Materiały źródłowe

• #1 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  If your symptoms respond to immunotherapy, the medicine doses are slowly lowered over time, known as tapering. You may take an oral corticosteroid at a lower dose for several weeks. Or you may get monthly doses of methylprednisolone or immunoglobulin through an IV for several months. […] If your symptoms aren’t improving, your healthcare professional also may recommend the medicines rituximab (Rituxan, Truxima, others), cyclophosphamide or tocilizumab (Actemra, Tofidence, Tyenne). These medicines can improve symptoms and help lower the chances of AE coming back. […] Most people recover with treatment. The earlier you receive treatment, the more quickly you may recover. Early treatment also lowers the chances of having lasting symptoms due to AE or having another bout of autoimmune encephalitis.

• #2 Autoimmune Encephalitis: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/autoimmune-encephalitis

  The effectiveness of treatment for AE varies from case to case. Some people with AE respond very quickly to the first medications they receive within days. Others will need treatment that lasts for weeks or months. […] In general, getting treatment early in the course of autoimmune encephalitis tends to decrease your risk of long-term complications and relapse. […] Autoimmune encephalitis generally responds well to treatment and goes away. But this can take a long time for some people. AE can also relapse (come back), especially if you have cancer that’s not responding to treatment.

• #3 Treatment strategies for autoimmune encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5784571/

  Autoimmune encephalitis is one of the most rapidly growing research topics in neurology. Along with discoveries of novel antibodies associated with the disease, clinical experience and outcomes with diverse immunotherapeutic agents in the treatment of autoimmune encephalitis are accumulating. Retrospective observations indicate that early aggressive treatment is associated with better functional outcomes and fewer relapses. Immune response to first-line immunotherapeutic agents (corticosteroids, intravenous immunoglobulin, plasma exchange, and immunoadsorption) is fair, but approximately half or more of patients are administered second-line immunotherapy (rituximab and cyclophosphamide). A small but significant proportion of patients are refractory to all first- and second-line therapies and require further treatment. Although several investigations have shown promising alternatives, the low absolute number of patients involved necessitates more evidence to establish further treatment strategies. In this review, the agents used for first- and second-line immunotherapy are discussed and recent attempts at finding new treatment options are introduced.

• #4 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. […] Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. […] As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. […] Several retrospective studies have shown that early and aggressive immunotherapy is associated with better outcomes in AE patients.

• #5 Autoimmune Encephalitis Treatment | AE Alliance

  https://aealliance.org/patient-support/treatment/

  If the combination of symptoms and test results suggest that a person has “possible AE”, the current recommendations are to start first line medications that treat inflammation in the brain, such as steroids, IV immune globulin, and/or plasma exchange. […] In addition to the medications that act on the immune system to decrease brain inflammation, many people with AE will need supportive medications to treat the AE symptoms that cause suffering and disability. […] As the results return from the workup for AE, including anti-neuronal antibody testing, health care providers will be able to decide if a patient meets the criteria for “definite AE” or “probable AE” and further treatment decisions will be discussed. […] Some people with AE improve within days of receiving their first line treatments and don’t need additional medications that act on the immune system.

• #6 Diagnosis and Management of Autoimmune Encephalitis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/diagnosis-and-management-of-autoimmune-encephalitis

  A: Treatment guidelines for AE are based on a combination of expert opinion, case series and case reports.911 Treatment initiation and escalation depends on the certainty of diagnosis of AE along with the clinical severity. […] First-line therapies we employ include high-dose intravenous corticosteroids, intravenous immunoglobulin, or plasmapheresis. These can be used sequentially or in combination with little evidence favoring one modality over the other. […] Second-line agents commonly used include rituximab (a monoclonal antibody targeting CD20-positive lymphocytes) and cyclophosphamide (an alkylating agent that targets rapidly dividing cells).12 Although these agents are classified as second-line therapies, they can be used in the acute phase of the disease either due to the disease severity of the presentation or to reduce the risk of relapse.

• #7 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The 2016 AE clinical criteria emphasise the importance of starting immunotherapy once AE is highly suspected and infectious etiologies are excluded based on CSF results (cell-count, glucose, viral PCR, gram stain). […] Empiric treatment with intravenous methylprednisolone at a dose of 1 g per day for 3-7 days is a common reasonable approach to achieve initial immunosuppressive and anti-inflammatory effect in AE patients. […] On our AEACN survey, 84% of responders chose corticosteroids alone (65%) or in combination with other agents (19%) for initial immunotherapy in patients with a general AE presentation. […] IVIg can be more readily available than PLEX in some centres and it does not require a central line. […] PLEX (5-10 sessions every other day) is an effective option for acute immunomodulation when corticosteroids are contraindicated or ineffective.

• #8 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Autoimmune-Encephalitis-Treatment.aspx

  Autoimmune encephalitis (AIE) is a term used to describe a group of conditions in which autoantibodies are formed against various antigens present in or on the neurons and appear as neurologic manifestations involving the central nervous system. […] Treatment of autoimmune encephalitides includes immunotherapy, either corticosteroids or intravenous immunoglobulins (IVIG). […] Provided a synaptic or cell-surface antibody has been detected, and the patient has symptoms suggestive of AIE, first-line therapy is promptly given, as aggressive and early treatment of ill patients, with escalation if improvement is not satisfactory, generally leads to better outcomes and fewer relapses. […] First-line therapy consists of intravenous, high dose corticosteroids and IVIG/plasmapheresis. […] Failure of first-line therapy to reverse the symptoms occurs in about 50%, and leads to the prompt use of second-line drugs.

• #9 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  Autoimmune encephalitis treatment focuses on the immune system, which is mistakenly attacking brain cells. If a tumor is causing autoimmune encephalitis, the first step is to diagnose and treat the tumor. […] Immunotherapy works by reducing the immune system’s activity and improving inflammation. […] Two types of immunotherapy are given through an IV in a vein in the arm. Most people get this therapy in a hospital. Medicines include: Methylprednisolone (Solu-Medrol). This high-dose steroid is given daily for 3 to 7 days. Immunoglobulin (IVIg). This medicine may be given daily for 2 to 5 days. […] Other treatment options include: Oral corticosteroids. With this medicine, you start with a larger dose and then slowly lower the dose over weeks to months, known as tapering. Plasma exchange. This therapy gets rid of antibodies that are causing the immune system to attack brain cells. During plasma exchange, the liquid part of your blood is removed and separated from your blood cells. The blood cells are put back into your body and your body makes more plasma.

• #10 Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management S | IJGM

  https://www.dovepress.com/anti-nmda-receptor-autoimmune-encephalitis-diagnosis-and-management-st-peer-reviewed-fulltext-article-IJGM

  There is consensus that corticosteroids should be the first agent used in anti-NMDAR encephalitis, often in conjunction with IVIG and/or PLEX. […] Although there is no compelling evidence to suggest the superiority of any specific regimen, IV steroids are frequently the first choice. Steroids alone, however, may be insufficient to ameliorate the autoantibody-mediated immune process, and direct removal or neutralization of autoantibodies from the circulation by PLEX and IVIG, respectively, may have a synergistic effect. There is increasing evidence to support use of steroid combination therapy over steroid alone. […] After completing pulsed IV corticosteroid therapy, a common strategy is to start oral prednisone 12 mg/kg/day immediately followed by a gradual taper over weeks to months overlapping with long-term immunotherapy if indicated. This is used, in theory, to avoid possible relapses.

• #11

  https://indianpediatrics.net/july2020/july-662-670.htm

  Corticosteroids form the cornerstone of treatment. They have good penetration across the blood brain barrier and have a broad spectrum of anti-inflammatory activity. […] If AIE is suspected, empirical therapy has to be initiated immediately. Waiting for the results of antibody tests is not an essential pre-requisite. […] A significant proportion of patients respond to first line therapy, showing benefit of treatment within the first 1-2 weeks of treatment initiation. Non-responders are treated with 2nd line agents viz, rituximab or Cyclophosphamide. […] Third line agents are needed when both 1st and 2nd line agents fail. […] Mycophenolate mofetil (MMF), methotrexate and azathioprine have been used as steroid-sparing agents in paediatric anti-NMDAR encephalitis. […] Symptomatic management should be given along with immunosuppressive treatment. […] Relapses tend to be uncommon in AIE. However, when they do occur, they are managed with repeat dosing of the first line agents.

• #12 Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management S | IJGM

  https://www.dovepress.com/anti-nmda-receptor-autoimmune-encephalitis-diagnosis-and-management-st-peer-reviewed-fulltext-article-IJGM

  IVIG is a pooled preparation of human immunoglobulins obtained from several thousand healthy donors. For patients with medical conditions that restrict the use of corticosteroids, such as uncontrolled hypertension or diabetes or there is concern for active infection, IVIG is often the initial treatment. IVIG has a better side effect profile than corticosteroids and is often more accessible than PLEX. […] PLEX is a non-selective extracorporeal blood purification method involving elimination of patient plasma and subsequent substitution with another solution. In AE, PLEX is often used in combination with steroids or following steroids rather than initial first-line therapy. […] The majority of patients respond to first-line immunotherapy. In those who fail to improve within two weeks of initiation of two or more first-line therapies, second-line treatment is recommended over continuing or repeating first-line therapies, with rituximab preferred over cyclophosphamide.

• #13 IVIG for Autoimmune Encephalitis | AmeriPharma® Specialty

  https://ameripharmaspecialty.com/ivig/a-guide-to-ivig-therapy-for-autoimmune-encephalitis/

  Autoimmune encephalitis is a rare and complex condition that can affect the brain and cause fatal consequences. Providers have recently become familiar with this disease. One treatment for this condition is IVIG, or intravenous immunoglobulin therapy. IVIG is effective against autoimmune encephalitis, and as someone dealing with this condition, you should learn more about this treatment. […] Patients who don’t receive IVIG for autoimmune encephalitis, or undergo another treatment, can experience serious consequences, such as coma, permanent brain damage, and even death. […] Treatment involves a combination of immunotherapy and supportive care. The most common therapies include: […] Intravenous immunoglobulin (IVIG) for autoimmune encephalitis is a promising approach. The treatment involves the administration of IVIG to help manage the symptoms of AE.

• #14 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. […] We reviewed common forms of AIE and discuss their diagnostic approach and treatment. […] Autoimmune encephalitis has a wide variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders and seizures. […] We reviewed common causes of AIE and discuss their pathophysiology, diagnostic approach and management. […] Various treatment approaches including corticosteroids, intravenous immunoglobulin, plasma exchange, rituximab and cyclophosphamide are currently used. […] Evidence suggests that early immunotherapy improves outcome, thus treatment for AIE should not be delayed. […] Patients should receive either methylprednisolone 1 g IV for 3-5 days and intravenous immunoglobulin (0.4 g/kg/day for five days) or methylprednisolone and plasmapheresis.

• #15 Treatment Of Autommune Encephalitis | Immunotherapy Treatment

  https://autoimmune-encephalitis.org/treatment/

  IVIG offers an important advantage of being unlikely to make an infectious encephalitis worse. IVIG is a blood product prepared from the serum of more than 1,000 donors that contains a broad range of antibodies. Some of these antibodies target a patient’s autoantibodies and neutralize them, along with other pro-inflammatory aspects of the immune system. IVIG can inhibit humoral and cellular immunity and regulate immune responses through a variety of mechanisms. […] Plasmapheresis is also unlikely to significantly worsen infectious encephalitis. Plasmapheresis (also called PLEX) “cleans” the blood of autoantibodies by replacing the liquid plasma portion of a patient’s blood with that of a donor thereby improving the disease. Plasmapheresis also changes T and B cells in favorable ways. […] The response to immune therapy is generally good, particularly if the more effective treatments are used promptly. However, powerful immune suppression may be needed for weeks or months in difficult cases and treatment may take many months to reach its full effects.

• #16 Treatment Of Autommune Encephalitis | Immunotherapy Treatment

  https://autoimmune-encephalitis.org/treatment/

  IVIG offers an important advantage of being unlikely to make an infectious encephalitis worse. IVIG is a blood product prepared from the serum of more than 1,000 donors that contains a broad range of antibodies. Some of these antibodies target a patient’s autoantibodies and neutralize them, along with other pro-inflammatory aspects of the immune system. IVIG can inhibit humoral and cellular immunity and regulate immune responses through a variety of mechanisms. […] Plasmapheresis is also unlikely to significantly worsen infectious encephalitis. Plasmapheresis (also called PLEX) “cleans” the blood of autoantibodies by replacing the liquid plasma portion of a patient’s blood with that of a donor thereby improving the disease. Plasmapheresis also changes T and B cells in favorable ways. […] The response to immune therapy is generally good, particularly if the more effective treatments are used promptly. However, powerful immune suppression may be needed for weeks or months in difficult cases and treatment may take many months to reach its full effects.

• #17 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  Autoimmune encephalitis treatment focuses on the immune system, which is mistakenly attacking brain cells. If a tumor is causing autoimmune encephalitis, the first step is to diagnose and treat the tumor. […] Immunotherapy works by reducing the immune system’s activity and improving inflammation. […] Two types of immunotherapy are given through an IV in a vein in the arm. Most people get this therapy in a hospital. Medicines include: Methylprednisolone (Solu-Medrol). This high-dose steroid is given daily for 3 to 7 days. Immunoglobulin (IVIg). This medicine may be given daily for 2 to 5 days. […] Other treatment options include: Oral corticosteroids. With this medicine, you start with a larger dose and then slowly lower the dose over weeks to months, known as tapering. Plasma exchange. This therapy gets rid of antibodies that are causing the immune system to attack brain cells. During plasma exchange, the liquid part of your blood is removed and separated from your blood cells. The blood cells are put back into your body and your body makes more plasma.

• #18

  https://www.nhs.uk/conditions/encephalitis/treatment/

  plasmapheresis a procedure which removes the substances that are attacking the brain from the blood […] surgery to remove abnormal growths (tumours) if encephalitis was triggered by a tumour somewhere in the body […] antibiotics or antifungal medicine used if encephalitis is caused by a bacterial or fungal infection. […] If there’s no treatment for the underlying cause, treatment is given to support the body, relieve symptoms, and allow the best chance of recovery. […] Most people need treatment to relieve these symptoms and to support certain bodily functions until they’re feeling better. […] This may involve: fluids given into a vein to prevent dehydration […] painkillers to reduce discomfort or a high temperature […] medicine to control seizures or fits […] medicine to help the person relax if they’re very agitated

• #19 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The 2016 AE clinical criteria emphasise the importance of starting immunotherapy once AE is highly suspected and infectious etiologies are excluded based on CSF results (cell-count, glucose, viral PCR, gram stain). […] Empiric treatment with intravenous methylprednisolone at a dose of 1 g per day for 3-7 days is a common reasonable approach to achieve initial immunosuppressive and anti-inflammatory effect in AE patients. […] On our AEACN survey, 84% of responders chose corticosteroids alone (65%) or in combination with other agents (19%) for initial immunotherapy in patients with a general AE presentation. […] IVIg can be more readily available than PLEX in some centres and it does not require a central line. […] PLEX (5-10 sessions every other day) is an effective option for acute immunomodulation when corticosteroids are contraindicated or ineffective.

• #20 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The 2016 AE clinical criteria emphasise the importance of starting immunotherapy once AE is highly suspected and infectious etiologies are excluded based on CSF results (cell-count, glucose, viral PCR, gram stain). […] Empiric treatment with intravenous methylprednisolone at a dose of 1 g per day for 3-7 days is a common reasonable approach to achieve initial immunosuppressive and anti-inflammatory effect in AE patients. […] On our AEACN survey, 84% of responders chose corticosteroids alone (65%) or in combination with other agents (19%) for initial immunotherapy in patients with a general AE presentation. […] IVIg can be more readily available than PLEX in some centres and it does not require a central line. […] PLEX (5-10 sessions every other day) is an effective option for acute immunomodulation when corticosteroids are contraindicated or ineffective.

• #21 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Autoimmune-Encephalitis-Treatment.aspx

  This includes Rituximab, which is a monoclonal anti-CD20 antibody, and its use therefore prevents the employment of plasmapheresis which would remove it from the blood. […] The treatment of relapse is with second-line therapy, though first-line treatment may be tried first. […] Tumor removal is essential in most cases to remove the source of the antibodies and to improve the prognosis of the tumor itself. […] Behavioral and psychotic symptoms may require the use of antipsychotics. […] Steroids may cause psychosis, however, benzodiazepines and clonidine, among others, may help induce sleep in sleep-deprived individuals. […] Prognostic factors included: Early therapy, Second-line treatment in case of failure of first-line therapy.

• #22 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis patients who fail to improve after 10-14 days should receive second-line therapies such as rituximab or cyclophosphamide, or both. […] Better prognosis has been associated with early treatment, no requirement for intensive care admission and non-paraneoplastic AIE. […] The treatment response and relapse rate vary among patients with AIE. […] Relapses may occur in 31% of patients with anti-LGI1 encephalitis and 10% of those with anti-CASPR2 encephalitis, sometimes years after the first episode. […] The nature of the antibody, and to a lesser extent the clinical syndrome, determines the risk and type of an underlying malignancy. […] Tumor treatment is essential for neurological improvement.

• #23 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Autoimmune-Encephalitis-Treatment.aspx

  Autoimmune encephalitis (AIE) is a term used to describe a group of conditions in which autoantibodies are formed against various antigens present in or on the neurons and appear as neurologic manifestations involving the central nervous system. […] Treatment of autoimmune encephalitides includes immunotherapy, either corticosteroids or intravenous immunoglobulins (IVIG). […] Provided a synaptic or cell-surface antibody has been detected, and the patient has symptoms suggestive of AIE, first-line therapy is promptly given, as aggressive and early treatment of ill patients, with escalation if improvement is not satisfactory, generally leads to better outcomes and fewer relapses. […] First-line therapy consists of intravenous, high dose corticosteroids and IVIG/plasmapheresis. […] Failure of first-line therapy to reverse the symptoms occurs in about 50%, and leads to the prompt use of second-line drugs.

• #24 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  If the initial clinical picture is severe (eg, NMDAR-antibody encephalitis, NORSE, severe dysautonomia), clinicians may consider using combined first-line therapies from the beginning despite the lack of high quality evidence to support this practice. […] If there is no meaningful clinical or radiological response to optimised first-line therapy after 24 weeks, the addition of a second-line agent with both rapid and sustained immunosuppressive effects can improve the outcome. […] On the AEACN survey, 50% of responders indicated they would consider adding a second-line agent in the acute setting only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in the acute setting on all patients regardless of the response to first-line therapy.

• #25 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. […] Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. […] As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. […] Several retrospective studies have shown that early and aggressive immunotherapy is associated with better outcomes in AE patients.

• #26 Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management S | IJGM

  https://www.dovepress.com/anti-nmda-receptor-autoimmune-encephalitis-diagnosis-and-management-st-peer-reviewed-fulltext-article-IJGM

  Rituximab is a B-cell depleting, partially humanized monoclonal antibody directed against CD20 with established efficacy in many autoimmune neurological diseases. […] Cyclophosphamide is an alkylating agent that impairs DNA replication or transcription, eventually leading to programmed cell death. In addition to anti-mitotic and anti-replicative effects, it has immunosuppressive as well as immunomodulatory properties that affect both B and T cells. […] The use of second-line immunotherapy is still variable globally and can be considerably less frequent in some countries. […] Relapses can occur in 10-30% of cases, usually within the first two years from onset. There is no convincing evidence for chronic maintenance immunotherapy in anti-NMDAR encephalitis except for patients with relapses despite second-line immunotherapy or those with severe and prolonged impairments. Re-dosing of rituximab, monthly IVIG for greater than 6 months, and long-term maintenance of prednisolone or steroid-sparing agents, such as azathioprine and mycophenolate mofetil, are among the options that have been used so far.

• #27 Simplified regimen of combined low-dose rituximab for autoimmune encephalitis with neuronal surface antibodies | Journal of Neuroinflammation | Full Text

  https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-022-02622-8

  Currently, the empirical protocols for AE are mainly derived from other disease processes such as lymphoma or rheumatoid arthritis (RA), including 375 mg/m2 weekly for 4 consecutive weeks or two doses of 1000 mg 2 weeks apart, then followed by reinfusions at fixed intervals for immunosuppressive maintenance, because of circulating B cell below the detectable range for 6-8 months after administration. […] Thus, in present study, we performed a simplified regimen in AE treatment, including regular induction of 3 cycles for acute and bridging management, and subsequent reinfusions (100 mg once) at fixed interval (every 6 months) for sustained immunosuppression. […] Altogether, in present study, the simplified regimen of combined low-dose rituximab (100 mg once) with common first-line therapy for AE with NSAbs, to our knowledge, firstly showed effective for short-term and long-term improvement, in parallel with reduced immunosuppressant and relapses, suggesting the advantages and benefits for combination of low-dose rituximab in the disease course.

• #28 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. […] Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. […] As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. […] Several retrospective studies have shown that early and aggressive immunotherapy is associated with better outcomes in AE patients.

• #29 The Diagnosis and Treatment of Autoimmune Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4712273/

  Although there are not randomized treatment trials, protocols have been proposed for anti-NMDAR encephalitis, and these approaches have been applied to other diseases in the cell-surface/synaptic autoantibody category. […] If the patient remains significantly impaired after first-line therapy, second-line treatments are typically used. […] Rituximab is a monoclonal antibody targeting CD20, so plasmapheresis generally should not be done after it is administered. […] Rituximab is thought to be generally effective against neurological diseases where the autoantibodies are of the IgG4 subtype. […] Cyclophosphamide has several important toxicities, including a risk of infertility, especially in young women who received repeated doses. […] In autoimmune encephalitis, relapse tends to follow a similar clinical course to the initial attack. […] The risk of relapse in anti-NMDAR encephalitis in approximately 12% over two years (but continues beyond that) and is highest in untreated patients, intermediate in patients who had only first-line therapy, and lowest in patients treated with second-line therapies.

• #30

  https://link.springer.com/article/10.1007/s11940-024-00786-7

  Third-line immunotherapy includes tocilizumab and bortezomib. […] The decision to start maintenance immunotherapy is still debated among experts. […] Current international consensus recommendations for treatment of NMDARE based on expert opinion recommend a similar treatment approach in both relapsing and refractory disease. […] A more recent study using a survival model demonstrated that rituximab treatment during the initial attack reduces the risk of relapse by 51%. […] Treatment outcomes in pediatric cohorts are largely published in the context of anti-NMDARE. […] While there is strong data to support early immunotherapy to improve clinical outcomes, there is less data on long-term management and treatment strategies to reduce the risk for relapsing disease.

• #31 Simplified regimen of combined low-dose rituximab for autoimmune encephalitis with neuronal surface antibodies | Journal of Neuroinflammation | Full Text

  https://jneuroinflammation.biomedcentral.com/articles/10.1186/s12974-022-02622-8

  Autoimmune encephalitis (AE) with neuronal surface antibodies (NSAbs) presents pathogenesis mediated by B cell-secreting antibodies. Rituximab is a second-line choice for the treatment for AE with NSAbs, which can cause B cell depletion via targeting CD20. However, the optimal protocol and dosage of rituximab combined with first-line therapy for NSAbs-associated AE remains unclear so far. In this study, we explored the efficacy and safety of low-dose rituximab combined with first-line treatment for NSAbs-associated AE. […] Our simplified regimen of combined low-dose rituximab firstly showed significantly accelerating short-term recovery and long-term improvement for AE with NSAbs, in parallel with markedly reduced prednisone dosage and clinical relapses. Moreover, opportunity of protocol showed earlier initiation (3 months) with better long-term improvement.

• #32 Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management S | IJGM

  https://www.dovepress.com/anti-nmda-receptor-autoimmune-encephalitis-diagnosis-and-management-st-peer-reviewed-fulltext-article-IJGM

  Rituximab is a B-cell depleting, partially humanized monoclonal antibody directed against CD20 with established efficacy in many autoimmune neurological diseases. […] Cyclophosphamide is an alkylating agent that impairs DNA replication or transcription, eventually leading to programmed cell death. In addition to anti-mitotic and anti-replicative effects, it has immunosuppressive as well as immunomodulatory properties that affect both B and T cells. […] The use of second-line immunotherapy is still variable globally and can be considerably less frequent in some countries. […] Relapses can occur in 10-30% of cases, usually within the first two years from onset. There is no convincing evidence for chronic maintenance immunotherapy in anti-NMDAR encephalitis except for patients with relapses despite second-line immunotherapy or those with severe and prolonged impairments. Re-dosing of rituximab, monthly IVIG for greater than 6 months, and long-term maintenance of prednisolone or steroid-sparing agents, such as azathioprine and mycophenolate mofetil, are among the options that have been used so far.

• #33 How do we treat autoimmune encephalitis? – GENERATE – GErman NEtwork for Research on AuToimmune Encephalitis [English]

  https://en.generate-net.de/how-do-we-treat-autoimmune-encephalitis.html

  According to experts opinion, the following immunosuppressant dosage and interval regimens are mostly used: […] First-line therapy: Plasmapheresis/immunoadsorption (5-10 cycles), Intravenous immunoglobulins (2 g/kg body weight for 5 days), Intravenous methylprednisolone pulse therapy (1 g/day for 5 days). […] Second-line therapy: Rituximab (1000 mg i.v. twice at 14-day intervals, repeat after 6 months as needed) innumerable experts meanwhile give rituximab as first-line therapy, underpinned by its comparatively favorable side effect profile and its relatively selective action on the depletion of B cells, Cyclophosphamide for 6-12 months at a dose of 750-1000 mg/m body surface area i.v. every 4 weeks with dose escalation as a function of leukocyte nadir, Azathioprine (100-250 mg/day), MTX (7.5-25 mg/week), folic acid rescue.

• #34 The Diagnosis and Treatment of Autoimmune Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4712273/

  Although there are not randomized treatment trials, protocols have been proposed for anti-NMDAR encephalitis, and these approaches have been applied to other diseases in the cell-surface/synaptic autoantibody category. […] If the patient remains significantly impaired after first-line therapy, second-line treatments are typically used. […] Rituximab is a monoclonal antibody targeting CD20, so plasmapheresis generally should not be done after it is administered. […] Rituximab is thought to be generally effective against neurological diseases where the autoantibodies are of the IgG4 subtype. […] Cyclophosphamide has several important toxicities, including a risk of infertility, especially in young women who received repeated doses. […] In autoimmune encephalitis, relapse tends to follow a similar clinical course to the initial attack. […] The risk of relapse in anti-NMDAR encephalitis in approximately 12% over two years (but continues beyond that) and is highest in untreated patients, intermediate in patients who had only first-line therapy, and lowest in patients treated with second-line therapies.

• #35 SciELO Brazil – Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adul

  https://www.scielo.br/j/anp/a/X4rrnygw43tryfbBp8Dvn8C/?lang=en

  Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. […] Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. […] The first-line treatment should preferably be the combination of MP and IVIG or MP and PLX. […] The second-line treatment should be initiated after 10 to 14 days of the start of the initial treatment if there is no satisfactory clinical response. […] The options for second-line treatment are rituximab alone or in combination with cyclophosphamide.

• #36 Treatment strategies for autoimmune encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5784571/

  Approximately 20-50% of patients with AE show inadequate responses to second-line therapies, and exhibit persistent neurological issues. […] A small number of studies reported a more targeted therapy with monoclonal antibodies or direct infusion of immune mediators. […] Tocilizumab is a monoclonal antibody targeting the IL-6 receptor. […] A recent observational study by Lee and colleagues showed that tocilizumab potentially improves clinical symptoms of AE in patients who do not respond adequately to rituximab. […] Lim and colleagues evaluated the feasibility of low-dose IL-2 therapy with 10 patients with refractory AE. […] Bortezomib is a proteasome inhibitor particularly effective at depleting plasma cells and is approved for the treatment of multiple myeloma. […] AE is not always monophasic and relapse of AE has been noted even after 5-10 years.

• #37 Canadian Consensus Guidelines for the Diagnosis and Treatment of Autoimmune Encephalitis in Adults | Canadian Journal of Neurological Sciences | Cambridge Core

  https://www.cambridge.org/core/journals/canadian-journal-of-neurological-sciences/article/canadian-consensus-guidelines-for-the-diagnosis-and-treatment-of-autoimmune-encephalitis-in-adults/DC88204486B656B85324E93AF2CCCFC7

  We suggest a low threshold to escalate immunotherapy, particularly in patients with severe AIE. […] There are a number of third-line and experimental immunotherapies (tocilizumab, bortezomib) which have been employed in cases refractory to second-line immunotherapy. […] Early initiation of appropriate treatment is essential to optimize outcomes. […] All patients with severe AIE should receive high dose corticosteroids with IVIg or PLEX as initial therapy; treatment with steroid monotherapy can be considered in mild/moderate cases but input from a specialist in autoimmune neurology is recommended. […] Second-line therapy should be offered to all patients with severe AIE who fail to improve or worsen 5-10 days after initiation of first-line therapy (2-4 weeks for mild/moderate cases). […] Early involvement of a specialist in autoimmune neurology is strongly recommended for all patients who fail first-line treatment.

• #38 Treatment strategies for autoimmune encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5784571/

  Approximately 20-50% of patients with AE show inadequate responses to second-line therapies, and exhibit persistent neurological issues. […] A small number of studies reported a more targeted therapy with monoclonal antibodies or direct infusion of immune mediators. […] Tocilizumab is a monoclonal antibody targeting the IL-6 receptor. […] A recent observational study by Lee and colleagues showed that tocilizumab potentially improves clinical symptoms of AE in patients who do not respond adequately to rituximab. […] Lim and colleagues evaluated the feasibility of low-dose IL-2 therapy with 10 patients with refractory AE. […] Bortezomib is a proteasome inhibitor particularly effective at depleting plasma cells and is approved for the treatment of multiple myeloma. […] AE is not always monophasic and relapse of AE has been noted even after 5-10 years.

• #39 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  If your symptoms respond to immunotherapy, the medicine doses are slowly lowered over time, known as tapering. You may take an oral corticosteroid at a lower dose for several weeks. Or you may get monthly doses of methylprednisolone or immunoglobulin through an IV for several months. […] If your symptoms aren’t improving, your healthcare professional also may recommend the medicines rituximab (Rituxan, Truxima, others), cyclophosphamide or tocilizumab (Actemra, Tofidence, Tyenne). These medicines can improve symptoms and help lower the chances of AE coming back. […] Most people recover with treatment. The earlier you receive treatment, the more quickly you may recover. Early treatment also lowers the chances of having lasting symptoms due to AE or having another bout of autoimmune encephalitis.

• #40 Treatment strategies for autoimmune encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5784571/

  Approximately 20-50% of patients with AE show inadequate responses to second-line therapies, and exhibit persistent neurological issues. […] A small number of studies reported a more targeted therapy with monoclonal antibodies or direct infusion of immune mediators. […] Tocilizumab is a monoclonal antibody targeting the IL-6 receptor. […] A recent observational study by Lee and colleagues showed that tocilizumab potentially improves clinical symptoms of AE in patients who do not respond adequately to rituximab. […] Lim and colleagues evaluated the feasibility of low-dose IL-2 therapy with 10 patients with refractory AE. […] Bortezomib is a proteasome inhibitor particularly effective at depleting plasma cells and is approved for the treatment of multiple myeloma. […] AE is not always monophasic and relapse of AE has been noted even after 5-10 years.

• #41 How do we treat autoimmune encephalitis? – GENERATE – GErman NEtwork for Research on AuToimmune Encephalitis [English]

  https://en.generate-net.de/how-do-we-treat-autoimmune-encephalitis.html

  Some specialists recommend a second-line therapy (including a combination of enhancement therapy drugs, e.g. rituximab together with cyclophosphamide) after as early as 10-14 days of no response. In therapy-refractory patients, more recent observations suggest a positive effect of bortezomib, the proteasome inhibitor used to deplete mainly mature plasma cells.

• #42

  https://link.springer.com/article/10.1007/s40263-022-00943-z

  A minority of patients with autoimmune encephalitis may remain refractory even to second-line therapies and they represent a major clinical challenge. In these cases, treatment strategies are controversial, and no guidelines exist. Treatments proposed for refractory autoimmune encephalitis include cytokine-based drugs, plasma cell-depleting agents, and treatments targeting intrathecal immune cells or their trafficking through the bloodbrain barrier. The evidence of efficacy of these treatments is mostly based on case reports or small case series, controlled studies and systematic reviews are rare. […] Different molecules have been proposed as potential treatments for refractory AEs and include plasma cell-depleting agents (bortezomib, daratumumab), cytokine-based drugs (interleukin-2/basiliximab, tocilizumab, anakinra, tofacitinib), and treatments targeting intrathecal immune cells or their trafficking through the BBB (intrathecal methotrexate, natalizumab).

• #43 Recovery – Autoimmune Encephalitis | IAES

  https://autoimmune-encephalitis.org/recovery/

  Those who remain refractory to this treatment have benefited from IL6 blockade (tocilizumab) or plasma cell-specific therapy (proteasome inhibitors). […] The speed of recovery, degree of residual deficit, and frequency of relapse vary according to the type of autoimmune encephalitis. […] Rapid diagnosis and treatment at disease onset as well as for continued and customized cognitive rehabilitation to improve the long-term outcome is of vital importance. […] To prevent relapses and maximize the response to acute phase therapy, maintenance immunotherapy should be instituted especially if early relapses during steroid taper occur. […] Commonly used drugs in this phase include oral corticosteroids, IVIG, and steroid-sparing agents such as mycophenolate mofetil, azathioprine, and rituximab.

• #44 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis patients who fail to improve after 10-14 days should receive second-line therapies such as rituximab or cyclophosphamide, or both. […] Better prognosis has been associated with early treatment, no requirement for intensive care admission and non-paraneoplastic AIE. […] The treatment response and relapse rate vary among patients with AIE. […] Relapses may occur in 31% of patients with anti-LGI1 encephalitis and 10% of those with anti-CASPR2 encephalitis, sometimes years after the first episode. […] The nature of the antibody, and to a lesser extent the clinical syndrome, determines the risk and type of an underlying malignancy. […] Tumor treatment is essential for neurological improvement.

• #45 Anti-NMDA Receptor Autoimmune Encephalitis: Diagnosis and Management S | IJGM

  https://www.dovepress.com/anti-nmda-receptor-autoimmune-encephalitis-diagnosis-and-management-st-peer-reviewed-fulltext-article-IJGM

  Rituximab is a B-cell depleting, partially humanized monoclonal antibody directed against CD20 with established efficacy in many autoimmune neurological diseases. […] Cyclophosphamide is an alkylating agent that impairs DNA replication or transcription, eventually leading to programmed cell death. In addition to anti-mitotic and anti-replicative effects, it has immunosuppressive as well as immunomodulatory properties that affect both B and T cells. […] The use of second-line immunotherapy is still variable globally and can be considerably less frequent in some countries. […] Relapses can occur in 10-30% of cases, usually within the first two years from onset. There is no convincing evidence for chronic maintenance immunotherapy in anti-NMDAR encephalitis except for patients with relapses despite second-line immunotherapy or those with severe and prolonged impairments. Re-dosing of rituximab, monthly IVIG for greater than 6 months, and long-term maintenance of prednisolone or steroid-sparing agents, such as azathioprine and mycophenolate mofetil, are among the options that have been used so far.

• #46 Recovery – Autoimmune Encephalitis | IAES

  https://autoimmune-encephalitis.org/recovery/

  Those who remain refractory to this treatment have benefited from IL6 blockade (tocilizumab) or plasma cell-specific therapy (proteasome inhibitors). […] The speed of recovery, degree of residual deficit, and frequency of relapse vary according to the type of autoimmune encephalitis. […] Rapid diagnosis and treatment at disease onset as well as for continued and customized cognitive rehabilitation to improve the long-term outcome is of vital importance. […] To prevent relapses and maximize the response to acute phase therapy, maintenance immunotherapy should be instituted especially if early relapses during steroid taper occur. […] Commonly used drugs in this phase include oral corticosteroids, IVIG, and steroid-sparing agents such as mycophenolate mofetil, azathioprine, and rituximab.

• #47 Autoimmune encephalitis: clinical spectrum and management | Practical Neurology

  https://pn.bmj.com/content/21/5/412

  Second-line therapies include rituximab, cyclophosphamide and other corticosteroid-sparing agents. […] In our experience, intravenous corticosteroids are generically highly effective agents, so relative contraindications (eg, pre-existing diabetes or psychiatric diseases) are often carefully managed in the acute phase but rarely considered absolute contraindications. […] We also find plasma exchange to be very effective, often used if patients show a limited or inadequate response to corticosteroids, or for patients with a rapid deterioration whose trajectory may otherwise be intensive care unit admission. […] In our experience, oral prednisolone should be maintained for around 2436 months, as shorter durations of corticosteroids are often associated with relapses. […] By contrast, rituximab appears more effective but longer-term follow-up is awaited.

• #48 Treatment strategies for autoimmune encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5784571/

  One study found that additional monthly rituximab after 4 weekly infusions contributed to further mRS improvement. […] The appropriate duration of maintenance therapy is currently unknown and the length of empirical use ranges widely from 6 months to several years according to the patients status and clinicians opinion. […] Although AE responds to immunotherapy and the majority of patients recover from the self-destructive autoimmune process, many patients fail to regain baseline cognitive and functional status. Early aggressive therapy is recommended in AE but steroid abuse should be avoided to prevent potential cognitive and other adverse effects. Each immunotherapeutic agent has its own strengths and weaknesses, and an appropriate combination of these agents is often needed to complement each other and achieve synergistic effects.

• #49 Encephalitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/encephalitis/diagnosis-treatment/drc-20356142

  Autoimmune encephalitis caused by tumors may require treatment of those tumors. This may include surgery, radiation, chemotherapy or a combination of treatments. […] People who are hospitalized with serious encephalitis might need: Breathing assistance, as well as careful monitoring of breathing and heart function. Intravenous fluids to ensure proper hydration and levels of essential minerals. Anti-inflammatory medicines, such as corticosteroids, to reduce swelling and pressure within the skull. Anti-seizure medicines to stop or prevent seizures. […] If you experience complications of encephalitis, you might need additional therapy, such as: Brain rehabilitation to improve cognition and memory. Physical therapy to improve strength, flexibility, balance, motor coordination and mobility. Occupational therapy to develop everyday skills and to use adaptive products that help with everyday activities. Speech therapy to relearn muscle control and coordination to produce speech. Psychotherapy to learn coping strategies and new behavioral skills to improve mood disorders or address personality changes.

• #50 Autoimmune Encephalitis: What It Is, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/autoimmune-encephalitis

  Autoimmune encephalitis is treatable with immunosuppressant medications. […] The main treatment goals for autoimmune encephalitis include immunosuppression and tumor removal (if applicable). […] First-line therapy works to decrease brain inflammation. It includes corticosteroids, IV immunoglobulins, and plasmapheresis. […] If this therapy isn’t effective, the next treatment options include rituximab (a monoclonal antibody injection) and cyclophosphamide (a chemotherapy medication). […] If you have a tumor, your healthcare team will recommend surgically removing it and/or chemotherapy. This helps speed up the improvement of AE. […] You may need additional medications to manage the symptoms of AE, including antiseizure medications to reduce or prevent seizures, medications that manage anxiety and depression, like antidepressants and anxiolytics, and antipsychotic medications if you’re experiencing psychosis.

• #51 Autoimmune encephalitis: clinical spectrum and management | Practical Neurology

  https://pn.bmj.com/content/21/5/412

  Given that immunotherapy is more effective than antiseizure medications in treating LGI1-antibody-associated seizures, early treatment with immunotherapy has shown great promise for preventing otherwise incipient cognitive impairment and functional disability. […] In NMDAR-antibody encephalitis, early treatment independently predicted good outcome (modified Rankin score 2) whereas delays in immunotherapy of 4 weeks were associated with poor functional outcomes at 1 year. […] There are several options for acute and long-term immunotherapies in both the inpatient and outpatient settings. […] Initial inpatient therapy often involves corticosteroids, intravenous immunoglobulins and/or plasma exchange. […] While awaiting autoantibody results, we start first-line immunotherapy when we are clinically confident of the diagnosis.

• #52 Autoimmune Encephalitis Treatment | AE Alliance

  https://aealliance.org/patient-support/treatment/

  It may be helpful for patients with AE to have a psychiatrist on their treatment team to discuss the different reasoning, risks and benefits of available psychiatric medications. […] After initial diagnosis and treatments, both adults and children with AE enter the period of ongoing treatment and recovery. […] This is a challenging stage of disease for patients with slow recoveries and those with major behavioral changes or psychiatric symptoms. […] Close follow-up with health care providers is important since changes in treatments may be needed.

• #53 Autoimmune Encephalitis: What Psychiatrists Need to Know

  https://www.psychiatrictimes.com/view/autoimmune-encephalitis-what-psychiatrists-need-know

  Many cases of autoimmune encephalitis are also treated with intravenous administration of rituximab or cyclophosphamide, thought to be beneficial in refractory cases, cases with delay to treatment initiation, and in preventing/decreasing relapses. […] Recovery is often a months-to-years long process, especially for patients with fulminant manifestations such as coma and catatonia. […] Agents such as amantadine, modafinil, stimulants, mood stabilizers, and selective serotonin reuptake inhibitors can all be safely considered to target these sequelae.

• #54 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Autoimmune-Encephalitis-Treatment.aspx

  This includes Rituximab, which is a monoclonal anti-CD20 antibody, and its use therefore prevents the employment of plasmapheresis which would remove it from the blood. […] The treatment of relapse is with second-line therapy, though first-line treatment may be tried first. […] Tumor removal is essential in most cases to remove the source of the antibodies and to improve the prognosis of the tumor itself. […] Behavioral and psychotic symptoms may require the use of antipsychotics. […] Steroids may cause psychosis, however, benzodiazepines and clonidine, among others, may help induce sleep in sleep-deprived individuals. […] Prognostic factors included: Early therapy, Second-line treatment in case of failure of first-line therapy.

• #55 Autoimmune Encephalitis – EMCrit Project

  https://emcrit.org/ibcc/ae/

  Fever may be managed with external cooling devices to achieve normothermia. […] Significant hypoventilation may require intubation. […] Medications typically used for paroxysmal sympathetic hyperactivity may be helpful in some patients (e.g., alpha-2 agonists and propranolol). […] Hyperkinetic disorders: dystonia, dyskinesia, or chorea. Cholinergic neurotransmission seems to be an important substrate for these abnormalities, so anticholinergic agents can be rapidly effective. […] Myoclonus results from cortical irritability, due to encephalitis. Treatments may include antiepileptic therapies, such as levetiracetam or sodium valproate.

• #56 Neurologic Syndrome and Autoimmune Encephalitis: Overview & Treatment

  https://www.ganeshdiagnostic.com/blog/neurologic-syndrome-and-autoimmune-encephalitis-overview-and-treatment

  Rituximab: A monoclonal antibody called rituximab targets B cells, a kind of white blood cell that makes antibodies. The blood level of autoantibodies is decreased to treat autoimmune encephalitis. […] Cyclophosphamide: Cyclophosphamide is a chemotherapy medication that is used to suppress the immune system in autoimmune encephalitis. It is often used in cases where other medications have not been effective. […] Antiepileptic medications: These medications are used to treat seizures, which can be a symptom of neurological syndromes and autoimmune encephalitis. Examples include levetiracetam, valproic acid, and carbamazepine. […] Muscle relaxants: Muscle relaxants are used to reduce stiffness and spasms, which can be a symptom of neurological syndromes and autoimmune encephalitis. Examples include baclofen and tizanidine.

• #57 Encephalitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/encephalitis/diagnosis-treatment/drc-20356142

  Autoimmune encephalitis caused by tumors may require treatment of those tumors. This may include surgery, radiation, chemotherapy or a combination of treatments. […] People who are hospitalized with serious encephalitis might need: Breathing assistance, as well as careful monitoring of breathing and heart function. Intravenous fluids to ensure proper hydration and levels of essential minerals. Anti-inflammatory medicines, such as corticosteroids, to reduce swelling and pressure within the skull. Anti-seizure medicines to stop or prevent seizures. […] If you experience complications of encephalitis, you might need additional therapy, such as: Brain rehabilitation to improve cognition and memory. Physical therapy to improve strength, flexibility, balance, motor coordination and mobility. Occupational therapy to develop everyday skills and to use adaptive products that help with everyday activities. Speech therapy to relearn muscle control and coordination to produce speech. Psychotherapy to learn coping strategies and new behavioral skills to improve mood disorders or address personality changes.

• #58

  https://www.nhs.uk/conditions/encephalitis/treatment/

  plasmapheresis a procedure which removes the substances that are attacking the brain from the blood […] surgery to remove abnormal growths (tumours) if encephalitis was triggered by a tumour somewhere in the body […] antibiotics or antifungal medicine used if encephalitis is caused by a bacterial or fungal infection. […] If there’s no treatment for the underlying cause, treatment is given to support the body, relieve symptoms, and allow the best chance of recovery. […] Most people need treatment to relieve these symptoms and to support certain bodily functions until they’re feeling better. […] This may involve: fluids given into a vein to prevent dehydration […] painkillers to reduce discomfort or a high temperature […] medicine to control seizures or fits […] medicine to help the person relax if they’re very agitated

• #59 Autoimmune Encephalitis – EMCrit Project

  https://emcrit.org/ibcc/ae/

  Fever may be managed with external cooling devices to achieve normothermia. […] Significant hypoventilation may require intubation. […] Medications typically used for paroxysmal sympathetic hyperactivity may be helpful in some patients (e.g., alpha-2 agonists and propranolol). […] Hyperkinetic disorders: dystonia, dyskinesia, or chorea. Cholinergic neurotransmission seems to be an important substrate for these abnormalities, so anticholinergic agents can be rapidly effective. […] Myoclonus results from cortical irritability, due to encephalitis. Treatments may include antiepileptic therapies, such as levetiracetam or sodium valproate.

• #60 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  You may need treatment for complications, such as epilepsy, sleep conditions and trouble with movements. You also may need rehabilitation if AE affected your memory, thinking skills or speech. Occupational and speech therapists, along with mental health professionals and other specialists, can help in your recovery. […] The long-term outlook can vary from person to person. Full recovery may take months or years. Many people continue to have symptoms related to thinking and behavior for longer than a year. But treatment continues to improve symptoms for 18 months to two years. […] Some people fully recover while others may have lasting symptoms that are mild or more serious. Getting treated early helps improve your long-term outlook.

• #61 Encephalitis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/encephalitis/diagnosis-treatment/drc-20356142

  Autoimmune encephalitis caused by tumors may require treatment of those tumors. This may include surgery, radiation, chemotherapy or a combination of treatments. […] People who are hospitalized with serious encephalitis might need: Breathing assistance, as well as careful monitoring of breathing and heart function. Intravenous fluids to ensure proper hydration and levels of essential minerals. Anti-inflammatory medicines, such as corticosteroids, to reduce swelling and pressure within the skull. Anti-seizure medicines to stop or prevent seizures. […] If you experience complications of encephalitis, you might need additional therapy, such as: Brain rehabilitation to improve cognition and memory. Physical therapy to improve strength, flexibility, balance, motor coordination and mobility. Occupational therapy to develop everyday skills and to use adaptive products that help with everyday activities. Speech therapy to relearn muscle control and coordination to produce speech. Psychotherapy to learn coping strategies and new behavioral skills to improve mood disorders or address personality changes.

• #62 Autoimmune Encephalitis: Kayla’s Long Journey to Diagnosis and Recovery | Children’s Hospital of Philadelphia

  https://www.chop.edu/stories/autoimmune-encephalitis-kayla-s-long-journey-diagnosis-and-recovery

  Once they knew for certain what they were fighting, doctors from Neurology and Immunology at CHOP started Kayla on plasmapheresis, or plasma exchange. During plasmapheresis, her blood was removed. Then her blood cells were separated from the plasma, mixed with fresh plasma and returned to her body. Her plasma carried antibodies that were attacking her immune system, specifically in her brain. […] She then began receiving intravenous immunoglobulin (IVIG) treatments to restore healthy, infection-fighting antibodies to her system. […] Kayla also had physical, occupational and speech-language therapy to retrain her muscles and brain to do some basic functions: Pick up utensils to feed herself, walk steadily, catch and throw a ball. […] She returned periodically to CHOP for IVIG treatments, which required overnight stays to watch for complications. The interval between IVIG sessions increased from three weeks to six months before her final treatment in January 2018.

• #63 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis patients who fail to improve after 10-14 days should receive second-line therapies such as rituximab or cyclophosphamide, or both. […] Better prognosis has been associated with early treatment, no requirement for intensive care admission and non-paraneoplastic AIE. […] The treatment response and relapse rate vary among patients with AIE. […] Relapses may occur in 31% of patients with anti-LGI1 encephalitis and 10% of those with anti-CASPR2 encephalitis, sometimes years after the first episode. […] The nature of the antibody, and to a lesser extent the clinical syndrome, determines the risk and type of an underlying malignancy. […] Tumor treatment is essential for neurological improvement.

• #64 Autoimmune Encephalitis Clinic at University Hospitals in Cleveland, Ohio | University Hospitals

  https://www.uhhospitals.org/services/neurology-and-neurosurgery-services/conditions-and-treatments/multiple-sclerosis-and-neuroimmunology/autoimmune-encephalitis

  Currently, there are no treatments approved by the U.S. Food and Drug Administration (FDA) for autoimmune encephalitis and treatment is guided by observational studies and clinical experience. Patients with severe symptoms will require admission to the hospital for treatment. […] These measures are commonly used to treat autoimmune encephalitis: […] High dose of intravenous corticosteroids […] Intravenous immunoglobulins […] Plasma exchange […] Immunosuppressant agents in severe or recurrent cases. […] In general, paraneoplastic cases may stabilize after treatment of the associated tumor, while idiopathic autoimmune encephalitis is usually fully or partially responsive to the treatments listed above. Some patients may have disease recurrence, so clinical monitoring and periodic cancer screening are often needed after recovery from the first attack. Patients with recurrent disease may need long-term immunosuppressive therapy. Many patients will require cognitive rehabilitation after recovery from the attack and some will require long-term management of residual seizures, involuntary movements, spasticity and psychiatric symptoms.

• #65 Autoimmune encephalitis // Middlesex Health

  https://middlesexhealth.org/6D451120-E1D2-9288-17CEEAF7E672AFCA

  You may need treatment for complications, such as epilepsy, sleep conditions and trouble with movements. You also may need rehabilitation if AE affected your memory, thinking skills or speech. Occupational and speech therapists, along with mental health professionals and other specialists, can help in your recovery. […] The long-term outlook can vary from person to person. Full recovery may take months or years. Many people continue to have symptoms related to thinking and behavior for longer than a year. But treatment continues to improve symptoms for 18 months to two years. […] Some people fully recover while others may have lasting symptoms that are mild or more serious. Getting treated early helps improve your long-term outlook.

• #66 Autoimmune Encephalitis: A Physician’s Guide to the Clinical Spectrum Diagnosis and Management

  https://www.mdpi.com/2076-3425/12/9/1130

  It is generally recommended not to delay treatment with first-line immune therapies while awaiting paired CSF/serum confirmation of the presence of neuronal autoantibodies in individuals strongly suspected to have autoimmune encephalitis based on the typical clinical presentations and the paraclinical findings indicative of inflammatory processes. […] Second-line agents, including rituximab or cyclophosphamide, are considered for those who show inadequate response to first-line immune therapies or develop relapse despite appropriate maintenance therapy. […] Maintenance therapy for 1–2 years is generally recommended to prevent relapse. Options for maintenance therapy include monthly IVIG infusions, high dose intravenous methylprednisolone pulse therapy, oral prednisone tapering, and steroid-sparing agents such as azathioprine and mycophenolate. […] It must be emphasized that the recovery course is typically protracted, and that complete or near-complete recovery may take up to 2 years, despite timely treatment with proper immune therapies.

• #67 Azthena logo with the word Azthena

  https://www.news-medical.net/health/Autoimmune-Encephalitis-Treatment.aspx

  This includes Rituximab, which is a monoclonal anti-CD20 antibody, and its use therefore prevents the employment of plasmapheresis which would remove it from the blood. […] The treatment of relapse is with second-line therapy, though first-line treatment may be tried first. […] Tumor removal is essential in most cases to remove the source of the antibodies and to improve the prognosis of the tumor itself. […] Behavioral and psychotic symptoms may require the use of antipsychotics. […] Steroids may cause psychosis, however, benzodiazepines and clonidine, among others, may help induce sleep in sleep-deprived individuals. […] Prognostic factors included: Early therapy, Second-line treatment in case of failure of first-line therapy.

• #68 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis patients who fail to improve after 10-14 days should receive second-line therapies such as rituximab or cyclophosphamide, or both. […] Better prognosis has been associated with early treatment, no requirement for intensive care admission and non-paraneoplastic AIE. […] The treatment response and relapse rate vary among patients with AIE. […] Relapses may occur in 31% of patients with anti-LGI1 encephalitis and 10% of those with anti-CASPR2 encephalitis, sometimes years after the first episode. […] The nature of the antibody, and to a lesser extent the clinical syndrome, determines the risk and type of an underlying malignancy. […] Tumor treatment is essential for neurological improvement.

• #69 Autoimmune Encephalitis Targeted by a Novel Cell Therapy

  https://www.insideprecisionmedicine.com/topics/precision-medicine/novel-cell-therapy-targets-autoimmune-encephalitis/

  Researchers at the German Center for Neurodegenerative Diseases (DZNE) and the Charité University Hospital in Berlin, Germany, have engineered a new type of specialized T cell that can be used to treat autoimmune encephalitis. […] Autoimmune encephalitis is difficult to treat and current treatment often requires escalated immunotherapy that can affect the entire immune system. This can be problematic, especially for cancer patients. […] “Instead of suppressing the entire immune system and eliminating not only the misdirected antibodies but also the more than 99% of beneficial antibodies, as we have done in the past, we set out to find a targeted approach,” said Harald Prüss, a professor at the DZNE and Charité and a corresponding author on the study published in Cell, in a press release.

• #70 Inebilizumab as a Potential Therapy for Autoimmune Encephalitis: Gregory Day, MD, MSc, MSCI, FAAN

  https://www.neurologylive.com/view/inebilizumab-potential-therapy-autoimmune-encephalitis-gregory-day

  We’ve known about antiNMDA receptor encephalitis for the last 15 years now and continuing to see more and more cases. But up until the launch of this trial, we haven’t had any clinical trials that have really been focused narrowly and specifically on patients with this disease. […] One potential therapy for patients with NMDAR encephalitis is inebilizumab (Uplizna, Horizon), a humanized anti-CD19 monoclonal antibody previously approved for neuromyelitis optica spectrum disorder. […] The phase 2 ExTINGUISH trial (NCT04372615), will assess safety and efficacy in 300 mg doses of inebilizumab (as a therapy for moderate-to-severe NMDAR encephalitis. […] Day, a neurologist at Mayo Clinic in Jacksonville Florida, also talked about the current available treatments for patients with the disease along with inebilizumab’s potential as a therapy.

• #71 State of Care for Autoimmune Encephalitis and the ExTINGUISH Trial of Inebilizumab

  https://www.neurologylive.com/view/state-care-autoimmune-encephalitis-extinguish-trial-inebilizumab

  Gregory Day, MD, MSc, MSCI, FAAN, a neurologist at Mayo Clinic in Jacksonville Florida, talked about the current state of treating autoimmune encephalitis and the significance of the ExTINGUISH trial. […] A lack of approved therapies for N-methyl-D-aspartate receptor (NMDAR) encephalitis has led to substantial variability in how the condition is treated. Inebilizumab (Uplizna, Horizon), a humanized anti-CD19 monoclonal antibody, may be a potentially efficacious treatment for NMDAR encephalitis, as the agent also has demonstrated early, robust, and sustained suppression of NMDAR autoantibodies, CD19+ plasmablasts and plasma cells that could lead to better longterm outcomes. […] The ExTINGUISH trial (NCT04372615), a phase 2b randomized double-blind placebo-controlled study, evaluates the safety and efficacy of inebilizumab 300 mg for the acute treatment of moderate-to-severe NMDAR encephalitis.

• #72 Autoimmune Encephalitis Targeted by a Novel Cell Therapy

  https://www.insideprecisionmedicine.com/topics/precision-medicine/novel-cell-therapy-targets-autoimmune-encephalitis/

  To develop a targeted treatment for NMDAR encephalitis, the researchers extracted T cells from the blood of patients and genetically modified them. The resulting chimeric autoantibody receptor (CAAR) T cells are programmed so they can precisely identify and target the cells that produce anti-NMDAR autoantibodies, without affecting “healthy” immune cells around them. […] As a next step, the DAZN and Charité researchers want to organize a clinical trial to test their therapy in humans. To start with, they will develop one targeted therapy per NMDAR encephalitis patient but hope to develop an off-the-shelf treatment in the future.

• #73 A Potential Treatment for Autoimmune Encephalitis | Technology Networks

  https://www.technologynetworks.com/neuroscience/news/a-potential-treatment-for-autoimmune-encephalitis-370823

  In autoimmune encephalitis, a rare but serious and sometimes life-threatening inflammation of the central nervous system, the bodys own defences are directed against the central nervous system. […] Researchers from Braunschweig, Jena, Leipzig and Berlin have developed a new potential treatment for this disease. […] With their translational research approach, the group has been able to discover a potential therapeutic agent. The molecule consists of a part of an NMDA receptor and a fragment of a human antibody. The pathogenic antibodies then bind to this fusion construct rather than to the NMDA receptors. […] We plan to try to apply this therapeutic approach to other forms of autoimmune encephalitis. In the long term, we hope that basic neuroscience research will help improve treatment options in neurology and psychiatry.

• #74 Immunotherapy for autoimmune encephalitis | Cell Death Discovery

  https://www.nature.com/articles/s41420-025-02459-z

  A subset of AE patients remains refractory to standard immunotherapy, necessitating novel treatment approaches. […] Long-lived plasma cells and intrathecal immune responses contribute to disease persistence, posing challenges to current therapeutic strategies. […] Large-scale randomized controlled trials (RCTs) are essential to refine treatment protocols and improve long-term outcomes. […] The consensus supports a framework of first-line, second-line, and maintenance immunotherapies. […] This review aims to provide a summary and discussion of the immunotherapeutic strategies available for treating AE. […] Corticosteroids, either alone or in combination with other treatments such as IVIG or PE, were chosen as the first-line treatment by 84% of respondents for patients with a typical presentation. Regarding the preferred second-line treatment, 80% of respondents opted for RTX, whereas only 10% selected CP in a clinical context where antibody status was unknown. […] If there is no clear clinical improvement within 2-3 weeks, second-line options such as RTX or CP should be initiated immediately. Third-line therapies, such as TCZ and BTZ, are becoming more popular and are the focus of ongoing studies, although more evidence is needed.

• #75 Treatment Of Autommune Encephalitis | Immunotherapy Treatment

  https://autoimmune-encephalitis.org/treatment/

  The treatment of patients should be multidisciplinary and involve neurologists, as well as neuroimmunologists, oncologists, rheumatologists, and psychiatrists. Autoimmune Encephalitis is treated with immunotherapy. Immunotherapy slows down the over-excited immune system. By slowing down the immune system it slows down the process the immune system is engaging in, which is creating the foreign antibodies that are attacking healthy brain cells. Suppressing the immune system attempts to stop the attack that is occurring. […] There is no uniform standard immunotherapy. Initially, high dose steroids are used to slow down the immune system and bring down the inflammation in the brain in a broad way. Many patients experience the return of mental clarity after their initial 3-5 days of one gram IV Solumedrol. However, treatment for autoimmune encephalitis is a marathon and not a sprint as treatment is on-going and usually involves several combinations of treatment in the protocol.

• #76 SciELO Brazil – Autoimmune encephalitis: a review of diagnosis and treatment Autoimmune encephalitis: a review of diagnosis and treatment

  https://www.scielo.br/j/anp/a/mqLMtVDZLLF566CwstHwyhy/

  Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. […] We reviewed common forms of AIE and discuss their diagnostic approach and treatment. […] Autoimmune encephalitis has a wide variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders and seizures. […] We reviewed common causes of AIE and discuss their pathophysiology, diagnostic approach and management. […] Various treatment approaches including corticosteroids, intravenous immunoglobulin, plasma exchange, rituximab and cyclophosphamide are currently used. […] Evidence suggests that early immunotherapy improves outcome, thus treatment for AIE should not be delayed. […] Patients should receive either methylprednisolone 1 g IV for 3-5 days and intravenous immunoglobulin (0.4 g/kg/day for five days) or methylprednisolone and plasmapheresis.

• #77 Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management | Journal of Neurology, Neurosurgery & Psychiatry

  https://jnnp.bmj.com/content/92/7/757

  The 2016 AE clinical criteria emphasise the importance of starting immunotherapy once AE is highly suspected and infectious etiologies are excluded based on CSF results (cell-count, glucose, viral PCR, gram stain). […] Empiric treatment with intravenous methylprednisolone at a dose of 1 g per day for 3-7 days is a common reasonable approach to achieve initial immunosuppressive and anti-inflammatory effect in AE patients. […] On our AEACN survey, 84% of responders chose corticosteroids alone (65%) or in combination with other agents (19%) for initial immunotherapy in patients with a general AE presentation. […] IVIg can be more readily available than PLEX in some centres and it does not require a central line. […] PLEX (5-10 sessions every other day) is an effective option for acute immunomodulation when corticosteroids are contraindicated or ineffective.

• #78 SciELO Brazil – Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adul

  https://www.scielo.br/j/anp/a/X4rrnygw43tryfbBp8Dvn8C/?lang=en

  Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. […] Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. […] The first-line treatment should preferably be the combination of MP and IVIG or MP and PLX. […] The second-line treatment should be initiated after 10 to 14 days of the start of the initial treatment if there is no satisfactory clinical response. […] The options for second-line treatment are rituximab alone or in combination with cyclophosphamide.

• #79

  https://continentalhospitals.com/diseases/autoimmune-encephalitis/

  It is important to note that early diagnosis and prompt initiation of treatment are crucial in autoimmune encephalitis. In some cases, hospitalization may be necessary to closely monitor symptoms and administer appropriate therapies. Furthermore, supportive care plays a vital role in managing symptoms associated with autoimmune encephalitis. […] It is worth mentioning that each case of autoimmune encephalitis is unique, and treatment plans should be tailored to individual needs under the guidance of a healthcare professional experienced in treating this condition. Regular follow-up visits are essential to monitor progress and make any necessary adjustments to the treatment plan. […] In conclusion, while there is no cure for autoimmune encephalitis at present, various treatments exist that aim to control symptoms by modulating the immune response. With proper medical management and ongoing care, individuals with this condition can experience improved quality of life and symptom control.

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