Materiały źródłowe

• #1 Autoimmune epilepsy | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/autoimmune-epilepsy?content_id=CON-20573820

  Autoimmune epilepsy is a type of epilepsy where seizures are caused by the immune system mistakenly attacking brain cells. […] Antiseizure medicines usually don’t do enough to manage seizures in people with autoimmune epilepsy. Instead, immunotherapy medicines help reduce the immune response on the brain. […] When immunotherapy is started early, it can reduce inflammation and improve seizures. […] You may not be able to prevent autoimmune epilepsy. But getting cancer screenings can help your healthcare professional find tumors and treat them early. This could prevent autoimmune encephalitis triggered by cancers, which can be a cause of epilepsy.

• #1 Mayo Clinic Health Library – Autoimmune epilepsy | Swiss Medical Network

  https://www.swissmedical.net/en/healtcare-library/con-20573820

  Autoimmune epilepsy is a type of epilepsy where seizures are caused by the immune system mistakenly attacking brain cells. […] You may not be able to prevent autoimmune epilepsy. But getting cancer screenings can help your healthcare professional find tumors and treat them early. This could prevent autoimmune encephalitis triggered by cancers, which can be a cause of epilepsy. Talk with your healthcare professional about your cancer risk and if you should get screened for certain cancers.

• #1 Autoimmune epilepsy | Altru Health System

  https://www.altru.org/health-library/conditions/autoimmune-epilepsy

  Autoimmune epilepsy is a type of epilepsy where seizures are caused by the immune system mistakenly attacking brain cells. […] Antiseizure medicines usually don’t do enough to manage seizures in people with autoimmune epilepsy. Instead, immunotherapy medicines help reduce the immune response on the brain. […] When immunotherapy is started early, it can reduce inflammation and improve seizures. […] You may not be able to prevent autoimmune epilepsy. But getting cancer screenings can help your healthcare professional find tumors and treat them early. This could prevent autoimmune encephalitis triggered by cancers, which can be a cause of epilepsy. Talk with your healthcare professional about your cancer risk and if you should get screened for certain cancers.

• #1 Autoimmune Epilepsy: Symptoms, Causes, Diagnosis, Treatment

  https://www.healthline.com/health/epilepsy/autoimmune-epilepsy

  There are some identified risk factors for autoimmune epilepsy, such as cancer and thyroid disease, but most people who have these risk factors do not develop autoimmune epilepsy. […] Its not known why some people develop this complication, so its typically defined as being idiopathic. That means it does not have an identifiable cause. There are no reliable guidelines for preventing it. […] Taking general steps to reduce your risk of cancer can potentially reduce your risk. This includes: avoiding or quitting smoking to reduce the risk of lung cancer (this can be difficult, but a doctor can build a cessation plan that works for you), protecting your skin from the sun, limiting alcohol consumption, eating a balanced diet, maintaining a moderate weight.

• #1

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

  Prevention is a crucial aspect when it comes to autoimmune encephalitis, a condition characterized by inflammation in the brain caused by an immune system malfunction. While there is no surefire way to completely prevent autoimmune encephalitis, there are steps that can be taken to reduce the risk and minimize its impact. First and foremost, maintaining a healthy lifestyle is essential. This includes eating a balanced diet rich in nutrients, exercising regularly, getting enough sleep, and managing stress levels. A strong immune system can help ward off infections and reduce the likelihood of triggering an autoimmune response. Additionally, staying up-to-date with vaccinations is important. Vaccines protect against various infectious diseases that can potentially lead to autoimmune encephalitis. By ensuring that you and your loved ones are immunized according to recommended schedules, you can significantly lower the risk of developing this condition. Furthermore, it is crucial to be mindful of any potential triggers or underlying conditions that may contribute to autoimmune encephalitis. Certain infections or illnesses have been linked to its development. Seek medical attention promptly if you experience any unusual symptoms or if you have a history of autoimmune disorders. Lastly, maintaining open communication with healthcare professionals is key in prevention efforts. Regular check-ups and consultations allow for early detection of any warning signs or risk factors associated with autoimmune encephalitis. By working closely with medical experts, appropriate preventive measures can be implemented based on individual circumstances.

• #1 Epilepsy: What It Is, Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17636-epilepsy

  Although many causes of epilepsy are out of your control and unpreventable, you can reduce your chance of developing a few conditions that might lead to epilepsy, such as: […] To lower your risk of traumatic brain injury (from blows to your head), always wear your seatbelt when driving and drive defensively; wear a helmet when biking; clear your floors of clutter and power cords to prevent falls; and stay off ladders. […] To lower your risk of stroke, eat a healthy diet (such as the Mediterranean diet), maintain a healthy weight and exercise regularly. […] Seek therapy for substance abuse. Alcohol and other illegal drugs can damage your brain, which can then lead to epilepsy.

• #1 What causes seizures in adults with no history?

  https://www.moleculera.com/seizures-adults-with-no-history/

  Autoimmune factors may play a bigger role in seizure disorders than previously suspected. […] But now, studies indicate that an autoimmune cause appears to be the potential culprit in at least 20% of [those] cases. […] Mounting evidence also indicates that an immune dysfunction or abnormal autoimmune activity can elicit a sudden onset of seizures in adults with no history of the illness. […] Its well-established that people with certain autoimmune disorders, such as lupus, type 1 diabetes, celiac disease, multiple sclerosis, rheumatoid arthritis, and psoriasis, are at greater risk of developing epileptic seizures. […] Autoimmune factors may play a role in the onset of seizures in adults with no history and who do not respond to standard medication. […] Identifying whether an autoimmune condition is causing the seizures or drug resistant epilepsy is important since treatment with immunotherapies can dramatically impact chances of recovery.

• #1 Epilepsy Prevention: Several Strategies and Precautions

  https://orlando-epilepsy.com/epilepsy-prevention-strategies-and-approaches/

  Many epilepsy cases are linked to factors affecting brain development during pregnancy or early childhood. […] Treat autoimmune conditions promptly to minimize brain inflammation.

• #1 Increased Risk of Epilepsy Among Autoimmune Disease Patients | Newsroom | Boston Children’s Hospital

  https://www.childrenshospital.org/newsroom/news-and-events/increased-risk-epilepsy-among-autoimmune-disease-patients

  Patients with an autoimmune disease have a 3.8-fold increased risk of developing epilepsy, according to a new population-level study from Boston Childrens Hospital based on health insurance claim data. […] Findings also revealed that autoimmunity patients on select immunosuppressing medications had a lower risk of developing epilepsy. […] „These findings suggest a new approach to treating seizures, one based on quieting the immune system,” Mandl said. […] the risk association between epilepsy and autoimmunity suggests that both rheumatologists and neurologists should consider screening their patients for signs of the associated condition. […] „There are many ways in which autoimmunity can affect the brain and cause seizures,” said Gorman. „The next step is to find out what they are.”

• #1 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  The pathomechanism of autoimmune epilepsy can be categorized as shown in Fig. 2. […] The recommended regimen for autoimmune epilepsy is a 1,000-mg daily IV infusion for 35 days. […] IVIg is recommended as a 400-mg/kg daily IV infusion for 5 days. […] The recommended regimen of PLEX in autoimmune epilepsy is 11.5 plasma-volume exchanges with 5% albumin replacement fluid. […] Rituximab has been classified as a second-line therapy, but due to successful outcomes in autoimmune encephalitis, administration of rituximab in combination with steroid or IVIg has recently been considered. […] Previous studies have shown that the earlier initiation of immunotherapy will result in faster recovery and a better prognosis. […] Early empirical immunotherapy should be actively considered for these patients.

• #1 Dynamic Link Display

  https://journals.lww.com/neurotodayonline/fulltext/2017/12070/in_the_clinic_autoimmune_epilepsy__immunotherapy.5.aspx

  Researchers reported that early use of immunotherapy reduced the number of seizures by half and prevented cognitive impairment that has been associated with faciobrachial dystonic seizures. […] Now, a team of scientists at the University of Oxford offer new data to suggest that while antiepileptic treatments do not work, early treatment with immunotherapies can reduce seizures and prevent cognitive impairment that is often seen in this condition. […] The cognitive impairments typically arrive after the seizures, so they wanted to understand whether early treatment with immunotherapy could prevent these cognitive symptoms. […] Prompt immunotherapy appears to prevent development of cognitive impairment, Dr. Irani said. […] We showed that time-to-treat is critical to how quickly they become seizure free and their long-term outcome. […] These findings are in keeping with our own clinical experience. Future work should focus on ways to reduce steroid burden in these patients. […] Simply put, rapid time to immunotherapy treatment is the strongest known predictor of good outcome.

• #1 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] Therefore, after performing a thorough evaluation of infection, it is possible to consider NORSE as potentially autoimmune epilepsy requiring active immunotherapy. […] Since NORSE is an emergency condition, immunotherapy should be administered as soon as possible, preferably within hours or (at worst) days.

• #1 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  As empirical immunotherapy, IVIg can be the first treatment of choice because it is both effective in autoimmune epilepsy and safe in viral encephalitis. […] If a patient does not fully respond to the initial immunotherapy, clinicians should move to an alternative immunotherapy such as rituximab, tocilizumab, anakinra, or cyclophosphamide. […] Early empirical immunotherapy should be actively considered for these patients. […] The duration of immunotherapy maintenance should also be addressed. […] The first step is to control seizures by the appropriate administration of AEDs. However, an AED is an adjuvant treatment in autoimmune epilepsy, and few cases of seizure are prevented by an AED alone. […] The recommended regimen for autoimmune epilepsy is a 1,000-mg daily IV infusion for 35 days.

• #1 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] Therefore, after performing a thorough evaluation of infection, it is possible to consider NORSE as potentially autoimmune epilepsy requiring active immunotherapy. […] The treatment of autoimmune epilepsy can be categorized into two axes: 1) disease-modifying treatment by immunotherapy and 2) the administration of appropriate AEDs.

• #1 Autoimmune epilepsy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-epilepsy/diagnosis-treatment/drc-20576912

  Autoimmune epilepsy treatment differs from the treatment used for other types of epilepsy. Healthcare professionals use immunotherapy to reduce the immune system activity and treat seizures. […] Depending on your response to the initial treatments, your healthcare professional also may recommend longer acting medicines that work on the immune system. These may include rituximab (Rituxan, Truxima, others), cyclophosphamide, mycophenolate (Cellcept, Myhibbin), azathioprine (Azasan, Imuran), or tocilizumab (Actemra, Tofidence, Tyenne). […] Your healthcare professional also may recommend plasma exchange. A healthcare professional removes the liquid part of your blood and separates it from your blood cells. Then the blood cells are put back into your body and your body makes more plasma. This therapy helps remove the antibodies that are causing the immune system to attack brain cells.

• #1 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  The recommended regimen of PLEX in autoimmune epilepsy is 11.5 plasma-volume exchanges with 5% albumin replacement fluid. […] Rituximab has been classified as a second-line therapy, but due to successful outcomes in autoimmune encephalitis, administration of rituximab in combination with steroid or IVIg has recently been considered. […] The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] The pathogenesis of autoimmune encephalitis caused by synaptic antibodies are mediated by B cells. […] The initial immunotherapy in this group of patients involves IVIg and high-dose corticosteroids, which target a broad spectrum of immune responses. […] The presence of a clinicoradiologic mismatch in which there is no or only a minimal MRI lesion but severe clinical deterioration is suggestive of antibody-mediated encephalitis. […] The sequential application of immunotherapies including IVIg, rituximab, tocilizumab, and anakinra should then be cautiously but promptly considered in an hourly and daily fashion.

• #1 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  The duration of immunotherapy maintenance should also be addressed. […] The presence of a clinicoradiologic mismatch in which there is no or only a minimal MRI lesion but severe clinical deterioration is suggestive of antibody-mediated encephalitis. […] The sequential application of immunotherapies including IVIg, rituximab, tocilizumab, and anakinra should then be cautiously but promptly considered in an hourly and daily fashion.

• #1 Identification and Treatment of Autoimmune Epilepsy – Contemporary Advances in Epilepsy

  https://www.medpagetoday.com/resource-centers/contemporary-advances-epilepsy/identification-and-treatment-autoimmune-epilepsy/2088

  Among the one-third of adults with epilepsy of unknown etiology, an autoimmune cause appears to be the potential culprit in at least 20% of cases, according to new research published by Divyanshu Dubey, MD, and colleagues in JAMA Neurology. […] The study investigators devised an antibody prevalence in epilepsy (APE) score useful for predicting positive serologic findings. […] Moreover, the investigators showed that seropositive patients exhibited favorable responses to immunomodulatory therapy. […] Immunomodulatory therapy reduced seizure frequency in patients seropositive for autoantibodies. […] Patients with autoimmune-induced seizures tend to have recalcitrant disease that does not respond to antiepileptic drugs. […] Accurately diagnosing these individuals not only helps avoid unnecessary therapy, but also offers the opportunity to treat patients with immunomodulatory therapies that have been shown to offer benefit, such as corticosteroids and intravenous immune globulin.

• #1 Identification and Treatment of Autoimmune Epilepsy – Contemporary Advances in Epilepsy

  https://www.medpagetoday.com/resource-centers/contemporary-advances-epilepsy/identification-and-treatment-autoimmune-epilepsy/2088

  Good seizure outcomes (ie, a >50% reduction in seizure frequency at the first follow-up visit) were strongly tied to the use of immunomodulatory therapy. […] If you diagnose autoimmune epilepsy in these patients early and treat them, you can actually cure them. […] Assessing this issue prospectively is really important, and that’s where this paper really has a unique position.

• #1 Autoimmune Epilepsies.pptx

  https://www.slideshare.net/slideshow/autoimmune-epilepsiespptx/256163891

  A RITE2 score of 7 has 88% sensitivity and 84% specificity of a favorable seizure outcome, defined as at least a 50% reduction in seizure frequency, following initiation of immunotherapy. […] Autoimmune epilepsies are an important subset of epilepsies. Autoimmune encephalitis is the most important type. They are in general treatable with good outcomes. Low threshold for diagnostic testing as epilepsy is often intractable and causes significant neuro-cognitive and behavioural sequelae.

• #1 Autoimmune epilepsy – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-epilepsy/diagnosis-treatment/drc-20576912

  Although antiseizure medicines don’t work as well in people with autoimmune epilepsy, they may still play a role in your treatment. However, the effectiveness of these medicines may be limited. If you take immunotherapy and become seizure free, your healthcare professional may suggest slowly reducing your dose of antiseizure medicines over several months to see if seizures return.

• #1 Autoimmune Epilepsy Identification and Treatment

  https://www.cureepilepsy.org/webinars/identification-and-treatment-of-autoimmune-epilepsy/

  Autoimmune epilepsy is important to diagnose because one of the hallmarks of this condition is that it does not generally respond to typical anti-seizure medications. Immunotherapy is often used to treat people with this condition, by reducing inflammation in the brain. […] So, what do I do if initial therapy doesn’t work, is I try more. But that being said, sometimes you do palliative surgeries. If one area is structurally very damaged, you could consider surgery or neurostimulation too with various nerve stimulators or even invasive neural stimulators have been used in patients that are autoimmune as well. So, you don’t give up. […] So, we don’t have a good answer to that. We don’t even know who needs long term therapy. So, I’ll just tell you what I do, which is what people more senior with more gray hair did. And so I just do it that way. So basically, it kind depends. I never wean seizure meds and immunotherapy at the same time. Because if you have a breakthrough seizure, you’re not going to know which one you actually need. So that’s rule number one.

• #1 Epilepsy Studies Uncover New Options for Shortening Seizures & Extending Lives | NYU Langone News

  https://nyulangone.org/news/epilepsy-studies-uncover-new-options-shortening-seizures-extending-lives

  A growing body of knowledge, informed by recent investigations and clinical trials at NYU Langones Comprehensive Epilepsy Center, could equip physicians and family members with new tools for stemming seizures and preventing sudden death in patients with epilepsy and related disorders. […] Two will explore autoimmune factors, believed to trigger epilepsy in some cases. […] A third group is looking at a blood biomarker involved in inflammation, which is hypothesized to exacerbate seizures.

• #1 Autoimmune Epilepsy Testing – Mayo Clinic LaboratoriesplayEpilepsyGABA-A receptor antibodies

  https://news.mayocliniclabs.com/neurology/autoimmune-neurology/epilepsy/

  Autoimmune epilepsy is increasingly recognized in the spectrum of immune-mediated neurological disorders, which can be characterized by detection of neural autoantibodies in serum or spinal fluid and responsiveness to immunotherapy. […] Identifying epilepsy as autoimmune-mediated is crucial because patients may benefit from immune suppression, while traditional antiepileptic therapy may not be effective. […] GABA-A receptor antibodies are biomarkers of autoimmune encephalopathy, which may occur at any age and disproportionately affects children. […] Because disease associated with the GABA-A receptor antibody is responsive to immunotherapy if treated early, accurate and timely testing is critical. […] 75% of GABA-A receptor-positive patients had a partial or complete recovery with proper treatment.

• #1 Autoimmune Epilepsies.pptx

  https://www.slideshare.net/slideshow/autoimmune-epilepsiespptx/256163891

  The neural injury is generally reversible. The neural injury is generally irreversible. Good response to immunotherapy. Poor response to immunotherapy […] Suspect autoimmune etiology when epilepsy is: New onset epilepsy with frequent seizures New onset refractory epilepsy NORSE or FIRES Inflammatory changes in CSF or serum Suggestive patterns on MRI brain/ PET scan Occurrence of anti neural antibodies Seizures/ epilepsy with personal or family history of autoimmune illness New onset unprovoked seizures […] The pathogenesis of autoimmune epilepsy can be categorized into two axes: the targets of autoimmunity and the types of autoimmunity. […] Treatment should be initiated at the earliest based on clinical suspicion aided by MRI, CSF and EEG studies, after reasonable exclusion of other competing diagnosis. Do not wait for antibody test results.

• #2 :: JCN :: Journal of Clinical Neurology

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] Therefore, after performing a thorough evaluation of infection, it is possible to consider NORSE as potentially autoimmune epilepsy requiring active immunotherapy. […] The treatment of autoimmune epilepsy can be categorized into two axes: 1) disease-modifying treatment by immunotherapy and 2) the administration of appropriate AEDs.

• #2 Autoimmune epilepsy | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/autoimmune-epilepsy

  You may not be able to prevent autoimmune epilepsy. But getting cancer screenings can help your healthcare professional find tumors and treat them early. This could prevent autoimmune encephalitis triggered by cancers, which can be a cause of epilepsy. Talk with your healthcare professional about your cancer risk and if you should get screened for certain cancers.

• #2 Autoimmune epilepsy | Health Library | Memorial Health System

  https://www.mhsystem.org/health-library/con-20573820/

  Autoimmune epilepsy is caused by the immune system attacking brain cells and leading to seizures. […] You may not be able to prevent autoimmune epilepsy. But getting cancer screenings can help your healthcare professional find tumors and treat them early. This could prevent autoimmune encephalitis triggered by cancers, which can be a cause of epilepsy. […] Talk with your healthcare professional about your cancer risk and if you should get screened for certain cancers.

• #2 What Is Autoimmune Epilepsy?

  https://www.icliniq.com/articles/neurological-health/autoimmune-epilepsy

  Autoimmune epilepsy can be prevented in the following ways: […] Prevent traumatic brain injuries. […] Avoid getting infections. […] Eat properly washed and cooked food. […] Do deworming of the body regularly. […] Do exercise regularly. […] Eat a well-balanced diet. […] Avoid alcohol and smoking.

• #2 Increased Risk of Epilepsy Among Autoimmune Disease Patients | Newsroom | Boston Children’s Hospital

  https://www.childrenshospital.org/newsroom/news-and-events/increased-risk-epilepsy-among-autoimmune-disease-patients

  Patients with an autoimmune disease have a 3.8-fold increased risk of developing epilepsy, according to a new population-level study from Boston Childrens Hospital based on health insurance claim data. […] Findings also revealed that autoimmunity patients on select immunosuppressing medications had a lower risk of developing epilepsy. […] „These findings suggest a new approach to treating seizures, one based on quieting the immune system,” Mandl said. […] the risk association between epilepsy and autoimmunity suggests that both rheumatologists and neurologists should consider screening their patients for signs of the associated condition. […] „There are many ways in which autoimmunity can affect the brain and cause seizures,” said Gorman. „The next step is to find out what they are.”

• #2 Epilepsy Prevention: Several Strategies and Precautions

  https://orlando-epilepsy.com/epilepsy-prevention-strategies-and-approaches/

  Many epilepsy cases are linked to factors affecting brain development during pregnancy or early childhood. […] Treat autoimmune conditions promptly to minimize brain inflammation.

• #2 Dynamic Link Display

  https://journals.lww.com/neurotodayonline/fulltext/2017/12070/in_the_clinic_autoimmune_epilepsy__immunotherapy.5.aspx

  Researchers reported that early use of immunotherapy reduced the number of seizures by half and prevented cognitive impairment that has been associated with faciobrachial dystonic seizures. […] Now, a team of scientists at the University of Oxford offer new data to suggest that while antiepileptic treatments do not work, early treatment with immunotherapies can reduce seizures and prevent cognitive impairment that is often seen in this condition. […] The cognitive impairments typically arrive after the seizures, so they wanted to understand whether early treatment with immunotherapy could prevent these cognitive symptoms. […] Prompt immunotherapy appears to prevent development of cognitive impairment, Dr. Irani said. […] We showed that time-to-treat is critical to how quickly they become seizure free and their long-term outcome. […] These findings are in keeping with our own clinical experience. Future work should focus on ways to reduce steroid burden in these patients. […] Simply put, rapid time to immunotherapy treatment is the strongest known predictor of good outcome.

• #2 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  Autoimmune epilepsy is a newly emerging area of epilepsy. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. […] A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. […] Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. […] Therefore, after performing a thorough evaluation of infection, it is possible to consider NORSE as potentially autoimmune epilepsy requiring active immunotherapy. […] Since NORSE is an emergency condition, immunotherapy should be administered as soon as possible, preferably within hours or (at worst) days.

• #2 Clinical Approach to Autoimmune Epilepsy

  https://www.kci.go.kr/kciportal/landing/article.kci?arti_id=ART002632162

  Autoimmune epilepsy is a newly emerging area of epilepsy. The concept of autoimmune as an etiology has recently been revisited thanks to advances in autoimmune encephalitis and precision medicine with immunotherapies. […] The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. Autoimmune epilepsy often manifests as new-onset refractory status epilepticus (NORSE). A patient classified as NORSE should receive empirical immunotherapy as soon as possible. […] The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy.

• #2 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  As empirical immunotherapy, IVIg can be the first treatment of choice because it is both effective in autoimmune epilepsy and safe in viral encephalitis. […] If a patient does not fully respond to the initial immunotherapy, clinicians should move to an alternative immunotherapy such as rituximab, tocilizumab, anakinra, or cyclophosphamide. […] Early empirical immunotherapy should be actively considered for these patients. […] The duration of immunotherapy maintenance should also be addressed. […] The first step is to control seizures by the appropriate administration of AEDs. However, an AED is an adjuvant treatment in autoimmune epilepsy, and few cases of seizure are prevented by an AED alone. […] The recommended regimen for autoimmune epilepsy is a 1,000-mg daily IV infusion for 35 days.

• #2 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  The recommended regimen of PLEX in autoimmune epilepsy is 11.5 plasma-volume exchanges with 5% albumin replacement fluid. […] Rituximab has been classified as a second-line therapy, but due to successful outcomes in autoimmune encephalitis, administration of rituximab in combination with steroid or IVIg has recently been considered. […] The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] The pathogenesis of autoimmune encephalitis caused by synaptic antibodies are mediated by B cells. […] The initial immunotherapy in this group of patients involves IVIg and high-dose corticosteroids, which target a broad spectrum of immune responses. […] The presence of a clinicoradiologic mismatch in which there is no or only a minimal MRI lesion but severe clinical deterioration is suggestive of antibody-mediated encephalitis. […] The sequential application of immunotherapies including IVIg, rituximab, tocilizumab, and anakinra should then be cautiously but promptly considered in an hourly and daily fashion.

• #2 Identification and Treatment of Autoimmune Epilepsy – Contemporary Advances in Epilepsy

  https://www.medpagetoday.com/resource-centers/contemporary-advances-epilepsy/identification-and-treatment-autoimmune-epilepsy/2088

  Good seizure outcomes (ie, a >50% reduction in seizure frequency at the first follow-up visit) were strongly tied to the use of immunomodulatory therapy. […] If you diagnose autoimmune epilepsy in these patients early and treat them, you can actually cure them. […] Assessing this issue prospectively is really important, and that’s where this paper really has a unique position.

• #3 Dynamic Link Display

  https://journals.lww.com/neurotodayonline/fulltext/2017/12070/in_the_clinic_autoimmune_epilepsy__immunotherapy.5.aspx

  Researchers reported that early use of immunotherapy reduced the number of seizures by half and prevented cognitive impairment that has been associated with faciobrachial dystonic seizures. […] Now, a team of scientists at the University of Oxford offer new data to suggest that while antiepileptic treatments do not work, early treatment with immunotherapies can reduce seizures and prevent cognitive impairment that is often seen in this condition. […] The cognitive impairments typically arrive after the seizures, so they wanted to understand whether early treatment with immunotherapy could prevent these cognitive symptoms. […] Prompt immunotherapy appears to prevent development of cognitive impairment, Dr. Irani said. […] We showed that time-to-treat is critical to how quickly they become seizure free and their long-term outcome. […] These findings are in keeping with our own clinical experience. Future work should focus on ways to reduce steroid burden in these patients. […] Simply put, rapid time to immunotherapy treatment is the strongest known predictor of good outcome.

• #3 :: JCN :: Journal of Clinical Neurology

  https://www.thejcn.com/DOIx.php?id=10.3988/jcn.2020.16.4.519

  The recommended regimen of PLEX in autoimmune epilepsy is 11.5 plasma-volume exchanges with 5% albumin replacement fluid. […] Rituximab has been classified as a second-line therapy, but due to successful outcomes in autoimmune encephalitis, administration of rituximab in combination with steroid or IVIg has recently been considered. […] The prognosis depends on the depth of autoimmunity and the presence of neuronal damage. […] The pathogenesis of autoimmune encephalitis caused by synaptic antibodies are mediated by B cells. […] The initial immunotherapy in this group of patients involves IVIg and high-dose corticosteroids, which target a broad spectrum of immune responses. […] The presence of a clinicoradiologic mismatch in which there is no or only a minimal MRI lesion but severe clinical deterioration is suggestive of antibody-mediated encephalitis. […] The sequential application of immunotherapies including IVIg, rituximab, tocilizumab, and anakinra should then be cautiously but promptly considered in an hourly and daily fashion.

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