.85, 95% CI 2.22-6.69), male sex (adjusted OR 2.23, 95% CI 1.20-4.15), and septic shock (adjusted OR 7.88, 95% CI 2.48-25.09). Median time to onset was 97 days (interquartile range 15-291 days), with early and late peaks. This real-world study highlights clinically relevant safety signals of satralizumab in NMOSD, identifies vulnerable patient subgroups, and supports risk-adapted monitoring strategies in neurological practice.

#5

Early anti-C5 therapy in NMOSD: rationale and clinical evidence.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026 Mar 14

Complement-mediated astrocyte injury is central to aquaporin-4 immunoglobulin G-positive neuromyelitis optica spectrum disorder (AQP4-IgG+ NMOSD). Although C5 inhibitors are approved for relapse prevention, their role as rescue therapy during severe acute attacks remains unclear.We describe a 39-year-old woman with AQP4-IgG+ NMOSD presenting with longitudinally extensive myelitis, optic neuropathy, and a basal ganglia lesion. Despite two courses of intravenous methylprednisolone, five plasma exchanges, and intravenous immunoglobulin, disability progressed to an Expanded Disability Status Scale (EDSS) score of 9.0. Ravulizumab was initiated on day 34 after meningococcal vaccination and prophylactic antibiotics. Clinical course, cerebrospinal fluid (CSF) parameters, and total hemolytic complement activity (CH50) were followed for 1 year. After a 2400-mg loading dose of ravulizumab, CH50 became undetectable, indicating effective terminal complement inhibition, whereas C3/C4 remained within reference ranges. Neurological deterioration stopped promptly, followed by marked recovery in motor and visual function. EDSS improved from 9.0 to 6.0 at transfer and to approximately 1.5-2.0 at 1 year. CSF pleocytosis and protein levels normalized in parallel with CH50 suppression. The patient regained ambulation with an orthosis, returned to work on day 220, and remained relapse-free without treatment-related adverse events during 8-weekly maintenance therapy. This case supports the biological plausibility and practical feasibility of early ravulizumab rescue therapy in severe refractory AQP4-IgG+ NMOSD. Prospective studies are needed to define optimal timing, patient selection, and the incremental benefit beyond prior acute immunotherapies.

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Frequency of AQP4 and MOG Antibodies in Patients With Optic Neuritis Fulfilling Minimal New Multiple Sclerosis MRI Criteria.

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Real-world safety of satralizumab in neuromyelitis optica spectrum disorder: a FAERS-based risk stratification study.

Journal of neurology
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Early anti-C5 therapy in NMOSD: rationale and clinical evidence.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
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Cureus
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The British and Irish orthoptic journal
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Microstructure-informed deep learning improves thalamic atrophy segmentation and clinical associations in multiple sclerosis and related neuroimmunological diseases.

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Clinical, radiological, and CSF features distinguishing spinal dural arteriovenous fistula from idiopathic transverse myelitis and seropositive NMOSD-/MOGAD-associated myelopathy: a retrospective observational study.

Scientific reports
2026

Impact of Age at Onset on Relapse and Disability in AQP4-IgG Neuromyelitis Optica Spectrum Disorder.

Neurology
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Trident Sign in Neuromyelitis Optica Spectrum Disorder.

Annals of Indian Academy of Neurology
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Safety and efficacy of ravulizumab in patients with NMOSD previously treated with rituximab: A post hoc analysis of the CHAMPION-NMOSD trial.

Multiple sclerosis (Houndmills, Basingstoke, England)
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ACSL4-mediated astrocyte ferroptosis augments neuroinflammation and exacerbates NMOSD pathology.

Cell death and differentiation
2026

Prognosis biomarkers in MOGAD: What can we learn from observational studies?

Revue neurologique
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Consistent Detection of Aquaporin-4 Antibodies: A Comparative Analysis Between Fixed and Live Cell-Based Assays.

Journal of clinical neurology (Seoul, Korea)
2026

The temporal sequence of myasthenia gravis and neuromyelitis optica spectrum disorder: a case report and systematic review of 74 patients.

Frontiers in neurology
2026

ACR Appropriateness Criteria® Demyelinating Diseases.

Journal of the American College of Radiology : JACR
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Safety of breastfeeding under monoclonal antibodies in the offspring of mothers with multiple sclerosis or neuromyelitis optica spectrum disorder.

Journal of neurology, neurosurgery, and psychiatry
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Aquaporin-4 (AQP4) antibody-positive neuromyelitis optica spectrum disorder (NMOSD) complicated with acute pancreatitis: A case report.

Medicine
2026

Optic neuropathies induced by immune checkpoint inhibitors: A case series and systematic review of the literature.

European journal of cancer (Oxford, England : 1990)
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Efficacy and Safety of Rituximab Versus Inebilizumab in Patients With Neuromyelitis Optica Spectrum Disorder: A Dual-Center, Real-World Cohort Study.

European journal of neurology
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Neuropathic Pain in Neuromyelitis Optica Spectrum Disorders: Prevalence and Management Strategies-A Systematic Review and Meta-Analysis.

Journal of clinical medicine
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Major depressive disorder in multiple sclerosis and AQP4-antibody positive neuromyelitis optica spectrum disorder: A cross-sectional study on clinical correlates in Iranian patients.

Journal of psychosomatic research
2026

Rare demyelinating diseases of the central nervous system: A diagnostic and therapeutic challenge - based on the case of a young woman with MOGAD.

Polski merkuriusz lekarski : organ Polskiego Towarzystwa Lekarskiego
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Comparative analysis of adverse event reporting signals between Satralizumab and Inebilizumab in neuromyelitis optica spectrum disorder: A pharmacovigilance study using the FDA Adverse Event Reporting System.

Intractable &amp; rare diseases research
2026

Long-term effectiveness and safety of satralizumab for neuromyelitis optica spectrum disorder in a real-world clinical setting in Japan: A 2.5-year final analysis of a multicenter medical chart review (The SAkuraBeyond Study).

Multiple sclerosis (Houndmills, Basingstoke, England)
2026

Progress in the Treatment of Neuromyelitis Optica Spectrum Disorder: From Pathogenic Insights to Biologics.

Internal medicine (Tokyo, Japan)
2026

A Modern Approach to Brown-Séquard Syndrome: A Case Report.

Cureus
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Current evidence and knowledge gaps in family planning and pregnancy in myasthenia gravis, NMOSD, and MOGAD.

The Lancet. Neurology
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Polymorphonuclear myeloid-derived suppressor cells inhibit natural killer cell function in neuromyelitis optica spectrum disorder via the CD155-TIGIT axis.

Clinical immunology (Orlando, Fla.)
2026

Monoclonal antibodies in neuromyelitis optica spectrum disorder: a systematic review and meta-analysis.

BMC neurology
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Neuromyelitis Optica Spectrum Disorder Initially Presenting With Meningitis-Like Symptoms: A Case Report.

Cureus
2026

Pseudoathetotic Pseudodystonia as a Manifestation of Isolated Medullary Demyelination in Neuromyelitis Optica Spectrum Disorder.

Tremor and other hyperkinetic movements (New York, N.Y.)
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Exploration of the source of AQP4 antibody: Based on paired serum and cerebrospinal fluid samples from 98 patients.

Clinica chimica acta; international journal of clinical chemistry
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C-Reactive Protein and Neurological Autoimmune Diseases: Bridging the Diagnostic and Pathogenic Gap.

International journal of molecular sciences
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Persistent parvovirus B19 DNAemia in B-cell depletion: Implications for family planning in multiple sclerosis and related disorders.

Journal of the neurological sciences
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Neuromyelitis optica - a cause for longitudinally extensive transverse myelitis: a rare case report from Nepal and literature review.

Annals of medicine and surgery (2012)
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Neuromyelitis optica spectrum disorder in an 11-year-old: a case report in a pediatric patient from Nepal.

Annals of medicine and surgery (2012)
2026

Diverticular Perforation with Normal C-reactive Protein in a Patient with Neuromyelitis Optica Spectrum Disorder Receiving Satralizumab.

Internal medicine (Tokyo, Japan)
2026

Complement Inhibition for Acute Neuromyelitis Optica Spectrum Disorder Attacks: Insights From an International Case Series.

Neurology(R) neuroimmunology &amp; neuroinflammation
2026

Natalizumab exacerbates astrocytopathy in NMOSD via blockade of endothelial VCAM1-astrocytic integrin α4 interaction.

Proceedings of the National Academy of Sciences of the United States of America
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AQP4-IgG-Induced Astrocyte-Derived Small Extracellular Vesicles Carrying Mitochondrial DNA Regulate the TLR9/MyD88/NF-κB Pathway to Drive Microglial Activation and Neuromyelitis Optica.

Neurochemical research
2025

Long-term Indian optic neuritis study (THE LION STUDY): clinical, imaging features and visual behavior of optic neuritis in Indian population over two decades.

Frontiers in ophthalmology
2026

Quantitative ophthalmic posterior segment optical coherence tomography angiography and neurologic conditions: a review.

Frontiers in neurology
2026

An Unusual Case of Hypersomnolence.

Annals of African medicine
2026

Biomarkers for satralizumab treatment in neuromyelitis optica spectrum disorders: a prospective case series.

BMC neurology
2026

Associations between personality traits and cognitive function in multiple sclerosis and neuromyelitis optica spectrum disorder: A cross-sectional study.

Multiple sclerosis and related disorders
2026

The immunoproteasome as a neuroimmune hub in the central nervous system: from proteostasis stress to inflammatory pathology.

Journal of neuroinflammation
2026

First demyelinating attack in children: A twelve year single center cohort.

Multiple sclerosis and related disorders
2026

Anti-IL-6 Receptor Antibody Suppresses Visual Dysfunction in AQP4 Peptide-Immunized Mice.

Translational vision science &amp; technology
2026

Demographic and Epidemiological Profile of Optic Neuritis in Colombia: A Retrospective Study Using Data from the Comprehensive Information System for Social Protection.

Neuro-ophthalmology (Aeolus Press)
2025

Double-seronegative neuromyelitis optica: it is not possible to interrupt treatment after 10 years of stability.

Arquivos de neuro-psiquiatria
2025

Double-seronegative neuromyelitis optica: it is possible to interrupt treatment after 10 years of stability.

Arquivos de neuro-psiquiatria
2026

[Neuromyelitis optica spectrum disorder with positive aquaporin-4 antibodies and anti-myelin oligodendrocyte glycoprotein antibodies].

Journal francais d'ophtalmologie
2026

Comparative Effectiveness of Disease-Modifying Treatments in Double Seronegative Neuromyelitis Optica Spectrum Disorder.

Neurology(R) neuroimmunology &amp; neuroinflammation
2026

Age, Low Immunoglobulin G, and M Serum Levels Predict Infections in People With AQP4-IgG+ NMOSD Treated With Rituximab-A Multicenter Cohort Study From the German Neuromyelitis Optica Study Group (NEMOS).

European journal of neurology
2026

Activation status of astrocytes drives the MS/NMOSD therapeutic paradox: Insights from IFNAR1 signaling.

Cell reports
2026

Microbial-Immune Interplay in CNS Autoimmune Diseases: Lessons from Animal Models and Clinical Studies.

European journal of immunology
2026

Natural killer cell reduction and dysfunction define a pathogenic and diagnostic axis in neuromyelitis optica spectrum disorder.

Journal of neuroinflammation
2026

Bilateral optic neuritis preceding a Baló concentric sclerosis lesion: A case report and literature review.

Journal of neuroimmunology
2026

Evaluation of blood-brain barrier integrity and clinical characteristics in autoimmune glial fibrillary acidic protein astrocytopathy.

Multiple sclerosis and related disorders
2026

Predictive factors for immunosuppression-associated severe and opportunistic infections in AQP4-IgG Neuromyelitis Optica Spectrum Disorder.

Multiple sclerosis (Houndmills, Basingstoke, England)
2025

Diagnostic biomarkers for differentiating AQP4-IgG- negative NMOSD from other nervous system autoimmune disorders: a retrospective study.

Frontiers in immunology
2026

Demographic and clinical characteristics of aquaporin-4 antibody positive neuromyelitis optica spectrum disorder in Canadian adults.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2026

Isolated Paramedian Pontine Reticular Formation (PPRF) syndrome in AQP4 antibody-positive neuromyelitis optica spectrum disorder: a case report.

BMC neurology
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Case Report: Organizing pneumonia associated with AQP4-antibody neuromyelitis optica spectrum disorder in children.

Frontiers in pediatrics
2026

Double Anti-NMO and Anti-MOG Positivity in a Patient With Metastatic Renal Carcinoma: First Reported Case.

Case reports in neurological medicine
2026

The impact of relapse on patient disability, clinical outcomes, and subsequent burdens to patients with neuromyelitis optica spectrum disorder and caregivers.

Multiple sclerosis journal - experimental, translational and clinical
2025

Outcomes and predictors of relapse and severe pneumonia in Chinese patients with AQP4-IgG-positive neuromyelitis optica spectrum disorder receiving inebilizumab: a prospective cohort study.

Frontiers in immunology
2026

Hearing Loss in Neuromyelitis Optica Spectrum Disorder: Case Report and Systematic Review.

Journal of clinical medicine
2026

Changes in Blood Cells and Complements During Relapse Prevention Therapies for Aquaporin-4 Antibody-Positive Neuromyelitis Optica Spectrum Disorder.

International journal of molecular sciences
2026

Unexpected pregnancy in a patient with AQP4-IgG + NMOSD after treatment with inebilizumab: a case report.

BMC neurology
2026

Therapeutic effects of IGF-2 on NMOSD by inhibiting astrocyte apoptosis through modulation of the IGF-1R/PI3K/AKT signaling pathway.

Scientific reports
2026

Tongue atrophy following unexplained nausea, vomiting, and hiccups.

European journal of internal medicine
2026

Consensus Recommendations for the Diagnosis and Treatment of Neuromyelitis Optica Spectrum Disorders (NMOSD): The MENACTRIMS Guidelines.

CNS drugs
2026

AQP4-IgG and mood disorders: Case series of neuromyelitis optica spectrum disorder.

Brain, behavior, &amp; immunity - health
2026

Low prevalence of inflammatory bowel disease among patients with seropositive neuromyelitis optica spectrum disorder in two large referral centers.

Multiple sclerosis and related disorders
2026

Baseline severe disability as a predictor of treatment escalation to rituximab in AQP4-IgG-positive NMOSD patients: A retrospective cohort study.

Multiple sclerosis and related disorders
2026

Neurological and paraclinical features differentiating Systemic lupus erythematosus, Sjögren's disease, and neuromyelitis optica spectrum disorders.

Multiple sclerosis and related disorders
2025

Dysregulated Tfh/B cells and their interactions in neuromyelitis optica spectrum disorder.

Frontiers in immunology
2025

Ravulizumab in NMOSD with associated interstitial pneumonitis in a 59-year-old female patient: a case report.

Frontiers in immunology
2026

Efficacy of fresh frozen plasma and human albumin in therapeutic plasma exchange for refractory neuromyelitis optica spectrum disorders and multiple sclerosis exacerbation: A two-tertiary care center study.

Multiple sclerosis and related disorders
2026

Neuromyelitis Optica Spectrum Disorder Developing after COVID-19 Vaccination Managed with Long-Term Eculizumab Treatment: A Case Report.

Case reports in neurology
2026

Dermatomal pruritus as a heralding symptom in AQP4-positive LETM with anti-Ro52 positivity.

BMJ case reports
2026

Urinary Tract Infection Masquerading as a Neuromyelitis Optica Spectrum Disorder Exacerbation: A Case Report.

Case reports in ophthalmology
2026

Prognostic Modeling for the Failure of Intravenous Methylprednisolone and the Risk of Visual Disability in AQP4-IgG Positive NMOSD-ON.

Translational vision science &amp; technology
2026

Pain in NMOSD is not always neuropathic: multifocal avascular osteonecrosis mimicking neuropathic pain.

BMJ case reports
2026

Relapse-related outcomes in the Danish population-based AQP4- antibody seropositive neuromyelitis optica spectrum disorder cohort.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2026

The future unfolded: CAR T cells and the transformation of treatment algorithms in autoimmune neurology.

Handbook of clinical neurology
2026

Immunotherapies for neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody-associated disease.

Handbook of clinical neurology
2026

The evolution and current status of anti-B-cell therapies in autoimmune neurologic diseases.

Handbook of clinical neurology
2026

Investigating the Frequency and Outcome of Central Vein Sign and Paramagnetic Rim Lesions in Children With MOGAD.

Neurology
2026

Outcomes of Children With Optic Neuritis as Their First Demyelinating Event.

Journal of child neurology
2026

Eculizumab as a rescue therapy in acute-phase aquaporin-4 (AQP4) antibody-positive neuromyelitis optica spectrum disorder.

Journal of neurology
2026

Myelitis-Predominant Aggressive Phenotype: Unveiling Unique Patterns of Late-Onset Neuromyelitis Optica Spectrum Disorders.

Annals of neurology
2026

Persistent Pathological Yawning due to Neuromyelitis Optica Spectrum Disorder.

Annals of Indian Academy of Neurology
2026

Real-world effectiveness and safety of eculizumab in AQP4-IgG-positive neuromyelitis optica spectrum disorder.

Journal of neurology
2026

OCT-Based Differentiation of First Acute Optic Neuritis: An International Study of 111 Patients With NMOSD and MOGAD.

Neurology(R) neuroimmunology &amp; neuroinflammation
2026

Patient Preferences for Neuromyelitis Optica Spectrum Disorder (NMOSD) Treatments: Results from a Discrete Choice Experiment Study in the USA.

The patient
2026

Aquaporin-4-IgG positive radiologically isolated neuromyelitis optica spectrum disorder: treat or observe?

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2025

Astrocyte-derived extracellular vesicles as antigen-specific therapy for neuromyelitis optica spectrum disorder in the mouse model.

bioRxiv : the preprint server for biology
2026

Folate metabolism disorder presenting as an opticospinal syndrome.

Practical neurology
2026

Real-World Efficacy and Safety of Neuromyelitis Optica Spectrum Disorder Disease-Modifying Treatments.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Cerebrospinal Fluid Autoantibody Profiles in Rheumatologic Disorders Affecting the Central Nervous System: A Systematic Review.

Cureus
2026

Remnant cholesterol and lipid ratios predict the relapse of neuromyelitis optica spectrum disorder.

Lipids in health and disease
2026

Screening for Biotinidase deficiency in children with spinal cord demyelination.

Multiple sclerosis and related disorders
2025

Apathy in patients with Neuromyelitis Optica Spectrum Disorder.

PloS one
2025

CAR T cells as novel therapeutic strategy for multiple sclerosis and other neuroimmune disorders.

Journal of neuroinflammation
2025

Prescription Drug Use in NMOSD: A Population-Based Study in Greece with Estimation of National Disease Administrative Prevalence.

Journal of clinical medicine
2025

Glymphatic dysfunction in neuromyelitis optica spectrum disorder.

Frontiers in immunology
2026

Optimizing kappa free light chain index thresholds for multiple sclerosis (MS) diagnosis in low-MS prevalence regions: Insights from an Asian cohort.

Multiple sclerosis (Houndmills, Basingstoke, England)
2026

Glymphatic transport and ocular diseases.

Progress in retinal and eye research
2025

Cerebellar microstructural and functional connectivity changes in patients with neuromyelitis optica spectrum disorders and their correlation with cognitive function: a female-dominated multimodal MRI study.

Frontiers in neurology
2025

The Optic Nerve as a New Diagnostic Frontier in Multiple Sclerosis: How the 2024 McDonald Criteria Leverage Multimodal Evaluation for Earlier Diagnosis.

Cureus
2025

Mitochondrial-Immune Overlap in Leber Hereditary Optic Neuropathy: A Case Report and Lessons Learned.

Reports (MDPI)
2025

Serum Aquaporin-4 Antibody Status and TGF-β in Neuromyelitis Optica Spectrum Disorder: Impact on Astrocyte Function and Correlation with Disease Activity and Severity.

Neurology international
2026

Complement C4d Informs the Differential Diagnosis of Inflammatory Demyelinating CNS Diseases.

Neurology(R) neuroimmunology &amp; neuroinflammation
2026

Progression Independent of Relapse Activity in Aquaporin-4-IgG-Positive NMOSD: A Decade-Long Cohort Study.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

Neuromyelitis Optica Spectrum Disorder Presenting With Longitudinally Extensive Transverse Myelitis and Pontine Involvement in a Patient With Systemic Lupus Erythematosus.

Cureus
2025

The impact of physical and psychological health outcomes on stigma in people with neuromyelitis optica spectrum disorder: A cross-sectional study.

Medicine
2026

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): Mechanisms, Syndromes, and Management.

International ophthalmology clinics
2026

Neuromyelitis Optica Spectrum Disorder.

International ophthalmology clinics
2025

Autoimmune thyroid disease and human health: a systematic review of Mendelian randomization studies.

Frontiers in immunology
2026

Cognitive performances in people with neuromyelitis optica spectrum disorder: a systematic review and meta-analysis.

Multiple sclerosis and related disorders
2025

Progressive Cerebral Atrophy and Cognitive Decline Without Cerebral Lesion in Neuromyelitis Optica Spectrum Disorder: A Case Report.

Cureus
2025

Drug-Induced Lupus Erythematosus After 14 Years of Carbamazepine Use in a Patient With Neuromyelitis Optica Spectrum Disorder: A Case Report.

The American journal of case reports
2025

Very-Late-Onset Neuromyelitis Optica Spectrum Disorder Presenting as Isolated Optic Neuritis in an 85-Year-Old Woman: A Diagnostic and Management Challenge.

Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society
2025

Complement activation in immunological neurological disorders: mechanisms and therapeutic strategies.

Frontiers in neurology
2025

Brain atrophy in NMOSD and MOGAD: a meta-analysis of volumetric and DTI biomarkers.

Frontiers in neurology
2025

Impact of Autoantibody Status on Visual Outcomes in Severe Optic Neuritis Patients Without Multiple Sclerosis.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2026

Anti-SRP antibody-positive polymyositis complicated by neuromyelitis optica spectrum disorder: a case report and literature review.

Modern rheumatology case reports
2025

Multifocal Presentations of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Case Series From a District General Hospital in South East England.

Cureus
2026

Efficacy of a fixed-interval, body surface area-adjusted rituximab regimen in Singaporean patients with CNS inflammatory demyelinating diseases.

Multiple sclerosis and related disorders
2026

Multiple sclerosis research in 2025: earlier diagnosis and halting progression.

The Lancet. Neurology
2025

Tocilizumab in Refractory Neuro Myelitis Optica Spectrum Disorder and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease - Experience from a Tertiary Care Centre.

Annals of Indian Academy of Neurology
2025

Short and Long-Term Effects of Intravenous Methylprednisolone and Plasma Exchange Combination in NMOSD Attacks.

Noro psikiyatri arsivi
2025

Satralizumab treatment in adults with aquaporin-4 immunoglobulin G-seropositive neuromyelitis optica spectrum disorder in clinical practice.

Multiple sclerosis journal - experimental, translational and clinical
2025

Cellular therapy in neuromyelitis optica spectrum disorder.

Frontiers in neurology
2025

Brain volumetry and spinal cord imaging in patients with AQP4-IgG+ NMOSD-a systematic review and meta-analysis.

Therapeutic advances in neurological disorders
2025

Inflammatory transcriptomic signatures in a human cellular NMOSD model reveal upregulation of NF-κB and IL6 pathways.

Scientific reports
2026

Cognitive impairment and multiparametric brain MRI in aquaporin-4-IgG positive neuromyelitis optica spectrum disorder.

Multiple sclerosis and related disorders
2025

Decoding tRNA dynamics in neuroimmune disorders: mechanistic insights, diagnostic innovations, and therapeutic opportunities.

Frontiers in immunology
2025

Alterations in the Expression of Let-7i, miR-21-5p, and miR-30b-5p in Plasma-Derived Extracellular Vesicles as the Possible Prognostic Markers in Central Demyelinating Diseases.

Molecular neurobiology
2026

Evaluation of the Efficacy and Safety of Satralizumab in a Pregnant NMOSD Patient With AQP4/MOG-IgG Dual Seropositive: A Case Report.

Annals of clinical and translational neurology
2026

Characterizing double seronegative neuromyelitis optica spectrum disorder: A distinct subgroup or part of the continuum?

Journal of neuroimmunology
2026

Adherence to the MIND diet and onset of neuromyelitis optica spectrum disorder: A case-control study.

Multiple sclerosis and related disorders
2025

Neuromyelitis Optica-Associated Acute Intermediate Uveitis.

International medical case reports journal
2025

Herpes simplex virus associated rhomboencephalitis mimicking demyelination: a case report.

BMC infectious diseases
2026

Inebilizumab: Pediatric Case Series in Anti-Aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorder.

Pediatric neurology
2026

A real-world comparative study on the efficacy and safety of tocilizumab and rituximab in patients with neuromyelitis optica spectrum disease and myelin oligodendrocyte glycoprotein antibody-associated disease.

Multiple sclerosis and related disorders
2026

Experience of bortezomib use in refractory autoimmune neurological disorders.

Multiple sclerosis and related disorders
2025

Effect of hyperbaric oxygen therapy on peripheral blood inflammatory markers in patients with neuromyelitis optica spectrum disorder: a retrospective cohort study.

Frontiers in neurology
2025

Microglial estrogen receptor 1 signal designates sexual dimorphism of AQP4 antibody-induced neuroinflammation and demyelination.

Cell reports
2026

Pain in neuromyelitis optica spectrum disorder: determination of prevalence and characteristics.

Acta neurologica Belgica
2025

Atypical Neuromyelitis Optica Spectrum Disorder: A Case of Unilateral Optic Neuritis and Partial Transverse Myelitis.

Cureus
2026

Bruton Tyrosine Kinase Inhibition Limits Multiple Sclerosis Disease-Driving Inflammation While Promoting Regulatory B Cells.

Neurology(R) neuroimmunology &amp; neuroinflammation
2026

Nationwide Survey of Atopic Myelitis and Plexin D1-Immunoglobulin G-Related Pain.

Annals of clinical and translational neurology
2025

Peripheral blood immune inflammatory markers in neuromyelitis optica spectrum disorders.

Frontiers in neurology
2025

Effects of eculizumab and rituximab on visual function, motor function and social quality in patients with NMOSD: a comparative study.

Frontiers in neurology
2026

Choroidal structure differs between multiple sclerosis and neuromyelitis optica spectrum disorder: An enhanced depth imaging optical coherence tomography study.

Multiple sclerosis and related disorders
2025

Inebilizuma treatment for acute serum AQP4 - IgG neuromyelitis optica: Case report.

Medicine
2026

Transcriptional expression patterns of orientation dispersion index of white matter in neuromyelitis optica spectrum disorder.

Clinical radiology
2025

CAR T Cell Therapy for Rheumatoid Arthritis.

Clinical reviews in allergy &amp; immunology
2025

Severe attacks and worse prognosis in patients with late-onset neuromyelitis optica spectrum disorder highlight the need for early highly effective treatment.

Journal of neurology
2025

Beyond the optic disc: Investigating gender-based differences in optic neuritis.

World journal of clinical cases
2026

Expanding Evidence of Leptomeningeal Involvement in MOGAD and Its Relevance to Its Pathophysiology.

Neurology(R) neuroimmunology &amp; neuroinflammation
2025

B cell receptor repertoire reconstitution in patients with neuromyelitis optica spectrum disorder receiving B-cell depletion therapy.

Frontiers in immunology
2025

Clinical features of three cases of neurosarcoidosis with a positive aquaporin-4 antibody requiring differentiation from neuromyelitis optica spectrum disorder.

Rinsho shinkeigaku = Clinical neurology
2025

Social alienation and related factors in patients with neuromyelitis optica spectrum disorder: a cross-sectional study.

Multiple sclerosis and related disorders
2025

Risk of hepatitis B and tuberculosis reactivation in patients with neuromyelitis optica spectrum disorder undergoing B cell depletion therapy.

Multiple sclerosis and related disorders
2025

Real-World Investigation of Satralizumab in Patients With Neuromyelitis Optica Spectrum Disease.

Annals of clinical and translational neurology
2025

Consensus recommendations for the diagnosis and management of neuromyelitis optica spectrum disorder: A Saudi expert panel review.

Multiple sclerosis and related disorders
2025

Dual ROCK1/2-MYLK4 Kinase Inhibition Preserves Visual Function in a Rat Model of Neuromyelitis Optica Spectrum Disorder Optic Neuritis.

Cells
2025

Hyperperfusion Improvement: A Potential Therapeutic Marker in Neuromyelitis Optica Spectrum Disorder (NMOSD).

Diagnostics (Basel, Switzerland)
2025

Double-negative NMOSD: from case report to a proposed diagnostic and therapeutic algorithm.

BMC neurology
2026

The real-world experience of biologics for neuromyelitis optica spectrum disorders as first-line and switched therapy.

Journal of neuroimmunology
2025

2025 Brazilian guidelines for the management of neuromyelitis optica spectrum disorder in adults and children.

Arquivos de neuro-psiquiatria
2025

Anterograde and trans-synaptic neurodegeneration in aquaporin-4-antibody neuromyelitis optica spectrum disorder patients with a history of transverse myelitis.

Brain communications
2025

Chimeric Antigen Receptor T-Cell Therapy and Autoimmune Diseases in the Nervous System.

Immunity, inflammation and disease
2025

Clinical value of plasma C1q, C3, and C4 in NMOSD and MOGAD.

Multiple sclerosis and related disorders
Ver todos os 1.718 no EuropePMC

Associações

Organizações que acompanham esta doença — pra ter apoio e orientação

Ainda não temos associações cadastradas para Espectro clínico de neuromielite óptica.

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Doença com base genética

Um médico geneticista pode ajudar no diagnóstico de Espectro clínico de neuromielite óptica e no aconselhamento genético da família.

Vai consultar um especialista? Confirme o registro dele no conselho.
Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

É uma doença autoimune rara que causa crises inflamatórias no sistema nervoso central. Seu impacto principal se dá no nervo óptico e na medula espinhal.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. Early anti-C5 therapy in NMOSD: rationale and clinical evidence.
    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2026PMID 41826590
  2. ORPHA:71211
    Orphanet
  3. GARD:6267
    GARD (NIH)
  4. Q611458
    Wikidata

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Citar este verbete

Raras. (s.d.). Espectro clínico de neuromielite óptica. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/espectro-clinico-de-neuromielite-optica

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Espectro clínico de neuromielite óptica

ORPHA:71211 · MONDO:0019100
🇧🇷 Brasil SUS
SIGTAP
2 procedimentos
Geral
Prevalência
1-9 / 100 000
Herança
Multigenic/multifactorial
CID-10
G36.0 · Neuromielite óptica [doença de Devic]
CID-11
Ensaios
86 ativos
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
2.071 (Worldwide)
MedGen
UMLS
C0027873
EuropePMC
Wikidata
Wikipedia
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Medicamentos aprovados FDA
fonte: FDA OpenFDA
Rara