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March 29, 2023 | Written by Dr. Robb Wesselingh. Edited by Dr Mastura Monif, Dr Loretta Piccenna, Ms Tiffany Rushen, Ms Amanda Wells (consumer representative) Ms Sasha Ermichina (consumer representative), and Ms. Michelle Mykytowycz.
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A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of Peripheral monocytes and soluble biomarkers in autoimmune encephalitis. This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers, and their families.
You can find out more about the Australian Autoimmune Encephalitis Consortium and its efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Source: R Wesselingh, S Griffith, J Broadley, D Tarlinton, K Buzzard, U Seneviratne, H Butzkueven, TJ O’Brien, M Monif, Peripheral monocytes and soluble biomarkers in autoimmune encephalitis, Journal of Autoimmunity, 2023; 135 https://doi.org/10.1016/j.jaut.2023.103000
Autoimmune encephalitis (AE) is a condition in which inflammation occurs in various regions of the brain. In AE a person’s immune system produces antibodies (proteins) that mistakenly targets components of the person’s own neurons (nerve structures). This can result in inflammation and nerve tissue damage. As a result, a person with AE can present with different neurological symptoms including seizures (sudden, uncontrolled electrical disturbances in the brain) and memory problems. There are different types of AE based on which protein the immune system is mistakenly targeting. Two of the most common types of AE are:
While we know antibodies play a key role in the disease, we do not know what changes occur in other parts of the immune system during the course of AE.
The innate immune system is a part of the immune system that acts as a broad first line of defence against foreign invaders to the body like viruses and bacteria. This system can often start or increase inflammation in the body as a protective mechanism. Monocytes are a major type of cell in the innate immune system that drive this response. Monocytes can alert and activate other parts of the immune system through release of small signalling proteins. These small signalling proteins can be released into the blood and tissues and are called cytokines. In AE it is unknown whether the innate immune system or monocytes play a role in the disease.
For this research, we set out to find out answers to following –
We recruited 40 people with AE and 28 healthy volunteers who provided blood samples. These blood samples were evaluated in the laboratory for:
These findings were then compared between people with AE and the healthy volunteers to see if there were any differences. We also compared these findings between people with different types and severities of AE.
This research showed that there is ongoing inflammation in the blood of people with AE. Also, monocytes and the innate immune system may play a role in the disease.
The research could help clinicians to –
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For more information and resources from Dr. Monif and her group at the Australian Autoimmune Encephalitis Consortium Project, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
January 25, 2023 | Written by Dr. Hannah Ford. Edited by Dr Mastura Monif, Dr Loretta Piccenna, Ms Sarah Griffith, Ms Tiffany Rushen, Ms Amanda Wells (consumer representative) and Ms Sasha Ermichina (consumer representative).
A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of typical psychiatric manifestations of autoimmune encephalitis This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers, and their families.
You can find out more about the Australian Autoimmune Encephalitis Consortium and its efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Autoimmune encephalitis is a disorder in which antibodies accidentally created by the immune system attack parts of the brain. This can lead to inflammation and nerve damage.
Psychiatric problems are common in autoimmune encephalitis and can imitate mental health conditions, for example psychotic illnesses like schizophrenia. It is important to separate patients with AE from those with mental illness as treatments are very different.
There are different subtypes of AE. Some cases are due to the presence of detectable auto antibodies (a protein targeting the person’s own nerve endings) which is known as ‘sero positive’ AE. In ‘sero negative’ AE, there is no detectable antibody when using currently available techniques for detection.
Within the ‘sero positive’ group are different AE categories depending on the type of antibody. We discuss this further in the next section.
Table 1.
Red Flags for Autoimmune Encephalitis in Psychiatric Presentations |
· Preceding physical symptoms such as fever, headache, stomach upset and dizziness · Seizures · Neurologic symptoms such as abnormal movements, speech difficulties, clumsiness, weakness and changes in sensation · “Catatonic” features such as abnormal posturing, repeating another person’s speech (echolalia), lack of movement or erratic movements · Memory problems · Psychotic symptoms that start rapidly and/or worsen quickly
|
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For more information and resources from Dr. Monif and her group at the Australian Autoimmune Encephalitis Consortium Project, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
October 28, 2022 | Written by Dr. Nabil Seery. Edited by Dr Mastura Monif, Ms Tiffany Rushen, Dr Loretta Piccenna, Ms Amanda Wells (consumer representative) and Ms Sasha Ermichina (consumer representative).
A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of how autoimmune encephalitis can affect cognitive abilities. This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers and their families. This blog has been facilitated by IAES Support Services coordinator Mari Wagner Davis, with input from IAES volunteers Sasha Ermichina (impacted by GFAP AE) and Amanda Wells (caregiver for her daughter with AE). These IAES representatives provide input from their unique perspectives, helping to educate researchers in the difficulties that patients and families face.
You can find out more about the Australian Autoimmune Encephalitis Consortium and their efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Source: Seery N, Butzkueven H, O’Brien TJ, Monif. M. Rare Antibody-Mediated and Seronegative Autoimmune Encephalitis: an Update. Autoimmunity Rev. 2022 May 18;21(7);103118. https://doi.org/10.1016/j.autrev.2022.103118
■ Autoimmune encephalitis (AE) is a form of autoimmune disease whereby immune cells in the body inappropriately target components of the nervous system. This causes dysfunction of nerve cells, and in some cases death of these cells, and further produces different clinical symptoms that are reversible. Such symptoms include (but are not limited to) cognitive symptoms, such as difficulties with memory and language, seizures, movement disorders, and psychiatric symptoms.
■ Antibodies are central to the diagnosis of many subtypes of autoimmune encephalitis. Generally, antibodies are proteins produced by the immune system to fight infections. In a proportion of patients with autoimmune encephalitis there can be an abnormal expression of antibodies, where, rather than targeting foreign molecules (e.g. viruses, bacteria), they mistakenly target self-proteins on nerve endings or self-proteins inside the nerve cell or neuron. In up to half of cases, an antibody is not detectable using current available tests or assays. This group of cases is called “seronegative” autoimmune encephalitis, i.e. denoting a lack of antibodies in the serum (a component of a patient’s blood) or cerebrospinal fluid (a clear fluid the surrounds the brain and spinal cord, obtained via a lumbar puncture, a procedure involving a fine needle being inserted in the lower back). ‘Seronegative’ autoimmune encephalitis most likely represents a broader collection of disorders.
■ Over the last two decades, antibody-mediated subtypes of autoimmune encephalitis continue to be discovered, with over ten such forms now recognised. Further, following the respective discovery of such new forms of autoimmune encephalitis, disease mechanisms and clinical features have been revealed. However, seronegative autoimmune encephalitis remains less well characterised, possibly in part to because of its heterogeneous nature – meaning that a variety of diseases forms may be included by the definition.
■ The purpose of our review was to explore advances regarding five rare antibody-mediated forms of autoimmune encephalitis, namely, anti-g-aminobutyric acid B (GABAB) receptor-, anti-a-amino-3hydroxy-5-methyl-4-isoxazolepropinoic receptor- (AMPAR), anti-GABAA receptor-and anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis and IgLON5 disease.
■ We also summarise current research and challenges in relation to ‘seronegative’ autoimmune encephalitis. For a detailed discussion of anti- NMDA autoimmune encephalitis, anti-LGI1 and anti-CASPR2 autoimmune encephalitis refer to (Contemporary advances in anti-NMDAR antibody (Ab)-mediated encephalitis -PubMed (nih.gov) (1) and Contemporary advances in antibody-mediated encephalitis: anti-LGI1 and anti-Caspr2 antibody (Ab)-mediated encephalitides -PubMed (nih.gov)) (2).
■ GABAB, AMPAR and GABAA autoimmune encephalitis have common and distinguishing clinical features. These three forms of autoimmune encephalitis are diagnosed by the presence of antibodies found in the blood or cerebrospinal fluid of suspected patients. All three are relatively rare, compared to some other antibody-mediated forms of autoimmune encephalitis such as anti-N-methyl-D-aspartate receptor (NMDAR) and anti-leucine-rich gliomainactivated 1 (LGI1) Ab-mediated encephalitis. GABAA encephalitis in particular is exceedingly rare, with approximately fifty cases reported overall as at a few years ago.
■In these diseases, antibodies target the GABAB, AMPAR and GABAA receptors (proteins present on nerve cell endings), causing neuronal dysfunction. GABAB and GABAA receptors both attract an inhibitory neurotransmitter called GABA. A neurotransmitter is a signalling molecule that helps with communication and transmission of impulses between neurons, and inhibitory neurotransmitters reduce the likelihood a given neuron will generate an electrical signal called an action potential.
■ Seizures in these diseases are a main feature, and may be particularly non-responsive to conventional anti-seizure treatment. Furthermore, cognitive and psychiatric symptoms are common in all three of these subtypes of autoimmune encephalitis. GABAB and AMPAR subtypes may have similar findings identified on MRI imaging of the brain, with inflammation and swelling seen in part of the brain called the mesial temporal lobe. The mesial temporal lobe is an area of the brain important for memory, emotion and behaviour.
■ The diagnosis of autoimmune encephalitis invariably necessitates that clinicians investigate for the possibility of a tumour (e.g. lung cancer, thyroid cancer, breast cancer) that may have triggered the disease. Treating the tumour or cancer where feasible and as promptly as possible has been linked to improvements in autoimmune encephalitis symptoms. Similarly, the presence of neurological symptoms, if preceding a cancer diagnosis, may allow for this to be facilitated more quickly than might have been the case otherwise, which may help afford a better chance of more effectively treating the underlying cancer.
■ In approximately half of patients diagnosed with GABAB encephalitis, an underlying tumour is found, most often small-cell lung cancer. In AMPAR encephalitis, almost two-thirds of patients have an underlying tumour, with thymus tumours and lung cancer most common. In GABAA encephalitis, approximately one third of patients have also been shown to have an underlying tumour.
■ DPPX encephalitis and IgLON5 disease are two rare and somewhat clinically unique forms of autoimmune encephalitis. In DPPX encephalitis, patients commonly present with profound weight loss or diarrhoea and have features of central-nervous system hyperexcitability. This is a state where the brain has increased responsiveness to a variety of external stimuli. In DPPX encephalitis, features attributed to CNS hyperexcitability include myoclonus, or rapid, involuntary muscle jerks, and tremor. IgLON5 disease on the other hand also has unique clinical features, such as a variety of sleep disturbances.
■ Seronegative autoimmune encephalitis overall requires further study and description to identify potential antibodies which may be the cause. Seronegative limbic encephalitis is a form of seronegative autoimmune encephalitis, where the limbic structures in the brain are affected. In this subset of the disease inflammation is observed in the mesial temporal lobes using Magnetic Resonance Imaging (MRI). Seronegative limbic encephalitis is typically seen in older patients, with conventional antibody testing not revealing an antibody. Patients typically have memory impairment, with or without psychiatric symptoms and seizures, and are treated with medications that lower effects of the immune system, as in other forms of autoimmune encephalitis.
■ These findings are intended to help researchers and clinicians better understand seronegative and rare forms of autoimmune encephalitis. By bringing this information together, it can assist with improving diagnosis and assisting with early treatment by clinicians.
■ It should be noted that antibody-related forms of autoimmune encephalitis are usually diagnosed as “possible autoimmune encephalitis” prior to the availability of antibody results, which can take up to a period of weeks. A diagnosis of autoimmune encephalitis is based on broad criteria involving consideration of a patient’s symptoms and test results, including MRI, electroencephalogram (EEG – a measure of the electrical activity of the brain) and cerebrospinal fluid biopsy results, combined with the exclusion other diseases, for example, viruses that could mimic the observed symptoms.
■ Prompt diagnosis of autoimmune encephalitis, and prompt exclusion of other causes such as viral encephalitis is very important, as there is a growing body of evidence indicating that earlier initiation of immune-lowering treatment for autoimmune encephalitis may be able to facilitate better recovery.
■ The seronegative form of autoimmune encephalitis can represent a large proportion of autoimmune encephalitis patients overall so its understanding is crucial for improvements in clinical care.
■ Regarding very rare subtypes of autoimmune encephalitis, an understanding of the characteristic features of these rare entities is crucial in forming a diagnostic workup plan. Further, awareness of the features of some of these rarer subtypes can ensure prompt and accurate investigation of underlying tumours. Knowledge of rarer subtypes may also be able to inform clinicians and patients about the possible outcomes of these conditions to inform day to day discussions with patients and their caregivers.
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For more information and resources from Dr. Monif and her group at the Australian Autoimmune Encephalitis Consortium Project, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
October 12, 2022 | Written by Dr. Robb Wesselingh. Edited by Dr Mastura Monif, Ms Tiffany Rushen, Dr Loretta Piccenna, Ms Amanda Wells (consumer representative) and Ms Sasha Ermichina (consumer representative).
A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of how autoimmune encephalitis can affect cognitive abilities. This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers and their families. This blog has been facilitated by IAES Support Services coordinator Mari Wagner Davis, with input from IAES volunteers Sasha Ermichina (impacted by GFAP AE) and Amanda Wells (caregiver for her daughter with AE). These IAES representatives provide input from their unique perspectives, helping to educate researchers in the difficulties that patients and families face.
You can find out more about the Australian Autoimmune Encephalitis Consortium and their efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Publication:
Source – Wesselingh, R., Broadley, J., Buzzard, K., Tarlinton, D., Seneviratne, U., Kyndt, C., Stankovich, J., Sanlippo, P., Nesbitt, C., D’Souza, W., Macdonell, R., Butzkueven, H., O’Brien, T. J., & Monif, M. (2022). Prevalence, risk factors, and prognosis of drugresistant epilepsy in autoimmune encephalitis. Epilepsy & behavior: E&B, 132, 108729. Advance online publication. https://doi.org/10.1016/j.yebeh.2022.108729
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Seizures (or sudden, uncontrolled electrical disturbances in the brain) are a common initial neurological symptom that occurs in people with autoimmune encephalitis. In autoimmune encephalitis a person’s immune system mistakenly targets different proteins in their brain causing damage and inflammation. For some people, the seizures can progress to very severe and ongoing seizures called status epilepticus, requiring treatment to stop them happening. While some patients will stop having seizures after immune system suppressing treatment, others will continue to have seizures that do not respond, even to increasing amounts of anti-seizure medications. This is known clinically as treatment- or drug-resistant epilepsy. Drug-resistant epilepsy has a significant impact on the quality of life of people with autoimmune encephalitis. We currently do not know why some patients with autoimmune encephalitis develop drug-resistant epilepsy whilst others do not.
It is important for doctors to be able to predict how and why people with autoimmune encephalitis develop drug-resistant epilepsy because it is a disabling complication that may be preventable. For this research, we wanted to find out answers to following questions –
We looked through the medical records of seven hospitals in Victoria (Australia) for people who met the diagnosis of autoimmune encephalitis and had an EEG when they first became unwell. Two hundred and eight patients were identified and selected for analysis. We then collected available data from 69 patients of their symptoms, seizures, treatment, and whether they developed drug-resistant epilepsy at 12 months after their initial illness.
We analysed EEGs from patients to find any brain wave irregularities or signatures (called EEG biomarkers) that were more common in those with autoimmune encephalitis who developed drug-resistant epilepsy than those that did not develop drug-resistant epilepsy. Finally, we combined all the factors and created a tool that doctors can use to predict an individual’s risk of developing drug-resistant epilepsy after autoimmune encephalitis.
The research could help clinicians to –
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For more information and resources from Dr. Monif and her group at the Australian Autoimmune Encephalitis Consortium Project, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
August 31, 2022 | Written by Sarah Griffith. Edited by Dr Mastura Monif, Ms Tiffany Rushen, Dr Loretta Piccenna, Ms Amanda Wells (consumer representative) and Ms Sasha Ermichina (consumer representative)
A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of how autoimmune encephalitis can affect cognitive abilities. This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers and their families. This blog has been facilitated by IAES Support Services coordinator Mari Wagner Davis, with input from IAES volunteers Sasha Ermichina (impacted by GFAP AE) and Amanda Wells (caregiver for her daughter with AE). These IAES representatives provide input from their unique perspectives, helping to educate researchers in the difficulties that patients and families face.
You can find out more about the Australian Autoimmune Encephalitis Consortium and their efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Source – Griffith, S., Wesselingh, R., Broadley, J., O’Shea, M., Kyndt, C., Meade, C., Long, B., Seneviratne, U., Reidy, N., Bourke, R., Buzzard, K., D’Souza, W., Macdonell, R., Brodtmann, A., Butzkueven, H., O’Brien, T. J., Alpitsis, R., Malpas, C. B., Monif, M., & Australian Autoimmune Encephalitis Consortium (2022). Psychometric deficits in autoimmune encephalitis: A retrospective study from the Australian Autoimmune Encephalitis Consortium. European journal of neurology, 10.1111/ene.15367. Advance online publication. https://doi.org/10.1111/ene.15367
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Autoimmune Encephalitis is a rare disease that affects different regions of the brain. Patients with this condition can have a variety of cognitive symptoms (for example, memory deficits, slow speed of information processing, attention lapses, word finding difficulties, trouble with following complex commands, difficulties in judgement, difficulties in comprehension of complex tasks, difficulties in forming new memories, and difficulties in understanding tasks or situations). These symptoms can change from one individual to another individual, and can fluctuate over the course of the illness. Also different types of autoimmune encephalitis can have differing presentation of the above symptoms.
Historically (and to this day) clinicians and researchers have used a scale called the modified Rankin Score as a tool to monitor an individual’s function – i.e. how independently they can function in their day to day life. However, this scale is very inaccurate and it does not capture complex and specific issues that might be troublesome for the patient. The modified Rankin Scale also does not reflect the gravity of the individual’s symptoms and does not provide in detail information about specific symptoms and complaints that the individual with autoimmune encephalitis might be experiencing. Importantly, the scale does not measure behavioural, mood or cognitive outcomes in autoimmune encephalitis. Cognitive changes can be associated with long term disease related morbidity and can reduce quality of life. Therefore we set out to gain a better understanding of the cognitive difficulties in autoimmune encephalitis.
What did the researchers do?
We gathered cognitive data from patients previously diagnosed with autoimmune encephalitis (retrospective data) from six hospitals in Victoria (Australia) to inform the analysis. Patients were identified retrospectively through medical records with a search for diagnosis of autoimmune encephalitis with a hospital admission between July 2008 and July 2019. Patients who met this criteria participated in a neuropsychology assessment of their cognitive function (i.e., memory, attention, language, judgement, planning, comprehension, and recall. We collected clinical data about each patient and also information of various clinical investigations (i.e. lumbar puncture results and MRI).
The average age of patients at diagnosis was 49 years old and more than half showing as seropositive autoimmune encephalitis (meaning they had identifiable antibodies in their blood or their cerebrospinal fluid). Of the seropositive group:
Forty two percent of patients had impairments on executive function tests (e.g. a set of tasks that includes working memory, planning, flexible thinking, and ability to remember multiple instructions, and capacity for self-regulation and ordering or prioritizing of tasks). The next most common impairment was on memory tests (e.g. the mental processes involved in acquiring, retaining and recalling information; 40.7% of patients).
For the first-time, we found 29 patterns of cognition among patients with autoimmune encephalitis in our analysis. The four most common patterns of cognition were:
But, given we found 29 patterns means that cognitive outcomes in patients with autoimmune encephalitis are complex and need further detailed investigation.
Our research highlighted that more detailed and systematic analysis of memory and executive function profiles in patients with autoimmune encephalitis is required. Impairments in memory and executive dysfunction can have huge implications for the patient and their caregivers, and tools that would better characterise and follow these symptoms in autoimmune encephalitis are needed. Understanding memory and executive function impairment in autoimmune encephalitis can help us in devising strategies that would assist patients with day to day function, but also monitor disease trajectory over time and delineate patient’s response to treatment.
We could not predict good cognitive outcome (e.g. having ‘intact’ cognition after autoimmune encephalitis) in patients. We recommend clinicians provide ongoing comprehensive cognitive monitoring in patients with autoimmune encephalitis, and reactive intervention when required. An individualised approach will assist in the management the long-term morbidity of this disease, to minimise the effect on the individual’s quality of life and any damaging psychological outcomes.
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To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
July 27, 2022 | by Dr. Robb Wesselingh
A message from IAES Blog Staff:
It is our honor & pleasure to present to all of you an overview of the use of an Electroencephalogram or EEG for diagnosis and prediction in the treatment of Autoimmune Encephalitis by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif. The International Autoimmune Encephalitis Society is proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project. Dr. Monif is on the board of directors for IAES and we work closely with them to best support AE patients, caregivers and their families. You can find out more about the team and their efforts to help those with AE and their families via the following link:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Publication:
R Wesselingh, J Broadley, K Buzzard, D Tarlinton, U Seneviratne, C Kyndt, J Stankovich, P Sanfilippo, C Nesbitt, W D’Souza, R Macdonell, H Butzkueven, TJ O’Brien, M Monif, Electroclinical biomarkers of autoimmune encephalitis, Epilepsy & Behaviour, 2022;128: 108571. https://doi.org/10.1016/j.yebeh.2022.108571
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Autoimmune encephalitis (AE) is a brain inflammation disorder caused by antibodies. A person’s immune system mistakenly targets different proteins in their brain causing damage and inflammation. This can result in different neurological symptoms including seizures (sudden, uncontrolled electrical disturbances in the brain) and memory problems. Autoimmune encephalitis can be classified into different subtypes based on the brain protein targeted by the antibodies produced. The most common subtypes are anti-NMDAR autoimmune encephalitis, anti-LGI-1 autoimmune encephalitis and seronegative autoimmune encephalitis (in which there is no identified antibody). While treatment is effective and available, the diagnosis of autoimmune encephalitis is not straightforward. Also, knowing which patients need more intensive treatment is tricky.
Patients thought to have autoimmune encephalitis usually have a few clinical tests to confirm the diagnosis. They include brain magnetic resonance imaging (MRI), an electroencephalogram (EEG), and blood or cerebrospinal fluid tests to analyse the presence of inflammation. The EEG is a procedure that measures brain electrical activity (brain waves) by using electrodes placed on the scalp. It can show different patterns or irregularities depending on the person’s health state. For example, an EEG can show seizure activity, or it can indicate drowsy or comatose states. In some situations, it can also show very subtle changes that could be useful in our understanding of autoimmune encephalitis and guiding management. It is important for patients with suspected autoimmune encephalitis to have a diagnosis as soon as possible because earlier treatment leads to better long-term recovery. But doing multiple clinical tests takes time, some can be invasive or may only be available in certain centres. For this research, we wanted to find out answers to following –
We looked through the medical records of seven hospitals in Victoria, Australia for people who had possible autoimmune encephalitis and had an EEG when they first became unwell. Overall, 208 patients were identified and selected for our analysis. We collected data from 131 patients of their symptoms, seizures, treatment, and their ability to return to normal day-to-day living. Key clinical characteristics of the patients can be seen below:
We analysed EEGs from patients to find any brain wave irregularities or signatures (called biomarkers) that would show different subtypes of autoimmune encephalitis. Other EEGs were analysed that could predict which patients might have impaired functional outcomes in the long term.
The brain wave signatures or biomarkers we identified can be useful for clinicians to recognise and use in practice as part of diagnosis and provide targeted treatment.
The research could help clinicians to –
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For more information and resources on anti-NMDAr encephalitis, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
May 11, 2022 | by Seery N, Butzkueven H, O’Brien TJ, Monif M.
A message from IAES Blog Staff:
It is our honor & pleasure to present to all of you an overview of anti-NMDAR Autoimmune Encephalitis by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif. The International Autoimmune Encephalitis Society is proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project. Dr. Monif is on the board of directors for IAES and we work closely with them to best support AE patients, caregivers and their families. You can find out more about the team and their efforts to help those with AE and their families via the following link: https://www.monash.edu/medicine/autoimmune-encephalitis
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Contemporary advances in anti-NMDAR antibody (Ab)-mediated encephalitis, Autoimmunity Reviews, April 2022, Volume 21, Issue 4, https://doi.org/10.1016/j.autrev.2022.103057
For more information and resources on anti-NMDAr encephalitis, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society cannot provide medical advice.
International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.
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