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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.
Figure 1: This figure shows a summary of our findings with 208 patients with autoimmune encephalitis, 16% had severe form of seizures (SE; status epilepticus), 75% of patients had 1 or more seizures, and 25% did not have seizures at their initial admission. Then after 12 months follow up, 16% of patients who completed follow up, had DRE (drug resistant epilepsy), and 33% of the patients were on anti-seizure medications (ASM) and 48% did not require ASMs.
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. For this interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
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 –
—-
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. For this interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
April-29-2020 | Claudia Lopez Lloreda, PennNeuroKnow
Seizures can be scary events both for people who suffer from them and for their loved ones. Symptoms of a seizure typically include muscle spasms; loss of consciousness; sudden, rapid eye movements; or sudden mood changes; among other symptoms, and these can last from seconds to minutes1. These are the most severe seizures, but mild seizures, with more moderate physical and behavioral symptoms — such as stiffness of the muscles, feelings of déjà vu, anxiety, temporary confusion, or nausea — can also happen and may negatively affect health. During seizures, the body parallels what is happening in the brain: uncontrolled movements of the body can result from uncontrolled bursts of electrical activity in the brain.
Seizures are a response to hyperexcitability, meaning increased activity, of neurons in the brain, and hypersynchrony, meaning more neurons fire at the same time than normal. Seizures are very different across and within conditions. They can be generalized, affecting the entire brain from the beginning of the seizure, or focal, affecting one specific area although it may later spread. Frequent, unprovoked seizures called recurrent seizures may indicate that the person has a condition called epilepsy. Epilepsy is a chronic neurological disorder in which seizures can cause periods of unusual behavior, sensations, and negative effects on cognition such as a loss of awareness. However, because abnormal electrical activity can happen in response to other alterations in the brain such as brain injury and in response to medications, seizures can also be seen in other conditions.
One of these conditions is autoimmune encephalitis (AE). In AE, the body attacks the brain by creating antibodies against important neuronal proteins. Because these proteins help neurons communicate, the antibodies alter neuronal activity. Altering neuronal activation can lead to the changes that are seen in seizures (hyperexcitability and hypersynchrony). In fact, research shows that seizures in some patients can be a common symptom during the acute phase (early on in disease) of AE2. It is believed that antibodies against the neuronal proteins contribute directly to the disease processes and the development of seizures. It’s also possible that the process of neuroinflammation associated with AE, which increases the amount of toxic inflammatory molecules in the brain, can also contribute to the development of seizures2. Even once the inflammation has been resolved, the brain can still be predisposed to seizures or developing epilepsy, especially if the inflammation resulted in neuronal death3. However, whether epilepsy, a chronic disease, is developed in response to AE is not entirely clear. Some studies suggest that the risk of developing chronic epilepsy is low, from 10-15%4.
In different types of AE, seizures appear differently. Apart from the well-known tonic-clonic seizures (associated with jerking muscle movements), seizures in AE can also show up as faciobrachial dystonic seizures. These are characterized by abrupt involuntary movements, typically on one half of the face and arm of the same side. The frequency, response to therapies, and symptoms of the seizures themselves can all vary. However, the AE that most frequently manifest with seizures and chronic epilepsy are those mediated by antibodies against the LGI1, GABABR, and GABAAR; all-important proteins involved in neuronal communication5.
Antiepileptic drugs are the standard of care for people with epilepsy. Since seizures are a result of uncontrolled electrical activity and an imbalance of excitation and inhibition in the brain, antiepileptic drugs work by trying to restore that balance. For example, the drug clonazepam prevents seizures by increasing the effectiveness of a molecule in the brain called GABA, which helps the brain dampen the uncontrolled brain activity.
Now, although the normal path for people with epilepsy is treatment with antiepileptic drugs, it may not be particularly effective for people with seizures associated with AE. A study looking at a population of AE patients found that resolution of seizures happened even after discontinued antiepileptic drugs therapy6. In these young patients with AE who experienced unprovoked seizures at the onset of the disease there was a remission rate of 94%, meaning they stopped suffering from seizures, after they stopped taking antiepileptic drugs. Rather, immunotherapy seemed to be the important factor in controlling seizures. The researchers suggested that “long-term use of antiepileptic drugs appears not to be necessary to control seizures in AE”6.
Other studies support the idea that immunotherapy is more effective in attacking seizures in AE. One study looked at three different types of autoimmune encephalitis (anti-LGI1, anti-NMDAR, and anti-GABABR) and their response to immunotherapy and antiepileptic drugs7. They found that seizure freedom was achieved faster and more frequently after the use of immunotherapy than after the use of antiepileptic drugs. However, there may be a specific window in which immunotherapy is effective at controlling seizures.
Importantly, the researchers do mention that differences in seizures characteristics and therefore response to treatment may be due to the specific type of encephalitis. For example, patients with anti-GABABR encephalitis had an increased risk of developing seizures, meaning that the development of seizures may depend on the type of encephalitis7.
These differences in treatment response between AE and epilepsy point to an important trait that needs to be considered: the cause of seizures. In AE, antibodies generated against important neuronal proteins make the brain go awry. Therefore, one of the most effective ways to treat seizures may be attacking the root of the problem with immunotherapy. However, due to the variable nature of AE and the seizures associated with the condition, proper treatment with immunotherapy and/or antiepileptic medication will change from patient to patient.
During the most severe seizures, the person may not be able to control their body movements. For this reason, you may help them clear the area around them to prevent possible injury. If possible, place them on their side and provide cushioning for their head. There are additional indications suggested by the Center for Disease Control (become familiar with these here).
Become an Advocate by sharing your story. It may result in an 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
References
Cover Image from Pixabay: https://pixabay.com/illustrations/epilepsy-seizure-stroke-headache-623346/
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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