Materiały źródłowe

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

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

  Autoimmune encephalitis defines brain inflammation caused by a misdirected immune response against self-antigens expressed in the central nervous system. It comprises a heterogeneous group of disorders that are at least as common as infectious causes of encephalitis. […] The California Encephalitis Project found that among persons under 30 years of age, N-methyl-D-aspartate receptor (NMDAR)-antibody encephalitis was more common than any individual infectious cause of encephalitis. […] Autoimmune causes of encephalitis have been reported to be at least as common as viral causes in Olmsted County, USA. […] The incidence of autoimmune encephalitis rose in the second 10-year epoch of this study, likely owing to growing awareness of these disorders and more widespread diagnostic capacities.

• #2 Biomedicines | Special Issue : Molecular Epidemiology and Pathophysiology of Autoimmune Encephalitis

  https://www.mdpi.com/journal/biomedicines/special_issues/Autoimmune_Encephalitis

  Autoimmune encephalitis encompasses a broad range of diseases with different pathophysiology. Largely due to their rarity and heterogenous nature, these encephalitises are challenging to treat, and frequently recur. Over the last decade, significant progress has been made, providing a better understanding of their physiopathology. […] The aim of this Special Issue is to provide a broad overview of the molecular epidemiology and pathophysiological mechanisms that drive autoimmune encephalitis. Overall, the goal is to highlight progress made in preclinical and translational research to improve our understanding of autoimmune encephalitis.

• #3 Autoimmune Encephalitis Epidemiology and a comparison to Infectious Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6011827/

  We evaluate incidence and prevalence of autoimmune encephalitis and compare the epidemiology of autoimmune and infectious encephalitis. […] The prevalence of autoimmune encephalitis on January 1, 2014 of 13.7/100,000 was not significantly different from that of all infectious encephalitides (11.6/100,000; p=0.63) or the viral subcategory (8.3/100,000; p=0.17). […] The incidence rates (1995-2015) of autoimmune and infectious encephalitis were 0.8/100,000 and 1.0/100,000 person-years respectively (p=0.58). […] This study shows that the prevalence and incidence of autoimmune encephalitis is comparable to infectious encephalitis and its detection is increasing over time. […] The prevalence of autoimmune encephalitis was higher among African-American population (38.3/100,000) than Caucasians (13.7/100,000; p=0.04), particularly for autoimmune GAD65 encephalitis and ADEM.

• #4 Nailing the Suspect: The Prevalence of Autoimmune Encephalitis Compared with Infectious Encephalitis – Insights

  https://news.mayocliniclabs.com/2018/04/29/nailing-the-suspect-the-prevalence-of-autoimmune-encephalitis-compared-with-infectious-encephalitis/

  Now, in a groundbreaking population-based study, Mayo Clinic has found that autoimmune encephalitis is as common as its infectious disease counterpart. […] Previous epidemiology studies of encephalitis have focused mostly on infectious causes, with “immune-mediated” syndromes occasionally analyzed as a subgroup. Mayo Clinic’s study is the first to determine the epidemiology of autoimmune encephalitis at a population level. […] In that study population, the prevalence of autoimmune encephalitis was 13.7 cases per 100,000 people, compared with 11.6 cases per 100,000 people for infectious encephalitis. […] Extrapolating our results to the world population suggests that about one million people are affected with autoimmune encephalitis. […] The study also found a nearly three-times higher incidence of autoimmune encephalitis for African-Americans: 38.3 cases per 100,000 people in the population group, which is largely of northern European descent.

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

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

  The assistant professor of neurology at Mayo Clinic spoke about understanding the phenotypes and epidemiology of autoimmune encephalitis at the 2023 AAN Annual Meeting. […] „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.

• #6 When the body attacks the brain: Immune system often to blame for encephalitis, study finds | ScienceDaily

  https://www.sciencedaily.com/releases/2018/02/180212133441.htm

  Encephalitis caused by the immune system attacking the brain is similar in frequency to encephalitis from infections, Mayo Clinic researchers report in Annals of Neurology. […] This study shows autoimmune encephalitis is an equally common cause. […] The researchers found about 14 per 100,000 people had autoimmune encephalitis in their lifetime, compared to 12 per 100,000 who had infectious encephalitis. […] This study allows us to estimate that approximately 1 million people worldwide had autoimmune encephalitis in their lifetime. […] We also estimate that, currently, about 90,000 people around the world develop autoimmune encephalitis each year. […] Our study showed that clinicians are now detecting more cases of autoimmune encephalitis than they were in the past because of the discovery of these new neural autoantibody markers.

• #7 Epidemiology of autoimmune encephalitis and comparison to infectious causes-Experience from a tertiary center – PubMed

  https://pubmed.ncbi.nlm.nih.gov/39030965/

  Objectives: The incidence of autoimmune encephalitis (AIE) has risen in the last decade, yet recent studies are lacking. We compared the epidemiology of autoimmune and infectious encephalitis cases in Tel-Aviv Sourasky Medical Center (TASMC) between 2010 and 2020. […] Results: Two hundred and twenty-five patients with encephalitis were identified. The most common identifiable cause was viral (42%), followed by autoimmune encephalitis (35%), bacterial (18%), and fungal/parasitic (5%). The incidence of AIE cases out of the yearly admitted cases increased substantially, from 3.8/100 K in 2010 to 18.8/100 K in 2020. […] Interpretation: AIE incidence has risen significantly in our institution during the past decade, with current rates comparable to those of all infectious causes combined. Based on this cohort, clinical clues for an autoimmune etiology include a non-inflammatory cerebrospinal fluid profile, the presence of seizures, and temporal lobe imaging abnormalities (also common in herpetic encephalitis). In light of its rising incidence and the importance of early treatment, AIE should be considered in the differential diagnosis of all encephalitis cases.

• #8 Autoimmune Encephalitis Epidemiology and a comparison to Infectious Encephalitis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6011827/

  The incidence of autoimmune encephalitis also was higher among African-Americans (2.8/100,000 person-years) than Caucasians (0.7/100,00 person-years, p=0.01). […] The incidence of autoimmune encephalitis increased from the 1995-2005 interval to the 2006-2016 interval (0.4 [1995-2005] to 1.2 [2006-2015], p=0.02). […] This study shows that the incidence and prevalence of autoimmune encephalitis approximates that of infectious encephalitis at a population level.

• #9

• #10

  https://samajournals.co.za/index.php/samj/article/view/780

  Since the identification of anti-N-methyl-D-aspartate (NMDA) receptor antibodies about 15 years ago, many patients with rapidly progressing psychiatric symptoms, abnormal movements, seizures or unexplained coma, have been diagnosed with autoimmune encephalitis (AE). […] While persons of all ages can be affected by AE, some types of AE affect more children and young adults and are more prevalent in women. […] This review will focus on encephalitides associated with neuronal cell-surface or synaptic antibodies, which can result in characteristic syndromes, and are often recognizable on clinical grounds. […] AE subtypes associated with antibodies against extracellular epitopes can occur with or without tumours. […] The first part of this series will introduce the topic, provide an overview of current neuronal surface antibodies and how they present, describe the most common subtype, anti-NMDA receptor encephalitis, and discuss the difficulties in recognizing patients with underlying AE amongst patients with new onset psychiatric disorders.

• #11 Autoimmune encephalitis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/autoimmune-encephalitis?lang=us

  The epidemiology of tumor-related autoimmune encephalitis mimics that of the underlying malignancy. Those with non-tumor related autoimmune encephalitis have a variable epidemiology. Overall, the most common encephalitis with antibodies to intracellular antigens is Hu antibody encephalitis, whilst the most common encephalitis with antibodies to cell surface antigens is LGI1 antibody encephalitis. Some autoimmune encephalitides have a sex and age predilection, for example, NMDAR antibody encephalitis is more common in young females, while LGI1 and CASPR2 antibody encephalitides are more common in older males. […] Various autoimmune encephalitides may be associated with an underlying malignancy or tumor, more commonly in those with antibodies against intracellular antigens.

• #12 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. […] The importance of early recognition and initiation of therapy for AE must be emphasised. […] 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.

• #13 Frontiers | A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making

  https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1408606/full

  A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making […] This challenge is exacerbated by the scarcity of epidemiological reporting of AIE. […] This SLR highlights a paucity of epidemiology data for AIE in children, which is likely reflective of difficulties in testing and diagnosis. […] Studies reporting the incidence and prevalence of AIE in children are scarce. […] A better understanding of the epidemiology of pediatric AIE and AIE subtypes could provide an estimate of the burden and distribution of AIE. […] This SLR identified a distinct lack of published data on the epidemiology of pediatric-onset AIE. […] Only nine studies reporting incidence data were identified, of which, only one reported incidence rates of definite AIE in a full pediatric population in children of all ages.

• #14

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

  Autoimmune encephalitis (AIE) is being increasingly recognized as a significant as well as frequent cause of encephalopathy in the pediatric age group. […] Data on the epidemiology of pediatric AIE is limited. A retrospective study of anti-NMDAR encephalitis conducted over seven years in Hong Kong estimated an incidence of 2.2/ million children per year. […] Anti-NMDAR encephalitis accounts for 4% of all encephalitis and is the most common cause of seropositive AIE in children. Almost 40% of all reported cases are below 18 years of age. […] Pediatric autoimmune encephalitis forms a group of acquired disorders with antibodies targeting cell-surface antigens or intracellular antigens that are treatable. Pediatric disease manifests differently from adults, with less frequent association with neoplasms and predominance of movement disorders, behavioral abnormalities and seizures. Anti-NMDAR encephalitis is the most common pediatric autoimmune encephalitis. It exhibits typical clinical features (limbic encephalitis) as well as imaging abnormalities (mesial temporal signal change) although these may be seen in only 30-40% of patients. Hence, clinical recognition is the key. It responds well to early therapy. Treatment involves immunomodulation which should be initiated empirically as soon as the diagnosis of autoimmune encephalitis is suspected, even prior to the availability of antibody test results.

• #15 Frontiers | A systematic review of the epidemiology of pediatric autoimmune encephalitis: disease burden and clinical decision-making

  https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2024.1408606/full

  The calculated incidence estimates presented here likely underestimate the true incidence of AIE in children. […] Further research at a national/international level, across a wider breadth of countries is critically necessary. […] Epidemiological data will play an instrumental role in informing research, health policies, clinical decision-making and also to better understand the worldwide burden of disease.

• #16

  https://link.springer.com/article/10.1007/s13311-020-00861-2

  The most common risk factors for patients with autoimmune encephalitis are what are thought to be the most likely triggers: malignancy and infections. […] Most autoimmune encephalitides will occur without an identified trigger, but each has several well-recognized associations with specific malignancies. […] Over the last three decades, modern techniques of cell-based assays and immunocytochemistry have demonstrated multiple antibodies responsible for limbic encephalitis.

• #17

  https://link.springer.com/article/10.1007/s00234-024-03318-x

  Autoimmune encephalitis (AE) refers to a spectrum of disorders characterized by inflammatory processes affecting the brain tissue and originating from an immune-mediated pathophysiological mechanism targeting neurons. The reported incidence ranges from 3/million to 8/million person-years in epidemiologic studies in the western world, but many authors argue that this disease category is still under-diagnosed and misdiagnosed. […] For instance, anti-Hu AE the most common paraneoplastic AE with a reported incidence of 0.4/million person-years is associated with tumors in 75-80% of cases (mainly small cell lung carcinoma, SCLC). On the other hand, Group II AE are more commonly non-paraneoplastic conditions that can affect patients belonging to a wide age range, including younger and sometimes pediatric patients. Anti-NMDAR (N-methyl D-aspartate receptor) AE and anti-LGI1 (leucine-rich glioma inactivated) AE the most common Group II AE, with a reported incidence of 0.5/million and 0.4/million person-years, respectively, show an association with neoplasms in 30%-40% and in 10% of cases, respectively.

• #18 Autoimmune Encephalitis – EMCrit Project

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

  Autoimmune disorders: 25% of patients with autoimmune encephalitis may have an underlying autoimmune disorder. Family history of autoimmune disease may also increase risk. […] Epidemiology: Most commonly affects women (4:1 ratio) in their 20s-30s (although elderly people can be affected). An underlying neoplasm may be present: In half of affected women, the disease is triggered by an ovarian teratoma. Teratomas should be aggressively sought (e.g., via transvaginal ultrasonography +/- MRI). Teratomas may rarely be found elsewhere in the body (e.g., head, neck, thyroid, testis). Less commonly, other malignancies may be involved (e.g., lung or breast carcinoma). […] Infection may trigger the disorder: HSV encephalitis may precede the development of anti-NMDA receptor encephalitis. This may cause diagnostic confusion, as it may appear that the HSV encephalitis has simply relapsed. Anti-NMDA receptor encephalitis may arise following COVID infection.

• #19 Azthena logo with the word Azthena

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

  In northern regions, encephalitis is somewhat rare at an incidence of about 2-3 for every 100,000 per year. Of these, approximately 20% are due to autoimmune mechanisms, including mostly anti-NMDAR and VGKC-complex antibody-positive encephalitis. […] AIE affects both men and women equally and in all age groups. In 94% of patients with anti-NMDAR encephalitis, an ovarian teratoma was detected, but this also depends upon race, sex, and age. However, AIE caused by cell-surface antibodies is less likely to be due to underlying tumors than those due to onconeural antigens.

• #20 UH Enrolls First US Patient in International Phas 3 Clinical Trial for autoimmune Encephalitis | University Hospitals

  https://www.uhhospitals.org/for-clinicians/articles-and-news/articles/2023/06/uh-enrolls-first-us-patient-in-international-phas-3-clinical-trial-for-autoimmune-encephalitis

  Individuals of any age can be affected by AE. While paraneoplastic AE is more common in the elderly, idiopathic AE is diagnosed more frequently in young adults. A personal or family history of other autoimmune disorders is common. The prevalence rate of 13.7/100,000 is likely underestimated. […] However, epidemiological studies suggest that AE is becoming one of the leading causes of encephalitis and is increasingly recognized as a reason for neurological hospitalization and neuro-ICU admissions.

• #21 Autoimmune Encephalitis – EMCrit Project

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

  Autoimmune encephalitis refers to a group of disorders which vary along numerous dimensions, as shown in the table below. This includes disorders associated with malignancy (paraneoplastic encephalitides), as well as postinfectious and idiopathic disorders. […] Our understanding of autoimmune encephalitis has advanced enormously in the past two decades. It is currently estimated that autoimmune encephalitis is as common as viral encephalitis (although historically, most cases of autoimmune encephalitis have eluded accurate diagnosis). With the increasing use of checkpoint inhibitors for treatment of malignancy, it’s conceivable that autoimmune encephalitis could become the most common form of encephalitis. […] Malignancy: Paraneoplastic encephalitides should be considered in patients with known malignancy or who are at-risk for malignancy (especially small cell lung carcinoma). Highest risk is associated with patients receiving checkpoint inhibitors.

• #22 Epidemiology of Autoimmune Encephalitis – ProQuest

  https://www.proquest.com/scholarly-journals/epidemiology-autoimmune-encephalitis/docview/1999521432/se-2

  The recent increase in autoimmune encephalitis incidence during the past decade may be largely attributable to the discovery and use of diagnostic autoantibodies.

• #23 SciELO Brazil – A critical review and update on autoimmune encephalitis: understanding the alphabet soup A critical review and update on autoimmune encephalitis: understanding the alphabet soup

  https://www.scielo.br/j/anp/a/gXJNV7zDM4BPfxwd3LPpbxh/

  Autoimmune encephalitis is not rare: the paradigm shifts in comparison with infectious encephalitis. Data from England estimates that incidence of all types of encephalitis is 2.73 to 8.66 cases per 100,000 a year. Approximately 40-50% of encephalitis cases remain without an established diagnosis, even when patients undergo extensive testing. Until the discovery of neuronal cell surface antibodies, infection was believed to be the main known cause of encephalitis. However, following the characterization of anti-N-methyl-d-aspartate-receptor (NMDAR) antibodies in 2007, we learned that autoimmune etiology is not rare. The California Encephalitis Project showed that in young people individuals (aged 30), anti-NMDAR (N-methyl-d-aspartate-receptor) was the most common type of encephalitis, surpassing individual viral etiology. In the Netherlands, annual incidence of anti-LGI1 (leucine-rich, glioma-inactivated 1) encephalitis was 0.83 per million, similar to other neurological diseases such as Creutzfeldt-Jakob Disease and Lambert-Eaton myasthenic syndrome. In Olmsted County, Minnesota, USA, a recent population-based comparative study showed that prevalence and incidence of autoimmune encephalitis is comparable to infectious encephalitis (13.7/100,000 for autoimmune encephalitis and 11.6/100,000 for all infectious encephalitis). The same study also demonstrated that detection of autoimmune encephalitis is increasing over time: incidence increased from 0.4/100,000 person-years from 1995-2005 to 1.2/100,000 person-years from 2006-2015, attributable to increased detection of autoantibodies-positive cases. Growing awareness of autoimmune encephalitis has led to adaption of previously proposed criteria for encephalitis (any cause or idiopathic) that focused on infectious causes. Required diagnostic criteria include changes in level of consciousness, fever, CSF (cerebrospinal fluid) pleocytosis, Magnetic Resonance Imaging (MRI) and EEG changes. Nowadays, it is well established that patients with AE without fever can present with memory deficits or behavioral changes without a decreased level of consciousness or fever, and normal CSF (cerebrospinal fluid) examination and brain Magnetic Resonance Imaging (MRI). Autoimmune encephalitis can occur in individuals of all ages, some, such as anti-NMDAR (N-methyl-d-aspartate-receptor) encephalitis, predominantly affecting children and young adults. […] The same study also demonstrated that detection of autoimmune encephalitis is increasing over time: incidence increased from 0.4/100,000 person-years from 1995-2005 to 1.2/100,000 person-years from 2006-2015, attributable to increased detection of autoantibodies-positive cases.

• #24 Antibody-Positive Autoimmune Encephalitis and Paraneoplastic Neurological Syndrome: Epidemiology and Outcome of Neuronal Antibody Testing in Sweden

  https://www.svarlifescience.com/news/antibody-positive-autoimmune-encephalitis-and-paraneoplastic-neurological-syndrome-epidemiology-and-outcome-of-neuronal-antibody-testing-in-sweden

  According to the study, the incidence of AE and PNS in Sweden has doubled from 2015 to 2019. This likely reflects the increased availability of testing and awareness of these conditions. […] According to the study, there was a double increase in the incidence of AE and PNS during the study period. Additionally, the overall number of patients who underwent testing for neuronal antibodies in serum and CSF also increased. […] The study sheds light on the prevalence of AE and PNS in Sweden. It emphasizes the significance of accurate testing for neuronal antibodies and the limitations of commercially available kits. This underscores the need for increased awareness and correct application of currently available methods. Overall, the study highlights that accurate testing is crucial for diagnosing and treating AE and PNS.

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

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

  In future, we predict that unbiased surveys in patients with encephalitis will show that the growing range of autoimmune causes significantly exceed those of infectious causes in developed countries. […] This field is of major clinical importance to all neurologists because these patients present with a wide variety of neurological features and typically respond to immunotherapies.

• #26 Autoimmune encephalitis: Epidemiology, pathophysiology and clinical spectrum (part 1)

  http://www.scielo.org.za/scielo.php?script=sci_arttext&pid=S0256-95742023000300006

  Autoimmune encephalitis: Epidemiology, pathophysiology and clinical spectrum (part 1) […] A recent epidemiological study from the USA suggests that AE may occur much more commonly than previously thought. In this population-based study, a prevalence of 13.7/100 000 was found, which was not significantly different from the prevalence of infectious causes of encephalitis. In addition, the detection of AE has also increased over the years. […] In contrast to this, an older hospital-based study from the UK in 2010 showed infective causes of encephalitis to be twice as common as autoimmune causes, likely reflecting the rising awareness and diagnostic advances of the last decade and possibly predicting a trend of autoimmune causes overtaking infectious aetiologies in frequency in the future. […] Whereas AE cases have been described in South Africa, the epidemiology currently remains unreported.

• #27 Autoimmune Encephalitis (AE) – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525951/autoimmune-encephalitis-ae-epidemiology?srsltid=AfmBOoo4tzEbNVmlkWMznSqNLR25oO-hGJf0O4QZZLzYLBztpHrF_Ihx

  This „Autoimmune Encephalitis (AE) – Epidemiology Forecast to 2032” report delivers an in-depth understanding of the disease, historical and forecasted Autoimmune Encephalitis (AE) epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. […] The Autoimmune Encephalitis (AE) epidemiology report gives a thorough understanding of the Autoimmune Encephalitis (AE) by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. […] The Autoimmune Encephalitis (AE) epidemiology division provides insights about historical and current patient pool and forecasted trend for every seven major countries. […] The Autoimmune Encephalitis (AE) epidemiology segment covers the epidemiology data in the US, EU5 countries (Germany, Spain, Italy, France, and the UK), and Japan from 2019 to 2032.

• #28 Autoimmune Encephalitis (AE) – Epidemiology Forecast – 2032

  https://www.researchandmarkets.com/reports/5525951/autoimmune-encephalitis-ae-epidemiology?srsltid=AfmBOoo4tzEbNVmlkWMznSqNLR25oO-hGJf0O4QZZLzYLBztpHrF_Ihx

  The Autoimmune Encephalitis (AE) report also provides the epidemiology trends observed in the 7MM during the study period, along with the assumptions undertaken. […] The Autoimmune Encephalitis (AE) Epidemiology Report and Model provide an overview of the global trends of Autoimmune Encephalitis (AE) in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan). […] The report provides insight into the historical and forecasted patient pool of Autoimmune Encephalitis (AE) in seven major markets covering the United States, EU5 (Germany, Spain, France, Italy, UK), and Japan. […] The report assesses the disease risk and burden and highlights the unmet needs of Autoimmune Encephalitis (AE). […] The calculated data are presented with relevant tables and graphs to give a clear view of the epidemiology at first sight. […] The report helps recognize the growth opportunities in the 7MM for the patient population.

• #29 Autoimmune Encephalitis Market: Epidemiology, Therapies,

  https://www.openpr.com/news/3996152/autoimmune-encephalitis-market-epidemiology-therapies

  Autoimmune Encephalitis emerging therapies such as Satralizumab, GLOVENIN-I (TAK-961), and others are expected to boost the Autoimmune Encephalitis Market in the upcoming years. […] DelveInsight has launched a new report on „Autoimmune Encephalitis – Market Insights, Epidemiology, and Market Forecast-2034” that delivers an in-depth understanding of the Autoimmune Encephalitis, historical and forecasted epidemiology as well as the Autoimmune Encephalitis market trends in the United States, EU5 (Germany, Spain, Italy, France, and United Kingdom) and Japan. […] The market is expected to experience significant growth due to increased adoption of existing treatments, the introduction of new therapies, and heightened disease awareness. […] The United States holds the largest share of the Autoimmune Encephalitis market, surpassing EU4 (Germany, Spain, Italy, France), the United Kingdom, and Japan.

• #30 Autoimmune encephalitis: an observational study from South India | The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Full Text

  https://ejnpn.springeropen.com/articles/10.1186/s41983-023-00624-8

  Ever since AE was acknowledged as a potentially treatable cause of encephalitis, it has been increasingly recognised worldwide. […] The epidemiology of AE in India is unknown. This calls for an increased awareness of the varying clinical, laboratory, electrophysiological and radiological presentations of the different types of autoimmune encephalitis. […] Cross-sectional studies and several Ambi-spective cohorts from India are emerging showing the prevalence of this condition here. […] The present study detailed the demographic, clinical, imaging, laboratory and EEG characteristics of 31 AE from a tertiary centre in Chennai, India. The finding concurred with the literature and demonstrate the diverse spectrum of clinical manifestations of patients with AE present. Moreover considering the robust response to treatment, when the condition is identified and promptly initiated, it becomes pertinent to be aware of the local prevalence. Large prospective cohorts are required to completely assess the efficacy of the treatment, long-term outcomes and the requirement of additional therapy in patients with AE.

• #31 Autoimmune encephalitis: Paving the way to better outcomes – Mayo Clinic

  https://www.mayoclinic.org/medical-professionals/neurology-neurosurgery/news/autoimmune-encephalitis-paving-the-way-to-better-outcomes/mac-20523925

  Autoimmune encephalitis primarily affects people from their early teenage years to age 50, and women more often than men. […] In a groundbreaking population-based study published in the January 2018 issue of the Annals of Neurology, Mayo Clinic researchers reported that autoimmune encephalitis is as common as infectious encephalitis. […] Mayo Clinic Laboratories was among the first centers to offer an antibody panel to test for autoimmune encephalitis. […] Although autoimmune neurology diseases are rare overall, they’re not rare at Mayo Clinic. […] As a nationwide center, Mayo Clinic has the experience and expertise needed to manage patients with the most challenging autoimmune encephalitis. […] Mayo Clinic researchers, including specialists in the Center for Multiple Sclerosis and Autoimmune Neurology, also continue to study autoimmune encephalitis. […] Dubey D, et al. Autoimmune encephalopathy epidemiology and a comparison to infectious encephalitis. Annals of Neurology. 2018;83:166.

• #32 Importance, Definitions, History, Classification, and Frequency of the Autoimmune Encephalitides (Chapter 1) – Autoimmune Encephalitis and Related Disorders of the Nervous System

  https://www.cambridge.org/core/books/autoimmune-encephalitis-and-related-disorders-of-the-nervous-system/importance-definitions-history-classification-and-frequency-of-the-autoimmune-encephalitides/D3DA58459CFE145E14BE8B7CBC20CF78

  About 20 years ago the group of diseases currently known as autoimmune encephalitis or antibody-mediated encephalitis was unknown and the entire field of autoimmune neurology non-existent. […] Up to 12.6 per 100,000 persons are affected by encephalitis annually. Of these, it has been estimated that 20-30% are caused by autoimmune mechanisms. […] The most frequently used classifications combine information related to three features: mechanisms of disease (cytotoxic T cell or antibody-mediated mechanisms), type of antigen (intracellular vs cell surface), and presence or absence of a tumour. […] The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. […] Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis.

• #33 Adult-onset Anti-NMDA Receptor Encephalitis | Cleveland Clinic

  https://my.clevelandclinic.org/departments/neurological/depts/multiple-sclerosis/ms-approaches/adult-onset-anti-nmda-receptor-encephalitis

  Anti-NMDAr encephalitis is one of the most frequently identified neuronal autoantibodies in autoimmune encephalitis with an estimated annual incidence of ~1.5 cases per million persons. […] A contemporary study in Rochester, MN (USA) illustrated that the incidence and prevalence of autoimmune encephalitis (including cases of anti-NMDAr) are comparable to infectious encephalitis and the detection of these disorders has increased over time. […] A retrospective study identified neurological symptoms in 94% of patients with anti-NMDAr encephalitis. […] Early diagnosis and treatment is associated with a better long-term outcome in anti-NMDAr encephalitis. […] Given that serological and CSF autoantibody testing can take days to weeks to result, formal diagnostic criteria for probable anti-NMDAr encephalitis does not include autoantibody testing in order to expedite treatment. […] The NEOS score has been found to be more useful for predicting the rate of recovery than the ultimate extent of recovery. […] Predictive factors associated with a poor outcome include younger age of onset and persistent neuropsychiatric symptoms following the initial treatment of the disorder.

• #34

  https://journals.lww.com/neur/fulltext/2024/05000/prevalence_and_factor_associated_with.3.aspx

  Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a severe autoimmune encephalitis due to immune production of anti-NMDAR antibodies against the NR1 subunit of the NMDA receptor which is present throughout the central nervous system. This condition had been reported to be prevalent in patients with certain medical conditions; however so far, there have been limited systematic reviews and meta-analyses on the prevalence and factors associated. […] The overall pooled prevalence of anti-NMDAR encephalitis among patients with medical conditions was 7.0% (95% CI = 4.4, 9.6). […] We found that almost one-tenth of patients with medical conditions had anti-NMDAR encephalitis; particularly those with first episode of psychosis or schizophrenia were among the high-risk medical conditions.

• #35

  https://journals.lww.com/neur/fulltext/2024/05000/prevalence_and_factor_associated_with.3.aspx

  About one-tenth (10%) of patients with medical comorbidities had anti-NMDAR encephalitis, particularly among those suffering from herpes simplex infection, teratoma/malignancy and first episode of psychosis/schizophrenia. […] The present meta-analysis included 11 studies involving a large population size (n = 873,078,410). This is the first study looking into the pooled prevalence of anti-NMDAR encephalitis, which was 7.0% among patients with medical conditions. […] The prevalence of anti-NMDAR encephalitis among patients with the first episode of psychosis/schizophrenia was 9.6% with almost six folds increased risk of getting anti-NMDAR encephalitis (odds ratio of 5.976).

• #36 An Autopsy-proven Case-based Review of Autoimmune Encephalitis

  https://www.en-journal.org/journal/view.html?uid=650

  Roughly half of all AIE cases are seronegative. […] The diagnostic criteria for AIE can be dependent on the specific subtype. […] The concept of seronegative AIEs initially ignited a debate, which has recently progressed with the establishment of a diagnostic criteria. […] AIE covers a spectrum of disorders characterized by diverse neurological symptoms, making clinical differentiation historically challenging.

• #37 Detection of antibodies in suspected autoimmune encephalitis diseases using machine learning | Scientific Reports

  https://www.nature.com/articles/s41598-025-95815-z

  In about half of all cases, patients with AE exhibit detectable antibodies against structures of the central nervous system. […] Recent studies have concluded that the prevalence of AE may be almost as high as that of infectious encephalitis. […] The expertise required for diagnosis limits the ability of inexperienced clinicians to diagnose the condition. […] Due to the problems described regarding a reliable and rapid diagnosis of AE, new and improved analytical methods are of great interest. […] Our proof-of-concept study demonstrates that Radiomics-based machine learning can reliably predict serostatus in suspected autoimmune encephalitis (AE).

• #38

  https://link.springer.com/article/10.1007/s00234-024-03318-x

  The Graus criteria also allow for the diagnosis of AE in the absence of neuronal antibodies. Antibody-negative AE mostly presents with a limbic phenotype (seronegative definite LE). In addition, patients not fulfilling criteria for LE can be diagnosed in a specific subgroup named antibody-negative but probable AE (ANPRA), which remains heterogeneous and poorly defined. Finally, a category named possible AE exists in the Graus criteria, but it should be intended as a work-in-progress category identifying patients that should undergo a thorough work-up for a definite diagnosis, including CSF testing. Overall, the existence of seronegative AE cases is due to a number of reasons, including some Ab not being detected by commercial assays (but exclusively by means of in-house assays in reference centers), new AE-associated Ab being constantly discovered, and some cell-mediated AE potential lacking associated Ab.

• #39

  https://aesnet.org/abstractslisting/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. […] The age and sex standardized DALYs of autoimmune seizures and epilepsy was 41.13 per 100,000 population in 2019. […] Although autoimmune seizure/epilepsy is a rare condition, it is associated with considerable clinical morbidity.

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

  https://thejcn.com/DOIx.php?id=10.3988/jcn.2023.0242

  Autoimmune etiologies are a common cause for encephalitis. […] We found 146 studies that produced outcomes data. The mortality rates were 6%19% and the relapse risks were 10%62%. […] While we found that lasting impairments were common among survivors of autoimmune encephalitis, additional research is needed to better understand the nature and impact of these sequelae. Standardized evaluation protocols are needed to improve the ability to compare outcomes across studies, guide rehabilitation strategies, and inform outcomes of interest in treatment trials as the field advances. […] The mortality rate ranged from 6% to 19%, while the reported relapse risk was 10%38% with the exception of two series, one on LGI-1 encephalitis that reported a relapse rate of 52% and one of MOG encephalitis patients in which it was 62%. […] Most studies focused on global outcomes, with functional assessments restricted to mRS scores.

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

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

  Relapses were found to be more common in patients without a tumour, after delayed immunotherapy as well as high antibody titres. […] 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.

• #42 Nailing the Suspect: The Prevalence of Autoimmune Encephalitis Compared with Infectious Encephalitis – Insights

  https://news.mayocliniclabs.com/2018/04/29/nailing-the-suspect-the-prevalence-of-autoimmune-encephalitis-compared-with-infectious-encephalitis/

  In this epidemiology study, the researchers took several steps to avoid overestimating the frequency of autoimmune encephalitis. […] The prevalence that we report may actually be an underestimate. […] The study also found that autoimmune encephalitis has a higher risk of relapse or recurrent hospitalization than infectious encephalitis.

• #43

  https://link.springer.com/article/10.1007/s13311-020-00861-2

  Autoimmune encephalitis (AE) is a group of disorders causing synaptic receptor dysfunction with a broad range of neurological symptoms that has been historically difficult to differentiate clinically. […] In this review, we will provide an overview of the epidemiology of NMDAr-AE, clinical features and treatments, and the basic science tools and techniques that were used to identify the cause, correlate symptoms to underlying pathophysiology, and to understand the mechanism of disease pathology. […] Encephalitis has an overall prevalence worldwide ranging from 0.07 to 12.6/100 k persons and is cryptogenic at least 40% of the time. […] In the United States, encephalitis had an estimated prevalence of 7/100 k persons and accounted for nearly $2 billion dollars in hospital-based charges in 2010.

• #44 Abstract Details

  https://www.aan.com/MSA/Public/Events/AbstractDetails/58147

  To compare the epidemiology of autoimmune and infectious encephalitis cases in a large tertiary center during the recent decade. […] The incidence of autoimmune encephalitis (AIE) has risen in the last decade, yet recent studies evaluating the current epidemiology of AIE and comparing it to the epidemiology of infectious encephalitis are lacking. […] The incidence of AIE cases out of the yearly admitted cases increased substantially, from 3.8/100K in 2010 to 18.8/100K in 2020. […] AIE incidence has risen significantly in our institution during the past decade, with current rates comparable to those of all infectious causes combined. […] In light of its rising incidence and the importance of early treatment, AIE should be considered in the differential diagnosis of all encephalitis cases.

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

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

  Recent epidemiological studies suggest that AE is possibly as common as infectious encephalitis with an estimated prevalence rate of 13.7/100 000. […] The heterogeneity of AE presentation and findings on ancillary testing hinder large-scale clinical trials and limit the quality of evidence behind AE management. […] The objective of this paper is to evaluate available evidence for each step in AE management and provide expert opinion when evidence is lacking. […] The AEACN is comprised of self-identified clinicians with interest and clinical expertise in AE management listed by the AE Alliance, a non-profit organisation founded by AE patients and families to establish a supportive community for patients and caregivers, enhance clinical collaboration, and facilitate AE scientific research.

• #46

  https://link.springer.com/article/10.1007/s13311-020-00861-2

  Given the high burden of disease, investigations into the etiology of encephalitis have discovered that the incidence of autoimmune encephalitis to be at least as common as infectious causes based on two different population based studies, the California Encephalitis Project and Olmsted County, Minnesota. […] Based on retrospective reviews, NMDAr-AE is the most commonly identified cause of AE. […] It has a high frequency in the young and may represent 1% of all young adults admitted to the ICU. […] However, this is subject to debate as the incidence of AE is increasing rapidly, most likely due to more awareness and testing. […] One limitation to these epidemiological studies is that patients may not be captured as they may be detected and treated prior to formal testing and diagnosis of autoimmune encephalitis.

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