Materiały źródłowe

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

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

  Neuromyelitis optica can’t be cured. But treatment can sometimes lead to a long-term period with no symptoms, known as remission. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks. […] In the early stage of an NMO attack, a healthcare professional might give a corticosteroid medicine such as methylprednisolone (Solu-Medrol). It’s given through a vein in the arm. The medicine is taken for about five days and then it’s usually tapered off slowly over several days. […] Plasma exchange is often recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from the body, and blood cells are mechanically separated from fluid called plasma. The blood cells are mixed with a replacement solution and the blood is returned to the body. This process can remove harmful substances and cleanse the blood.

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

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

  NMO isn’t curable, but thanks to continuing research, this condition is treatable. Because NMO is an autoimmune condition, the main treatments take two forms: acute treatment and long-term management. […] Acute treatment: This focuses on treating the immediate effects of an NMO attack, especially inflammation. Corticosteroids (or other kinds of prescription anti-inflammatory drugs) are most common for this. Acute treatment is extremely important, as it reduces the risk of permanent damage from an attack. […] Long-term management: NMO happens because your immune system mistakenly attacks your nervous system. Managing that involves suppressing or modulating your immune system, which minimizes its ability to damage your nervous system. This can prevent NMO attacks or at least limit how severe they are or how long they last. Experts recommend these treatments for people with and without AQP4 antibodies.

• #3 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Neuromyelitis optica (NMO) is an autoimmune demyelinating disease preferentially targeting the optic nerves and spinal cord. […] Preventive therapy in NMO has focused on a range of immunosuppressive medications, none of which have been validated in a rigorous randomized trial. […] Multiple retrospective and a few recent prospective studies have provided evidence for the use of six medications for the prevention of NMO exacerbations: azathioprine, rituximab, mycophenolate mofetil, prednisone, methotrexate and mitoxantrone. […] In the acute setting of an initial presentation or an exacerbation of NMO, treatment is focused on minimizing irreversible damage to the CNS and restoring neurologic function. […] The standard of care for the treatment of an acute optic neuritis or transverse myelitis associated with NMO is high dose intravenous methylprednisolone at a daily dose of 1000 mg for at least three to five days.

• #4 SciELO Brazil – Treatment of neuromyelitis optica: an evidence based review Treatment of neuromyelitis optica: an evidence based review

  https://www.scielo.br/j/anp/a/rdSpmcLbFzPkCPK4cMfmZsw/

  Neuromyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. […] Due to the severity of NMO attacks and the high risk for disability, treatment should be instituted as soon as the diagnosis is confirmed. […] Acute NMO attacks usually are treated with high dose intravenous corticosteroid pulse and plasmapheresis. Maintenance therapy is also required to avoid further attacks and it is based on low-dose oral corticosteroids and non-specific immunosuppressant drugs, like azathioprine and mycophenolate mofetil. […] New therapy strategies using monoclonal antibodies like rituximab have been tested in NMO, with positive results in open label studies. […] NMO treatment has two main objectives: one is to control the inflammatory damage in acute attacks and the other one is a maintenance treatment to avoid relapses. The former is based on high dose intravenous corticosteroids and plasmapheresis; the latter is based on low-dose corticosteroids and immunosuppressants.

• #5 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Neuromyelitis optica (NMO) is an autoimmune demyelinating disease preferentially targeting the optic nerves and spinal cord. […] Preventive therapy in NMO has focused on a range of immunosuppressive medications, none of which have been validated in a rigorous randomized trial. […] Multiple retrospective and a few recent prospective studies have provided evidence for the use of six medications for the prevention of NMO exacerbations: azathioprine, rituximab, mycophenolate mofetil, prednisone, methotrexate and mitoxantrone. […] In the acute setting of an initial presentation or an exacerbation of NMO, treatment is focused on minimizing irreversible damage to the CNS and restoring neurologic function. […] The standard of care for the treatment of an acute optic neuritis or transverse myelitis associated with NMO is high dose intravenous methylprednisolone at a daily dose of 1000 mg for at least three to five days.

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

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

  Neuromyelitis optica can’t be cured. But treatment can sometimes lead to a long-term period with no symptoms, known as remission. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks. […] In the early stage of an NMO attack, a healthcare professional might give a corticosteroid medicine such as methylprednisolone (Solu-Medrol). It’s given through a vein in the arm. The medicine is taken for about five days and then it’s usually tapered off slowly over several days. […] Plasma exchange is often recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from the body, and blood cells are mechanically separated from fluid called plasma. The blood cells are mixed with a replacement solution and the blood is returned to the body. This process can remove harmful substances and cleanse the blood.

• #7 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8395403/

  Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune central nervous system (CNS) inflammatory disorder that can lead to serious disability and mortality. […] With the increased understanding of these mechanisms underlying this serious autoimmune astrocytopathy, effective treatments for both active attacks and long-term immunosuppression to prevent relapses in NMOSD are increasingly available based on the evidence from retrospective observational data and prospective clinical trials. […] Knowledge on the indications and potential side effects of these medications are essential for a clear evaluation of the potential benefits and risks to NMOSD patients in a personalized manner. […] High dose intravenous steroids are traditionally used as first-line treatment of acute attacks of NMOSD, typically intravenous methylprednisolone (IVMP) at 1 g per day for 3-5 consecutive days.

• #8 Neuromyelitis optica spectrum disorder – Wikipedia

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

  There is no cure for NMO, but it is treatable. Some patients recover, but many are left with impairment of vision and limbs, which can be severe in some cases. […] Traditionally, attacks have been treated with short courses (3-5 days) of high dosage intravenous corticosteroids, such as methylprednisolone IV (Solu-Medrol). Early initiation of treatment with steroids has been shown to improve vision-related outcomes after acute attacks. […] Plasmapheresis can be an effective treatment when attacks progress after the administration of corticosteroids. […] Prophylactic treatment, to prevent relapses of NMO, is generally employed; but the exact duration of such treatment is debatable. […] FDA-approved pharmaceuticals against AQP4-IgG-positive NMOSD, shown to be effective in phase III clinical trials, first became available in 2019.

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

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

  Neuromyelitis optica can’t be cured. But treatment can sometimes lead to a long-term period with no symptoms, known as remission. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks. […] In the early stage of an NMO attack, a healthcare professional might give a corticosteroid medicine such as methylprednisolone (Solu-Medrol). It’s given through a vein in the arm. The medicine is taken for about five days and then it’s usually tapered off slowly over several days. […] Plasma exchange is often recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from the body, and blood cells are mechanically separated from fluid called plasma. The blood cells are mixed with a replacement solution and the blood is returned to the body. This process can remove harmful substances and cleanse the blood.

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

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

  Neuromyelitis optica can’t be cured. But treatment can sometimes lead to a long-term period with no symptoms, known as remission. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks. […] In the early stage of an NMO attack, a healthcare professional might give a corticosteroid medicine such as methylprednisolone (Solu-Medrol). It’s given through a vein in the arm. The medicine is taken for about five days and then it’s usually tapered off slowly over several days. […] Plasma exchange is often recommended as the first or second treatment, usually in addition to steroid therapy. In this procedure, some blood is removed from the body, and blood cells are mechanically separated from fluid called plasma. The blood cells are mixed with a replacement solution and the blood is returned to the body. This process can remove harmful substances and cleanse the blood.

• #11 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8395403/

  Plasma exchange (PLEX) has been shown to improve neurological functions in patients with severe attacks of central nervous system inflammatory demyelinating disorders who failed standard treatment with high dose intravenous steroid in a randomized sham-controlled trial. […] Immunoadsorption (IA) is an alternative apheresis therapy to PLEX when PLEX is contraindicated or unavailable. […] Intravenous immunoglobulins (IVIg) therapy is occasionally considered as a treatment option in NMOSD attacks, especially in patients with contraindications to IVMP and apheresis therapies. […] Table 2 lists the treatments available for relapse prevention in NMOSD whereas Table 3 lists the core clinical trials for relapse prevention in NMOSD. […] Azathioprine has been used to prevent relapse in NMOSD for decades, since a report on its efficacy in seven NMOSD patients who became relapse free after azathioprine treatment with a significant improvement in neurological function.

• #12

  https://link.springer.com/article/10.1007/s00415-023-11910-z

  The duration of low-dose add-on glucocorticoid treatment depends on the AQP4-IgG serostatus, disease activity, mode of action, and the expected time to the attack-preventive effect of the subsequent immunotherapy. […] Several retrospective studies have shown that an early start of apheresis therapy is associated with better outcomes in NMOSD patients. […] The second main goal of NMOSD therapy is to prevent further attacks. […] Four therapies, eculizumab, inebilizumab, and satralizumab and most recently ravulizumab have been approved for use in AQP4-IgG-positive NMOSD since 2019. […] Long-term immunotherapy must be offered to patients with AQP4-IgG-positive NMOSD already after the first attack. […] Eculizumab/ravulizumab, inebilizumab, rituximab, satralizumab and tocilizumab are highly effective therapies for AQP4-IgG-positive NMOSD.

• #13 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8395403/

  Plasma exchange (PLEX) has been shown to improve neurological functions in patients with severe attacks of central nervous system inflammatory demyelinating disorders who failed standard treatment with high dose intravenous steroid in a randomized sham-controlled trial. […] Immunoadsorption (IA) is an alternative apheresis therapy to PLEX when PLEX is contraindicated or unavailable. […] Intravenous immunoglobulins (IVIg) therapy is occasionally considered as a treatment option in NMOSD attacks, especially in patients with contraindications to IVMP and apheresis therapies. […] Table 2 lists the treatments available for relapse prevention in NMOSD whereas Table 3 lists the core clinical trials for relapse prevention in NMOSD. […] Azathioprine has been used to prevent relapse in NMOSD for decades, since a report on its efficacy in seven NMOSD patients who became relapse free after azathioprine treatment with a significant improvement in neurological function.

• #14 Neuromyelitis Optica Spectrum Disorder Treatment—Current and Future Prospects

  https://www.mdpi.com/1422-0067/22/6/2801

  Immunoadsorption (IA) is a more selective method of apheresis, which allows the removal of specific antibodies and immune complexes from systemic circulation, without total plasma exchange. […] Another option for NMO relapse treatment is intravenous immunoglobulins (IVIG). […] Maintenance therapy should be instituted shortly after treatment of relapse in order to prevent future exacerbations and accumulating disability. […] Although not officially approved and mostly used on an empirical basis or tested in short-term, open label trials, a few immunosuppressants have been applied in NMO as a single or combined therapy. […] There is more evidence available for the effective use of mycophenolate mofetil (MMF) in NMOSD. […] Rituximab (RTX) is a chimeric monoclonal antibody against the CD-20 antigen, which is expressed on the majority of B-cells.

• #15 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8395403/

  Plasma exchange (PLEX) has been shown to improve neurological functions in patients with severe attacks of central nervous system inflammatory demyelinating disorders who failed standard treatment with high dose intravenous steroid in a randomized sham-controlled trial. […] Immunoadsorption (IA) is an alternative apheresis therapy to PLEX when PLEX is contraindicated or unavailable. […] Intravenous immunoglobulins (IVIg) therapy is occasionally considered as a treatment option in NMOSD attacks, especially in patients with contraindications to IVMP and apheresis therapies. […] Table 2 lists the treatments available for relapse prevention in NMOSD whereas Table 3 lists the core clinical trials for relapse prevention in NMOSD. […] Azathioprine has been used to prevent relapse in NMOSD for decades, since a report on its efficacy in seven NMOSD patients who became relapse free after azathioprine treatment with a significant improvement in neurological function.

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

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

  There are several drugs and treatments that can help treat NMO, including: Anti-inflammatory drugs: These drugs reduce the inflammation of your nervous system. The most common drugs that do this are corticosteroids, such as prednisone. […] Plasma exchange (plasmapheresis): When steroids aren’t helpful, your healthcare provider may recommend a procedure called plasma exchange. […] Intravenous immunoglobulin (IVIG): This procedure involves infusing plasma into your body through an IV. […] Immune-suppressing drugs (immunosuppressants): These are medications you take regularly. […] Taking immune-suppressing drugs can help prevent NMO attacks, but it also reduces your body’s ability to fight certain infections, and it can raise your risk of certain types of tumors (both cancerous and noncancerous). […] The treatment and recovery timeline can vary with NMO because many factors play a role. Your healthcare provider is the best source of information on this. They can tell you the timeline that’s most likely in your case, and what you can do to help that process.

• #17 7 Neuromyelitis Optica Treatment Options | MyMSTeam

  https://www.mymsteam.com/resources/neuromyelitis-optica-treatment-options

  Monoclonal antibodies are human-made versions of immune proteins designed to specific target parts of the immune system involved in NMOSD attacks. […] These therapies are highly effective for NMOSD, particularly in reducing relapses. […] Corticosteroids are effective at reducing inflammation and stopping NMOSD attacks, but they cause serious side effects if used long term. […] Plasma exchange, or plasmapheresis, is often used for severe attacks of NMOSD that dont respond well to steroids. […] Intravenous immunoglobulin (IVIG) is a treatment option that delivers healthy antibodies from donated blood to help block the harmful ones causing damage in NMOSD. […] If you develop symptoms from your attacks, your doctor may recommend additional medications. […] Although medications and procedures are essential, managing NMOSD also means addressing overall health and well-being.

• #18 NMOSD | Neuromyelitis Optica Spectrum Disorder | Prognosis & Management | SRNA

  https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/prognosis-management/

  In NMOSD, the likelihood of recurrence of disease activity is greater than 90%. […] Therefore, it is generally thought that ongoing treatment with medications that suppress the immune system and prevent relapses is necessary. The three primary off-label therapies used in the US are mycophenolate mofetil (CellCept), rituximab (Rituxan), and azathioprine (Imuran), but new therapies have been FDA approved based on randomized clinical trials that demonstrated effective and safe strategies to treat NMO. […] SOLIRIS (eculizumab) was the first FDA-approved complement inhibitor indicated for the treatment of adults with AQP4 antibody-positive NMOSD. […] ULTOMIRIS (ravulizumab) was the second FDA-approved complement inhibitor indicated for the treatment of adults with AQP4 antibody-positive NMOSD.

• #19 Diagnosing and Managing Neuromyelitis Optica

  https://www.uspharmacist.com/article/diagnosing-and-managing-neuromyelitis-optica

  Monoclonal antibodies such as rituximab and other agents that earned FDA indication for chronic management of NMO/NMOSD, such as eculizumab, inebilizumab, and satralizumab, may be the solution that fits the bill. […] The use of biologic immunosuppression to prevent relapses in NMOSD is becoming more prevalent while simultaneously becoming more costly. The biologic agents eculizumab, inebilizumab, satralizumab, rituximab, and tocilizumab are used, with studies showing them to be clinically significant. […] Eculizumab was the first monoclonal antibody FDA approved for the treatment of NMO/NMOSD in adult patients who are seropositive for AQP-4 and may be considered a first-line treatment option. […] Inebilizumab is a monoclonal antibody that binds to CD19 and depletes lymphocytes. […] Satralizumab is a humanized monoclonal antibody that targets IL-6 receptors. […] Rituximab, a chimeric monoclonal antibody specific for the CD20 antigen on B lymphocytes, may be used to treat NMOSD. […] Tocilizumab is a humanized IL-6 receptor antagonist.

• #20 Neuromyelitis Optica Spectrum Disorder Treatment—Current and Future Prospects

  https://www.mdpi.com/1422-0067/22/6/2801

  Inebilizumab is a humanized monoclonal antibody against CD-19, a marker expressed on a wide range of B-cell lines. […] The first Il-6 receptor antagonist adapted for NMO treatment was tocilizumab, a humanized monoclonal antibody. […] Eculizumab is a humanized monoclonal antibody, targeted at terminal complement protein C5 and preventing its cleavage into C5a and C5b components. […] Directions of research in the field of future therapies for NMO include further exploration of already known mechanisms, as well as investigation of potential novel targets.

• #21 Neuromyelitis optica: New therapies offer hope – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/neurology-neurosurgery/news/neuromyelitis-optica-new-therapies-offer-hope/mac-20515747

  Mayo Clinic autoimmune neurologists advise physicians and patients to consider each therapy’s efficacy, convenience and cost: In the PREVENT eculizumab trial, published in The New England Journal of Medicine in 2019, 98% of patients receiving the therapy were relapse-free 144 weeks after starting treatment, compared with 45% in the placebo group. […] Dr. Pittock notes that a recent randomized controlled trial at a single institution in Japan found that rituximab was more effective than a placebo. […] Mayo Clinic is currently a site for a pharmaceutical company-funded study investigating ravulizumab, a monoclonal antibody inhibitor of complement activation that is similar to eculizumab but with a longer half-life. […] „At Mayo Clinic, our work goes from bench to bedside from biomarker discovery to treatments that can stop attacks,” he adds. „By stopping NMOSD attacks, we can potentially stop the accrual of disability.”

• #22 Neuromyelitis Optica Spectrum Disorder Treatment—Current and Future Prospects

  https://www.mdpi.com/1422-0067/22/6/2801

  Inebilizumab is a humanized monoclonal antibody against CD-19, a marker expressed on a wide range of B-cell lines. […] The first Il-6 receptor antagonist adapted for NMO treatment was tocilizumab, a humanized monoclonal antibody. […] Eculizumab is a humanized monoclonal antibody, targeted at terminal complement protein C5 and preventing its cleavage into C5a and C5b components. […] Directions of research in the field of future therapies for NMO include further exploration of already known mechanisms, as well as investigation of potential novel targets.

• #23 Neuromyelitis Optica Spectrum Disorder (NMOSD)

  https://www.massgeneral.org/neurology/treatments-and-services/about-neuromyelitis-optica-nmosd

  Patients may know that in late June of 2019, the first drug for NMOSD was approved by the FDA. This drug, eculizumab, produced by Alexion, has the brand name Soliris. […] One year later, in June of 2020, the second drug for aquaporin-4-positive patients got FDA approval. This drug, inebilizumab, known as Uplizna, is administered by infusion twice a year. […] In August, 2020, a third medication for AQP4+ patients with NMO was approved by the FDA. Enspryng, by Genentech, targets the interleukin 6 (IL-6) receptor which is believed to play a role in inflammation in NMO. […] For patients experiencing attacks, we recommend rapid intervention with: Intravenous corticosteroids (Solumedrol) and/or Plasma exchange (PLEX). […] Many patients who were diagnosed before 2019 were prescribed off-label medications for their disease. When these drugs are successful in preventing attacks, physicians may not change the therapy regimens.

• #24 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://www.mdpi.com/1422-0067/22/16/8638

  Table 2 lists the treatments available for relapse prevention in NMOSD whereas Table 3 lists the core clinical trials for relapse prevention in NMOSD. […] Azathioprine has been used to prevent relapse in NMOSD for decades, since a report on its efficacy in seven NMOSD patients who became relapse free after azathioprine treatment with a significant improvement in neurological function. […] Rituximab is a chimeric monoclonal antibody targeting CD20, initially developed for the treatment of B cell lymphoma. […] Eculizumab is a monoclonal antibody that inhibits the terminal complement component C5, and prevents its cleavage into C5a and C5b. […] This study provides evidence that inebulizumab reduced the risk of NMOSD attacks compared with placebo; hence, inebulizumab has potential as an evidence-based treatment for AQP4-IgG-seropositive NMOSD patients. […] Satralizumab is a subcutaneously administered humanized monoclonal antibody targeting the interleukin-6 (IL-6) receptor. […] […] Overall, rituximab is well tolerated in autoimmune diseases, including NMOSD. […] The main side effects are infusion reactions and infections (opportunistic and nonopportunisitic).

• #25 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://www.mdpi.com/1422-0067/22/16/8638

  Table 2 lists the treatments available for relapse prevention in NMOSD whereas Table 3 lists the core clinical trials for relapse prevention in NMOSD. […] Azathioprine has been used to prevent relapse in NMOSD for decades, since a report on its efficacy in seven NMOSD patients who became relapse free after azathioprine treatment with a significant improvement in neurological function. […] Rituximab is a chimeric monoclonal antibody targeting CD20, initially developed for the treatment of B cell lymphoma. […] Eculizumab is a monoclonal antibody that inhibits the terminal complement component C5, and prevents its cleavage into C5a and C5b. […] This study provides evidence that inebulizumab reduced the risk of NMOSD attacks compared with placebo; hence, inebulizumab has potential as an evidence-based treatment for AQP4-IgG-seropositive NMOSD patients. […] Satralizumab is a subcutaneously administered humanized monoclonal antibody targeting the interleukin-6 (IL-6) receptor. […] […] Overall, rituximab is well tolerated in autoimmune diseases, including NMOSD. […] The main side effects are infusion reactions and infections (opportunistic and nonopportunisitic).

• #26 NMOSD | Neuromyelitis Optica Spectrum Disorder | Prognosis & Management | SRNA

  https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/prognosis-management/

  UPLIZNA (inebilizumab-cdon) is the second FDA-approved treatment for NMOSD in adult patients who are AQP4 antibody positive. […] ENSPRYNG (satralizumab-mwge) is the first and only subcutaneous treatment for adults living with AQP4 antibody positive NMOSD. […] Azathioprine is the medication that has been around the longest, and, over the years, has been used most widely in NMOSD. […] Mycophenolate mofetil has a similar effect on the gastrointestinal system, though many report that the symptoms are milder with mycophenolate as compared with azathioprine. […] Rituximab is an intravascular infusion which works differently from the other two agents listed above. […] Low-dose prednisone is used as well, more often in other parts of the world.

• #27 Neuromyelitis Optica Spectrum Disorder (NMOSD)

  https://www.massgeneral.org/neurology/treatments-and-services/about-neuromyelitis-optica-nmosd

  Patients may know that in late June of 2019, the first drug for NMOSD was approved by the FDA. This drug, eculizumab, produced by Alexion, has the brand name Soliris. […] One year later, in June of 2020, the second drug for aquaporin-4-positive patients got FDA approval. This drug, inebilizumab, known as Uplizna, is administered by infusion twice a year. […] In August, 2020, a third medication for AQP4+ patients with NMO was approved by the FDA. Enspryng, by Genentech, targets the interleukin 6 (IL-6) receptor which is believed to play a role in inflammation in NMO. […] For patients experiencing attacks, we recommend rapid intervention with: Intravenous corticosteroids (Solumedrol) and/or Plasma exchange (PLEX). […] Many patients who were diagnosed before 2019 were prescribed off-label medications for their disease. When these drugs are successful in preventing attacks, physicians may not change the therapy regimens.

• #28 Neuromyelitis optica: New therapies offer hope – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/neurology-neurosurgery/news/neuromyelitis-optica-new-therapies-offer-hope/mac-20515747

  Mayo Clinic autoimmune neurologists advise physicians and patients to consider each therapy’s efficacy, convenience and cost: In the PREVENT eculizumab trial, published in The New England Journal of Medicine in 2019, 98% of patients receiving the therapy were relapse-free 144 weeks after starting treatment, compared with 45% in the placebo group. […] Dr. Pittock notes that a recent randomized controlled trial at a single institution in Japan found that rituximab was more effective than a placebo. […] Mayo Clinic is currently a site for a pharmaceutical company-funded study investigating ravulizumab, a monoclonal antibody inhibitor of complement activation that is similar to eculizumab but with a longer half-life. […] „At Mayo Clinic, our work goes from bench to bedside from biomarker discovery to treatments that can stop attacks,” he adds. „By stopping NMOSD attacks, we can potentially stop the accrual of disability.”

• #29 Neuromyelitis optica spectrum disorders (NMOSD) – MS Australia

  https://www.msaustralia.org.au/nmosd-and-mogad/neuromyelitis-optica-spectrum-disorders/

  Early identification and treatment are very important in managing NMOSD. Treatment is usually broken into two different phases: rescue therapy of the acute attack when it happens and relapse prevention, which is a more long-term approach. […] Rescue therapy currently typically involves high dose corticosteroid treatment (to reduce the inflammation at the site of the lesion), plasma exchange using a machine to clean the blood (to remove the harmful AQP4 antibodies) and occasionally immunoglobulin infusions. Relapse prevention usually involves a general immunosuppressive medication to prevent further attacks to the body and includes corticosteroids, azathioprine, mycophenolate, methotrexate, cyclophosphamide and rituximab. […] However, the approval of targeted treatments (similar to disease modifying treatments in MS) has significantly improved the management of NMOSD. On 1 April 2025, Ravulizumab (Ultomiris) was listed on the Pharmaceutical Benefits Scheme (PBS) for adults with NMOSD who are AQP4-antibody positive. This provides subsidised access to a highly effective treatment that can prevent relapses and reduce the severity of the disease. Ravulizumab is a long-acting monoclonal antibody that blocks the complement system, a part of the immune system that plays a key role in NMOSD-related damage. It is administered via intravenous infusion. […] The inclusion of ravulizumab on the PBS represents a significant milestone for people living with NMOSD, ensuring broader access to a life-changing therapy that can help prevent relapses and improve quality of life.

• #30 Treatment of Neuromyelitis Optica Spectrum Disorders

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8395403/

  Rituximab is a chimeric monoclonal antibody targeting CD20, initially developed for the treatment of B cell lymphoma. […] Eculizumab is a monoclonal antibody that inhibits the terminal complement component C5, and prevents its cleavage into C5a and C5b. […] This study provides evidence that inebulizumab reduced the risk of NMOSD attacks compared with placebo; hence, inebulizumab has potential as an evidence-based treatment for AQP4-IgG-seropositive NMOSD patients. […] Overall, rituximab is well tolerated in autoimmune diseases, including NMOSD.

• #31 Diagnosing and Managing Neuromyelitis Optica

  https://www.uspharmacist.com/article/diagnosing-and-managing-neuromyelitis-optica

  Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system that targets the optic nerves and the spinal cord, resulting in optic neuritis and myelitis, respectively. […] Treatment consists of medication management for acute relapses and symptomatic and maintenance therapy for relapse prevention. There are currently only three drugs that are FDA approved for NMO. […] Therefore, long-term management of symptoms involving immunotherapy is of utmost importance in managing NMO/NMOSD, considering its relapse-related disability, poor prognosis, and high risk of mortality in untreated patients. […] Recent studies suggest immediate initiation of long-term immunotherapy once the diagnosis of NMO/NMOSD is determined. There are three monoclonal antibody agents that recently earned FDA approval for management of NMO/NMOSD. Rituximab, an anti-CD20 agent approved in 1997, has the strongest evidence to support its use, while mycophenolate has comparable reductions in absolute relapse rate and Expanded Disability Status Scale (EDSS) scores.

• #32 Neuromyelitis Optica Spectrum Disorders

  https://practicalneurology.com/articles/2019-feb/neuromyelitis-optica-spectrum-disorders

  A retrospective study demonstrated improved outcomes when patients were treated with PLEX early in the course of an exacerbation. […] We recommend prompt initiation of long-term immunosuppression once a diagnosis of NMOSD is made. […] Given the available data, we prefer rituximab (RTX) for most patients with NMOSD. […] Treatment with RTX decreased relapse rate by up to 88%, with 2 of 3 patients achieving complete remission during a 10-year study period, whereas MMF reduced relapse rates by 87.4% with a 36% failure rate and AZA reduced relapse rates by 72% with a 53% failure rate even when used concurrently with prednisone. […] Adequate RTX dose is important in order to maintain remission. […] If a relapse is confirmed while on immunotherapy, it is important to determine if the medication is being optimally dosed.

• #33 SciELO Brazil – Treatment of neuromyelitis optica: an evidence based review Treatment of neuromyelitis optica: an evidence based review

  https://www.scielo.br/j/anp/a/rdSpmcLbFzPkCPK4cMfmZsw/

  There is some evidence from some case series that plasmapheresis is effective for acute relapse treatment in NMO patients who did not respond satisfactorily to high dose intravenous methylprednisolone. […] Azathioprine is a DNA synthesis inhibitor, as it is converted to a purine analogue, with interference in the purines synthesis (adenine and guanine). […] Rituximab is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells and it has the property to eliminate B cells selectively. […] Mitoxantrone hydrochloride is an anthracenedione used as an antineoplastic agent for prostate cancer and acute non-lymphocytic leukemia in adults. […] Mycophenolate mofetil has been largely used for the treatment of several autoimmune diseases. […] Cyclophosphamide is an alkylating agent that prevents cell division by cross-linking DNA strands and decreasing DNA synthesis.

• #34 NMOSD | Neuromyelitis Optica Spectrum Disorder | Prognosis & Management | SRNA

  https://wearesrna.org/living-with-myelitis/disease-information/neuromyelitis-optica-spectrum-disorder/prognosis-management/

  UPLIZNA (inebilizumab-cdon) is the second FDA-approved treatment for NMOSD in adult patients who are AQP4 antibody positive. […] ENSPRYNG (satralizumab-mwge) is the first and only subcutaneous treatment for adults living with AQP4 antibody positive NMOSD. […] Azathioprine is the medication that has been around the longest, and, over the years, has been used most widely in NMOSD. […] Mycophenolate mofetil has a similar effect on the gastrointestinal system, though many report that the symptoms are milder with mycophenolate as compared with azathioprine. […] Rituximab is an intravascular infusion which works differently from the other two agents listed above. […] Low-dose prednisone is used as well, more often in other parts of the world.

• #35 Neuromyelitis Optica Spectrum Disorders

  https://practicalneurology.com/articles/2019-feb/neuromyelitis-optica-spectrum-disorders

  A retrospective study demonstrated improved outcomes when patients were treated with PLEX early in the course of an exacerbation. […] We recommend prompt initiation of long-term immunosuppression once a diagnosis of NMOSD is made. […] Given the available data, we prefer rituximab (RTX) for most patients with NMOSD. […] Treatment with RTX decreased relapse rate by up to 88%, with 2 of 3 patients achieving complete remission during a 10-year study period, whereas MMF reduced relapse rates by 87.4% with a 36% failure rate and AZA reduced relapse rates by 72% with a 53% failure rate even when used concurrently with prednisone. […] Adequate RTX dose is important in order to maintain remission. […] If a relapse is confirmed while on immunotherapy, it is important to determine if the medication is being optimally dosed.

• #36 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Guidelines for preventive therapy in NMO have been crafted to balance effectiveness with short- and long-term side effects, but clinicians must also consider age, associated medical conditions, functional status, access to medications and response to previous preventative therapies. […] We recommend preventive therapy with one of the six immunosuppressive regimens that have multiple studies reporting efficacy in NMO – azathioprine, rituximab, mycophenolate mofetil, methotrexate, prednisone and mitoxantrone. […] The duration of preventive treatment in NMO that is needed has not been adequately studied. […] Azathioprine is effective in a variety of applications for immunosuppressive therapy. […] Mycophenolate is used in a variety of autoimmune conditions. […] Rituximab is a chimeric mouse/human monoclonal antibody directed against the CD20 antigen.

• #37 Recent Advances on the Diagnosis and Management of Seronegative Neuromyelitis Optica Spectrum Disorder – touchNEUROLOGY

  https://touchneurology.com/neuroimmunology/journal-articles/recent-advances-on-the-diagnosis-and-management-of-seronegative-neuromyelitis-optica-spectrum-disorder/

  Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory autoimmune disease of the central nervous system (CNS) with a worldwide distribution. The treatment of AQP4-IgG-positive NMOSD has improved over the last decades, but the alternatives for seronegative cases are still limited to off-label immunosuppressive drugs. The aim of NMOSD management is to treat acute attacks, prevent further attacks and reduce the disease-related burden in patients. An optimized acute management of attacks promotes a greater recovery of symptoms in patients with NMOSD, mainly due to the suppression of the inflammatory response and CNS injury. The treatment of NMOSD attacks should be initiated early and aggressively, as this can reduce the disability accrual in NMOSD. The use of high doses of intravenous methylprednisolone (IVMP), 1 g/day for 3-7 days, followed by oral corticosteroid therapy, may be the first-line acute treatment, considering that about 35% of patients may show good recovery. However, it is common to have a poor response to corticosteroids, requiring the use of five to seven sessions of therapeutic plasma exchange (PLEX) or immune adsorption (IA) on alternate days. PLEX/IA can be used as a first choice in severe or disabling attacks, as some studies suggest that a greater response can be observed with PLEX/IA when compared with treatment limited to IVMP. There are no data about the difference between the acute treatment of attacks in patients with seronegative and seropositive AQP4-IgG. Some preliminary studies demonstrated the benefits of intravenous human IgG, mainly associated with IVMP. However, this recommendation is still controversial. Prevention of attacks is recommended for all patients diagnosed with NMOSD, especially those with recurrent attacks. The aim is to avoid new attacks associated with the accrual of neurological disabilities. After 2019, three drugs were approved for AQP4-IgG-positive NMOSD. However, no drugs have been approved for patients who were seronegative. Over the last decades, drugs, such as azathioprine (AZA), mycophenolate mofetil (MMF), tacrolimus, mitoxantrone and cyclophosphamide, have been used as off-label preventive treatments for these patients, with previous studies showing a reduction in annualized relapse rate (ARR). The initial effect on immunosuppression by AZA and MMF starts within 4-6 months, therefore, it is common to use oral corticosteroids for the initial treatment period. Previous studies have reported a significant decrease in relapses and the disability level related to AZA. In 2017, a study demonstrated that the use of MMF was effective and safe as a first-line treatment for patients with NMOSD, independent of the serology status of AQP4-IgG. In addition, another commonly used first-line therapy is rituximab (RTX), a monoclonal antibody that selectively depletes CD20+ B cells. This therapy requires intravenous infusions every 6 months, and the biological effect may be monitored by CD19+ cell counting. RTX requires safety monitoring for immunoglobulin levels. A comparative study demonstrated a high efficacy of RTX in reducing attacks compared to placebo. This treatment was associated with decreased or stabilization of disability in 93% and a 60% reduction in the ARR in 5 years. A greater efficacy of RTX has been reported when compared with AZA and MMF. Despite the evidence indicating some effects of these immunosuppressive drugs in reducing attacks and disability for patients with seropositive and seronegative NMOSD, RTX showed a greater association with relapse-free after long-term follow-up. Tocilizumab (TCZ), a monoclonal antibody against the interleukin-6 (IL-6) receptor, has also been used as an off-label preventive treatment option for patients with NMOSD. One study showed a decrease in clinical and radiological activity of the disease in patients with a previous use of RTX. In a comparative study with AZA (Tocilizumab vs Azathioprine in Neuromyelitis Optica Spectrum Disorders [TANGO] study), TCZ was associated with a longer interval between attacks, as well as a reduction in the ARR. Randomized controlled trials (RCTs) have recently been published demonstrating the efficacy of three monoclonal antibodies for the treatment of patients with AQP4-IgG-positive NMOSD, but the results were inconclusive for patients who were seronegative. Despite the lack of significant results for patients with seronegative AQP4-IgG NMOSD in these pivotal studies, some authors consider that, based on the known immunopathogenic mechanisms of NMOSD versus other autoimmune CNS diseases, treatments targeting specific mechanisms, such as complement inhibition, may be more restricted to AQP4-IgG-seropositive NMOSD.

• #38 Neuromyelitis Optica Spectrum Disorders

  https://practicalneurology.com/articles/2019-feb/neuromyelitis-optica-spectrum-disorders

  A retrospective study demonstrated improved outcomes when patients were treated with PLEX early in the course of an exacerbation. […] We recommend prompt initiation of long-term immunosuppression once a diagnosis of NMOSD is made. […] Given the available data, we prefer rituximab (RTX) for most patients with NMOSD. […] Treatment with RTX decreased relapse rate by up to 88%, with 2 of 3 patients achieving complete remission during a 10-year study period, whereas MMF reduced relapse rates by 87.4% with a 36% failure rate and AZA reduced relapse rates by 72% with a 53% failure rate even when used concurrently with prednisone. […] Adequate RTX dose is important in order to maintain remission. […] If a relapse is confirmed while on immunotherapy, it is important to determine if the medication is being optimally dosed.

• #39 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Methotrexate has been used as therapy for NMO patients although the number of reported cases is fewer than 10. […] Mitoxantrone was first studied in five patients with NMO. […] Based on relatively small retrospective and prospective case series, several treatments appear to be likely effective in preventing attacks and stabilizing disability in NMO patients. […] We recommend starting with one of four options for first-line monotherapy treatments for NMO: azathioprine, mycophenolate, rituximab, or prednisone.

• #40 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Methotrexate has been used as therapy for NMO patients although the number of reported cases is fewer than 10. […] Mitoxantrone was first studied in five patients with NMO. […] Based on relatively small retrospective and prospective case series, several treatments appear to be likely effective in preventing attacks and stabilizing disability in NMO patients. […] We recommend starting with one of four options for first-line monotherapy treatments for NMO: azathioprine, mycophenolate, rituximab, or prednisone.

• #41 SciELO Brazil – Treatment of neuromyelitis optica: an evidence based review Treatment of neuromyelitis optica: an evidence based review

  https://www.scielo.br/j/anp/a/rdSpmcLbFzPkCPK4cMfmZsw/

  There is some evidence from some case series that plasmapheresis is effective for acute relapse treatment in NMO patients who did not respond satisfactorily to high dose intravenous methylprednisolone. […] Azathioprine is a DNA synthesis inhibitor, as it is converted to a purine analogue, with interference in the purines synthesis (adenine and guanine). […] Rituximab is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells and it has the property to eliminate B cells selectively. […] Mitoxantrone hydrochloride is an anthracenedione used as an antineoplastic agent for prostate cancer and acute non-lymphocytic leukemia in adults. […] Mycophenolate mofetil has been largely used for the treatment of several autoimmune diseases. […] Cyclophosphamide is an alkylating agent that prevents cell division by cross-linking DNA strands and decreasing DNA synthesis.

• #42 SciELO Brazil – Treatment of neuromyelitis optica: an evidence based review Treatment of neuromyelitis optica: an evidence based review

  https://www.scielo.br/j/anp/a/rdSpmcLbFzPkCPK4cMfmZsw/

  There is some evidence from some case series that plasmapheresis is effective for acute relapse treatment in NMO patients who did not respond satisfactorily to high dose intravenous methylprednisolone. […] Azathioprine is a DNA synthesis inhibitor, as it is converted to a purine analogue, with interference in the purines synthesis (adenine and guanine). […] Rituximab is a chimeric monoclonal antibody against the protein CD20, which is primarily found on the surface of B cells and it has the property to eliminate B cells selectively. […] Mitoxantrone hydrochloride is an anthracenedione used as an antineoplastic agent for prostate cancer and acute non-lymphocytic leukemia in adults. […] Mycophenolate mofetil has been largely used for the treatment of several autoimmune diseases. […] Cyclophosphamide is an alkylating agent that prevents cell division by cross-linking DNA strands and decreasing DNA synthesis.

• #43 Diagnosing and Managing Neuromyelitis Optica

  https://www.uspharmacist.com/article/diagnosing-and-managing-neuromyelitis-optica

  Monoclonal antibodies such as rituximab and other agents that earned FDA indication for chronic management of NMO/NMOSD, such as eculizumab, inebilizumab, and satralizumab, may be the solution that fits the bill. […] The use of biologic immunosuppression to prevent relapses in NMOSD is becoming more prevalent while simultaneously becoming more costly. The biologic agents eculizumab, inebilizumab, satralizumab, rituximab, and tocilizumab are used, with studies showing them to be clinically significant. […] Eculizumab was the first monoclonal antibody FDA approved for the treatment of NMO/NMOSD in adult patients who are seropositive for AQP-4 and may be considered a first-line treatment option. […] Inebilizumab is a monoclonal antibody that binds to CD19 and depletes lymphocytes. […] Satralizumab is a humanized monoclonal antibody that targets IL-6 receptors. […] Rituximab, a chimeric monoclonal antibody specific for the CD20 antigen on B lymphocytes, may be used to treat NMOSD. […] Tocilizumab is a humanized IL-6 receptor antagonist.

• #44 Recent Advances on the Diagnosis and Management of Seronegative Neuromyelitis Optica Spectrum Disorder – touchNEUROLOGY

  https://touchneurology.com/neuroimmunology/journal-articles/recent-advances-on-the-diagnosis-and-management-of-seronegative-neuromyelitis-optica-spectrum-disorder/

  Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory autoimmune disease of the central nervous system (CNS) with a worldwide distribution. The treatment of AQP4-IgG-positive NMOSD has improved over the last decades, but the alternatives for seronegative cases are still limited to off-label immunosuppressive drugs. The aim of NMOSD management is to treat acute attacks, prevent further attacks and reduce the disease-related burden in patients. An optimized acute management of attacks promotes a greater recovery of symptoms in patients with NMOSD, mainly due to the suppression of the inflammatory response and CNS injury. The treatment of NMOSD attacks should be initiated early and aggressively, as this can reduce the disability accrual in NMOSD. The use of high doses of intravenous methylprednisolone (IVMP), 1 g/day for 3-7 days, followed by oral corticosteroid therapy, may be the first-line acute treatment, considering that about 35% of patients may show good recovery. However, it is common to have a poor response to corticosteroids, requiring the use of five to seven sessions of therapeutic plasma exchange (PLEX) or immune adsorption (IA) on alternate days. PLEX/IA can be used as a first choice in severe or disabling attacks, as some studies suggest that a greater response can be observed with PLEX/IA when compared with treatment limited to IVMP. There are no data about the difference between the acute treatment of attacks in patients with seronegative and seropositive AQP4-IgG. Some preliminary studies demonstrated the benefits of intravenous human IgG, mainly associated with IVMP. However, this recommendation is still controversial. Prevention of attacks is recommended for all patients diagnosed with NMOSD, especially those with recurrent attacks. The aim is to avoid new attacks associated with the accrual of neurological disabilities. After 2019, three drugs were approved for AQP4-IgG-positive NMOSD. However, no drugs have been approved for patients who were seronegative. Over the last decades, drugs, such as azathioprine (AZA), mycophenolate mofetil (MMF), tacrolimus, mitoxantrone and cyclophosphamide, have been used as off-label preventive treatments for these patients, with previous studies showing a reduction in annualized relapse rate (ARR). The initial effect on immunosuppression by AZA and MMF starts within 4-6 months, therefore, it is common to use oral corticosteroids for the initial treatment period. Previous studies have reported a significant decrease in relapses and the disability level related to AZA. In 2017, a study demonstrated that the use of MMF was effective and safe as a first-line treatment for patients with NMOSD, independent of the serology status of AQP4-IgG. In addition, another commonly used first-line therapy is rituximab (RTX), a monoclonal antibody that selectively depletes CD20+ B cells. This therapy requires intravenous infusions every 6 months, and the biological effect may be monitored by CD19+ cell counting. RTX requires safety monitoring for immunoglobulin levels. A comparative study demonstrated a high efficacy of RTX in reducing attacks compared to placebo. This treatment was associated with decreased or stabilization of disability in 93% and a 60% reduction in the ARR in 5 years. A greater efficacy of RTX has been reported when compared with AZA and MMF. Despite the evidence indicating some effects of these immunosuppressive drugs in reducing attacks and disability for patients with seropositive and seronegative NMOSD, RTX showed a greater association with relapse-free after long-term follow-up. Tocilizumab (TCZ), a monoclonal antibody against the interleukin-6 (IL-6) receptor, has also been used as an off-label preventive treatment option for patients with NMOSD. One study showed a decrease in clinical and radiological activity of the disease in patients with a previous use of RTX. In a comparative study with AZA (Tocilizumab vs Azathioprine in Neuromyelitis Optica Spectrum Disorders [TANGO] study), TCZ was associated with a longer interval between attacks, as well as a reduction in the ARR. Randomized controlled trials (RCTs) have recently been published demonstrating the efficacy of three monoclonal antibodies for the treatment of patients with AQP4-IgG-positive NMOSD, but the results were inconclusive for patients who were seronegative. Despite the lack of significant results for patients with seronegative AQP4-IgG NMOSD in these pivotal studies, some authors consider that, based on the known immunopathogenic mechanisms of NMOSD versus other autoimmune CNS diseases, treatments targeting specific mechanisms, such as complement inhibition, may be more restricted to AQP4-IgG-seropositive NMOSD.

• #45 Neuromyelitis optica: New therapies offer hope – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/neurology-neurosurgery/news/neuromyelitis-optica-new-therapies-offer-hope/mac-20515747

  Mayo Clinic autoimmune neurologists advise physicians and patients to consider each therapy’s efficacy, convenience and cost: In the PREVENT eculizumab trial, published in The New England Journal of Medicine in 2019, 98% of patients receiving the therapy were relapse-free 144 weeks after starting treatment, compared with 45% in the placebo group. […] Dr. Pittock notes that a recent randomized controlled trial at a single institution in Japan found that rituximab was more effective than a placebo. […] Mayo Clinic is currently a site for a pharmaceutical company-funded study investigating ravulizumab, a monoclonal antibody inhibitor of complement activation that is similar to eculizumab but with a longer half-life. […] „At Mayo Clinic, our work goes from bench to bedside from biomarker discovery to treatments that can stop attacks,” he adds. „By stopping NMOSD attacks, we can potentially stop the accrual of disability.”

• #46

  https://link.springer.com/article/10.1007/s00415-023-11910-z

  The duration of low-dose add-on glucocorticoid treatment depends on the AQP4-IgG serostatus, disease activity, mode of action, and the expected time to the attack-preventive effect of the subsequent immunotherapy. […] Several retrospective studies have shown that an early start of apheresis therapy is associated with better outcomes in NMOSD patients. […] The second main goal of NMOSD therapy is to prevent further attacks. […] Four therapies, eculizumab, inebilizumab, and satralizumab and most recently ravulizumab have been approved for use in AQP4-IgG-positive NMOSD since 2019. […] Long-term immunotherapy must be offered to patients with AQP4-IgG-positive NMOSD already after the first attack. […] Eculizumab/ravulizumab, inebilizumab, rituximab, satralizumab and tocilizumab are highly effective therapies for AQP4-IgG-positive NMOSD.

• #47

  https://link.springer.com/article/10.1007/s00415-023-11910-z

  Long-term immunotherapy in AQP4-IgG-positive NMOSD should be initiated with one of the monoclonal antibodies eculizumab/ravulizumab, inebilizumab, rituximab, or satralizumab, whenever those are available and accessible. […] In case of treatment failure with classical immunosuppressive therapies, therapy should be switched to a monoclonal antibody. […] In case of treatment failure with a monoclonal antibody, therapy should be switched to another monoclonal antibody, preferably with a different mode of action. […] Immunotherapy should be continued in stable AQP4-IgG-positive NMOSD patients and patients must be closely monitored if treatment is temporarily or permanently discontinued due to side effects or patient choice.

• #48

  https://link.springer.com/article/10.1007/s00415-023-11910-z

  Long-term immunotherapy in AQP4-IgG-positive NMOSD should be initiated with one of the monoclonal antibodies eculizumab/ravulizumab, inebilizumab, rituximab, or satralizumab, whenever those are available and accessible. […] In case of treatment failure with classical immunosuppressive therapies, therapy should be switched to a monoclonal antibody. […] In case of treatment failure with a monoclonal antibody, therapy should be switched to another monoclonal antibody, preferably with a different mode of action. […] Immunotherapy should be continued in stable AQP4-IgG-positive NMOSD patients and patients must be closely monitored if treatment is temporarily or permanently discontinued due to side effects or patient choice.

• #49

  https://link.springer.com/article/10.1007/s00415-023-11910-z

  Long-term immunotherapy in AQP4-IgG-positive NMOSD should be initiated with one of the monoclonal antibodies eculizumab/ravulizumab, inebilizumab, rituximab, or satralizumab, whenever those are available and accessible. […] In case of treatment failure with classical immunosuppressive therapies, therapy should be switched to a monoclonal antibody. […] In case of treatment failure with a monoclonal antibody, therapy should be switched to another monoclonal antibody, preferably with a different mode of action. […] Immunotherapy should be continued in stable AQP4-IgG-positive NMOSD patients and patients must be closely monitored if treatment is temporarily or permanently discontinued due to side effects or patient choice.

• #50 Neuromyelitis optica (NMO)

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

  If you’re diagnosed with neuromyelitis optica (NMO) you’ll need treatment to stop the inflammation in your nerves and reduce the risk of relapses. […] When your symptoms first start or if you’re having a relapse, where symptoms come back, the main treatments are: high dose steroid injections, plasma exchange, where some of your blood is removed, treated and then returned to your body, immunoglobulins (antibodies in the blood) given through a drip into your vein. […] You’ll usually also need to take medicines long term that reduce the activity of your immune system to help prevent relapses. These may include: immunosuppressants, such as azathioprine, steroid tablets, such as prednisolone, biological medicines, such as rituximab. […] You may also be offered medicines to help with any ongoing symptoms, such as: painkillers, antidepressant or anticonvulsant medicines that help with nerve pain, such as amitriptyline or gabapentin, muscle relaxant or anticonvulsant medicines to treat muscle spasms, such as baclofen or carbamazepine, medicines for bladder problems, such as oxybutynin, medicines for bowel problems, such as laxatives. […] To help with symptoms such as muscle weakness and mobility problems you may be offered: physiotherapy, including advice on exercises and stretching, occupational therapy to help with daily activities.

• #51 Neuromyelitis optica (NMO)

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

  If you’re diagnosed with neuromyelitis optica (NMO) you’ll need treatment to stop the inflammation in your nerves and reduce the risk of relapses. […] When your symptoms first start or if you’re having a relapse, where symptoms come back, the main treatments are: high dose steroid injections, plasma exchange, where some of your blood is removed, treated and then returned to your body, immunoglobulins (antibodies in the blood) given through a drip into your vein. […] You’ll usually also need to take medicines long term that reduce the activity of your immune system to help prevent relapses. These may include: immunosuppressants, such as azathioprine, steroid tablets, such as prednisolone, biological medicines, such as rituximab. […] You may also be offered medicines to help with any ongoing symptoms, such as: painkillers, antidepressant or anticonvulsant medicines that help with nerve pain, such as amitriptyline or gabapentin, muscle relaxant or anticonvulsant medicines to treat muscle spasms, such as baclofen or carbamazepine, medicines for bladder problems, such as oxybutynin, medicines for bowel problems, such as laxatives. […] To help with symptoms such as muscle weakness and mobility problems you may be offered: physiotherapy, including advice on exercises and stretching, occupational therapy to help with daily activities.

• #52 Neuromyelitis Optica Spectrum Disorder (NMOSD) | Autoimmune Disorders | University Hospitals | Cleveland, OH | University Hospitals

  https://www.uhhospitals.org/services/neurology-and-neurosurgery-services/conditions-and-treatments/multiple-sclerosis-and-neuroimmunology/neuromyelitis-optica-spectrum-disorder

  Several symptomatic medications are available to treat nerve pain, tonic spasms, constipation, and bladder symptoms in NMOSD patients. […] Some NMOSD patients may require botulinum toxin injections or intrathecal baclofen pump to treat spasticity (increased stiffness and spasms of the legs and arms after NMOSD attacks). […] Some NMOSD patients may require spinal cord stimulators to address intractable pain and/or bladder stimulators for bladder dysfunction. […] Physical and occupational therapy: including gait/balance training and visual/motor occupational therapy.

• #53 Neuromyelitis optica (NMO)

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

  If you’re diagnosed with neuromyelitis optica (NMO) you’ll need treatment to stop the inflammation in your nerves and reduce the risk of relapses. […] When your symptoms first start or if you’re having a relapse, where symptoms come back, the main treatments are: high dose steroid injections, plasma exchange, where some of your blood is removed, treated and then returned to your body, immunoglobulins (antibodies in the blood) given through a drip into your vein. […] You’ll usually also need to take medicines long term that reduce the activity of your immune system to help prevent relapses. These may include: immunosuppressants, such as azathioprine, steroid tablets, such as prednisolone, biological medicines, such as rituximab. […] You may also be offered medicines to help with any ongoing symptoms, such as: painkillers, antidepressant or anticonvulsant medicines that help with nerve pain, such as amitriptyline or gabapentin, muscle relaxant or anticonvulsant medicines to treat muscle spasms, such as baclofen or carbamazepine, medicines for bladder problems, such as oxybutynin, medicines for bowel problems, such as laxatives. […] To help with symptoms such as muscle weakness and mobility problems you may be offered: physiotherapy, including advice on exercises and stretching, occupational therapy to help with daily activities.

• #54 Neuromyelitis optica (NMO)

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

  If you’re diagnosed with neuromyelitis optica (NMO) you’ll need treatment to stop the inflammation in your nerves and reduce the risk of relapses. […] When your symptoms first start or if you’re having a relapse, where symptoms come back, the main treatments are: high dose steroid injections, plasma exchange, where some of your blood is removed, treated and then returned to your body, immunoglobulins (antibodies in the blood) given through a drip into your vein. […] You’ll usually also need to take medicines long term that reduce the activity of your immune system to help prevent relapses. These may include: immunosuppressants, such as azathioprine, steroid tablets, such as prednisolone, biological medicines, such as rituximab. […] You may also be offered medicines to help with any ongoing symptoms, such as: painkillers, antidepressant or anticonvulsant medicines that help with nerve pain, such as amitriptyline or gabapentin, muscle relaxant or anticonvulsant medicines to treat muscle spasms, such as baclofen or carbamazepine, medicines for bladder problems, such as oxybutynin, medicines for bowel problems, such as laxatives. […] To help with symptoms such as muscle weakness and mobility problems you may be offered: physiotherapy, including advice on exercises and stretching, occupational therapy to help with daily activities.

• #55 Is there a Cure for Neuromyelitis Optica?

  https://www.dragarwal.com/blog/eye-wellness/is-there-a-cure-for-neuromyelitis-optica/

  Depending on the patients symptoms, medications such as pain relievers, muscle relaxants, and drugs to manage bladder and bowel dysfunction may be prescribed. […] Physical therapy and occupational therapy can help patients regain strength and independence after a relapse. […] Ongoing research offers hope for the future: […] Several experimental therapies are in development, including monoclonal antibodies that specifically target AQP4 antibodies, complement inhibitors, and other immune-modulating drugs. These therapies hold promise for preventing relapses and improving outcomes. […] Neuromyelitis Optica is a complex and challenging autoimmune disease that primarily affects the central nervous system. While there is currently no definitive cure for NMO, significant progress has been made in understanding the condition, diagnosing it more accurately, and developing targeted therapies to manage symptoms and prevent relapses.

• #56 Neuromyelitis optica (NMO)

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

  If you’re diagnosed with neuromyelitis optica (NMO) you’ll need treatment to stop the inflammation in your nerves and reduce the risk of relapses. […] When your symptoms first start or if you’re having a relapse, where symptoms come back, the main treatments are: high dose steroid injections, plasma exchange, where some of your blood is removed, treated and then returned to your body, immunoglobulins (antibodies in the blood) given through a drip into your vein. […] You’ll usually also need to take medicines long term that reduce the activity of your immune system to help prevent relapses. These may include: immunosuppressants, such as azathioprine, steroid tablets, such as prednisolone, biological medicines, such as rituximab. […] You may also be offered medicines to help with any ongoing symptoms, such as: painkillers, antidepressant or anticonvulsant medicines that help with nerve pain, such as amitriptyline or gabapentin, muscle relaxant or anticonvulsant medicines to treat muscle spasms, such as baclofen or carbamazepine, medicines for bladder problems, such as oxybutynin, medicines for bowel problems, such as laxatives. […] To help with symptoms such as muscle weakness and mobility problems you may be offered: physiotherapy, including advice on exercises and stretching, occupational therapy to help with daily activities.

• #57 Neuromyelitis Optica | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/neuromyelitis-optica.html

  Our physical and occupational therapists are especially skilled in treating people with NMO and other complex autoimmune and neurological disorders. […] Your rehabilitation and therapy may include: Physical therapy for improving mobility, strength, coordination, and balance, Occupational therapy for performing daily activities, such as getting dressed, cooking, and driving.

• #58 Neuromyelitis Optica | Stanford Health Care

  https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/neuromyelitis-optica.html

  Our physical and occupational therapists are especially skilled in treating people with NMO and other complex autoimmune and neurological disorders. […] Your rehabilitation and therapy may include: Physical therapy for improving mobility, strength, coordination, and balance, Occupational therapy for performing daily activities, such as getting dressed, cooking, and driving.

• #59 Journal of Rehabilitation Medicine – Multidisciplinary rehabilitation for adults with neuromyelitis optica spectrum disorders: A pilot study – HTML

  https://www.medicaljournals.se/jrm/content/html/10.2340/16501977-2587

  Objective: To provide detailed data on the effects of multidisciplinary rehabilitation for patients with neuromyelitis optica spectrum disorder with moderate to severe disability. […] Conclusion: These results suggest that multidisciplinary rehabilitation potentially promotes motor functional recovery in patients with neuromyelitis optica spectrum disorders. […] The aim of this study is to investigate the effects of multidisciplinary rehabilitation for patients with neuromyelitis optica spectrum disorders who have moderate to severe disability. […] These results confirm the short-term effectiveness of multidisciplinary rehabilitation in patients with neuromyelitis optica spectrum disorders in terms of improving impairment. […] The results also suggest that multidisciplinary rehabilitation is a safe and feasible therapy for adults with neuromyelitis optica who have severe disability.

• #60 Effects of physical therapy intervention in the management of neuromyelitis optica: a case report | Bulletin of Faculty of Physical Therapy | Full Text

  https://bfpt.springeropen.com/articles/10.1186/s43161-022-00111-w

  This case report showed physical therapy is effective in improving muscle strength, balance, gait impairments, and ADLs in patients with NMO. However, there is a need for further studies focusing on the role of physiotherapy in the management of NMO, and the inclusion of recent technologies like virtual reality, and TecnoBody needs to be addressed with better outcome measures for the study.

• #61 Neuromyelitis Optica Spectrum Disorder (NMOSD) | OHSU

  https://www.ohsu.edu/brain-institute/neuromyelitis-optica-spectrum-disorder-nmosd

  Nerve pain: NMOSD attacks can cause long-term pain, numbness and other nerve issues. Our team can recommend medications and help you find therapies such as acupuncture and meditation. […] Depression: It’s normal to feel sad or stressed after an NMOSD diagnosis. If your feelings interfere with your relationships or daily life, you may have depression that should be treated. Our team can help with counseling and medications.

• #62 Diet and Neuromyelitis Optica (NMO): A Three Step Approachmail2

  https://guthyjacksonfoundation.org/diet-and-nmo-a-three-step-approach/

  Fatty acids affect immune system function. Immune system suppression, which could be beneficial for NMO, is produced by omega-six fatty acids and especially by omega-three fatty acids. […] People with NMO may want to consider strategies that increase the intake of vitamin D and PUFAs: Take supplements of vitamin D if the blood level of vitamin D is low. […] Omega-three fatty acids are available in a wide variety of supplements, including fish oil, salmon oil, and cod liver oil. […] People with NMO should avoid or use caution with supplements that may increase immune system activity, cause significant side effects, or interact with medications.

• #63 Diet and Neuromyelitis Optica (NMO): A Three Step Approachmail2

  https://guthyjacksonfoundation.org/diet-and-nmo-a-three-step-approach/

  Fatty acids affect immune system function. Immune system suppression, which could be beneficial for NMO, is produced by omega-six fatty acids and especially by omega-three fatty acids. […] People with NMO may want to consider strategies that increase the intake of vitamin D and PUFAs: Take supplements of vitamin D if the blood level of vitamin D is low. […] Omega-three fatty acids are available in a wide variety of supplements, including fish oil, salmon oil, and cod liver oil. […] People with NMO should avoid or use caution with supplements that may increase immune system activity, cause significant side effects, or interact with medications.

• #64 Diet and Neuromyelitis Optica (NMO): A Three Step Approachmail2

  https://guthyjacksonfoundation.org/diet-and-nmo-a-three-step-approach/

  Many people with NMO and related conditions would like to use dietary approaches to try to control the disease. […] The dietary strategies discussed in this article should not be used instead of conventional medications. […] Unfortunately, no dietary approach has ever been systematically studied in NMO. Thus, for those who are only interested in absolutely proven therapies, there is not a dietary approach that can be formally recommended. This article provides information about approaches that are not absolutely proven but are low risk and may possibly benefit the underlying disease process in NMO. […] Two dietary approaches for which there is evidence for suppressing the immune system are vitamin D and fatty acids. […] Vitamin D has not been rigorously studied in NMO. It has been fairly extensively studied in MS.

• #65 Diet and Neuromyelitis Optica (NMO): A Three Step Approachmail2

  https://guthyjacksonfoundation.org/diet-and-nmo-a-three-step-approach/

  Fatty acids affect immune system function. Immune system suppression, which could be beneficial for NMO, is produced by omega-six fatty acids and especially by omega-three fatty acids. […] People with NMO may want to consider strategies that increase the intake of vitamin D and PUFAs: Take supplements of vitamin D if the blood level of vitamin D is low. […] Omega-three fatty acids are available in a wide variety of supplements, including fish oil, salmon oil, and cod liver oil. […] People with NMO should avoid or use caution with supplements that may increase immune system activity, cause significant side effects, or interact with medications.

• #66 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

  Acute treatment is critically important in NMOSD as exacerbations result in severe residual disability. Therefore, relapse therapies should be started early and aggressively. […] Relapses are commonly treated with high-dose IV methylprednisolone (IVMP) 1 g/daily for 3–7 days, followed by tapered oral steroids. […] Plasma exchange (PLEX) every other day for 2 weeks (1.5 L volume, 5–7 treatments) or immunoadsorption are recommended within 5 days from NMOSD relapse onset, when response to IVMP is poor or absent. […] New therapies for acute NMOSD relapses, including bevacizumab, ublituximab, NPB-1, and HBM9161, are currently under investigation. […] Long-term relapse prevention treatment is recommended for all patients who are diagnosed with NMOSD. […] Off label use of some older immunosuppressive agents such as AZA, MMF, and rituximab have shown reductions in ARR, with disability stabilization in retrospective, prospective, and meta-analysis studies. […] Eculizumab was the first monoclonal approved by the Food and Drug Administration to prevent NMOSD relapses in AQP4-ab-positive patients in June 2019, followed by Inebilizumab (June 2020) and satralizumab (August 2020). […] Long-term impact of recently developed drugs remains to be established.

• #67 Treatment of neuromyelitis optica: state-of-the-art and emerging therapies | Nature Reviews Neurology

  https://www.nature.com/articles/nrneurol.2014.141

  Potential therapeutic approaches include inhibition of AQP4-IgG binding (aquaporumab, small molecules), AQP4-IgG inactivation (endoglycosidase S, IgG-degrading enzyme), alternative complement inhibitors (C1inh, compstatin, CD59), anti-FcRIII antibody, VEGF inhibition (bevacizumab), and antigen-specific tolerization. […] Challenges for NMO therapeutics include optimization of drug penetration into NMO lesions, clinical trial design (given the small patient numbers), and management of seronegative patients.

• #68 Treatment of neuromyelitis optica: state-of-the-art and emerging therapies | Nature Reviews Neurology

  https://www.nature.com/articles/nrneurol.2014.141

  Potential therapeutic approaches include inhibition of AQP4-IgG binding (aquaporumab, small molecules), AQP4-IgG inactivation (endoglycosidase S, IgG-degrading enzyme), alternative complement inhibitors (C1inh, compstatin, CD59), anti-FcRIII antibody, VEGF inhibition (bevacizumab), and antigen-specific tolerization. […] Challenges for NMO therapeutics include optimization of drug penetration into NMO lesions, clinical trial design (given the small patient numbers), and management of seronegative patients.

• #69 Neuromyelitis Optica (NMO) Treatment | Rush

  https://www.rush.edu/services/neuromyelitis-optica-nmo-services

  Neuromyelitis optica specialists at Rush can prescribe a full range of care plans that address your whole brain health and aim to give you the best quality of life possible. […] Treatment is most effective when it begins as early as possible. Early intervention can slow or stop progression of the disease and prevent disabilities. […] Once you have a diagnosis, you will have a team of physicians from many different specialties all working together on a care plan that is personalized for your needs and goals. […] Their long-term goal is to prevent NMO attacks or relapses and give you the best quality of life possible. […] Our neurologists can provide you with all approved medications to treat NMO. This includes the latest infusion therapies. […] Getting a second opinion for an NMO diagnosis or prescribed treatment can help you make decisions about your care with confidence.

• #70 Treatment of Neuromyelitis Optica: Review and Recommendations

  https://pmc.ncbi.nlm.nih.gov/articles/PMC3926208/

  Guidelines for preventive therapy in NMO have been crafted to balance effectiveness with short- and long-term side effects, but clinicians must also consider age, associated medical conditions, functional status, access to medications and response to previous preventative therapies. […] We recommend preventive therapy with one of the six immunosuppressive regimens that have multiple studies reporting efficacy in NMO – azathioprine, rituximab, mycophenolate mofetil, methotrexate, prednisone and mitoxantrone. […] The duration of preventive treatment in NMO that is needed has not been adequately studied. […] Azathioprine is effective in a variety of applications for immunosuppressive therapy. […] Mycophenolate is used in a variety of autoimmune conditions. […] Rituximab is a chimeric mouse/human monoclonal antibody directed against the CD20 antigen.

• #71 Neuromyelitis Optica Spectrum Disorder (NMOSD)

  https://www.massgeneral.org/neurology/treatments-and-services/about-neuromyelitis-optica-nmosd

  Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system in which a persons immune system invades the optic nerves and spinal cord leading to vision loss and paralysis. […] Corticosteroids and plasmapheresis (removing and replacing components of a patients blood) are effective in stopping NMO relapses. […] In order to prevent additional injury from future attacks, it is imperative that people with NMOSD take medication regularly. NMOSD never goes away on its own, so preventive treatment is required lifelong. There are a number of medications that successfully suppress the immune system in NMOSD patients. […] Though there is no cure for NMOSD at this time, we are encouraged that the last fifteen years have brought an abundance of interest and a number of positive therapies, including three drugs approved for NMOSD by the FDA in 2019 and 2020.

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