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Espectro clínico de neuromielite óptica com anticorpos anti-AQP4
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Introdução

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Transtornos do espectro da neuromielite óptica (NMOSD) são um espectro de doenças autoimunes caracterizadas por inflamação aguda do nervo óptico e da medula espinhal (mielite). Episódios de neurite óptica e mielite podem ser simultâneos ou sucessivos. Um curso de doença com recaídas é comum, especialmente em pacientes não tratados. A neuromielite óptica (NMO) é uma doença específica dentro do espectro da NMOSD. É caracterizada por neurite óptica e mielite longitudinalmente extensa. Em mais de 80% dos casos de NMO, a causa são autoanticorpos de imunoglobulina G contra a aquaporina 4 (anti-AQP4), a proteína de canal de água mais abundante no sistema nervoso central.

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

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

BMC neurology2026 Jan 29

Paramedian pontine reticular formation (PPRF) syndrome is characterized by horizontal gaze palsy due to lesions in the PPRF or the abducens nucleus. It is relatively rare and is typically associated with medial longitudinal fasciculus (MLF) syndrome. A 53-year-old Japanese woman presented with right lateral gaze palsy and facial nerve palsy, with preserved convergence and left lateral gaze. Neurological evaluation confirmed PPRF syndrome and facial nerve palsy, with no involvement of the MLF. Brain MRI showed a lesion in the right dorsal pons, affecting the PPRF, abducens nucleus, and facial nerve. Serum testing revealed positive antinuclear and anti-AQP4 antibodies. The patient responded well to immunotherapy, with substantial clinical and radiological improvements. She was ultimately diagnosed with AQP4 antibody-positive NMOSD. This is the first documented case of isolated PPRF syndrome in AQP4 antibody-positive NMOSD. The findings expand the known phenotypic spectrum of NMOSD and highlight the importance of considering NMOSD, with targeted AQP4 antibody testing, in patients presenting with isolated PPRF syndrome.

#2

Progress on experimental models of neuromyelitis optica spectrum disease.

IBRO neuroscience reports2026 Jun

Neuromyelitis optica spectrum disease (NMOSD) is an inflammatory demyelinating disease of the central nervous system (CNS) that primarily affects the optic nerve and spinal cord. At present, the etiology and pathogenesis of NMOSD have not been fully clarified, and its treatment is not perfect, and ideal animal models are a key tool to explore the disease mechanism and screen therapeutic drugs. In recent years, the discovery and confirmation of neuromyelitis optica immunoglobulin G (anti-AQP4 antibodies) as aquaporin-4 (AQP4)-specific antibodies have greatly promoted the study of the pathogenesis of NMOSD, and the study of animal models of NMOSD has become a hot topic of current attention. This article reviews the research progress of NMOSD animal models in recent years, providing a reference for further promoting the in-depth understanding of NMOSD and the formulation of treatment plans.

#3

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

Case reports in neurology2026

Vaccine-associated neuromyelitis optica spectrum disorder (NMOSD) is rare, especially after COVID-19 vaccination. Furthermore, prior cases generally had specific symptoms. We describe a patient who developed NMOSD following vaccination against COVID-19, whose initial symptoms were intractable hiccups and nausea. Thorough clinical examinations, including blood tests and imaging, immediately after symptom onset did not reveal any abnormal findings, and the anti-aquaporin-4 (AQP4) antibody titer was low. The patient's neurological symptoms (left side-dominant abnormal tingling sensation, touch allodynia, and muscle weakness) progressed. Approximately 5 months after symptom onset, a spinal cord lesion was found along the entire cervical cord that partially involved the thoracic cord. Owing to high anti-AQP4 antibody titers, the patient was diagnosed with AQP4-positive NMOSD. Despite steroid pulse therapy, the patient's neurological symptoms progressed daily, eventually resulting in quadriplegia. Concomitant plasmapheresis gradually improved the patient's muscle strength, and after 2 months of rehabilitation, the patient was discharged home. Before discharge, the patient started maintenance therapy with eculizumab and low-dose steroid therapy. Although she continues to experience some pain and numbness, she has not experienced any relapse for at least 2 years. Owing to the risk of severe disease, such as NMOSD, in patients who initially present with nonspecific neurological symptoms following vaccination, careful examination and monitoring are essential to ensure treatment can be started as soon as possible. Once diagnosed, eculizumab is an effective treatment for patients with AQP4-positive NMOSD following vaccination. Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease that affects the brain and nervous system, including the spinal cord and optic nerves. The disease is caused by the immune system, which produces antibodies that mistakenly target a protein, known as aquaporin-4, on the surface of nerve cells. In some patients, this disease develops after vaccination against unrelated infectious diseases. In the last few years, there have been some patients who developed NMOSD after vaccination against SARS-CoV-2, the virus that causes COVID-19. Here, we describe our experience of a patient who developed nonspecific neurological symptoms, including hiccups and weakness, after being vaccinated against SARS-CoV-2. She was diagnosed with aquaporin-4-positive NMOSD about 5 months after the symptoms first appeared. Once this diagnosis was made, she started treatment with eculizumab, a drug that inhibits complement component 5 in the complement cascade, which is part of the immune system. Over 2 years since she started eculizumab, she has not experienced any signs of NMOSD recurrence, although some symptoms, including pain and numbness, continue. Our experience with this patient highlights the risk of developing autoimmune diseases, such as NMOSD, following vaccination. For patients diagnosed with aquaporin-4-positive NMOSD, eculizumab is a potential treatment option that could be considered.

#4

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

Cureus2026 Jan

We report a case of neuromyelitis optica spectrum disorder (NMOSD) relapse in a 45-year-old woman with a known 11-year history of NMOSD, who initially presented with meningitis-like symptoms, including fever, headache, and neck stiffness. Physical examination revealed neck stiffness and positive jolt accentuation, without any other neurological abnormalities. Cerebrospinal fluid (CSF) analysis revealed mononuclear-predominant pleocytosis and hypoglycorrhachia, raising concerns of meningitis. On hospital day 2, brain magnetic resonance imaging (MRI) revealed new hyperintense lesions in the right lateral midbrain and bilateral subcortical regions. That night, the patient developed acute right-sided visual impairment. The patient was diagnosed with NMOSD relapse and was treated with intravenous methylprednisolone pulse therapy, achieving gradual visual improvement. This case highlights that NMOSD can initially present with meningitis-like symptoms, and CSF profiles may resemble those of infectious meningitis. Clinicians should therefore maintain diagnostic vigilance for NMOSD in patients presenting with meningitis-like symptoms, particularly in those with a prior diagnosis of NMOSD or nonadherence to treatment.

#5

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 Feb

Neuromyelitis optica spectrum disorder (NMOSD) is an auto-immune inflammatory disorder of the central nervous system mediated by auto-antibodies against Aquaporin-4 (AQP4). It causes vision impairment, vomiting, and paralysis. We report a case of an 11-year-old Nepalese girl who initially presented with persistent vomiting for 2 months, followed by progressive limb weakness and facial asymmetry. She was initially misdiagnosed as gastroduodenitis with mesenteric lymphadenitis and later with major depressive illness and dissociative motor disorder. Neurological examination revealed multiple cranial nerve palsies and upper motor neuron signs. Magnetic resonance imaging (MRI) of the brain and cervical spine demonstrated long segment T2 hyper-intensities involving the medulla, cervicomedullary junction, and cervical spinal cord which was highly suggestive of transverse myelitis with postrema lesions. Serum anti-AQP4 antibody testing done by indirect immunofluorescence assay was positive which confirmed NMOSD. The patient was treated with intravenous methylprednisolone pulse therapy followed by intravenous immunoglobulin and transitioned to oral prednisolone with gradual improvement in neurological function. This case highlights diagnostic challenge of NMOSD, particularly when presented with area postrema syndrome without early neurological findings. Such atypical presentations are frequently mistaken for gastrointestinal or psychiatric conditions, delaying appropriate diagnosis and management. MRI findings and a positive anti-AQP-4 antibody remain key to confirming the diagnosis. Early initiation of immunotherapy is essential to prevent irreversible neurological deficits. NMOSD should be considered in children presenting with unexplained persistent vomiting and subsequent neurological deficits. A high index of suspicion, prompt antibody testing, and timely immunotherapy are vital for favorable outcomes.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 198

2026

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

Cureus
2026

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

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

BMC neurology
2025

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

Frontiers in pediatrics
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

Progress on experimental models of neuromyelitis optica spectrum disease.

IBRO neuroscience reports
2026

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

Case reports in neurology
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
2025

Changes in T lymphocyte subsets in patients with acute neuromyelitis optica spectrum disorder.

Frontiers in immunology
2025

Mycoplasma pneumoniae infection and concurrent aquaporin-4-positive neuromyelitis optica spectrum disorder: a case report and literature review.

BMC pediatrics
2025

Clinical insights and risk factors for disability in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder patients: a multicenter real-world study in Greece.

Multiple sclerosis and related disorders
2025

Clinical and epidemiological characteristics of late-onset neuromyelitis optica at a tertiary care hospital in Central America.

Multiple sclerosis and related disorders
2025

[AQP4 positive neuromyelitis optica spectrum disorder: an atypical pathology with nerve root enlargement and peripheral nervous system damage in demyelination].

Rinsho shinkeigaku = Clinical neurology
2025

Effective Control of Neuropathic Pain With Amitriptyline in Neuromyelitis Optica Spectrum Disorder: A Case Report.

Cureus
2025

Efficacy of mycophenolate mofetil for the maintenance therapy of anti-AQP4 antibody-positive NMOSD complying with probable sjögren's disease in a 2-year-old girl: a case report.

Pediatric rheumatology online journal
2025

[A case of recurrence after more than 20 years from transverse myelitis suspected to be neuromyelitis optica spectrum disorder].

Rinsho shinkeigaku = Clinical neurology
2025

The role of IL-39 in autoimmune diseases: From general to immunopathogenesis.

Cytokine
2025

Early neuromyelitis optica antibody-induced molecular changes in aquaporin 4 and associated proteins at astrocyte endfeet in murine brain tissues.

Journal of pharmacological sciences
2025

Anti-IL-6R antibody treatment changes microglial phenotype in AQP4 peptide-immunized mice, leading to suppression of myelitis severity.

Journal of neuroimmunology
2025

Confusing Onset of MOGAD in the Form of Focal Seizures.

Neurology international
2025

Transcriptome signature in the blood of neuromyelitis optica spectrum disorder under steroid tapering.

Frontiers in immunology
2025

Novel Automated Chemiluminescent Immunoassay for the Detection of Autoantibodies Against Aquaporin-4 in Neuromyelitis Optica Spectrum Disorders.

Diagnostics (Basel, Switzerland)
2025

Neuromyelitis Optica Spectrum Disorder with Interstitial Pneumonia and Marked HyperCKemia as an Initial Presentation: A Case Report and Literature Review.

The Tohoku journal of experimental medicine
2024

Inebilizumab treatment in a patient with co-occurring AQP4-IgG positive neuromyelitis optica spectrum disorder and myasthenia gravis: a case report and literature review.

Frontiers in immunology
2025

Clinical characteristics of anti-myelin oligodendrocyte glycoprotein antibody among aquaporin-4 negative neuromyelitis optica spectrum disorders in Egyptian patients.

Scientific reports
2024

Prevalence of, and Disability Due to, Multiple Sclerosis and Neuromyelitis Optica Spectrum Disorder in Japan by the Fifth Nationwide Survey.

Neurology
2024

[Efficacy and Safety of Inebilizumab, an Anti-CD19 Monoclonal Antibody, for the Treatment of Neuromyelitis Optica Spectrum Disorder: Based on the N-MOmentum Trial].

Brain and nerve = Shinkei kenkyu no shinpo
2024

Polyautoimmunity and multiple autoimmune syndrome in patients with neuromyelitis optica spectrum disorder.

Multiple sclerosis and related disorders
2024

A rare case of systemic lupus erythematosus-associated neuromyelitis optica spectrum disorder with cystic lesions and dual seropositivity for anti-AQP4 and anti-MOG antibodies.

Clinical case reports
2024

Humanized-Aquaporin-4-Expressing Rat Created by Gene-Editing Technology and Its Use to Clarify the Pathology of Neuromyelitis Optica Spectrum Disorder.

International journal of molecular sciences
2024

Hematopoietic stem cell transplantation for neuromyelitis optica spectrum disorder. Can immune tolerance be reestablished?

Handbook of clinical neurology
2024

Relapsing meningitis and limbic encephalitis in anti-AQP4-Ab-associated neuromyelitis optica spectrum disorder.

Multiple sclerosis (Houndmills, Basingstoke, England)
2024

Neuromyelitis in a Patient with Rheumatoid Arthritis: A Case Report.

Archives of Iranian medicine
2024

Cancer-associated neuromyelitis optica spectrum disorder: a case report with literature review.

Rinsho shinkeigaku = Clinical neurology
2024

Successful treatment with efgartigimod as an add-on therapy in acute attack of anti-AQP4 antibody-positive NMOSD: a case report.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2024

Neuromyelitis Optica Spectrum Disorders (NMOSDs) Diagnosed After Surgery for the Ossification of the Posterior Longitudinal Ligament of the Cervical and Thoracic Spine: A Case Report.

Cureus
2024

Comparison of efficacy of plasma exchange versus intravenous immunoglobulin as an add-on therapy in acute attacks of neuromyelitis optica spectrum disorder.

Journal of clinical apheresis
2024

An Aberrant Case of Neuromyelitis Optica Spectrum Disorder With a Review of Literature.

Cureus
2024

Whole spinal transverse myelitis in neuromyelitis optica spectrum disorder.

Multiple sclerosis and related disorders
2024

Correlation between severe attacks and serum aquaporin-4 antibody titer in neuromyelitis optica spectrum disorder.

Journal of neurology
2024

Stage-dependent immunity orchestrates AQP4 antibody-guided NMOSD pathology: a role for netting neutrophils with resident memory T cells in situ.

Acta neuropathologica
2024

Establishment of novel cell lines that maintain the features of B cells derived from patients with neuromyelitis optica spectrum disorder.

Immunological medicine
2024

Clinical Research into Central Nervous System Inflammatory Demyelinating Diseases Related to COVID-19 Vaccines.

Diseases (Basel, Switzerland)
2024

Infrequent patterns in cerebrospinal fluid isofocusing test: Clinical significance and contribution of IgG index and Reiber diagram to their interpretation.

Multiple sclerosis and related disorders
2024

Peripheral memory B cells in multiple sclerosis vs. double negative B cells in neuromyelitis optica spectrum disorder: disease driving B cell subsets during CNS inflammation.

Frontiers in cellular neuroscience
2024

B cells orchestrate tolerance to the neuromyelitis optica autoantigen AQP4.

Nature
2024

CD11chigh B Cell Expansion Is Associated With Severity and Brain Atrophy in Neuromyelitis Optica.

Neurology(R) neuroimmunology & neuroinflammation
2024

Anti-aquaporin-4 immune complex stimulates complement-dependent Th17 cytokine release in neuromyelitis optica spectrum disorders.

Scientific reports
2024

Neuromyelitis optica associated with the use of Atezolizumab in a patient with advanced lung adenocarcinoma.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

Neuromyelitis optica spectrum disorder with herpes simplex viral infection presenting with syndrome of inappropriate antidiuretic hormone: A case report.

Medicine
2023

[A case of neuromyelitis optica spectrum disorder after human immunodeficiency virus infection treated with rituximab].

Rinsho shinkeigaku = Clinical neurology
2024

Long-term Effects of IL-6 Receptor Blockade Therapy on Regulatory Lymphocytes and Neutrophils in Neuromyelitis Optica Spectrum Disorder.

Neurology(R) neuroimmunology & neuroinflammation
2024

Probable eculizumab-associated hepatotoxicity in a patient with neuromyelitis optica: a case report.

The International journal of neuroscience
2023

Neuromyelitis Optica Spectrum Disorder Mimicking Pontine Stroke: A Case Report and Systematic Literature Review.

Cureus
2024

Overlap syndrome of anti-aquaporin 4 positive neuromyelitis optica spectrum disorder and primary Sjögren's syndrome: a systematic review of individual patient data.

Rheumatology international
2023

Overlap syndrome of anti-aquaporin-4 positive neuromyelitis optica spectrum disorder and systemic lupus erythematosus: A systematic review of individual patient data.

Lupus
2023

Secondary immunoreaction in patients with neurosyphilis and its relevance to clinical outcomes.

Frontiers in neurology
2023

[Neuromyelitis optica spectrum disorder in a patient with human immunodeficiency virus].

Revista medica del Instituto Mexicano del Seguro Social
2023

Establishment of a comprehensive diagnostic model for neuromyelitis optica spectrum disorders based on the analysis of laboratory indicators and clinical data.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2023

Effectiveness of rituximab versus oral immunosuppressive therapies in neuromyelitis optica spectrum disorder in a racially diverse cohort of subjects: A single-center retrospective study.

Multiple sclerosis and related disorders
2023

Clinical characteristics of late-onset neuromyelitis optica spectrum disorder.

Multiple sclerosis and related disorders
2022

A case report of neuromyelitis optica spectrum disorder induced by pembrolizumab treatment for lung adenocarcinoma: a clinical and immunohistochemical study.

BMC neurology
2022

Autoimmune Myelitis and Myocarditis in a Patient With Anti-Aquaporin-4, Antinuclear, and Antiphospholipid Autoantibodies: The Neuromyelitis Optica-Systemic Lupus Erythematosus (NMO-SLE) Overlap Syndrome.

Cureus
2024

Neuromyelitis optica spectrum disorder: pathophysiological approach.

The International journal of neuroscience
2023

A Quality Assessment of Aquaporin-4 & Myelin Oligodendrocyte Glycoprotein Antibody Testing.

The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques
2022

A Case of Neuromyelitis Optica with Systemic Lupus Erythematosus.

Journal of the College of Physicians and Surgeons--Pakistan : JCPSP
2022

Double seropositive neuromyelitis optica associated with COVID-19: A case report.

Frontiers in neurology
2022

A novel aquaporin-4-associated optic neuritis rat model with severe pathological and functional manifestations.

Journal of neuroinflammation
2023

First manifestation of AQP4-IgG-positive neuromyelitis optica spectrum disorder following the COVID-19 mRNA vaccine BNT162b2.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2022

The Prevalence, Demographics, Clinical Features, Neuroimaging, and Inter-ethnic Differences of MOGAD in Malaysia with Global Perspectives.

Multiple sclerosis and related disorders
2022

Enlarged perivascular spaces, neuroinflammation and neurological dysfunction in NMOSD patients.

Frontiers in immunology
2022

Re-examining the characteristics of pediatric multiple sclerosis in the era of antibody-associated demyelinating syndromes.

European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society
2022

Neuromyelitis Optica Spectrum Disorders in Black African: Experience of Togo (2015-2020).

Journal of neurosciences in rural practice
2022

Neuromyelitis optica spectrum disorders and anti-myelin oligodendrocyte glycoprotein positive optic neuropathies.

International journal of ophthalmology
2022

[Local experience of IL-6 pathway inhibition with satralizumab for patients with neuromyelitis optica spectrum disorder].

Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova
2022

Clinical features of organizing pneumonia in anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders.

Respiratory investigation
2022

Strategies to Screen Anti-AQP4 Antibodies from Yeast Surface Display Libraries.

Antibodies (Basel, Switzerland)
2023

Neuromyelitis optica spectrum disorder: do patients positive and negative for anti-aquaporin-4 antibodies present distinct entities? A Colombian perspective.

Neurologia
2022

Immuno-pathogenesis of neuromyelitis optica and emerging therapies.

Seminars in immunopathology
2022

Late onset neuromyelitis optica spectrum disorders (LONMOSD) from a nationwide Portuguese study: Anti-AQP4 positive, anti-MOG positive and seronegative subgroups.

Multiple sclerosis and related disorders
2022

Follow-up of retinal thickness and optic MRI after optic neuritis in anti-MOG antibody-associated disease and anti-AQP4 antibody-positive NMOSD.

Journal of the neurological sciences
2022

[Seroconversion of anti-aquaporin-4 antibody in a patient with neuromyelitis optica spectrum disorder: a case report].

Rinsho shinkeigaku = Clinical neurology
2022

Neuromyelitis Optica Spectrum Disorder in Central America and the Caribbean: A Multinational Clinical Characterization Study.

Neurology international
2022

Treatment regimens for neuromyelitis optica spectrum disorder attacks: a retrospective cohort study.

Journal of neuroinflammation
2022

Pathological observations of a long spinal cord lesion in a patient with multiple sclerosis.

Neuropathology : official journal of the Japanese Society of Neuropathology
2022

Anti-aquaporin-4 immunoglobulin G colorimetric detection by silver nanoparticles.

Nanomedicine : nanotechnology, biology, and medicine
2022

CLINICAL CASE OF NEUROMYELITIS OPTICA SPECTRUM DISORDERS IN YOUNG WOMAN TREATED WITH RITUXIMAB.

Wiadomosci lekarskie (Warsaw, Poland : 1960)
2022

Pediatric neuromyelitis optica spectrum disorders in Portugal: A multicentre retrospective study.

Multiple sclerosis and related disorders
2022

Optical coherence tomography angiography (OCTA) in differential diagnosis of aquaporin-4 antibody seronegative NMOSD and multiple sclerosis.

Multiple sclerosis and related disorders
2023

Development of Paraneoplastic Neuromyelitis Optica after Lung Resection in a Patient with Squamous Cell Carcinoma.

Annals of thoracic and cardiovascular surgery : official journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
2021

Acute Demyelinating Syndromes: A report of child neurology department of Sfax University Hospital.

Multiple sclerosis and related disorders
2021

Regulatory T cells protect against brain damage by alleviating inflammatory response in neuromyelitis optica spectrum disorder.

Journal of neuroinflammation
2021

Inebilizumab for treatment of neuromyelitis optica spectrum disorder.

Neurodegenerative disease management
2021

Five-year visual outcomes after optic neuritis in anti-MOG antibody-associated disease.

Multiple sclerosis and related disorders
2021

Are Zika virus cross-reactive antibodies against aquaporin-4 associated to Neuromyelitis Optica Spectrum Disorder?

Journal of neuroimmunology
2021

[A case of neuromyelitis optica associated with pulmonary Mycobacterium avium complex disease].

Rinsho shinkeigaku = Clinical neurology
2021

Anti-AQP4 autoantibodies promote ATP release from astrocytes and induce mechanical pain in rats.

Journal of neuroinflammation
2021

Conus medullaris involvement in demyelinating disorders of the CNS: A comparative study.

Multiple sclerosis and related disorders
2021

Serum Immunoglobulin G Level and Neutrophils to Lymphocytes Ratio Associated with the Prognosis of Neuromyelitis Optica Spectrum Disorder.

Current neurovascular research
2021

Status of the neuromyelitis optica spectrum disorder in Latin America.

Multiple sclerosis and related disorders
2021

Molecular Level Characterization of Circulating Aquaporin-4 Antibodies in Neuromyelitis Optica Spectrum Disorder.

Neurology(R) neuroimmunology & neuroinflammation
2021

Clinical characteristics of intractable or persistent hiccups and nausea associated with herpes zoster.

Clinical neurology and neurosurgery
2021

[A Case of Neuromyelitis Optica Spectrum Disorder Who Relapsed Under Oral Corticosteroid Treatment with Multiple Cerebrospinal Lesions and Severe Neurological Deficits].

Journal of UOEH
2021

Difference in the Source of Anti-AQP4-IgG and Anti-MOG-IgG Antibodies in CSF in Patients With Neuromyelitis Optica Spectrum Disorder.

Neurology
2021

Paraneoplastic neuromyelitis optica spectrum disorder associated with malignant melanoma: A case report.

Thoracic cancer
2021

Dementia and Parkinson-like syndrome with basal ganglia lesion in neuromyelitis optica spectrum disorders.

Neurocase
2021

[Clinical characteristics in 40 patients with longitudinally extensive transverse myelitis and connective tissue disease].

Zhonghua nei ke za zhi
2021

Longitudinally Extensive Transverse Myelitis: A Retrospective Study Differentiating Neuromyelitis Optica Spectrum Disorder From Other Etiologies.

Cureus
2021

Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD.

BMC neurology
2021

Anti-aquaporin 4 IgG Is Not Associated With Any Clinical Disease Characteristics in Neuromyelitis Optica Spectrum Disorder.

Frontiers in neurology
2021

Longitudinal Evaluation of Serum MOG-IgG and AQP4-IgG Antibodies in NMOSD by a Semiquantitative Ratiometric Method.

Frontiers in neurology
2021

Meningitis as a recurrent manifestation of anti-AQP4/anti-MOG negative neuromyelitis optica spectrum disorder: a case report.

BMC neurology
2021

The efficacy of rituximab in patients with neuromyelitis optica spectrum disorder: A real-world study from Turkey.

International journal of clinical practice
2021

Satralizumab: an interleukin-6 (IL-6) receptor antagonist for the treatment of neuromyelitis optica spectrum disorders.

Drugs of today (Barcelona, Spain : 1998)
2020

Efficacy for the Annual Relapse Rate after the Immunosuppressive Therapy in Patients Associated with Anti-AQP4 or Anti-MOG Antibody-Positive Optic Neuritis.

Journal of ophthalmology
2021

Optic neuritis after ocular trauma in anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder.

Brain and behavior
2021

[A case of spinal cord infarction accompanied with neuromyelitis optica spectrum pathophysiology].

Rinsho shinkeigaku = Clinical neurology
2021

Anti-MOG associated disorder-Clinical and radiological characteristics compared to AQP4-IgG+ NMOSD-A single-center experience.

Multiple sclerosis and related disorders
2021

Aquaporin-4-antibody-positive Neuromyelitis Optica Spectrum Disorder in a Patient with Charcot-Marie-Tooth Disease Type 1A.

Internal medicine (Tokyo, Japan)
2021

Benefits of eculizumab in AQP4+ neuromyelitis optica spectrum disorder: Subgroup analyses of the randomized controlled phase 3 PREVENT trial.

Multiple sclerosis and related disorders
2020

A case of anti-AQP4 antibody-positive neuromyelitis optica spectrum disorder with MRI-proven lesions in lumbar nerve roots.

Multiple sclerosis and related disorders
2021

A case of anti-aquaporin-4 antibody-positive optic neuritis treated by selective immunoadsorption.

Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
2021

Advances in the Treatment of Neuromyelitis Optica Spectrum Disorder.

Neurologic clinics
2020

Rituximab efficacy at different initial and maintenance doses in neuromyelitis optica spectrum disorder: Experience from a national health institute in México.

Journal of the neurological sciences
2020

Recurrent optic neuritis in a patient with Sjogren syndrome and neuromyelitis optica spectrum disorder: A case report.

Medicine
2020

Two cases of NMOSD with MRI findings mimicking CADASIL.

Multiple sclerosis and related disorders
2020

Clinical outcomes of transverse myelitis with myelin oligodendrocyte glycoprotein antibody versus negative cases among adults in Isfahan, Iran: A comparative study.

Current journal of neurology
2020

Aquaporin-4 Expression during Toxic and Autoimmune Demyelination.

Cells
2020

Eculizumab in the treatment of neuromyelitis optica spectrum disorder.

Immunotherapy
2020

Mitochondrial DNA enhance innate immune responses in neuromyelitis optica by monocyte recruitment and activation.

Scientific reports
2020

Recurrent episodes of syncope requiring pacemaker implantation as an initial presentation of neuromyelitis optica spectrum disorder.

Multiple sclerosis and related disorders
2021

An update on epidemiology, diagnosis & management of NMO-SD in the tertiary neurology department of Marrakech (Morocco).

Revue neurologique
2020

The relationship between aquaporin-4 antibody status and visual tract integrity in neuromyelitis optica spectrum disorders: A visual evoked potential study.

Multiple sclerosis and related disorders
2020

Unilateral Optic Neuritis in Children: Experience of a Tertiary Centre.

European neurology
2020

Effect of rituximab on disease activity in latin American patients with anti-aquaporin-4 (+) neuromyelitis optica spectrum disorder.

Clinical neurology and neurosurgery
2020

Anti-MOG and Anti-AQP4 positive neuromyelitis optica spectrum disorder in a patient with myasthenia gravis.

Multiple sclerosis and related disorders
2020

Moving beyond anti-aquaporin-4 antibodies: emerging biomarkers in the spectrum of neuromyelitis optica.

Expert review of neurotherapeutics
2019

A new M23-based ELISA assay for anti-aquaporin 4 autoantibodies: diagnostic accuracy and clinical correlation.

Auto- immunity highlights
2020

Current and emerging biologics for the treatment of neuromyelitis optica spectrum disorders.

Expert opinion on biological therapy
2020

Neuromyelitis optica spectrum disorders in Arabian Gulf (NMOAG); establishment and initial characterization of a patient registry.

Multiple sclerosis and related disorders
2020

Clinical and immunological differences between MOG associated disease and anti AQP4 antibody-positive neuromyelitis optica spectrum disorders: Blood-brain barrier breakdown and peripheral plasmablasts.

Multiple sclerosis and related disorders
2020

Two Cases of Very-Late-Onset Neuromyelitis Optica Spectrum Disorder (NMOSD) in Patients over the Age of 80.

Case reports in neurology
2020

Use of ganglion cell layer analysis for diagnosing anti-glycoprotein neuromyelitis optica of oligodendrocyte myelin.

Archivos de la Sociedad Espanola de Oftalmologia
2020

Tacrolimus is effective for neuromyelitis optica spectrum disorders with or without anti-AQP4 antibody.

Multiple sclerosis and related disorders
2020

HLA-G Ins/Del polymorphism and +3142C/G SNP are not related to neuromyelitis optica spectrum disorder (NMOSD) development, disability status or anti-aquaporin 4 presence in Brazilian patients.

Journal of neuroimmunology
2019

Predictive Value of Serum Antibodies and Point Mutations of AQP4, AQP1 and MOG in A Cohort of Spanish Patients with Neuromyelitis Optica Spectrum Disorders.

International journal of molecular sciences
2019

[Optic neuropathy in positive anti-MOG antibody syndrome].

Journal francais d'ophtalmologie
2020

HA280 immunoadsorption, an alternative treatment for neuromyelitis optica spectrum disorders?

Multiple sclerosis and related disorders
2020

Neuromyelitis optica spectrum disorders in South of Tunisia: A rare entity with low seroprevalence of anti-aquaporin 4 autoantibodies.

Revue neurologique
2020

Affinity-matured 'aquaporumab' anti-aquaporin-4 antibody for therapy of seropositive neuromyelitis optica spectrum disorders.

Neuropharmacology
2019

GRP 78 antibodies are associated with clinical phenotype in neuromyelitis optica.

Annals of clinical and translational neurology
2019

The accuracy of flow cytometric cell-based assay to detect anti-myelin oligodendrocyte glycoprotein (MOG) antibodies determining the optimal method for positivity judgement.

Journal of neuroimmunology
2019

Intractable axonal neuropathy with multifocal peripheral nerve swelling in neuromyelitis optica spectrum disorders: A case report.

Multiple sclerosis and related disorders
2019

Incidence of pediatric neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody-associated disease in Denmark 2008‒2018: A nationwide, population-based cohort study.

Multiple sclerosis and related disorders
2019

Disease course, progression and activity of neuromyelitis optica (NMOSD) in patients who were treated with Rituximab, 6 and 12 months after receiving the first dose of drug, in Isfahan city.

Multiple sclerosis and related disorders
2019

Optic neuritis, encephalitis and leptomeningeal enhancement in a patient with anti-MOG antibodies: A case study.

Multiple sclerosis and related disorders
2019

Assessment of lesions on magnetic resonance imaging in multiple sclerosis: practical guidelines.

Brain : a journal of neurology
2019

The clinical spectrum of anti-MOG associated acquired demyelinating disorders: Three case-reports.

Multiple sclerosis and related disorders
2019

HLA-alleles class I and II associated with genetic susceptibility to neuromyelitis optica in Brazilian patients.

Arquivos de neuro-psiquiatria
2019

Early B cell tolerance defects in neuromyelitis optica favour anti-AQP4 autoantibody production.

Brain : a journal of neurology
2019

Human Serum Anti-aquaporin-4 Immunoglobulin G Detection by Cell-based Assay.

Journal of visualized experiments : JoVE
2019

Case report: an area postrema syndrome revealing a neuromyelitis optica spectrum disorder associated with central nervous system tuberculosis in a young Togolese (black African) woman.

BMC neurology
2019

Inhibitors of Mammalian Aquaporin Water Channels.

International journal of molecular sciences
2019

Quantitative 7T MRI does not detect occult brain damage in neuromyelitis optica.

Neurology(R) neuroimmunology & neuroinflammation
2019

[Neuromyelitis optica spectrum disorders positive for anti-AQP4 antibody associated with myasthenia gravis: A literature review].

Journal francais d'ophtalmologie
2019

Pontine hemorrhage accompanied by neuromyelitis optica spectrum disorder.

Journal of neuroimmunology
2019

Astrocytic damage in glial fibrillary acidic protein astrocytopathy during initial attack.

Multiple sclerosis and related disorders
2020

Area postrema syndrome: Another feature of anti-GFAP encephalomyelitis.

Multiple sclerosis (Houndmills, Basingstoke, England)
2017

Neurological Complications of Sjögren's Syndrome: Diagnosis and Management.

Current treatment options in rheumatology
2019

Neuromyelitis optica spectrum disorder: Pathogenesis, treatment, and experimental models.

Multiple sclerosis and related disorders
2019

Clinical characteristics of 153 Brazilian patients with neuromyelitis optica spectrum disorder (NMOSD).

Multiple sclerosis and related disorders
2018

Rituximab-induced hypogammaglobulinemia in patients with neuromyelitis optica spectrum disorders.

Neurology(R) neuroimmunology & neuroinflammation
2018

Prevalence of anti-aquaporin 4 antibody in a diagnostic cohort of patients being investigated for possible neuromyelitis optica spectrum disorder in Western Australia.

Journal of neuroimmunology
2018

[Demyelinating disorders].

Medicina
2018

Diagonistic Apraxia: A Unique Case of Corpus Callosal Disconnection Syndrome and Neuromyelitis Optica Spectrum Disorder.

Frontiers in neurology
2018

Devic's syndrome and mixed connective tissue disease: an unusual association.

Acta reumatologica portuguesa
2019

Helicobacter pylori infection and demyelinating disease of the central nervous system.

Journal of neuroimmunology
2018

Intractable nausea and vomiting as an uncommon presentation in an anti-aquaporin 4-positive patient.

The Journal of international medical research
2018

Neuromyelitis optica in Sub-Saharan Africa: the first case report from Togo.

Medecine et sante tropicales
2018

Immunoadsorption plasmapheresis treatment for the recurrent exacerbation of neuromyelitis optica spectrum disorder with a fluctuating anti-aquaporin-4 antibody level.

Journal of artificial organs : the official journal of the Japanese Society for Artificial Organs
2018

Refractory neuromyelitis optica spectrum disorder in systemic lupus erythematosus successfully treated with rituximab.

Lupus
2018

Recombinant IgG1 Fc hexamers block cytotoxicity and pathological changes in experimental in vitro and rat models of neuromyelitis optica.

Neuropharmacology
2018

Neuromyelitis optica spectrum disorder secondary to treatment with anti-PD-1 antibody nivolumab: the first report.

BMC cancer
2017

The single nucleotide polymorphism site of aquaporin-4 gene in patients with neuromyelitis optica.

Experimental and therapeutic medicine
2017

Subclinical retinal atrophy in the unaffected fellow eyes of multiple sclerosis and neuromyelitis optica.

Journal of neuroimmunology
2017

Radiculopathy in neuromyelitis optica. How does anti-AQP4 Ab involve PNS?

Multiple sclerosis and related disorders
2017

[A case of recurrent myelitis associated with anti-myelin oligodendrocyte glycoprotein antibody that developed only as localized short spinal cord lesions].

Rinsho shinkeigaku = Clinical neurology
2017

Gender differences among Chinese patients with neuromyelitis optica spectrum disorders.

Multiple sclerosis and related disorders
2017

Anti-aquaporin-4 titer is not predictive of disease course in neuromyelitis optica spectrum disorder: A multicenter cohort study.

Multiple sclerosis and related disorders
2017

A case of MOG antibody-positive bilateral optic neuritis and meningoganglionitis following a genital herpes simplex virus infection.

Multiple sclerosis and related disorders
2017

Diagnostics of the neuromyelitis optica spectrum disorders (NMOSD).

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2017

[Clinical characteristics of neuromyelitis optica spectrum disorders associated with syringomyelia].

Zhonghua yi xue za zhi
2017

Glucose-regulated protein 78 autoantibody associates with blood-brain barrier disruption in neuromyelitis optica.

Science translational medicine
2017

Aquaporin-4 antibody positive neuromyelitis optica spectrum disorder associated with esophageal cancer.

Journal of neuroimmunology
2017

A Case of Primary Sjögren's Syndrome Presenting as Mass-Like Encephalitis, With Progression to Neuromyelitis Optica Spectrum Disorder.

Archives of rheumatology
2017

Screening for onconeural antibodies in neuromyelitis optica spectrum disorders.

BMC neurology
2017

Deletional tolerance prevents AQP4-directed autoimmunity in mice.

European journal of immunology

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Isolated Paramedian Pontine Reticular Formation (PPRF) syndrome in AQP4 antibody-positive neuromyelitis optica spectrum disorder: a case report.
    BMC neurology· 2026· PMID 41612254mais citado
  2. Progress on experimental models of neuromyelitis optica spectrum disease.
    IBRO neuroscience reports· 2026· PMID 41550976mais citado
  3. Neuromyelitis Optica Spectrum Disorder Developing after COVID-19 Vaccination Managed with Long-Term Eculizumab Treatment: A Case Report.
    Case reports in neurology· 2026· PMID 41550453mais citado
  4. Neuromyelitis Optica Spectrum Disorder Initially Presenting With Meningitis-Like Symptoms: A Case Report.
    Cureus· 2026· PMID 41700230mais citado
  5. 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· PMID 41675751mais citado
  6. Low prevalence of inflammatory bowel disease among patients with seropositive neuromyelitis optica spectrum disorder in two large referral centers.
    Mult Scler Relat Disord· 2026· PMID 41576642recente
  7. Double-negative NMOSD: from case report to a proposed diagnostic and therapeutic algorithm.
    BMC Neurol· 2025· PMID 41225357recente
  8. Clinical insights and risk factors for disability in aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder patients: a multicenter real-world study in Greece.
    Mult Scler Relat Disord· 2025· PMID 41027273recente
  9. Clinical and epidemiological characteristics of late-onset neuromyelitis optica at a tertiary care hospital in Central America.
    Mult Scler Relat Disord· 2025· PMID 40974625recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:592850(Orphanet)
  2. MONDO:0035663(MONDO)
  3. Neuromielite Optica(PCDT · Ministério da Saúde)
  4. GARD:22372(GARD (NIH))
  5. Busca completa no PubMed(PubMed)

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.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Espectro clínico de neuromielite óptica com anticorpos anti-AQP4
Compêndio · Raras BR

Espectro clínico de neuromielite óptica com anticorpos anti-AQP4

ORPHA:592850 · MONDO:0035663
🇧🇷 Brasil SUS
CEAF
1AEculizumabeRavulizumabeSatralizumabeInebilizumabe
Geral
CID-10
G36.0 · Neuromielite óptica [doença de Devic]
CID-11
MedGen
UMLS
C5680295
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