Materiały źródłowe

• #1 Autoimmune Epilepsy

  https://practicalneurology.com/diseases-diagnoses/epilepsy-seizures/autoimmune-epilepsy/30185/

  Approximately 30% of patients with epilepsy do not achieve seizure freedom despite medical therapy, and the etiology of many epilepsies remains unknown. In 2017, the International League Against Epilepsy (ILAE) recognized an epilepsy resulting directly from an immune disorder with seizures as a core symptom. For an epilepsy to meet this classification, evidence of autoimmune-mediated central nervous system (CNS) inflammation is required. Currently, no strict diagnostic guidelines exist for autoimmune epilepsy. This article seeks to provide a basic clinical approach to diagnosis and treatment. […] Recommendations for tumor screening are antibody specific and covered later in this article, although often a specific antibody is not detected. When no specific antibody is detected, the clinical features and other test results guide diagnosis of autoimmune epilepsy, and broad cancer screening is also performed.

• #2 Autoimmune Epilepsy

  https://practicalneurology.com/articles/2018-oct/autoimmune-epilepsy

  Approximately 30% of patients with epilepsy do not achieve seizure freedom despite medical therapy, and the etiology of many epilepsies remains unknown. In 2017, the International League Against Epilepsy (ILAE) recognized an epilepsy resulting directly from an immune disorder with seizures as a core symptom. Currently, no strict diagnostic guidelines exist for autoimmune epilepsy. […] Recommendations for tumor screening are antibody specific and covered later in this article, although often a specific antibody is not detected. When no specific antibody is detected, the clinical features and other test results guide diagnosis of autoimmune epilepsy, and broad cancer screening is also performed. […] Patients with autoimmune epilepsy typically have multifocal seizures with an unusually high (ie, daily or weekly) seizure frequency at onset and can also present in nonconvulsive status epilepticus.

• #3 Autoimmune Epilepsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6694338/

  The true incidence of autoimmune epilepsy remains unknown. There are no population-based studies providing age- and sex-adjusted incidence and prevalence of autoimmune epilepsy. […] A recent population-based study from Olmsted county, MN, estimated the incidence and prevalence of autoimmune encephalitis to be 0.8/100,000 person-years and 13.7/100,000 people, respectively. […] A hospital-based prospective study reported that 20% of adult patients with epilepsy of unknown etiology were seropositive for neural specific antibodies associated with autoimmune epilepsy or encephalopathy. […] Epilepsies of unknown etiology are estimated to constitute one-third of all epilepsies among adults. Therefore, the rate of autoimmune epilepsies based on these studies can be inferred to be around 5 to 7% of all epilepsies, at least in adults. […] The frequency of autoantibodies in pediatric epilepsy is more unclear. Studies from Europe and Australia have reported the presence of autoantibodies in about 10% of pediatric patients with new-onset epilepsy.

• #4

  https://aesnet.org/abstractslisting/population-based-epidemiology-study-of-autoimmune-seizures-and-epilepsy

  Population-Based Epidemiology Study of Autoimmune Seizures and Epilepsy […] In 2017, a new classification of epilepsy by the International League Against Epilepsy acknowledged the concept of epilepsy of immune etiology. However, there is a scarcity of epidemiological data and information regarding the impact of these disorders on healthcare planning and resource allocation. This study aims to estimate the prevalence and incidence of autoimmune seizures and epilepsy, as well as evaluate the disease burden associated with these conditions. […] On December 31, 2019, age and sex-adjusted overall prevalence of autoimmune seizures and epilepsy was estimated at 6.87 per 100,000 population, with adjusted prevalence of 5.51 per 100,000 for AAE and 5.12 per 100,000 for active epilepsy. […] The incidence rate of autoimmune seizure/epilepsy from 1996-2019 was 0.33 per 100,000 person-years. The incidence rate of seropositive and seronegative autoimmune seizure/epilepsy were 0.14 and 0.18 per 100,000 respectively. […] Although autoimmune seizure/epilepsy is a rare condition, it is associated with considerable clinical morbidity.

• #5 Autoimmune epilepsy: Symptoms, causes, and treatment

  https://www.medicalnewstoday.com/articles/autoimmune-epilepsy

  Autoimmune epilepsy is a rare type of epilepsy that occurs as a result of an immune system imbalance. It develops due to immune antibodies targeting the brain. […] A 2021 case study notes that it is rare, affecting 14 in every 100,000 people. […] According to a 2021 review, people with systemic autoimmune disorders (SADs) might have a higher risk of epilepsy. […] An estimated 5% to 35% of individuals who begin to develop seizures may have autoimmune epilepsy, although the exact figure is unclear. […] Early diagnosis of autoimmune epilepsy can help increase a person’s chances of stopping seizures long term. […] While autoimmune epilepsy typically does not respond well to antiseizure medications, effective treatments are available. […] Autoimmune epilepsy is a rare type of epilepsy that occurs due to the immune system attacking specific proteins in the brain, leading to seizures. […] The types include autoimmune limbic encephalitis, FIRES, and epilepsy induced by systemic autoimmune disorders.

• #6

  https://link.springer.com/article/10.1007/s13311-019-00750-3

  The true incidence of autoimmune epilepsy remains unknown. There are no population-based studies providing age- and sex-adjusted incidence and prevalence of autoimmune epilepsy. […] A recent population-based study from Olmsted county, MN, estimated the incidence and prevalence of autoimmune encephalitis to be 0.8/100,000 person-years and 13.7/100,000 people, respectively. […] A hospital-based prospective study reported that 20% of adult patients with epilepsy of unknown etiology were seropositive for neural specific antibodies associated with autoimmune epilepsy or encephalopathy. […] Epilepsies of unknown etiology are estimated to constitute one-third of all epilepsies among adults. Therefore, the rate of autoimmune epilepsies based on these studies can be inferred to be around 5 to 7% of all epilepsies, at least in adults. The frequency of autoantibodies in pediatric epilepsy is more unclear. Studies from Europe and Australia have reported the presence of autoantibodies in about 10% of pediatric patients with new-onset epilepsy.

• #7 Autoimmune epilepsy – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/autoimmune-epilepsy/symptoms-causes/syc-20576892

  Autoimmune epilepsy is a type of epilepsy where seizures are caused by the immune system mistakenly attacking brain cells. […] Autoimmune epilepsy is caused by the immune system attacking brain cells and leading to seizures. […] More recently, research has concluded that epilepsy can be an autoimmune disease. […] Autoimmune epilepsy causes may include: Autoimmune encephalitis is the most common cause of autoimmune epilepsy. […] The risk of autoimmune epilepsy is low, but it can occur in both adults and children. […] Autoimmune epilepsy complications can include serious seizures that last more than five minutes or occur one after another.

• #8 Antibody Prevalence in Epilepsy and Encephalopathy (APE2) Score – Insights

  https://news.mayocliniclabs.com/2019/10/21/antibody-prevalence-in-epilepsy-and-encephalopathy-ape2-score/

  Autoimmune encephalitis and autoimmune epilepsy were once considered extremely rare, but lately this perception has changed. A considerable proportion of these cases have neural-specific antibody biomarkers. […] In a population-based study, performed in Olmsted County, Minnesota, we found that the incidence of autoimmune encephalitis nearly tripled over two decades, increasing from 0.4/100,000 to 1.2/100,000. This was mostly due to increased recognition of antibody-positive cases. […] We have noticed similar trends among patients with epilepsy of unknown etiology. Nearly 15–20% of patients with cryptogenic epilepsies with or without other features of encephalitis have serological biomarkers suggestive of an autoimmune etiology. […] An APE2 score of more than or equal to four performed well as a predictor of neural autoantibody positivity. The sensitivity for neural-specific antibody positivity among patients with epilepsy or encephalopathy for an APE2 score of more than or equal to four was 98%, whereas the specificity was approximately 84%.

• #9 Diagnostic Criteria, Phenotypes and Epidemiology of Autoimmune Encephalitis: Divyanshu Dubey, MBBS

  https://www.neurologylive.com/view/diagnostic-criteria-phenotypes-epidemiology-autoimmune-encephalitis-divyanshu-dubey

  Autoimmune encephalitis was found to be as common as infectious encephalitis, if not slightly more common. If you extrapolate this data to the entire world, I would say there are about 1 million autoimmune encephalitis cases in the world, and 90,000 people develop this condition every year. […] Dubey also talked about the epidemiology of the condition and how it compares with infectious encephalitis.

• #10 Autoimmune Epilepsy Panel, Serum and CSF | Test Fact Sheet

  https://arupconsult.com/ati/autoimmune-epilepsy-panel

  Autoimmune epilepsy accounts for 15-20% of epilepsies previously considered to be cryptogenic. […] In patients with new-onset seizure activity, the Antibody Prevalence in Epilepsy and Encephalopathy (APE2) score can be used to predict the likelihood of the presence of an antineural antibody and should therefore be considered before ordering antineural antibody testing. […] Factors associated with autoimmune epilepsy include autonomic dysfunction, brain magnetic resonance imaging (MRI) findings suggestive of encephalitis, elevated cerebrospinal fluid (CSF) protein or pleocytosis, faciobrachial dystonic seizures, history of autoimmunity, history of malignancy, neuropsychiatric changes, orofacial dyskinesias, seizures refractory to antiseizure drugs, and viral prodrome. […] It is important to note that autoimmune epilepsy may exist in the absence of detectable, known antineural antibodies, and empiric immunotherapy trials may be considered in the appropriate clinical context.

• #11 Biomarkers for epileptogenesis in patients with autoimmune epilepsy

  https://www.oaepublish.com/articles/2347-8659.2020.29

  Autoimmune epilepsy (AE) is a general term to describe recurrent seizures that have an immune-mediated origin. It is increasingly being recognized as a cause of epilepsy due to accumulating evidence supporting an immune-mediated pathogenesis in patients who have shown resistance to traditional antiepileptic drugs (AEDs). The diagnosis of AE is one of the exclusions. Currently, there are no strict diagnostic guidelines for AE, and it is similarly under-recognized. The importance of early diagnosis of AE cannot be overstated, as prompt immunotherapy is important for seizure reduction. Further investigations into potential biomarkers are needed for early detection of AE and include targeted immunotherapies in combination with AEDs. […] While the true prevalence of AE is unknown, it is suspected that between 14% and 20% of patients with refractory epilepsy of unknown etiology can be attributed to an immune basis and as much as a third of patients do not benefit when current symptomatic epilepsy management strategies are used.

• #12 Population-Level Evidence for an Autoimmune Etiology of Epilepsy

  https://pmc.ncbi.nlm.nih.gov/articles/PMC4324719/

  Epilepsy is a debilitating condition, often with neither a known etiology nor an effective treatment. Autoimmune mechanisms have been increasingly identified. […] To conduct a population-level study investigating the relationship between epilepsy and several common autoimmune diseases. […] The risk of epilepsy is significantly heightened among patients with autoimmune diseases (OR 3.8, 95% CI 3.64.0, P0.001), and is especially pronounced in children (OR 5.2, 95% CI 4.16.5; P0.001). Elevated risk is consistently observed across all 12 autoimmune diseases. […] Epilepsy and AD frequently co-occur and patients with either condition should undergo surveillance for the other. The potential role of autoimmunity must be given due consideration in epilepsy, so we are not overlooking a treatable etiology. […] The risk of epilepsy is consistently higher in children with AD, compared with adults with the same AD. […] Our data provide important epidemiology evidence that lends support to the hypothesis, and highlights the need for in-depth investigation.

• #13 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. […] The researchers found that there was a 3.8-fold increase in the risk of epilepsy for patients with an autoimmune disease and 17.5 percent of epilepsy patients also had an autoimmune disease. […] The increase in risk was greater among children (5.2-fold) than adults (4.3-fold). […] Findings also revealed that autoimmunity patients on select immunosuppressing medications had a lower risk of developing epilepsy. […] The risk association between epilepsy and autoimmunity suggests that both rheumatologists and neurologists should consider screening their patients for signs of the associated condition. […] The team’s data did not allow them to explore possible mechanisms behind the association, but they hope to work with collaborators to look for biological links. […] There are many ways in which autoimmunity can affect the brain and cause seizures.

• #14 Autoimmune Diseases Frequently Co-occur with Epilepsy

  https://www.hcplive.com/view/autoimmune-diseases-frequently-co-occur-with-epilepsy

  A new retrospective study suggests that patients with autoimmune disorders should be monitored for epilepsy, and vice versa. […] According to the study authors, the risk of epilepsy was significantly heightened among patients with autoimmune diseases and was especially pronounced in children. In addition, the elevated risk was consistently observed across all 12 autoimmune diseases. […] The study authors concluded that epilepsy and autoimmune disease frequently co-occur, (so) patients with either condition should undergo surveillance for the other. The potential role of autoimmunity must be given due consideration in epilepsy so that we are not overlooking a treatable cause. […] Based on those findings, Gorman recommended launching a concerted effort between neurologists and rheumatologists to control the association between epilepsy and autoimmune disorders.

• #15 Azthena logo with the word Azthena

  https://www.news-medical.net/news/20140514/Autoimmune-epilepsy-diagnostics-an-interview-with-Dr-Joseph-J-Higgins-Medical-Director-Neurology-Athena-Diagnostics-a-business-of-Quest-Diagnostics.aspx

  A growing body of literature demonstrates an autoimmune basis in the etiology of some forms of epilepsy. […] A study published in the March 2014 edition of JAMA Neurology found that autoimmune disorders were associated with elevated risk of epilepsy, and that patients with either condition should undergo surveillance for the other. […] The findings indicate the risk of epilepsy is almost four times higher for patients with an autoimmune disease, with 17.5 percent of epilepsy patients also having an autoimmune disease. […] Of these, as many as 10 percent may be categorized as autoimmune epilepsy. […] The JAMA study is disturbing because it found a strong association between incidence of epilepsy and twelve autoimmune diseases, some of which are prevalent. […] Autoimmune epilepsy requires different diagnostic techniques. […] To identify autoimmune epilepsy, patients are also screened for a broad range of neural autoantibodies while MRI and cerebrospinal fluid testing may help identify inflammatory changes. […] We believe this test therefore reflects a significant diagnostic advance for autoimmune epilepsy.

• #16

  https://www.jci.org/articles/view/125178

  The rapid expansion in the number of encephalitis disorders associated with autoantibodies against neuronal proteins has led to an incremental increase in use of the term autoimmune epilepsy, yet has occurred with limited attention to the physiopathology of each disease and genuine propensity to develop epilepsy. […] The risk of epilepsy is higher for disorders in which the antigens are intracellular (often T cell-mediated) compared with disorders in which the antigens are on the cell surface (antibody-mediated). […] The exact frequency of neuronal antibody-mediated encephalitis is unknown. It has been estimated to constitute 10% to 15% of all cases of encephalitis (which have an annual incidence of 5 to 10 cases per 100,000 persons). […] In 2017, a new classification of epilepsy by the ILAE introduced the concept of epilepsy of immune etiology for patients whose epilepsy results directly from an immune disorder in which seizures are a core symptom of the disorder. […] The acute phase of most antibody-mediated encephalitis with seizures can last several months, yet the risk of epilepsy is small. […] A common feature of all types of autoimmune epileptic seizures is the refractoriness to antiepileptic drugs unless immunotherapy is concurrently used.

• #17 Autoimmune-associated epilepsy in an outpatient epilepsy clinic: A retrospective study | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-articulo-autoimmune-associated-epilepsy-in-an-outpatient-S2667049623001291

  The main objective of this study is to analyse cases in which AAEp was suspected in the outpatient epilepsy clinic of Cruces University Hospital (CUH), a national epilepsy referral centre in Spain, between 2014 and 2021. Primary aims of the study were to determine how many patients with clinical suspicion were finally diagnosed with AAEp and to analyse differences according to autoimmune test results and response to immunotherapy. Understanding these differences could lead to better and earlier identification of patients with AAEp. […] A secondary study objective was to assess whether diagnostic, therapeutic, and/or prognostic differences exist between patients with chronic/isolated AAEp and patients with ASS-AEn seen in our hospital. […] Overall, 1749 patients were seen during the study period. In 30 (1.71%), autoimmune testing was performed due to clinical suspicion of AAEp, 60% of whom were women, with a mean age of 28.2 years at seizure-onset. A total of 30 patients were included: 18 women (60%); mean age 28.2 years at seizure-onset. AAEp was diagnosed in 14 (46.6%), on the basis of antineuronal antibodies, CSF pleocytosis/OCB (oligoclonal bands), MRI with neuroinflammation, and/or PET hypermetabolism.

• #18 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. […] Among 112 consecutive adult patients with unexplained epilepsy, 20.5% were found to have neurologic antibodies strongly suggesting an autoimmune etiology. […] Applying an antibody prevalence in epilepsy (APE) score based on clinical characteristics helped predict which epilepsy patients were likely to have an autoimmune etiology. […] Immunomodulatory therapy reduced seizure frequency in patients seropositive for autoantibodies. […] The primary aim of the Dubey et al. study was to determine the prevalence of neurologic autoantibodies among adult patients with new-onset or established epilepsy of unknown etiology.

• #19 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

  Of 112 consecutive patients who fit these criteria, 35% had serum antibodies suggesting that autoimmunity may be to blame for the seizures. […] Presence of these antibodies, all of which are highly suggestive of an autoimmune cause of epilepsy, was evident in 20.5% of patients. […] New-onset seizures, neuropsychiatric changes, autonomic dysfunction, a viral prodrome, a brain MRI indicative of limbic encephalitis, and faciobrachial dystonic seizures were all significantly more common in antibody-positive cases than in antibody-negative cases. […] The Dubey group has now proven some of these clinical features on a prospective level. […] The team prospectively assigned a score to each patient in the study to estimate the probability of an autoimmune etiology prior to knowing the antibody test results.

• #20 Antibody Prevalence in Epilepsy and Encephalopathy (APE2) Score – Insights

  https://news.mayocliniclabs.com/2019/10/21/antibody-prevalence-in-epilepsy-and-encephalopathy-ape2-score/

  To conclude, APE2 scoring is a useful predictive model based on clinical criteria, which can aid in diagnosis and management of autoimmune epilepsy and encephalopathy. Very low APE2 scores may be useful in identifying patients who are more likely to have any negative neural-specific autoantibody evaluation. Furthermore, in some cases with epilepsy of unknown etiology, after reasonable exclusion of alternative etiologies, this model can help in early diagnosis and early immunotherapy initiation.

• #21 Early predictors of new-onset immune-related seizures: a preliminary study | BMC Neurology | Full Text

  https://bmcneurol.biomedcentral.com/articles/10.1186/s12883-022-03042-0

  NSAbs and APE2 scores could act as early predictors of initial immune-related seizures. […] Approximately 60% of patients with autoimmune encephalitis (AE) exhibit secondary acute symptomatic seizures. […] The sensitivity and specificity of APE24 points for predicting AE were 73.9% and 100%. […] The results of multivariate analysis showed that the NSAbs and APE2 scores independently influenced the early prediction of initial immune-related seizures (P<0.05). [...] The results showed that NSAbs and APE2 scores were independent factors for the early prediction of initial immune-related seizures (P<0.05). [...] This study found that immune-related seizures were more common with positive NSAbs and APE2 score≥4, and neuroimaging facilitated the diagnosis of the disease. [...] We demonstrated that NSAbs and APE2 scores could be used as early predictors of initial immune-related seizures.

• #22 Autoimmune Epilepsy

  https://practicalneurology.com/diseases-diagnoses/epilepsy-seizures/autoimmune-epilepsy/30185/

  Currently, no data from randomized controlled trials is available. Observational studies and clinical experience suggest that a response to immunotherapy in epilepsy (RITE) score 7 predicts a favorable response to immunotherapy. […] Early diagnosis and treatment can drastically alter the course of the disease.

• #23 Autoimmune Epilepsy

  https://practicalneurology.com/articles/2018-oct/autoimmune-epilepsy

  Seizures of autoimmune epilepsy are most commonly focal temporal lobe seizures but seizure onset is often seen elsewhere. […] If there is a positive antibody result, cancer screening for the most common associated cancer type should be done. If antibody testing is negative, ovarian or testicular ultrasound and thoracic, abdominal/pelvic CT and full body PET scans are reasonable follow up tests. […] Currently, no data from randomized controlled trials is available. Observational studies and clinical experience suggest that a response to immunotherapy in epilepsy (RITE) score 7 predicts a favorable response to immunotherapy. […] If patients worsen or there is no response within 2 weeks of a first-line therapy, second-line therapies are rituximab (1g IV) given twice 2 weeks apart or cyclophosphamide (750mg/m2 monthly up to 6 months). […] Early diagnosis and treatment can drastically alter the course of the disease.

• #24 Autoimmune-associated epilepsy in an outpatient epilepsy clinic: A retrospective study | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-articulo-autoimmune-associated-epilepsy-in-an-outpatient-S2667049623001291

  The delay between epilepsy-onset and autoimmune testing was longer in patients with negative autoimmune-testing and in non-responders. Diagnosis and treatment delay were longer in AAEp than in ASS-AEn. This may indicate that currently there is less capacity to detect AAEp than ASS-AEn. […] Isolated/chronic AAEp is a rare, drug-resistant epileptic disorder. Early diagnosis is essential for immunotherapy. However, diagnostic and therapeutic delay is longer in AAEp than in ASS-AEn.

• #25

  https://link.springer.com/article/10.1007/s40265-022-01826-9

  Autoimmune-associated epilepsy is often falsely used to describe patients with AIE presenting with seizures. […] Autoimmune-associated epilepsy patients generally do not present the clinical features of acute AIE, but can test positive for neural autoantibodies, and do not respond well to immunotherapy. […] The fact that persistent seizure-freedom can be obtained after immunotherapy (with or, in some cases even without ASM), contradicts the conceptual definition of epilepsy as an enduring predisposition to generate epileptic seizures and by the neurobiological, cognitive, psychological, and social consequences of this condition as defined by the ILAE. […] Rapid initiation of immunomodulation in immune-mediated seizures is crucial as ASMs often fail and are usually only applied add-on for symptomatic control of seizures.

• #26 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. […] More recently, research has concluded that epilepsy can be an autoimmune disease. […] Autoimmune epilepsy is caused by the immune system attacking brain cells and leading to seizures. […] The risk of autoimmune epilepsy is low, but it can occur in both adults and children. […] Autoimmune epilepsy complications can include serious seizures that last more than five minutes or occur one after another. […] Autoimmune epilepsy treatment differs from the treatment used for other types of epilepsy. […] Immunotherapy to reduce the immune system activity and treat seizures is used for autoimmune epilepsy. […] Many people whose seizures are due to autoimmune encephalitis become seizure free after taking immunotherapy. […] Rasmussen syndrome, which mainly affects children, usually doesn’t respond to medicines.

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

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

  Autoimmune epilepsy is a newly defined group of conditions characterized by recurrent seizures associated with autoimmunity. […] The International League Against Epilepsy has recognized autoimmune epilepsy since 2017. […] In a 2018 study, researchers reported an incidence of autoimmune encephalitis in Olmsted Country, Minnesota, of 0.4 people per 100,000 people per year from 1995 to 2005 and 1.2 people per 100,000 people per year from 2006 to 2015. […] Its thought that autoimmune seizures could make up as many as 1 in 7 to 1 in 20 new seizures. […] Symptoms of autoimmune epilepsy can develop rapidly from the onset of the condition. […] Additionally, epilepsy caused by autoimmune inflammation has a risk of causing seizures that progress to status epilepticus. […] An early initiation of immunotherapy is associated with a favorable outcome.

• #28 Autoimmune encephalitis: Epidemiology, pathophysiology and clinical spectrum (part 2)

  http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742023000400001

  Autoimmune encephalitis (AE) represents a growing number of severe autoimmune-inflammatory diseases affecting both the white and grey matter of the brain. […] In part 1 of this series, we focused on the epidemiology, pathophysiology and clinical presentation of this condition, with two illustrative cases. […] The disease predominantly affects younger patients, and ~80% are female. […] Prognosis varies depending on AE subtype, associated antibodies, possible paraneoplastic origin, patient age and severity of disease. […] While spontaneous clinical improvement is rare, with prompt and effective therapy, the potential for a good recovery is high. […] More than 80% of patients with anti-NMDA receptor encephalitis have a good outcome and are able to function independently after 2 years. […] The importance of early recognition and initiation of therapy for AE must be emphasised. […] Mortality due to AE is low in most cohorts, and often associated with prolonged admission to ICU and complications arising from mechanical ventilation, sepsis and status epilepticus.

• #29 Autoimmune-associated epilepsy in an outpatient epilepsy clinic: A retrospective study | Neurology perspectives

  https://www.elsevier.es/en-revista-neurology-perspectives-17-articulo-autoimmune-associated-epilepsy-in-an-outpatient-S2667049623001291

  Autoimmune epilepsy (AEp) refers to epilepsy of autoimmune aetiology. The link between the immune system and epilepsy has long been described, but AEp was not included in the International League Against Epilepsy (ILAE) seizure classification until 2017. However, the definition of AEp is still highly controversial: AEp is considered a broad term that encompasses different types of conditions, and conceptual distinctions are emerging between acute symptomatic seizures (ASS) secondary to autoimmune encephalitis (AEn) and autoimmune-associated epilepsy (AAEp). The first refers to seizures occurring in the setting of the active phase of AEn, while the second refers to chronic seizures which are the unique or the main symptom of a disorder that is caused by autoimmune brain diseases. Epilepsy, as defined by the ILAE, is considered a chronic disease. On this basis, AEp should be used to refer to a chronic disorder in which seizures are the most prominent symptom and an autoimmune origin is confirmed by the presence of either antineuronal antibodies or chronic brain inflammation. However, the proposed diagnostic criteria for AEp and rating scales predicting antineuronal antibody prevalence in epilepsy (APE-score) and the chance of response to immunotherapy in epilepsy (RITE-score) are strongly focused on ASS-AEn. As such, the available diagnostic criteria for AEp as an isolated symptom or as a chronic disorder are inadequate. Moreover, although increasing evidence is emerging on when to suspect AEp in an acute setting, it remains unclear when AEp should be suspected in chronic or isolated epileptic disorders, despite this being the most common scenario in the outpatient care setting. In real-world practice, most epilepsy experts need to manage chronic seizure disorders of unknown aetiology that might be autoimmune in origin, but no guidelines or recommendations are available on when to perform autoimmune testing or when to start immunotherapy in this setting. In response to this lack of information, this study aimed to provide more data in the area of chronic/isolated AAEp, in order to optimise the selection of patients who would benefit from autoimmune testing and an immunotherapy trial in outpatient clinical practice.

• #30 Immunity, Inflammation, and Epilepsy (Chapter 23) – Autoimmune Encephalitis and Related Disorders of the Nervous System

  https://www.cambridge.org/core/books/autoimmune-encephalitis-and-related-disorders-of-the-nervous-system/immunity-inflammation-and-epilepsy/BB7A5E6820254290607B832706B8BB30

  In this chapter we review the inflammatory mechanisms involved in epileptogenesis, the caveats and limitations of the term autoimmune epilepsy, and two epileptic syndromes of autoimmune origin: epilepsy associated with GAD antibodies and Rasmussen encephalitis. […] The International League Against Epilepsy (ILAE) recently proposed the definition of acute symptomatic seizures secondary to autoimmune encephalitis for the seizures that occur in the setting of the active phase of immune-mediated encephalitis. […] In contrast, the term autoimmune epilepsy applies to chronic seizures considered to be secondary to autoimmune brain diseases. […] If promptly diagnosed and treated, patients with symptomatic seizures due to antibody-mediated encephalitis rarely evolve to develop epilepsy and, therefore, they do not fulfil criteria of autoimmune epilepsy.

• #31 Immunity, Inflammation, and Epilepsy (Chapter 23) – Autoimmune Encephalitis and Related Disorders of the Nervous System

  https://www.cambridge.org/core/books/autoimmune-encephalitis-and-related-disorders-of-the-nervous-system/immunity-inflammation-and-epilepsy/BB7A5E6820254290607B832706B8BB30

  Scoring systems to predict autoimmune seizures are not very useful because they rely on the presence of additional neurological manifestations or diagnostic tests included in the definition of autoimmune encephalitis. […] Antibodies against neuronal surface antigens occur in a minority (5%) of patients with isolated epilepsy; the significance of these antibodies is unclear as the spectrum of symptoms of these patients is not different from that of seronegative cases. […] In contrast, antibodies against GAD (an intracellular protein) occur in a small subset of patients with temporal lobe epilepsy that is usually refractory to anti-seizure medication.

• #32 Autoimmune Epilepsy Panel, Serum and CSF | Test Fact Sheet

  https://arupconsult.com/ati/autoimmune-epilepsy-panel

  These serum and CSF antineural antibody panel tests can be used for the evaluation of patients with a neurologic phenotype consisting predominantly of new, acute to subacute onset of epilepsy that is refractory to more than two antiseizure medications. […] Testing for the presence of antineural antibodies in both serum and CSF is recommended to improve diagnostic yield. […] Regardless of the panel chosen, order only one panel for serum and/or one panel for CSF; many antineural antibodies are redundant between these panels, and choosing based on the predominant phenotype will provide the most meaningful results. […] This panel does not include every antibody that has been associated with autoimmune epilepsy: […] As testing for newly described antibodies becomes available and their clinical relevance is established, these panels will evolve to reflect these discoveries. […] A diagnosis of autoimmune epilepsy is not excluded.

• #33

  https://link.springer.com/article/10.1007/s40265-022-01826-9

  Seizures and epilepsy can result from various aetiologies, yet the underlying cause of several epileptic syndromes remains unclear. […] Autoimmune-mediated pathophysiological mechanisms have been gaining attention in the past years and were included as one of the six aetiologies of seizures in the most recent classification of the International League Against Epilepsy. […] The increasing number of anti-neuronal antibodies identified in patients with encephalitic disorders has contributed to the establishment of an immune-mediated pathophysiology in many cases of unclear aetiology of epileptic syndromes. […] Given the overlapping clinical presentation of immune-mediated seizures originating from different aetiologies, a clear distinction among the aetiological entities is crucial when it comes to discussing pathophysiological mechanisms, therapeutic options, and long-term prognosis of patients.

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

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

  People with cancer-related autoimmune epilepsy generally have worse outcomes than people who develop autoimmune epilepsy with other underlying causes. […] 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.

• #35 Autoimmune epilepsy | Shilkina | Epilepsy and paroxysmal conditions

  https://www.epilepsia.su/jour/article/view/777?locale=en_US

  The psychopathology of NMDAR-antibody encephalitis in adults: a systematic review and phenotypic analysis of individual patient data. […] Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. […] Anti-NMDA-R encephalitis: should we consider extreme delta brush as electrical status epilepticus? […] Problems of paraneoplastic limbic encephalitis diagnosis. […] Paraneoplastic limbic encephalitis in neurological and oncological practice. […] Nonconvulsive status epilepticus in patients with limbic encephalitis. […] Pediatric LGI1 and CASPR2 autoimmunity associated with COVID 19: Morvan syndrome. […] Genetic predisposition in anti-LGI1 and anti-NMDA receptor encephalitis. […] Encephalitis and AMPA receptor antibodies: novel findings in a case series of 22 patients.

• #36 Autoimmune epilepsy | Shilkina | Epilepsy and paroxysmal conditions

  https://www.epilepsia.su/jour/article/view/777?locale=en_US

  Investigation of autoimmune epilepsy (AIE) has been attracting increasingly more attention due to discovery of neuronal antibodies and improved understanding of the mechanisms related to such immune-mediated syndromes. […] The necessity of regular monitoring patients with AIE is indicated, preferably by an epileptologist together with a neuroimmunologist. […] With a deeper understanding of the mechanisms of antibody-mediated and autoantigen-specific T-cell-mediated AIE syndromes, the use of antiepileptic drugs and immunotherapy can be further optimized. […] Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. […] Autoimmune epilepsy: clinical characteristics and response to immunotherapy. […] Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy.

Zobacz więcej