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The Unique Nature of Seizures in Autoimmune Encephalitis

The Unique Nature of Seizures in Autoimmune Encephalitis

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April-29-2020 | Claudia Lopez Lloreda, PennNeuroKnow 

IAES PNK Partnership logo 300x251 - The Unique Nature of Seizures in Autoimmune EncephalitisWhat are seizures?

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.


Are seizures associated with AE treated the same way as in epilepsy?


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.


What do these findings mean for people with AE?


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.


What to do if someone is having a seizure?


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).

Seizures in AE Handout 


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  1. Epilepsy. (2019, August 10). Retrieved March 6, 2020, from
  2. Rana, A., & Musto, A. E. (2018). The role of inflammation in the development of epilepsy. Journal of Neuroinflammation, 15(1). doi: 10.1186/s12974-018-1192-7
  3. Vezzani, A., Fujinami, R. S., White, H. S., Preux, P.-M., Blümcke, I., Sander, J. W., & Löscher, W. (2015). Infections, inflammation and epilepsy. Acta Neuropathologica, 131(2), 211–234. doi: 10.1007/s00401-015-1481-5
  4. Steriade, C., Moosa, A. N., Hantus, S., Prayson, R. A., Alexopoulos, A., & Rae-Grant, A. (2018). Electroclinical features of seizures associated with autoimmune encephalitis. Seizure, 60, 198–204. doi: 10.1016/j.seizure.2018.06.021
  5. Spatola, M., & Dalmau, J. (2017). Seizures and risk of epilepsy in autoimmune and other inflammatory encephalitis. Current Opinion in Neurology, 30(3), 345–353. doi: 10.1097/wco.0000000000000449
  6.  Huang, Q., Ma, M., Wei, X., Liao, Y., Qi, H., Wu, Y., & Wu, Y. (2019). Characteristics of Seizure and Antiepileptic Drug Utilization in Outpatients with Autoimmune Encephalitis. Frontiers in Neurology, 9. doi: 10.3389/fneur.2018.01136
  7. Bruijn, M. A. D., Sonderen, A. V., Coevorden-Hameete, M. H. V., Bastiaansen, A. E., Schreurs, M. W., Rouhl, R. P., … Titulaer, M. J. (2019). Evaluation of seizure treatment in anti-LGI1, anti-NMDAR, and anti-GABABR encephalitis. Neurology, 92(19). doi: 10.1212/wnl.0000000000007475


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KETO Diet: What is it? Who is it for? And why?

KETO Diet: What is it? Who is it for? And why?

Keto Diet Blog FB post - KETO Diet: What is it? Who is it for? And why?January-28-2020 | Daria Muir, M.D.

It is important that we all stay well informed. When a person receives a diagnosis of autoimmune encephalitis, they want to do whatever they can to improve their health. Some will reach for what appears to them to be a positive action and decide to change their diet. This can have very negative unintended consequences. To ensure your safety, and in IAES’ commitment to providing accurate information to assist you in being well informed on your road to recovery, today I’m going to give you a simple explanation about the Keto Diet.

What is it?


KETO is a diet that excludes carbohydrates completely. Basically, you eat just proteins and fats. Carbohydrates are the main fuel generator for the body (and especially the brain). They transform into glucose (the fuel). By switching to the KETO diet, one leaves the body without the fuel source. So, the body learns to turn other stuff into fuel. In this case, mainly fat. But also, the muscles. That’s what we call “putting the body into a state ok Ketosis). Is this good? Is it bad? For some people it is good. For some it is bad. For some it is an unnecessary risk, meaning there is no proof whatsoever that it helps, but there is proof that it can do some damage.

Who is a candidate for the Keto Diet?


Keto diet is recommended in a small number of diseases:

– severe Epilepsy, irresponsive to golden standard medication (being more efficient in children);

– Glucose Transporter Type 1 Deficiency Syndrome (a genetical disease that does not allow the body to produce GluT1 , so the brain does not get glucose- fuel. The ONLY treatment in this situation is keto diet!);

– it is also used by some nutritionists with patients that need to lose weight.

!!!! It needs to be assessed, recommended and monitored by a doctor!!!


There is no proof that the KETO Diet works in Autoimmune Encephalitis. (It can be recommended in severe Epilepsy post-Acquired Brain Injury in AE, but it does not treat AE, it treats the Epilepsy resulted from a brain injury from AE. As you know by now, Epilepsy and Seizures are not the same things. In AE we may have seizures, due to brain swelling and antibodies attacking our healthy brain cell. This is why we need immunosuppressant/ immunomodulatory treatment to control the disease.

Epilepsy is a disease resulted from the brain being injured and scarred.

Epilepsy can happen in AE but having seizures does not mean you have Epilepsy! Your doctor needs to asses that and tell you that you acquired Epilepsy!

Why? And why not the KETO Diet?


Now that you understand who an appropriate candidate for the Keto Diet would be, let’s discuss what can happen if you try the diet on your own without discussing it with your doctor.

Why does the Keto Diet need to be recommended by your doctor and monitored all the time?


Because it can be dangerous! Yes, it can be.

For diabetics. Also, people with kidney disease or who take medication that increases the risk of Kidney disease. For example, It can lead to major weight loss, below the normal BMI, that can endanger hormonal balance. It can increase pressure on the liver and kidney and eventually eats up your muscles too. The body can enter into ketosis (dangerous for some people).

KETO Diet is not easy, not harmless, not for anybody and not proven effective in AE.


Your doctor needs to assess and recommend that for you or for your child because it can be dangerous and can cause harm. If you have the conditions stated at point 2, then you might benefit from a very well supervised KETO Diet. Always ask your doctor! And please, don’t recommend it to other people, because it can harm them! Our concern is your safety and sharing accurate information for your best overall health.


Ketogenic Diet and Epilepsy: What We Know So Far

Ketogenic Diet in Patients with GLUT1 Deficiency Syndrome 

Ketogenic Diet 


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After 25 Years, a Proper Diagnosis-Autoimmune Encephalitis!

After 25 Years, a Proper Diagnosis-Autoimmune Encephalitis!

October 30-2019 | Lauren Mabry

lauren Mabry 1 300x225 - After 25 Years, a Proper Diagnosis-Autoimmune Encephalitis!In 1994 when I was 9 years old I was diagnosed with Epilepsy with partial complex seizures after my mom witnessed a bizarre event where my arm jerked wildly and I fell to the ground. These events occurred multiple times and I underwent EEG testing, MRI, and all the tests came back normal. We tried a few anti-epileptic drugs and eventually found one that seemed to stop the episodes. Fast-forward a few years and I started to have breakthrough seizures…

This became the routine: find a drug that worked, maybe have a breakthrough seizure, up the dose, breakthrough, worry about having a breakthrough, or maybe go years and be fine.Things were challenging for me off and on throughout college as I seemed to be unable to stabilize my episodes for a year or two. And then I was fine for many years up until I was told I had to switch medication to get pregnant and so I did. I switched to the “safest’ medication and things seemed to be fine until about halfway through my pregnancy with my first son in 2015 and then things went haywire. I suffered these partial seizures every ten minutes. I hardly slept. I had to go on short term disability from my job at 5 months pregnant. I could barely speak. Eventually things semi-stabilized.

The goal was always to go back on Topamax, the drug I had been on before my pregnancies as it had been working, but when I finally switched back to this drug in November 2018 my body appeared to just reject it and instead I just started to have multiple episodes per day. My doctor. sent me to the hospital for inpatient video EEG monitoring. I was in the hospital for about 3.5 days where I had 87 episodes. I barely slept.

On the last day in the hospital my doctor took bloodwork for an autoimmune panel. It was the last thing we did while I was in the hospital and at the time I barely thought anything of it. At my follow-up appointment my neurologist told me I have autoimmune encephalitis – my brain is on fire. We did a round of prednisone steroids where I could literally feel my brain cooling down. Within a couple of days of being on the prednisone my seizures that I had experienced multiple times a day for 4 years stopped completely.  We followed up the oral prednisone with IV Solu-Medrol and it’s now been 8 months and I’m still seizure free AND off my anti-epileptic medications.

Autoimmune encephalitis is still a very new disease in its infancy of research. It wasn’t even discovered and published until almost 12 years after my initial diagnosis of epilepsy. I saw neurologists in VA, at Johns Hopkins, and UCLA over the years and it wasn’t until I moved to Pittsburgh, PA that I was finally properly diagnosed.  I don’t say this to place blame on any of my previous doctors, but to raise awareness that it can save your life to get a second or even third opinion about what is going on in your body.

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Have You Thought About Clinical Trials?

Have You Thought About Clinical Trials?

February 10, 2017


Sometimes clinical trials go past us and we hesitate to participate because we don’t really understand how they work and what is involved.

Only through scientific investigation can we hope for new treatments and protocols to be developed. Participants contribute to these discoveries. The link below leads to an article from the Epilepsy Foundation but is a simple and thorough explanation of all clinical trials. I urge you to get the facts and if offered the chance to participate consider being a part of how science can help victims of AE.

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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.




Autoimmune Encephalitis Trivia Playing Cards

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