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• #1 Neuromyelitis Optica – EyeWiki

  https://eyewiki.org/Neuromyelitis_Optica

  Neuromyelitis optica (NMO) is an inflammatory disease that causes demyelination of the central nervous system, primarily affecting the optic nerve (optic neuritis) and the spinal cord. […] The precise etiology of NMOSD remains to be completely defined. In the past there was debate as to whether NMO represented a variant of multiple sclerosis (MS), recent evidence however suggests that NMOSD has a completely different pathogenesis, pathology, mechanism of disease, presentation, course, treatment, and prognosis than MS. […] The pathophysiology of NMOSD mainly involves the humoral immune system. NMO is characterized by a disease specific IgG antibody against the astrocytic aquaporin 4 (AQP4) water channel (also known as the aquaporin-4 autoantibody (anti-AQP4 or AQP4-IgG). […] There are still unknown elements in the pathophysiology of NMOSD including the mechanism for loss of tolerance and anti-AQP4 formation, pathogenesis of seronegative NMOSD, and the mechanisms that anti-AQP4 breach the blood brain barrier.

• #2 Neuromyelitis Optica Spectrum Disorder | SpringerLink

  https://link.springer.com/10.1007/978-3-031-60006-7_16

  Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system, also known as Devics syndrome, that typically manifests with optic neuritis and transverse myelitis and, like other antibody-mediated autoimmune diseases, primarily affects women. Most NMO cases are caused by a circulating autoantibody termed NMO-IgG or AQP4-IgG that targets the astrocytic water channel protein aquaporin-4 (AQP4). Some NMO cases are caused by a circulating autoantibody against the myelin oligodendrocyte glycoprotein termed MOG-IgG. A small proportion of NMO cases, termed seronegative NMO, are not associated with an autoantibody. AQP4-IgG binds extracellular conformational epitopes on AQP4, activates complement, which in turn causes inflammatory cell infiltration, demyelination and pan-necrosis. […] The discoveries of AQP4-IgG and MOG-IgG have shown that NMO is a distinct entity from multiple sclerosis with fundamentally different pathophysiology and treatment.

• #3 Neuromyelitis optica spectrum disorder | MedLink Neurology

  https://www.medlink.com/articles/neuromyelitis-optica-spectrum-disorders

  The etiology of neuromyelitis optica spectrum disorder remains unknown, as with other autoimmune diseases. About 10% to 20% of patients with neuromyelitis optica spectrum disorder have another coexisting autoimmune disease, notably Sjgren disease, myasthenia gravis, systemic lupus erythematosus, and systemic sclerosis. A greater percentage (approximately 50%) have autoantibodies, such as antinuclear antibodies, extractable nuclear antigens, Sjgren syndrome antigen A/B (SSA/SSB), antithyroglobulin, antithyroid microsomal, antigastroparietal cell, and p-antineutrophil cytoplasmic antibodies (some of which can be disease-causing). It appears that neuromyelitis optica spectrum disorder occurs as a separate comorbid condition in patients with preexisting systemic autoimmune disease. Patients with neuromyelitis optica spectrum disorder rarely have autoantibodies to paraneoplastic disease markers, such as acetylcholine receptors, glutamate decarboxylase 65, voltage-gated potassium channels, and voltage-gated calcium-channels. The association of neuromyelitis optica spectrum disorder with a variety of autoantibodies suggests widespread B cell polyclonal activation.

• #4 What Is Neuromyelitis Optica Spectrum Disorder (NMOSD)? Symptoms, Causes, Diagnosis, Treatment, and Prevention

  https://www.everydayhealth.com/neurology/neuromyelitis-optica-spectrum-disorder/guide/

  Neuromyelitis optica spectrum disorder (NMOSD) happens when your immune system targets myelin, a substance that forms a protective layer around nerves. Its unknown what causes this autoimmune attack. […] In all autoimmune disorders including NMOSD, the immune system mistakenly attacks your bodys own healthy tissue the way it normally attacks outside invaders like bacteria or viruses. Some people with NMOSD report having an upper respiratory infection before the onset of their symptoms, but this possible connection isnt well understood. […] Since doctors dont know what leads to the autoimmune attack on your nerves in NMOSD, the condition is not considered preventable.

• #5 Neuromyelitis Optica (NMO): What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/9858-neuromyelitis-optica-nmo

  Neuromyelitis optica (NMO) is an autoimmune disease, meaning it happens when your immune system attacks parts of your nervous system. […] Neuromyelitis optica occurs when the protective coating around part of certain nerve cells degrades, leading to nerve damage. […] Neuromyelitis optica (NMO) is a rare chronic condition that affects your eyesight and your ability to move. Its an autoimmune disorder, meaning it happens because your immune system mistakenly attacks parts of your central nervous system. […] Experts previously thought that NMO was a rare type of multiple sclerosis (MS). However, they know now that its a separate condition. […] NMO is much more likely to affect females, who make up about 80% to 90% of cases. […] NMO overall is rare. The average number of people with NMO per 100,000 is between 0.3 and 4.4.

• #6 Neuromyelitis optica Information | Mount Sinai – New York

  https://www.mountsinai.org/health-library/diseases-conditions/neuromyelitis-optica

  Neuromyelitis optica (NMO) is a disorder of the central nervous system. NMO is a type of autoimmune disorder. Autoimmune disorders are conditions where the body’s immune system destroys healthy cells and tissues. NMO causes damage to a type of nerve cell called astrocytes as well as the myelin sheath of nerve cells. There is no identifiable cause for NMO. It may occur after an infection. People with a personal or family history of autoimmune disorders may also be at risk. […] NMO is often confused with and misdiagnosed as multiple sclerosis (MS) because they have similar symptoms. However, the two conditions are different and have different treatments.

• #7 Neuromyelitis Optica | National Institute of Neurological Disorders and Stroke

  https://www.ninds.nih.gov/health-information/disorders/neuromyelitis-optica

  Neuromyelitis optica (NMO) is an autoimmune disease of the central nervous system (CNS) that mainly affects the optic nerves and spinal cord. […] The disease is caused by abnormal autoantibodies that bind (attach) to a protein called aquaporin-4, activating other parts of the immune system and causing inflammation and damage to cells. […] NMO is different from multiple sclerosis (MS). […] Most individuals with NMO experience clusters of attacks days to months or years apart, followed by partial recovery during periods of remission. […] African Americans are at greater risk of the disease. […] The onset of NMO varies from childhood to adulthood, with two peaks, one in childhood and the other in adults in their 40s.

• #8 Neuromyelitis optica spectrum disorders: from pathophysiology to therapeutic strategies | Journal of Neuroinflammation | Full Text

  https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-021-02249-1

  Neuromyelitis optica (NMO) is caused by a pathogenic serum IgG antibody against the water channel aquoporin 4 (AQP4) in the majority of patients. […] AQP4-antibody (AQP4-ab) presence is highly specific, and differentiates NMO from multiple sclerosis. […] Binding of AQP4-ab to astrocyte AQP4 channels triggers classical complement cascade activation, followed by granulocyte, eosinophil, and lymphocyte infiltration, culminating in injury first to astrocytes, then oligodendrocytes, demyelination, neuronal loss, and neurodegeneration. […] AQP4-ab-positive NMOSD is characterized by AQP4 loss, dystrophic astrocytes, and absence of cortical demyelination. […] The frequency of MOG-ab and AQP4-ab coexistence was exceptionally reported, suggesting that both have different immunopathogenic mechanisms.

• #9 Neuromyelitis optica spectrum disorders: from pathophysiology to therapeutic strategies | Journal of Neuroinflammation | Full Text

  https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-021-02249-1

  The autoimmune response in NMOSD primarily affects astrocytes and is initiated by autoantibody-mediated loss of AQP4. […] AQP4-ab in NMO serum are IgG1, a subclass of mature IgG that requires help from T cells, indicating that AQP4-specific CD4+ T cells participate in the genesis of this adaptive humoral response. […] AQP4-ab binds to extracellular epitopes of AQP4 present on the astrocyte plasma membrane. […] In CDC, classical pathway activation begins when the multivalent protein C1q binds to the conformational Fc determinant on IgG or IgM antibody-antigen complexes, producing cellular injury by formation of a pore-like membrane attack complex (MAC). […] Studies in experimental animal models as well as in human NMOSD lesions show polymorphonuclear leukocytes (PMNs) as the key determinants of BBB permeability and NMOSD lesion formation.

• #10 Neuromyelitis Optica Spectrum Disorder | National MS SocietyNational Multiple Sclerosis Society LogoNational Multiple Sclerosis Society LogoOpen searchExpand SectionExpand SectionExpand SectionExpand SectionExpand SectionExpand SectionExpand SectionExpand

  https://www.nationalmssociety.org/understanding-ms/what-is-ms/related-conditions/nmosd

  Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease in which the immune system thinks the healthy tissues of the central nervous system are a threat and attacks them. […] We do not know the root cause of the demyelination and inflammation that occurs in NMOSD. However, scientists do understand what happens in the body. The immune system attacks the central nervous system, producing inflammation. The inflammation causes damage and demyelination, which produces the symptoms you experience. […] In most people with NMOSD, blood tests detect specific antibodies (anti-AQP4) that attack Aquaporin-4 (AQP4) in the central nervous system. AQP4 is a protein on astrocytes, a type of cell that helps the nerves in the central nervous system work properly. This attack leads to inflammation and damage to the astrocytes, as well as to the nerve cells and their myelin insulation. The resulting damage disrupts the messages from the brain and spinal cord to the rest of the body, causing weakness, worsened vision or other symptoms.

• #11 Neuromyelitis optica symptoms and causes

  https://www.medicalnewstoday.com/articles/symptoms-of-neuromyelitis-optica

  Researchers do not know the exact cause of NMO. While more than 95% of people with NMO have no family history of the disease, 50% have a personal or family history of other autoimmune disorders, according to the National Organization for Rare Disorders. […] In NMO, the immune system attacks proteins on the surface of supportive cells in the brain, spine, and optic nerves. This damages the cells and leads to the loss of protective fatty tissues (called myelin) that surround the nerves, in a process called demyelination. […] For an estimated 80% of people with NMO, the body develops AQP4 antibodies (NMO-IgG or anti-AQP4 antibody) that attack the AQP4 water channel. This channel allows water to pass through cell membranes. […] Up to 1 in 3 people who do not test positive for AQP4 antibodies have another antibody that attacks myelin, called myelin oligodendrocyte glycoprotein (MOG).

• #12 Neuromyelitis optica spectrum disorder – Wikipedia

  https://en.wikipedia.org/wiki/Neuromyelitis_optica_spectrum_disorder

  Neuromyelitis optica (NMO) is a particular disease within the NMOSD spectrum. It is characterised by optic neuritis and longitudinally extensive myelitis. In more than 80% of NMO cases, the cause is immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the central nervous system. […] NMOSD is caused by an autoimmune attack on the nervous system. In more than 80% of cases, IgG autoantibodies against aquaporin-4 (anti-AQP4+) are the cause, and in 10-40% of the remaining cases, IgG antibodies against MOG are the cause. The cause of the remaining cases is still unknown, and it is likely heterogeneous. […] Why autoimmunity develops is largely unknown. Multiple genetic and environmental factors are known to increase the risk of developing NMOSD. The strongest risk factor is being female, especially in AQP4-IgG-positive NMOSD. Multiple human leukocyte antigen (HLA) alleles are associated with NMOSD.

• #13 Neuromyelitis Optica | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/neuromyelitis-optica

  Neuromyelitis optica is an autoimmune disorder, which means the body mistakenly attacks its own healthy tissue. In NMO, the immune system targets the optic nerves and spinal cord, causing inflammation and swelling that results in injury and loss of function. […] As with other autoimmune diseases, the cause of NMO remains unknown. About 10% to 20% of patients with NMO have another autoimmune disorder, such as systemic lupus erythematosus, Sjogren syndrome, myasthenia gravis, or systemic sclerosis. […] Most patients with NMO have elevated levels of an antibody called aquaporin-4 (anti-AQP4, or NMO-IgG) which is believed to play a direct role in the cause of the disease. Some NMO syndromes are associated with anti-myelin oligodendrocyte glycoprotein (MOG) antibodies instead of anti-AQP4 antibodies, and these patients may have clinically less severe disease with fewer relapses.

• #14 Improving the Diagnosis of Neuromyelitis Optica Spectrum Disorder – Ophthalmology Advisor

  https://www.ophthalmologyadvisor.com/cch/improving-the-diagnosis-of-neuromyelitis-optica-spectrum-disorder/

  Serum autoantibodies specific to myelin oligodendrocyte glycoprotein (MOG) found on myelin sheath surfaces present a new potential marker in support of a diagnosis of AQP4-seronegative NMOSD. […] The antibodies have been identified in the serum of 25% to 42% of AQP4-seronegative patients, suggesting an alternate pathophysiology that might lead to NMOSD.

• #15 Orphanet: Neuromyelitis optica spectrum disorder

  https://www.orpha.net/en/disease/detail/71211

  Etiology is unknown but NMO is believed to be an autoimmune disease associated with autoantibodies to aquaporin-4 (AQP4) in 70% and autoantibodies to myelin oligodendrocyte glycoprotein (MOG) in 15% of patients. […] 15% of patients are double seronegative for these antibodies. […] Patients with autoantibodies to MOG have overlapping clinical features, but have demographic and clinical differences from patients with autoantibodies to AQP4 and patients with these antibodies are diagnosed with MOG associated disorders (MOGAD).

• #16 Neuromyelitis Optica: Symptoms, Causes, Diagnosis & Treatment

  https://www.healthline.com/health/neuromyelitis-optica/neuromyelitis-optica

  Neuromyelitis optica (NMO) is disease thats caused by an autoimmune attack on your optic nerve and spinal cord. […] Neuromyelitis optica (NMO) is caused by the malfunction of your immune system. Your body creates antibodies that attack a type of protein that moves water through your cells, called an anti-AQP4 antibody. […] In some people, anti-MOG antibodies may also be present. These are the antibodies common in MS that strip the insulating layer of nerves and exposes them to damage. […] Unlike other autoimmune diseases, NMO rarely has a genetic connection. Less than 3 percent of people with NMO have an affected relative. […] Its been suggested that NMO affects people from some ethnic backgrounds like Indian, Asian, and Black more than others, but a 2018 study found no real difference in prevalence between ethnic groups. […] Some risk factors that have been identified in people with NMO include: gastrointestinal infections like H. pylori and C. Diff, vitamin D deficiency, smoking, having other autoimmune diseases.

• #17 What Is the Life Expectancy of Someone With NMO? NMO vs. MS

  https://www.medicinenet.com/what_is_the_life_expectancy_of_someone_with_nmo/article.htm

  Neuromyelitis optica is a rare inflammatory autoimmune disorder that primarily affects the optic nerve and the spinal cord, and sometimes the brain. […] NMO is the result of an abnormal attack on healthy nerve cells by the immune system. Antibodies (IgG) in the immune system, for unknown reasons, consider certain proteins in the central nervous system a threat and attack them. NMO is caused by two types of antibodies that attack different proteins in the optic nerve and spinal cord: […] In the relapsing type of NMO, anti-AQP4 antibodies (NMO-IgG) form and attack aquaporin-4 protein, damaging the myelin and nerve fibers. […] Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies attack MOG, a component of myelin. […] It is not clear what exactly triggers the autoimmune attack in NMO and it doesn’t appear to be an inherited condition. Only about 3% of NMO patients report having a relative with the condition, but approximately 50% of patients have a personal or family history of other autoimmune disorders.

• #18 Neuromyelitis optica spectrum disorder | MedLink Neurology

  https://www.medlink.com/articles/neuromyelitis-optica-spectrum-disorders

  Neuromyelitis optica spectrum disorder has been associated with infections, such as herpes viruses (human herpesvirus 6, HSV-2, and varicella), Mycoplasma pneumoniae, tuberculosis, Epstein-Barr virus, and HIV. Neuromyelitis optica spectrum disorder could be triggered by these chronic infections in hosts who have preexisting vulnerability or by B-cell activation. […] The role of genetic factors in neuromyelitis optica spectrum disorder is unknown. There are reports of familial occurrence, but no conclusive evidence exists. There is evidence that neuromyelitis optica spectrum disorder is associated with human leukocyte antigen alleles DR1*801, DP1 501, and DPA1 202, but not with DRB1*1501, which is associated with multiple sclerosis. A 2018 study looking at neuromyelitis optica spectrum disorder and healthy controls using whole genome sequencing suggested that certain genetic variants in the MHC region play a role in the pathology of neuromyelitis optica spectrum disorder.

• #19 Neuromyelitis Optica Spectrum Disorder (NMOSD) – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK572108/

  Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon yet impactful inflammatory and demyelinating disorder of the central nervous system. […] The etiology of NMOSD is still unclear. In the past, NMOSD was considered a variant of MS, but recent studies have elucidated the differences in the disease process, manifestations, and management. Whole-genome sequencing has identified some at-risk genotypes for NMOSD, and major histocompatibility complexes (including HLA-DRB1*03:01) have been identified. […] The genetic variant of major histocompatibility complexes of NMOSD is more similar to systemic lupus erythematosus (SLE) than MS.

• #20 A whole-genome sequence study identifies genetic risk factors for neuromyelitis optica | Nature Communications

  https://www.nature.com/articles/s41467-018-04332-3

  Neuromyelitis optica (NMO) is a rare autoimmune disease that affects the optic nerve and spinal cord. Most NMO patients (70%) are seropositive for circulating autoantibodies against aquaporin 4 (NMO-IgG+). […] We identify two independent signals in the major histocompatibility complex (MHC) region associated with NMO-IgG+, one of which may be explained by structural variation in the complement component 4 genes. […] Mendelian Randomization analysis reveals a significant causal effect of known systemic lupus erythematosus (SLE), but not multiple sclerosis (MS), risk variants in NMO-IgG+. Our results suggest that genetic variants in the MHC region contribute to the etiology of NMO-IgG+ and that NMO-IgG+ is genetically more similar to SLE than MS. […] To elucidate genetic factors driving NMO risk and to clarify the genetic architecture of this disease, we analyzed up to 6.8 million single-nucleotide polymorphisms (SNPs) and performed copy number variation (CNV) analysis on 215 cases and 1244 controls of European ancestry.

• #21 A whole-genome sequence study identifies genetic risk factors for neuromyelitis optica | Nature Communications

  https://www.nature.com/articles/s41467-018-04332-3

  In this study, we find two independent significant genetic signals in the major histocompatibility complex (MHC) region associated with NMO. […] We also provide initial evidence that suggest NMO-IgG+ is genetically more similar to systemic lupus erythematosus (SLE) than to MS. […] A significant association was identified in the NMO-IgG+ group (OR=6.21, P=1.0109) for reduced copy number in the region annotated by a complex structural site on the location of complement component 4 (C4). […] The cumulative evidence suggests that C4 deletions may be the functional driver of the association with NMO-IgG+. […] The association of the same C4 deletions in SLE and NMO-IgG+ and the genetic link observed in the Mendelian Randomization study between SLE and NMO-IgG+ suggest a potential common strategy for therapeutic targeting of autoantibody production.

• #22 Neuromyelitis optica (NMO)

  https://www.nhs.uk/conditions/neuromyelitis-optica/

  Neuromyelitis optica (NMO) is an autoimmune condition. It happens when your immune system attacks nerves in your eyes or your spinal cord. […] It’s not known what causes it. Sometimes it happens after a virus such as flu. […] NMO is not an inherited condition. But it’s more common in people with other autoimmune conditions, and those with close relatives who have autoimmune conditions. […] It’s more common in women and people of Asian or Black African origin.

• #23 Neuromyelitis Optica – Child Neurology Foundation

  https://www.childneurologyfoundation.org/disorder/neuromyelitis-optica/

  No single factor has been shown to cause neuromyelitis optica. […] We do know that: […] Some infections can trigger NMO […] Smoking and exposure to cigarette smoke increases the risk of NMO.

• #24 What Causes Neuromyelitis Optica? | MyMSTeam

  https://www.mymsteam.com/resources/what-causes-neuromyelitis-optica

  Some people with NMOSD have antibodies against myelin oligodendrocyte glycoprotein (MOG), another protein involved in protecting nerves. […] People with a history of other autoimmune conditions have a higher risk of developing NMOSD, which is also most common in females. […] Researchers have identified several factors that may raise the risk of neuromyelitis optica spectrum disorder. […] Certain environmental factors are more likely to raise your risk of neuromyelitis optica. […] A few studies point to a possible link between the microbiome (gut bacteria) and NMOSD.

• #25 Neuromyelitis optica spectrum disorder | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/neuromyelitis-optica-spectrum-disorder?lang=us

  Neuromyelitis optica spectrum disorder (NMOSD) is a severe demyelinating diseases, which in seropositive cases, is caused by an autoantibody to the aquaporin-4 (AQP4) water channel. […] Generally, the condition is sporadic, although some overlap in immunogenic features between certain viruses and aquaporin-4 water channel have been identified.

• #26 Neuromyelitis optica (NMO): Symptoms, treatment, and more

  https://www.medicalnewstoday.com/articles/170302

  Neuromyelitis optica (NMO), or Devics disease, is an inflammation of the sheath that protects the nerves in the eyes and the spinal cord. […] The specific causes of NMO are unknown. It is an autoimmune disorder, and it causes the immune system to mistakenly attack healthy cells in the optic nerve and spinal cord. This causes the symptoms. […] In some rare cases, tuberculosis and some environmental organisms may play a role in causing it, though scientists have not been able to isolate the specific organisms responsible. […] Many people with NMO have an antibody called neuromyelitis optica immunoglobin G in their blood. Research suggests that this antibody may damage aquaporin-4, the water channel that surrounds the optic nerve and spinal cord cells, causing the characteristic inflammation. […] However, confirming this and identifying the full range of causes requires more research.

• #27 Neuromyelitis Optica (NMO) Disease: Symptoms, Causes, and Treatment | Apollo Hospitals

  https://www.apollohospitals.com/diseases-and-conditions/neuromyelitis-optica-nmo-disease-symptoms-causes-and-treatment

  Neuromyelitis Optica may develop after an infection or is linked to another autoimmune disease. […] While NMOSD is considered to be a autoimmune disease, the exact cause of the autoimmunity is not known. […] Autoimmune disorders happen when the natural defenses of the body against disease or invading organisms (like bacteria), for reasons not known, suddenly start attacking healthy tissue. […] In some people with NMOSD, especially those with a non-relapsing variant, antibodies to MOG-IgG (myelin oligodendrocyte glycoprotein) were discovered. […] There is a strong link with personal or family history of autoimmunity that are present in 50 per cent of cases.

• #28 Neuromyelitis Optica Spectrum Disorder: Up-to-Date Advance Researches for a Novel Therapeutic Target

  https://www.journal-dtt.org/journal/view.html?year=&uid=47&vmd=Full

  The prevalence of NMO ranges from 0.1 to 4.4 cases per 100,000 individuals, with a disease onset age of 35-45 years; this distinguishes NMO from MS, where the disease onset is much earlier at 25-29 years of age. […] The ratio of female and male patients with NMO revealed that the disease was more prevalent in women at a ratio of approximately 9:1. […] AQP4-IgG activated astrocytes therefore induce prominent microglia activation. This results in the activated pro-inflammatory cytokine as well as including TNF-A, IL-1beta, and IL-6. Subsequently, NMO is characterized pathologically by significant astrocyte injuries, initiating secondary responses, such as demyelination, oligodendrocyte, and neuronal loss.

• #29 Multiple Sclerosis Related Diseases: Causes, Symptoms & Diagnosis

  https://www.clevelandclinicabudhabi.ae/en/health-hub/health-resource/diseases-and-conditions/multiple-sclerosis-related-diseases

  Neuromyelitis Optica Spectrum Disorder (NMOSD) or Neuromyelitis Optica (NMO), is a disease that resembles MS. […] Most cases of NMO are caused by a specific protein in the blood called NMO antibody (anti-aquaporin-4 antibody), which is produced by the white blood cells. […] While we understand the mechanisms that cause NMO (specifically the NMO antibody), just like in MS, we do not know what causes NMO. […] It is more common in women, and occurs all over the world, with African, Asian, and Native American populations being more at risk. […] MOGAD is caused by a specific protein in the blood called the MOG antibody (Myelin Oligodendrocyte Glycoprotein antibody), which is produced by our white blood cells. […] It is not known why the white blood cells of the immune system mistakenly attack the nervous system.

• #30 Neuromyelitis optica spectrum disorder (NMOSD): Clinical features and diagnosis – UpToDate

  https://www.uptodate.com/contents/neuromyelitis-optica-spectrum-disorders-nmosd-clinical-features-and-diagnosis

  Neuromyelitis optica spectrum disorder (NMOSD; previously known as Devic disease or neuromyelitis optica [NMO]) is an inflammatory disorder of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and the spinal cord. […] It was previously believed that NMOSD and multiple sclerosis represented one disease entity with variable phenotypes and expression. However, the discovery of a disease-specific serum NMO-immunoglobulin G (IgG) antibody that selectively binds aquaporin-4 (AQP4) has led to increased understanding that NMOSD is distinct from classic relapsing-remitting multiple sclerosis with respect to pathogenesis, imaging features, biomarkers, neuropathology, and treatment. […] Neuropathology – In NMOSD, florid demyelination and inflammation involve multiple spinal cord segments and the optic nerves with associated astrocyte death, axonal loss, perivascular lymphocytic infiltration, and vascular proliferation. Unlike multiple sclerosis, necrosis and cavitation typically involve both gray and white matter. The neuropathologic features of NMOSD at autopsy are those of a much more severe necrotic lesion of the cord rather than incomplete demyelination. This may result from the distribution of aquaporin-4 (AQP4) receptors, which predominate in astrocytes rather than oligodendrocytes.

• #31 Neuromyelitis optica spectrum disorder | MedLink Neurology

  https://www.medlink.com/articles/neuromyelitis-optica-spectrum-disorders

  Lesions in neuromyelitis optica spectrum disorder show perivascular and parenchymal leukocyte infiltration, deposition of IgG, IgM, and complement, loss of GFAP and AQP4 staining, hemorrhagic exudation, edema, capillary proliferation, vascular fibrosis, hyalinization, tissue necrosis, and cavitation. Both white and gray matter are affected. Lesions are more similar to necrotizing vasculitis than to the predominantly demyelinating lesions of multiple sclerosis or acute disseminated encephalomyelitis.

• #32 Neuromyelitis Optica

  https://www.tenderpalm.com/neuromyelitis-optica-treatment-lucknow-india

  Neuromyelitis Optica (NMO), also known as Devic’s Disease in medical terms, is a rare autoimmune disorder that primarily affects the optic nerves and spinal cord, causing the immune system of the human body to mistakenly attack protein, i.e., aquaporin-4, and triggering inflammation and damage over the region. […] The exact cause of NMO is unknown, but it is believed to result from a combination of genetic predisposition and environmental triggers. The bodys immune system produces antibodies, particularly anti-aquaporin-4 antibodies (AQP4-IgG), which mistakenly target the optic nerves and spinal cord. While the cause is not fully understood, NMO has been linked to infections and other autoimmune disorders, such as lupus or Sjgrens syndrome.

• #33 Neuromyelitis optica – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/neuromyelitis-optica/diagnosis-treatment/drc-20375655

  Diagnosing neuromyelitis optica involves a physical exam and tests. […] A healthcare professional might test the blood for the autoantibody that binds to proteins and causes NMO. The autoantibody is called aquaporin-4-immunoglobulin G, also known as AQP4-IgG. […] Other biomarkers such as serum glial fibrillary acidic protein, also called GFAP, and serum neurofilament light chain help detect relapses. […] The spinal fluid might show a very high level of white blood cells during NMO episodes. […] Neuromyelitis optica can’t be cured. […] Monoclonal antibodies have been shown in clinical trials to be effective in reducing the risk of NMO relapses. […] Your healthcare professional also might recommend taking a medicine that suppresses the immune system.

• #34 Neuromyelitis Optica Spectrum Disorder: From Basic Research to Clinical Perspectives

  https://www.mdpi.com/1422-0067/23/14/7908

  Despite important breakthroughs in the understanding of AQP4 and MOG antibodies and their involvement in NMOSD, the genetic factors underlying the disease pathogenesis have not been fully understood. […] The potential molecular mechanisms underlying AQP4-seropositive NMOSD may be related to proteins encoded by the novel genes involved in complement activation, antigen presentation, antibody-dependent cytotoxicity, and immune regulation.

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