The Long Road to Somewhere …. Wherever That is

by Alanna | Sep 26, 2023 | AE Warrior Personal Stories

September 27, 2023 | By Amanda Wells

Introduction from the IAES Blog Team:

The staff at IAES is proud to present to you the heart wrenching AE story of Hannah Wells written by her biggest fan and advocate, her mom! This journey travels a very long and bumpy road but also is one of resilience, never ever giving up and, mostly, this is a story of love!

——-

Have you ever noticed how many people, when they are encouraging you, will say, ‘Oh, don’t worry you’ll get there’ but what if you no longer remember where ‘there’ is?

When my youngest daughter Hannah was born, she was 6 weeks premature. On Christmas day of 1983, the paediatrician called and said, ‘If she doesn’t fight, we will lose her, it’s now up to her’! New Year’s Day 1984 he called again, ‘Come and pick her up, take her home, she is a real little fighter, she did it!’ That day when she came home in our arms, we had no idea how that little fighter would have to dig very deep, again, and fight for her life 35 years later!

Hannah was our social butterfly, she was popular with everyone, and everyone seemed to know her! Once she started to talk, she never stopped. Her Grade 1 teacher said to her, ‘Hannah if you can stop talking in class for a whole day, I will give you $20.’ Hannah couldn’t stop talking so the $20 was left wanting!

At age 35 she had been working as a Practice Manager in a Dental Surgery for 10 years. She was amazing at her job. Dentists and patients alike loved her. She was chatty, never took a sick day, and was always organized. Hannah would call me every day from work just to fill me in on what was happening and to touch base.

Around the end of April/ May 2018, Hannah got the flu. She was suffering from headaches and became deaf in her right ear. She went to a general practitioner, and he said, on the third visit, to see a psychologist, he did not believe her headaches were real!

Then one day, in early June, my husband (who was still alive) and I had just been to IKEA and were on our way home when my mobile phone rang. It was Hannah. She had called me the night before to say goodnight and was fine, but this call was anything BUT fine. She was paranoid, delusional, and hearing voices. She had made up some unbelievable story of what was happening next door. I got off the phone and remember feeling ice in my veins, my husband, Hannah’s dad, and I looked at each other and said, ‘What alien has stolen our daughter?’

The days that followed that fateful June day were horrendous. Hannah was in full-blown psychosis. She would call me up to 30 times all night long, as she had insomnia. Her thoughts were disorganized, she could no longer work, and she couldn’t remember how to turn a computer on, copy and paste or even how to unlock a door. She couldn’t change her clothes because she couldn’t remember what clothes went where. She had no idea what day it was or even who our Prime Minister was. She would take off in the car and we didn’t know where she was. She didn’t even know where she was. The internal trauma and also one of her symptoms were now causing unimaginable rage.

When this all began Hannah developed dyskinesia or involuntary and jerking movements. She lost an extreme amount of weight and was now 35 kgs (between 75-80 pounds). Her anxiety was through the roof, and she had become aggressive. Her hair was falling out in spots, and she had tremors in her hands. Hannah knew something wasn’t right but by now had no idea how to articulate it. She was living in a mental prison that was terrorizing her. She knew, in all this chaos, something was wrong in her brain but had almost stopped talking. When she did talk, she would repeat the same sentence for hours, therefore she was unable to articulate how she was feeling.

She/we needed help, this was not our daughter, and I was desperate.

I took her to a nearby hospital emergency department hoping they could help her, but sadly this is where the nightmare really began. Not one test was done except a blood test for drugs. The on-call psychiatrist took me aside to ask what happened. I told her, ‘Hannah has never taken drugs, doesn’t drink, has no past or present trauma, and no mental health in the family. I said this happened, literally, overnight’. I will never forget her reply, ‘That means absolutely nothing.’

Hannah was sectioned under the Mental Health Act and put into the psych ward. The only test done was daily drug testing, no neurological testing was ever done or even suggested. The psych wards in our state are still lock-up wards. She was started on Risperidone, but Hannah refused to take it and would spit it out, as she told us much later, it was making her feel worse.

After getting her released from that psych unit, we decided to take her to one of our large teaching hospitals. They took her from me, and I had no further contact with her. I wasn’t asked what led to our decision to bring her there. They placed Hannah in a room with no water, bathroom, or blanket and took her phone away from her for 12 hours, no one checked on her. They then sectioned her under the Mental Health Act again.

I was trying to speak to a doctor and when I finally did, he told me she had been on drugs, ICE, and that’s why the dyskinesia in her face and weight loss. I asked if he had her chart in front of him and the blood test to confirm this. He admitted he didn’t! Again, no tests were done on Hannah, we couldn’t get information about her from the staff, and by now we were desperately trying to get her out of that hospital and under a private doctor’s care.

To this day I have no idea how my husband got her out of there and got her an urgent appointment with a private psychiatrist, but somehow, he did! She was immediately admitted to a private psych hospital. They did an ECG (on her heart!!!) tested her blood for drugs and that was it. They put Hannah into 2 weeks of CBT, cognitive behavioral training for someone, who cognitively, was a mess! Her short-term memory was non-existent. She was unable to read, write or comprehend 2/ 3 sentences. They also prescribed new antipsychotics! Hannah’s memory and executive functioning abilities were in total chaos, but not one person suggested a neurological workup! As her Mum, I had never felt so hopeless in my life. My daughter was there on the outside, but on the inside, it was like she had left us permanently.

By the end of September and Hannah’s initial psychosis was at last dissipating. There were still the outbursts of rage, strange seizures where she would seem to be frozen, dyskinesia, and limited speech that was often slurred. She would still repeat a phrase or thought for hours, we called it her hamster wheel.

2018 turned into 2019. Trying to get through 2019 was difficult, to say the least. Not only had the disease traumatized her but the auditory hallucinations and repeated hospitalizations had bullied her. She was still very sick, and the doctors had no answers at all!

In 2020 I decided to take her to my dermatologist to check the alopecia (hair loss) that had begun back in 2018 when this all first began. She did a blood test and told Hannah she needed an endocrinologist immediately. She felt she had a thyroid problem. Her blood samples showed abnormal levels. The endocrinologist who treated my husband for osteoporosis, treated Hannah for Graves’ disease, but soon realized this was not her thyroid, there was something else going on and as she said, ‘it was above her pay grade.’ She sent Hannah to her brother who is an infectious and rare disease doctor.

During this time, I went with Hannah back to her psychiatrist who still insisted on seeing her monthly. I was stunned when we told him about the blood tests and the endocrinologists’ findings. He said, ‘I believe this is just a false positive and she just has anxiety and stress.’

By November 2020 we now had seen the rare diseases doctor who flagged her for Autoimmune Encephalitis, but he was stumped and believed she needed a neurologist. We found a neurologist who knew about AE and waited 6 weeks for an appointment.

During all this, my husband, Hannah’s dad passed away a few days before Christmas 2020. I had been caring for him for the last year and for Hannah also. I was exhausted!

Hannah and I were now both grieving the loss of a great husband, an incredible dad, and for Hannah, and the life that she once had.

It was 2021 by the time we saw the neurologist. He ordered an MRI, lumbar puncture (LP), and EEG. He told us the MRI came back normal, as did the LP, but the EEG indicated slowing in the temporal lobes, He said he believed she had Autoimmune Encephalitis. He put her on high-dose steroids tapering down for 3 months. There was some improvement after 3 months, so he repeated the high-dose steroids. He then put her on Cellcept and steroids. He told her to come back if she needed him.

In April 2021 Hannah had a horrific reaction to Cellcept. The neurologist discontinued Cellcept, but she remained on steroids. He said he felt it was now probably too late to treat her! During this time, Hannah began to have breathing issues, but a CT scan did not reveal anything amiss. I was very concerned about Hannah’s bones with all the steroids and told her neurologist, as osteoporosis runs in the family.

April/ May 2022 Hannah seemed to be in a relapse. The only answer they had was more high-dose steroids. Hannah’s GP (general practitioner) was concerned and referred her to another neurologist for a 2^ndopinion. This new neurologist, also, said it was Autoimmune Encephalitis and put Hannah back on another round of high-dose steroids and wanted Hannah to have full cognitive testing.

This journey was beginning to feel like one big roundabout that had no exit. Trying to get any proper treatment was almost impossible.

Her cognitive testing showed deficits with her executive functioning, word processing, short-term memory, and visual abilities were all very badly affected. Hannah was told she needed brain rehab immediately. The report said that this was caused by Autoimmune Encephalitis and damage to the temporal lobe.

The neurologist then said I will see you in 6 months and since it had been 4 years since this all began it is too late to treat Hannah. Her MRI showed a small amount of inflammation, but again ‘It’s too late to treat her now.’

At this point in this long terrifying journey, I was ready to scream. Hannah was now traumatized by the very system that is supposed to heal and help you navigate through a rare disease.

Exactly one week and one day after the neurologist sent us on our way for 6 months to Rehab which was possibly never going to happen because of the long wait list, Hannah ended up in a Private hospital Emergency Dept on her birthday, she was extremely sick. They suggested we go back to her endocrinologist. I was so frustrated we were back at the beginning again! Hannah was devastated. She lost her career, her friends, her life! This disease is harrowing and traumatic, and you have to walk the road to somewhere …… alone!

I was able to obtain Hannah’s complete medical chart that included all prior testing and information. I was beyond incredulous at what I was looking at. Her breathing issues back in 2021 when they said the testing showed nothing amiss was a rib fracture. I was right, this looked like osteoporosis! All her blood tests showed inflammatory markers through the roof since her relapse in May 2022. Her GP couldn’t believe how sick she was. She was now having around 4/5 seizures a day. Her dyskinesia had returned, and anxiety and OCD through the roof. The GP prescribed Azathioprine and steroids, and seizure medication.

I asked for a bone density and her bones were a mess, at 39 she now had osteoporosis!

I thoroughly researched all possible doctors we could contact and turn to review Hannah’s case. I found 2 immunologists; one was a neuroimmunologist whose interest was in Autoimmune Encephalitis. Both had 12-month waits. I wrote to both doctors a letter pleading for help and succinctly outlining Hannah’s case history and her present-day symptoms. Her endocrinologist was stunned she was back where she started 4 ½ years ago, she even contacted the immunologists. Both immunologists denied her an appointment. Their reasoning? She was too complicated and was in the system for too long.

Then out of the blue Dr Martin Newman, the neuro immunologist with experience in AE had a change of heart and called wanting to see her in three days. Her medical file had almost 100 pages (and this was missing most of the beginning files!) and after 5 minutes he said to her, ‘So have they treated you with IVIG or Rituximab?’ Hannah said, ‘No.’ His response was ‘Why not’! Hannah cried, as she sat before someone who just maybe would now help her come back from the brink of the darkness of shame, guilt, isolation, and feeling lost, and she asked him, ‘Will you be able to help me?’ His answer was, “I certainly will Hannah.’ He started Hannah on IVIG in early May 2023 followed by two Ritux infusions. She will most likely be on both for the foreseeable future. Hannah is now, also, in weekly Brain Injury Rehab.

It is now 5 years since Hannah stood on the edge of insanity and a harrowing nightmare. Hannah had almost forgotten who she was and what it’s like to be ‘normal’ and healthy.

I am so thankful to Tabitha Orth, President and Founder of IAES and the IAES family. They were always there, whether I needed a rant or information, their resources seemed limitless. I had no choice but to learn everything I could about Autoimmune Encephalitis. I realized, for an advocate, knowledge is power! I am so thankful for IAES who gave me so much knowledge so as to keep advocating for my daughter.

After 4 months we have seen great improvement. She is mentally and physically up and down. Each day can be different, which we were told to expect, but her progress especially cognitively has amazed all those caring for her medically.

IVIG dealt with a lot of her physical problems. Rituximab kicked in around 9 weeks and she was amazed at some of the improvements cognitively. Where almost ‘there,’ even though ‘there’ is different from what I had expected, but ‘there’ is more than OK. One of the remaining issues with AE is loss of identity and confidence. Hannah’s motto through all this is: ‘I was enough before and I’m enough now!’

After 5 harrowing and terrifying years, we have learned a lot. If you are an advocate/caregiver or even a patient trying to advocate for yourself, don’t give up! There will be someone out there who has a small beacon of light, a life raft for you to jump in, and most of all hope to give you the strength to keep going. IAES and their personal care held a torch for me to see and guided me to the tugboat whose name was ‘DON’T QUIT!’

Just find that light and keep your eye on it!

Click here or the image below to subscribe to our mailing list :

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Neuro MythBusters: The truth behind 10 common myths about your brain

by Alanna | Sep 12, 2023 | PennNeuroKnow

September 13, 2023 | by Catrina Hacker, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

When it comes to evidence-based answers about all thing’s neurology and neuro myth busting, who you going to call? Well, here at IAES it will be the PNK team of course! We hope you enjoy and learn from this myth busting blog as much as we have!!

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

—-

Introduction

Many people find neuroscience fascinating because learning about our brains teaches us about ourselves. Unfortunately, popular interest in brain research has led to several pervasive myths that misrepresent how our brains work. Combatting these neuromyths is difficult because the truth is often much more complicated than the myth and buried in intimidating scientific literature. However, correcting misconceptions about how our brains work can have important benefits for our everyday lives. In this post I’ll break down what some of these neuromyths claim, where they came from, whether there’s any truth behind them, and why we should care about correcting them.

Myth #1: Humans only use 10% of our brains.

This is arguably the most common neuromyth¹, inspiring movies like Limitless (2011) and Lucy (2014) in which characters gain superhuman abilities by tapping into the large unused portions of their brains. It’s appealing to think that we all have potential superpowers sitting in our brains waiting to be unleashed, but there’s nothing to support this claim. The reality is that neuroscientists observe activity throughout the entire brain.

While nobody is certain where it came from, some believe that this myth originates from work done by neuroscientist Wilder Penfield in the 1930s¹. Penfield was a neurosurgeon who studied the effects of stimulating the brains of patients undergoing neurosurgery to learn what each part of the brain was responsible for. He found that stimulating a large portion of the brain didn’t cause any noticeable effect², meaning he could not learn what its function might be. However, new and less invasive methods of recording brain activity show that these “silent” parts of the brain are actually active. In fact, a network of brain regions called the default mode network is even active when we are at rest³.

The bottom line: We use 100% of our brains.

Why it matters: The number of drugs and treatments that claim to enhance brain function, collectively called neuroenhancers, is on the rise. While we can always learn and grow, understanding that there is no “hidden” brain waiting to be unlocked can protect you from wasting your money.

Myth #2: Right-brained people are creative while left-brained people are analytical.

The idea that you can be either right-brained or left-brained has captured the attention of people on social media and even teachers in classrooms. It’s tempting to think that people can be categorized so easily and that differences can be attributed to our brains, but the truth isn’t that simple. While people can tend to be more creative than analytical or vice versa, those differences cannot be explained by dominance of one half of the brain over the other⁴.

This myth has been tricky to combat because there is some important truth behind it. There are some differences between the two halves of your brain, but creativity versus logical reasoning isn’t one of them. Your brain has two hemispheres, left and right, that communicate via a bundle of neural connections called the corpus callosum. While almost everything we do involves communication between the two halves of our brain, sometimes one half of the brain contributes a little more than the other. For example, the left hemisphere typically takes the lead in language processing⁵, the right hemisphere seems to play an especially important role in visual attention⁶, and the left and right hemispheres might do slightly different things to aid in face processing⁷. Things like creativity and emotional processing rely on both hemispheres and complicated networks of brain activations^8,9.

The bottom line: Being right-brained or left-brained can’t explain why some people are more creative than others, but there are some differences in what your left and right hemispheres do when it comes to things like language, attention, and face recognition.

Why it matters: Categorizing people as one thing or another (left-brained or right-brained) is restrictive and ultimately harmful. Many “logical” tasks require creativity and “creative” tasks require logic. If teachers, mentors, and bosses make these assumptions about members of their teams or classrooms they risk mischaracterizing people or preventing them from working up to their true potential.

Myth #3: Listening to Mozart makes babies smarter.

This neuromyth, sometimes called the “Mozart effect”, started in 1991 when Alfred Tomatis shared his thoughts about how listening to Mozart could help children with speech and auditory disorders¹⁰. When a group of researchers showed in 1993 that listening to 10 minutes of Mozart’s K. 448 improved college students’ ability to visualize and manipulate mental images¹¹, the media took this result and ran with it. The effects in the original study only lasted 10 to 15 minutes and only impacted mental manipulation of images, but the media wrote about general boosts to intelligence and implied that they lasted much longer. Despite the original study being done with college students, the myth was somehow generalized to include babies. Several studies published since 1993 have provided alternate explanations for the original result or have failed to replicate it while studying the same or different skills¹².

Although listening to Mozart can’t make you smarter, there is some truth behind this myth. Stimulating an infant’s brain helps with their development, but activities like direct interactions with a parent, reading a book, or talking and singing with an infant are much more effective^13,14. When it comes to music, passively listening might not impact development, but learning to play an instrument positively impacts a child’s cognitive abilities and their performance in school¹⁵.

The bottom line: Listening to Mozart doesn’t make babies smarter, but stimulation from things like singing to your child is an important part of their development, and children who learn to play an instrument tend to perform better in school.

Why it matters: Belief in the Mozart effect and similar claims led many people to show their children the popular Baby Einstein videos in the early 2000s. However, in 2007 a study showed that not only did viewing these baby DVDs not improve children’s intelligence, children who watched the videos tended to have a worse vocabulary than other children¹⁶.

Myth #4: Everybody has a distinct style in which they learn best.

Many people have memories like mine of being asked if they are a visual, auditory, or kinesthetic learner as a child. You may even have filled out a survey to learn what your learning style is. Even today, many teachers collect this information and personalize their teaching to each student’s supposed learning style. While this seems logical, there is no evidence that each person has a specific learning style in which they learn best^17,18, and some research suggests that teaching to learning styles is more harmful than helpful¹⁹. While it’s true that people vary in ability on different kinds of tasks and that teachers should work with students as individuals to help them succeed, when “visual learners” are tasked with learning through auditory tasks, they do just as well¹⁹.

The bottom line: Everybody has different preferences, but matching teaching to a preferred learning style does not improve learning.

Why it matters: It is a waste of time and resources to focus on tailoring education to preferred learning styles when it has no impact on learning. In fact, teaching based on learning styles might actually harm students by limiting them to certain modalities and subjects that match their learning style and discouraging them from exploring²⁰.

Myth #5: Your handwriting reveals aspects of your personality.

The use of handwriting to learn about someone’s personality is called graphology. Graphology became popular in the late 1800s, with German scientist William Preyer commenting that handwriting is “brain writing”^21,22. Despite its dubious scientific validity, graphology was used to make decisions about a person’s value to society, such as in determining whether a person was trustworthy or a criminal. Fortunately, modern experiments have conclusively shown that handwriting cannot predict a person’s personality. In controlled settings, graphologists are no better at using a person’s handwriting to make judgments about them than if they were guessing²³. However, many people still believe that aspects of a person’s personality can be learned from their handwriting, and some computer scientists are still trying to build computer models that can predict things like criminality and work ethic from handwriting²⁴, repeating the mistakes of the past.

Despite the dubious link between handwriting and personality, there are some reliable links between handwriting and brain health. Our brains control the muscles that move as we write, and some neurological disorders can cause changes in the brain that impact handwriting²¹. For example, one early symptom of Parkinson’s Disease can be small, cramped handwriting²⁵. For this and related disorders, handwriting can act as a window into brain health and an early warning sign that can lead to faster care and better outcomes.

The bottom line: A person’s handwriting cannot reliably predict their personality, but changes to handwriting can be early signs of neurological disorders like Parkinson’s Disease.

Why it matters: Despite there being no connection between a person’s handwriting and their personality, in 2017 then President Donald Trump tweeted about being able to tell from his handwriting that former United States Secretary of the Treasury, Jack Lew, “is secretive”²². Some scientists are still trying to build tools that can determine a person’s personality based on their handwriting to help with hiring decisions²⁴. Without widespread acceptance that handwriting cannot predict personality, we risk repeating the mistakes of the past and using handwriting to unfairly discriminate against certain people.

Myth #6: A common sign of dyslexia is seeing letters backwards.

Dyslexia, characterized by difficulty reading, affects an estimated 20% of the population and is the most common neuro-cognitive disorder²⁶. It is a popular misconception that a common sign of dyslexia is seeing words and letters backwards. People with dyslexia don’t see words and letters backwards, but they do have difficulty naming letters and words (think saying “was” while reading “saw”)²⁷. When it comes to writing, there is some evidence that dyslexic children may be more likely than others to write letters and words backwards, a phenomenon called reversals. However, reversals are common in all children learning to read and write²⁸, and not all children with dyslexia make reversals²⁹.

There are many other reliable indicators that a person may have dyslexia. The signs of dyslexia change throughout a lifetime and range from preschool children who struggle to identify the letters in their names to high school students who struggle to read unfamiliar words³⁰. Visit this fact page from the Yale Center for Dyslexia & Creativity for a full list of signs of dyslexia for all age groups.

The bottom line: Dyslexic children don’t see letters backwards, although they may read and write letters backwards. However, not all dyslexic readers write letters backwards and not all children who write letters backwards are dyslexic.

Why it matters: If parents and educators expect dyslexic children to describe seeing letters backwards or adults think they must see letters backwards to have dyslexia, then many people could go undiagnosed and not get the support they need to succeed.

Myth #7: Human memory works like a camera, perfectly recording what you experience.

As a child, one of my favorite book series starred Cam Jansen, a fifth grader who solves mysteries utilizing her flawless photographic memory. Any time she wanted to remember something she would say “click” and it would be perfectly captured in her memory. As an adult, I’ve watched plenty of TV shows and movies featuring similar characters who can use their perfect memory to save the day. Unfortunately, this kind of memory doesn’t exist outside books and other media.

For the rest of us here on earth, our brains forget and fill in details of our memories, even when we feel certain we remember things perfectly. A great example of this is the visual Mandela effect, wherein people consistently report strong false memories of things like whether Curious George has a tail or the Monopoly man wears a monocle (neither is true, but people consistently believe that they are)³¹. In general, it’s a good thing that our brains work in this imperfect way. We don’t want to get bogged down with irrelevant details of memories, so our brains act as a filter, prioritizing memory for the things that matter most and filling in the details and moments that are less important.

If our memory is so imperfect, where does the idea of photographic memory come from? This myth might have started after psychologist Ralph Haber noticed that a small percentage of children seemed to be able to hold pictures in their mind’s eye for seconds or minutes after they were removed from sight³². He called this kind of memory eidetic memory (often used interchangeably with “photographic memory” in popular media). However, these studies only looked at memory for short periods of time, and later research demonstrated that this “memory” is far from perfect³³.

The bottom line: Some people can remember things better or longer than others, but nobody’s memory works like a camera.

Why it matters: Our criminal justice system still relies heavily on eyewitness reports. If police officers, lawyers, and jurors don’t realize that memory is flawed, they risk inflating the value of this kind of testimony and incarcerating innocent people³⁴.

Myth #8: People with bigger brains are smarter.

We’ve all heard or used the term “big-brained” to describe someone who does something smart, but the size of their brain has nothing to do with their intellect. If size was all that mattered, then elephants, whose brains are 3x heavier than ours, would be 3x smarter than us³⁵. Even if we’re just looking at human brains, Albert Einstein’s brain was no bigger than average, and despite years of studying his brain, neuroscientists haven’t found any clear differences in its structure compared to other human brains³⁶.

The myth that smarter people have bigger brains has a particularly harmful history. In the 1800s, scientists measured the skulls of people of different races and genders as an estimate of brain size to provide “scientific” evidence that Caucasian men were superior to women and other races. There are many reasons this approach was flawed, not least of which is that correcting for body size can account for many of the reported differences³⁷. In 1898, a woman named Alice Lee challenged this idea by storming into the all-male meeting of the Anatomical Society at Trinity College Dublin, measuring the skulls of several prominent men in the audience, and demonstrating that many of these supposedly intelligent men had rather small skulls³⁸.

Read my previous post, “The Problem of Brain Size”, for a more detailed look at this myth.

The bottom line: Brain size has nothing to do with intelligence.

Why it matters: Flawed measurements of brain size have historically been used as scientific “proof” that women and racial minorities are not as intelligent as Caucasian men. Dispelling this myth is critical to reverse the harm done by the claims made in these studies and to prevent making the same mistakes in the future.

Myth #9: Playing brain games makes you smarter.

We’ve all seen ads for games that claim to train your brain to make you smarter, or measure your IQ. However, these claims are misleading and overinflated. One study conclusively proved this by having over 11,000 people play online brain games for six weeks. At the end of the six weeks, people had gotten a little better at the specific games that they played, but they were no better at any other tests³⁹. In other words, playing one memory game could make people better at that game, but it didn’t improve their memory overall.

In 2016, the brain game company Lumosity paid a $2 million settlement to the Federal Trade Commission (FTC) who filed false advertising charges against them⁴⁰. The FTC asserted that Lumosity’s claims that playing their games could improve performance on everyday tasks, delay age-related cognitive decline, and reduce the effects of brain injuries like stroke were unfounded. Since the settlement, Lumosity has been forced to alter their claims so that they do not mislead consumers.

The bottom line: Playing brain games makes you better at those particular games, but not any smarter overall.

Why it matters: Before investing time and money into products that claim to improve brain function by playing fun brain training games, it’s important to understand that these effects are often small and improve performance on specific tasks, but don’t generalize.

Myth #10: Different regions of your tongue are specialized for different kinds of tastes.

There are five basic tastes: bitter, salty, sour, sweet, and umami⁴¹. The myth goes that there are different parts of your tongue that are specialized to sense different tastes, so sweet and salty tastes are sensed on the tip of your tongue, while bitter tastes are sensed toward the back. I remember learning this myth for the first time at a girl scout meeting where we tasted different foods by placing them on different parts of our tongue. Since then, I heard it repeated many times in school and even in some of my neuroscience classes as an undergrad. In fact, many textbooks that are still being used today include this false claim. However, the truth is that although some parts of the tongue might be more sensitive to one taste or another, all five basic tastes are sensed across the entire tongue⁴².

The tongue map myth started with a 1901 paper in which German scientist David Hänig measured how much taste sensitivities changed across the tongue. He noticed that some parts of the tongue were more sensitive to a particular taste than others, and he drew some graphs to show how those sensitivities changed across the tongue. In 1940, another scientist adapted these graphs for a book about the different senses. In his adaptation, he simplified things by showing a single taste that was most sensitive on each part of the tongue rather than the relative sensitivities of each taste. This gave the false impression that each region of the tongue could sense just one taste, and this oversimplified figure has been copied thousands of times into science textbooks to teach the neuroscience of taste.

The bottom line: Sensitivity to each taste varies somewhat across the tongue, but each part of the tongue senses all the basic tastes.

Why it matters: The negative consequences of this myth might not be as harmful as the others, but it’s always worth correcting our understanding of ourselves and our bodies.

Now that you’ve learned the truth behind 10 popular neuromyths, it’s worth asking how so many neuromyths have leaked into popular press and what we can do to prevent them in the future. Preventing the spread of disinformation about the brain starts at all levels. Scientists should be careful not to overgeneralize or oversimplify their findings and to always consider alternative explanations and how their work might be misinterpreted. Journalists and science communicators should carefully report the results of scientific studies and not overstate what a given experiment shows. Non-scientists should think critically about what they read, and fact check things they read from unknown sources on social media. And most importantly, now that you know the truth behind the myth, the best thing you can do is to teach it to others who still believe in these popular neuromyths.

References

1. Jarrett, C. All You Need To Know About the 10 Percent Brain Myth, in 60 Seconds. Wired.

2. Ferrier Lecture – Some observations on the cerebral cortex of man. Proc. R. Soc. Lond. Ser. B – Biol. Sci. 134, 329–347 (1947).

3. Raichle, M. E. The Brain’s Default Mode Network. Annu. Rev. Neurosci. 38, 433–447 (2015).

4. Nielsen, J. A., Zielinski, B. A., Ferguson, M. A., Lainhart, J. E. & Anderson, J. S. An Evaluation of the Left-Brain vs. Right-Brain Hypothesis with Resting State Functional Connectivity Magnetic Resonance Imaging. PLoS ONE 8, e71275 (2013).

5. Bradshaw, A. R., Thompson, P. A., Wilson, A. C., Bishop, D. V. M. & Woodhead, Z. V. J. Measuring language lateralisation with different language tasks: a systematic review. PeerJ 5, e3929 (2017).

6. Chica, A. B. et al. Attention networks and their interactions after right-hemisphere damage. Cortex 48, 654–663 (2012).

7. Meng, M., Cherian, T., Singal, G. & Sinha, P. Lateralization of face processing in the human brain. Proc. R. Soc. B Biol. Sci. 279, 2052–2061 (2012).

8. Amir, O. & Biederman, I. The Neural Correlates of Humor Creativity. Front. Hum. Neurosci. 10, (2016).

9. Fossati, P. Neural correlates of emotion processing: From emotional to social brain. Eur. Neuropsychopharmacol. 22, S487–S491 (2012).

10. Tomatis, Alfred. Pourqoi Mozart? (Diffusion, Hachette, 1991).

11. Rauscher, F. H., Shaw, G. L. & Ky, K. N. Music and spatial task performance. Nature 365, (1993).

12. Cong, A. FROM MOZART TO MYTHS: Dispelling the ‘Mozart Effect’. Young Sci. J. 49–53 (2014).

13. California Childcare Health Program, UCSF School of Nursing. Building Baby’s Intelligence: Why Infant Stimulation Is So Important. (2002).

14. Walker, S. P. et al. Cognitive, psychosocial, and behaviour gains at age 31 years from the Jamaica early childhood stimulation trial. J. Child Psychol. Psychiatry 63, 626–635 (2022).

15. Román-Caballero, R., Vadillo, M. A., Trainor, L. J. & Lupiáñez, J. Please don’t stop the music: A meta-analysis of the cognitive and academic benefits of instrumental musical training in childhood and adolescence. Educ. Res. Rev. 35, 100436 (2022).

16. Zimmerman, F. J., Christakis, D. A. & Meltzoff, A. N. Associations between Media Viewing and Language Development in Children Under Age 2 Years. J. Pediatr. 151, 364–368 (2007).

17. Pashler, H., McDaniel, M., Rohrer, D. & Bjork, R. Learning Styles: Concepts and Evidence. Psychol. Sci. Public Interest 9, 105–119 (2008).

18. Cuevas, J. Is learning styles-based instruction effective? A comprehensive analysis of recent research on learning styles. Theory Res. Educ. 13, 308–333 (2015).

19. Riener, C. & Willingham, D. The Myth of Learning Styles. Change Mag. High. Learn. 42, 32–35 (2010).

20. Newton, P. M. & Salvi, A. How Common Is Belief in the Learning Styles Neuromyth, and Does It Matter? A Pragmatic Systematic Review. Front. Educ. 5, 602451 (2020).

21. The Telltale Hand. Dana Foundation https://www.dana.org/article/the-telltale-hand/.

22. Trubek, A. Sorry, Graphology Isn’t a Real Science. JSTOR Daily https://daily.jstor.org/graphology-isnt-real-science/ (2017).

23. Dazzi, C. & Pedrabissi, L. Graphology and Personality: An Empirical Study on Validity of Handwriting Analysis. Psychol. Rep. 105, 1255–1268 (2009).

24. Bandhu, K. C., Litoriya, R., Khatri, M., Kaul, M. & Soni, P. Integrating graphology and machine learning for accurate prediction of personality: a novel approach. Multimed. Tools Appl. (2023) doi:10.1007/s11042-023-15567-8.

25. Small Handwriting | Parkinson’s Foundation. https://www.parkinson.org/understanding-parkinsons/non-movement-symptoms/small-handwriting.

26. Dyslexia FAQ. Yale Dyslexia https://dyslexia.yale.edu/dyslexia/dyslexia-faq/.

27. Shaywitz, S. E. & Shaywitz, B. A. Dyslexia (Speciﬁc Reading Disability).

28. Cornell, J. M. Spontaneous mirror-writing in children. Can. J. Psychol. Rev. Can. Psychol. 39, 174–179 (1985).

29. Brooks, A. D., Berninger, V. W. & Abbott, R. D. Letter Naming and Letter Writing Reversals in Children With Dyslexia: Momentary Inefficiency in the Phonological and Orthographic Loops of Working Memory. Dev. Neuropsychol. 36, 847–868 (2011).

30. Signs of Dyslexia. Yale Dyslexia https://dyslexia.yale.edu/dyslexia/signs-of-dyslexia/.

31. Prasad, D. & Bainbridge, W. A. The Visual Mandela Effect as Evidence for Shared and Specific False Memories Across People. Psychol. Sci.

32. Haber, R. N. Twenty years of haunting eidetic imagery: where’s the ghost? Behav. Brain Sci. 2, 583–594 (1979).

33. Gray, C. R. & Gummerman, K. The Enigmatic Eidetic Image: A Critical Examination of Methods, Data, and Theories.

34. Report Urges Caution in Handling and Relying Upon Eyewitness Identifications in Criminal Cases, Recommends Best Practices for Law Enforcement and Courts | National Academies. https://www.nationalacademies.org/news/2014/10/report-urges-caution-in-handling-and-relying-upon-eyewitness-identifications-in-criminal-cases-recommends-best-practices-for-law-enforcement-and-courts.

35. Herculano-Houzel, S. et al. The elephant brain in numbers. Front. Neuroanat. 8, (2014).

36. Hines, T. Neuromythology of Einstein’s brain. Brain Cogn. 88, 21–25 (2014).

37. Gould, S. The Mismeasure of Man. (WW Northon & Company, 1996).

38. McNeill, L. The Statistician Who Debunked Sexist Myths About Skull Size and Intelligence. Smithsonian Magazine https://www.smithsonianmag.com/science-nature/alice-lee-statistician-debunked-sexist-myths-skull-size-intelligence-180971241/.

39. Owen, A. M. et al. Putting brain training to the test. Nature 465, 775–778 (2010).

40. Lumosity to Pay $2 Million to Settle FTC Deceptive Advertising Charges for Its “Brain Training” Program. Federal Trade Commission https://www.ftc.gov/news-events/news/press-releases/2016/01/lumosity-pay-2-million-settle-ftc-deceptive-advertising-charges-its-brain-training-program (2016).

41. sarah. Accounting for taste. Curious https://www.science.org.au/curious/people-medicine/accounting-taste (2016).

42. Caballar, R. D. Do Different Parts of the Tongue Taste Different Things? https://www.brainfacts.org:443/thinking-sensing-and-behaving/taste/2018/do-different-parts-of-the-tongue-taste-different-things-010319.

Cover photo made by Catrina Hacker in Biorender.com using image by GraphicMama-team from Pixabay.

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

why zebra - Aphasia as a Symptom of Autoimmune Encephalitis

image - Neuro MythBusters: The truth behind 10 common myths about your brain

10 Big Unanswered Questions in Neuroscience Part 1

by Alanna | Aug 29, 2023 | PennNeuroKnow

August 30, 2023 | by Sophie Liebergall, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

As AE Warriors, caregivers, friends, family, loved ones and medical personnel we have been unwittingly thrown into a world we may or may not have been at all curious about previously. No matter where we are in our individual AE journeys, neurology and neuroscience are terms we all know well. AE may have sent us on this journey deep into the amazing world of neurology, but we all have found out just how interesting and fascinating our brains can be! In the first of a two-part series, Sophie Liebergall has helped us to better understand 10 big unanswered questions in neuroscience! We hope you enjoy this and look forward to Part II.

——

Introduction

This past year, astrophysicists used NASA’s James Webb Space telescope to observe a star that is over 33 billion light years away from earth. Back on earth, particle physicists used the Large Hadron Collider in Switzerland to confirm the existence of incomprehensibly tiny subatomic particles. But despite these astounding scientific and technologic advances, we still have a lot to learn about what is going on in the organ inside our own skulls! In part one of what will be a two-part series, we discuss a few of the fundamental questions about the brain that have remained mysterious to neuroscientists.

1. Where do our memories go when we put them in long-term storage?

For a brain to perform complex tasks, such as telling the body to execute a series of movements or being able to recognize and evade a predator, it must be able to recall information that was gathered during previous experiences. Neuroscience researchers divide memory storage into two stages: short-term memory and long-term memory.¹ When the brain senses something in its environment, it can hold that information for a few seconds to minutes as a short-term memory.² Over time, scientists have gathered clues that short-term memories (at least those of conscious facts and events) are stored in the hippocampus, an almond-size region nestled on either side below the brain’s surface on either side.³ But sometimes the brain needs to hold onto information for longer periods of time (up to a lifetime) so that is can be recalled later. We’re fairly certain that long-term memories aren’t stored in the hippocampus; but where exactly these long-term memories go remains a mystery. Several recent studies seem to suggest that, unlike short-term memories, long-term memories may be widely distributed in the cerebral cortex (the large surface of the brain that is used for complex thought), with different features of the memory spread across different regions.^4,5 You can read more about the process of memory formation and how it can go wrong in some diseases here!

2. Why do we need to sleep?

Evolution has shaped the human body into an elegant and efficient machine, with a versatile digestive system, a continuously beating heart, and a thinking brain. However, one of our basic biologic functions, sleep, seems like something that should have been stamped out by evolution many generations ago. When we sleep, we are essentially unconscious for up to one-third of the day. For our ancestors, this is a time when they were particularly vulnerable to predators and unable to gather food. So why, then, has sleep survived the test of natural selection?

Sleep is absolutely necessary for all animals (from armadillos, who sleep up to twenty hours each day, to giraffes who need just two hours of sleep a day).⁶ After just a couple days of total sleep deprivation, many people will start to show symptoms of psychosis.⁷ And if the sleep deprivation continues, it can even be deadly. In a study from the 1980s, which would likely be forbidden under contemporary ethical standards, researchers subjected a group of rats to total sleep deprivation. All of the rats died by the 32^nd day of the study.⁸ The ultrarare genetic disease Fatal familial insomnia gives further insight into the danger of insomnia in humans. Patients with Fatal familial insomnia slowly lose their ability to fall and stay asleep.⁹ Tragically, these patients always die soon after they completely lose their ability to sleep.

Sleep is important for a variety of our body and brain’s normal functions: solidifying events that occurred during the day as long term memories,¹⁰ recalibrating the strength of the connections between brain cells,^11,12 balancing the hormones that control our appetites and metabolism,¹³ and clearing the toxic byproducts of brain cell activity.¹⁴ But scientists still do not know what function (or functions) of sleep are the primary reason why it is essential for survival. Read more about the possible hypothesis for why we sleep in this PNK article!

3. Why do we dream?

Even more mysterious than the question of why we sleep is the question of why we dream. Though sleep has been a target of neuroscience research for decades, there are inherent challenges to studying dreaming that prevent us from using some of the traditional tools of neuroscience research. The study of dreams still largely relies on dream reports, when a person wakes up and verbally reports or writes down whether they were dreaming and what their dream was about. Dream reports are often unreliable because of the bias and imperfect memory of the dreamer. This can prevent researchers from making objective scientific conclusions from dream reports. Furthermore, all animals clearly display some form of sleep, but there is no conclusive evidence that other animals have dreams. This makes it challenging or even impossible to study dreaming using laboratory animals, which generally allow us to perform important experiments that would take too long or be too dangerous in humans.

Though we have recently developed more sophisticated tools that allow us to correlate the dream reports of humans with measures of brain activity, many of these studies have only raised more questions. It was once thought that dreaming only occurred during rapid eye movement (REM) sleep, the phase of sleep during which brain waves look most similar to the waking state. But more recent evidence suggests that dreams occur during both REM and non-REM sleep (though dreams that occur during REM sleep do seem to be more vivid than the dreams that occur during non-REM sleep).^15,16

Another strange aspect of dreaming is called the dream-lag effect, which describes a phenomenon in which you’re most likely to dream about real life events that happened 5-7 days ago.¹⁷ And we still don’t have a clue as to why some people are prone to sleepwalking: a state in which individuals are clearly deep in a dream, but somehow are aware of their surroundings enough to navigate a space, consume food, or even drive a motor vehicle.¹⁸ You can learn more about the neuroscience of dreaming here!

4. How do the general anesthesia drugs used during surgery make you unconscious?

General anesthetics, the class of drugs which cause temporary unconsciousness, have made it possible for doctors to perform lifesaving and life-altering surgeries that would otherwise be impossibly painful for patients. General anesthetics are some of the most safe and reliable medications that are administered by doctors. But we still don’t have an understanding of where general anesthetics act in the brain, or of what their ultimate effects are on brain processes. Even though anesthetic drugs all have the same end effect of making a patient unconscious, anesthetics can come in all different shapes and sizes. Some, like xenon gas, have a structure as simple a single atom, whereas others, like alfaxalone, have a complex structure with many branches and rings.^19,20 Some are inhaled as a gas, whereas others are injected into the bloodstream. And, strangely, general anesthetics don’t just sedate animals with complex brains like humans. They also impair the movement and environmental responsiveness of plants and even single-celled organisms!²¹ You can learn more about the possible mechanisms of general anesthetics and their relationship with sleep in this PNK article.

5. How does each area of the brain know what function it is supposed to perform?

In the mid-19^th century, in the early days of modern neuroscience, the French physician Paul Broca learned of a patient with a unique neurologic condition. This patient had lost the ability to generate speech, but had somehow maintained the ability to comprehend speech.²² When this patient died, Broca performed an autopsy, where he discovered that the patient had sustained an injury to a very specific area of their frontal lobe. Broca’s work inspired other physicians of his age to look for injuries to specific areas of their brains in their patients with specific neurologic symptoms. If multiple patients with the same symptoms had an injury in the same region, then it could be assumed that an injury to that region was the cause of the symptom. These studies of localized brain injuries led neurologists to believe that different regions of the brain are responsible for the different functions of the brain. For example, one region of the brain is required for the ability to move a hand, whereas another region of the brain is required to read language.

Modern-day neuroscientists and neurologists take the idea that certain regions of the brain are responsible for certain functions for granted. But there is a great deal of complexity to this picture that we have yet to understand. The exact mapping of the functions of the brain can vary between individuals – sometimes in dramatic ways. For example, most people have the speech control area of their brain somewhere on the left side of their brain. But occasionally, in people who are left-handed, the speech control area is instead found on the right side.²³ This variability between individuals suggests that the process of assigning a function to a specific brain region doesn’t follow a simple blueprint. But we still don’t know how the brain knows which functions it needs to perform. And we also don’t know each function is assigned to a particular region of the brain.

Stay tuned for part two with five more big unanswered questions in neuroscience coming this summer!

References

1. Cowan, N. What are the differences between long-term, short-term, and working memory? Prog Brain Res 169, 323–338 (2008).

2. Atkinson, R. C. & Shiffrin, R. M. Human Memory: A Proposed System and its Control Processes11This research was supported by the National Aeronautics and Space Administration, Grant No. NGR-05-020-036. The authors are indebted to W. K. Estes and G. H. Bower who provided many valuable suggestions and comments at various stages of the work. Special credit is due J. W. Brelsford who was instrumental in carrying out the research discussed in Section IV and whose overall contributions are too numerous to report in detail. We should also like to thank those co-workers who carried out a number of the experiments discussed in the latter half of the paper; rather than list them here, each will be acknowledged at the appropriate place. in Psychology of Learning and Motivation (eds. Spence, K. W. & Spence, J. T.) vol. 2 89–195 (Academic Press, 1968).

3. Duff, M. C., Covington, N. V., Hilverman, C. & Cohen, N. J. Semantic Memory and the Hippocampus: Revisiting, Reaffirming, and Extending the Reach of Their Critical Relationship. Frontiers in Human Neuroscience 13, (2020).

4. Yadav, N. et al. Prefrontal feature representations drive memory recall. Nature 608, 153–160 (2022).

5. Roy, D. S. et al. Brain-wide mapping reveals that engrams for a single memory are distributed across multiple brain regions. Nat Commun 13, 1799 (2022).

6. Campbell, S. S. & Tobler, I. Animal sleep: a review of sleep duration across phylogeny. Neurosci Biobehav Rev 8, 269–300 (1984).

7. Waters, F., Chiu, V., Atkinson, A. & Blom, J. D. Severe Sleep Deprivation Causes Hallucinations and a Gradual Progression Toward Psychosis With Increasing Time Awake. Front Psychiatry 9, 303 (2018).

8. Everson, C. A., Bergmann, B. M. & Rechtschaffen, A. Sleep deprivation in the rat: III. Total sleep deprivation. Sleep 12, 13–21 (1989).

9. Fatal Familial Insomnia – Symptoms, Causes, Treatment | NORD. https://rarediseases.org/rare-diseases/fatal-familial-insomnia/.

10. Diekelmann, S. & Born, J. The memory function of sleep. Nat Rev Neurosci 11, 114–126 (2010).

11. Frank, M. G. Erasing Synapses in Sleep: Is It Time to Be SHY? Neural Plast 2012, 264378 (2012).

12. Tononi, G. & Cirelli, C. Sleep function and synaptic homeostasis. Sleep Medicine Reviews 10, 49–62 (2006).

13. Sharma, S. & Kavuru, M. Sleep and Metabolism: An Overview. Int J Endocrinol 2010, 270832 (2010).

14. Xie, L. et al. Sleep Drives Metabolite Clearance from the Adult Brain. Science 342, 10.1126/science.1241224 (2013).

15. Foulkes, W. D. Dream reports from different stages of sleep. J Abnorm Soc Psychol 65, 14–25 (1962).

16. Hobson, J. A., Pace-Schott, E. F. & Stickgold, R. Dreaming and the brain: toward a cognitive neuroscience of conscious states. Behav Brain Sci 23, 793–842; discussion 904-1121 (2000).

17. Eichenlaub, J. et al. The nature of delayed dream incorporation (‘dream‐lag effect’): Personally significant events persist, but not major daily activities or concerns. J Sleep Res 28, e12697 (2019).

18. Cochen De Cock, V. Sleepwalking. Curr Treat Options Neurol 18, 6 (2016).

19. PubChem. Alfaxalone. https://pubchem.ncbi.nlm.nih.gov/compound/104845.

20. PubChem. Xenon. https://pubchem.ncbi.nlm.nih.gov/compound/23991.

21. Kelz, M. B. & Mashour, G. A. The Biology of General Anesthesia from Paramecium to Primate. Current Biology 29, R1199–R1210 (2019).

22. Dronkers, N. F., Plaisant, O., Iba-Zizen, M. T. & Cabanis, E. A. Paul Broca’s historic cases: high resolution MR imaging of the brains of Leborgne and Lelong. Brain 130, 1432–1441 (2007).

23. Packheiser, J. et al. A large-scale estimate on the relationship between language and motor lateralization. Sci Rep 10, 13027 (2020).

Cover photo made with biorender.com.

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

image - 10 Big Unanswered Questions in Neuroscience Part 1

A link between COVID-19 and autoimmune encephalitis?

by Alanna | Aug 22, 2023 | PennNeuroKnow

August 23, 2023 | by Kara McGaughey, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

——-

Introduction

The Covid 19 pandemic spread its insidious tentacles all over the world. Scientific papers, chapters of books and entire university courses can be counted on to outline and delve deep into the wide spread effects on all levels of society that Covid has caused. For the AE community we are not only affected by the general Covid effects but possibly, also, in relation to our own ongoing AE journeys. How does the Covid virus affect AE? Is there a link between Covid 19 and AE? Kara McGaughey from the PNK team has done a wonderful job helping us all better understand what can be understood about this possible relationship at this time and what it may mean for the future of AE research.

Coronavirus Disease (COVID-19) is an ongoing global health crisis with more than 760 million confirmed cases and nearly 7 million deaths reported by the World Health Organization as of June 2023.¹ However, as we enter into the fourth year of the pandemic, we’re beginning to understand that knowing the number of active cases of COVID-19 isn’t the whole story.

In this post, we will dive into the long-term consequences of COVID-19, with a focus on the potential link between COVID-19 and autoimmune encephalitis (AE). We will explore why scientists think these diseases might be connected as well as what implications these new, post-COVID cases can have for AE research.

What is the connection between COVID-19 and AE?

Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) is a newly-emerged virus that causes Coronavirus Disease 2019 (COVID-19). SARS-CoV-2 infection results in various systemic and respiratory symptoms such as fever, fatigue, cough, and difficulty breathing. In cases of severe disease, these symptoms can cause heart and lung failure, requiring hospitalization. However, the struggle isn’t always over once infection has subsided. Around 15% of patients have persistent symptoms for months after testing positive.^2-3These symptoms, often including fatigue and brain fog, can be debilitating. In many cases, a patient’s ability to carry out normal, everyday activities is profoundly affected. In a much, much smaller percentage of cases, the SARS-CoV-2 virus can also function as a trigger for some autoimmune diseases, like Guillain-Barré Syndrome (GBS), rheumatoid arthritis, and even autoimmune encephalitis (AE).^4-5

AE refers to a group of conditions that occur when the body’s immune system mistakenly attacks healthy brain tissue.⁵ The cause of AE is often unknown. However, experts say that, in some cases, exposure to certain bacteria or viruses may increase someone’s risk of AE. For example, infection with herpes simplex virus 1 (HSV-1) has been linked to later development of AE, particularly the anti-NMDA AE subtype.⁷

We are seeing something similar happening now with SARS-CoV-2 viral infections (and re-infections) leading to an uptick in the number of AE diagnoses. Case reports of this so-called “post-COVID AE” have come from all over the world — Iran, Canada, France, Italy, the United Kingdom, China, Sweden, India, Mexico, and the United States — and describe patients across a wide range of ages from 2 to 88.^4,8-9 A majority of these post-COVID AE diagnoses are for either limbic or anti-NMDA AE subtypes with patients experiencing headache, cognitive impairment, and seizures.⁴ Fortunately, a majority of patients respond well to treatment.⁴

How can SARS-CoV-2 infection lead to AE?

How exactly AE develops from SARS-CoV-2 infections is not yet fully understood. However, scientists do have some theories.

The “cytokine storm” and inflammatory cytokines:

Cytokines are small proteins that are crucial for controlling the immune system’s activity.¹⁰ Inflammatory cytokinesact as signals that tell the immune system to turn on, enabling the body to recognize and destroy foreign invaders (like the SARS-CoV-2 virus). Anti-inflammatory cytokines are responsible for dialing immune system activity back down once the threat has been neutralized. During the pandemic, you may have heard about COVID-19 causing the overproduction of inflammatory cytokines, known as a “cytokine storm.” With too many of these cytokines released in the body, immune system activity and inflammation can spiral out of control, leading to, in the worst cases, multi-organ failure.^4-6,11

Scientists think that one link between COVID-19 and AE is a particular inflammatory cytokine, IL-6, released during this storm.^5,12 Elevated levels of IL-6 are often found in patients with anti-NMDA AE.^11,13-14 In fact, they are considered a characteristic feature of this AE subtype.¹³ Given that many post-COVID AE cases are anti-NMDA, it is possible that high levels of IL-6 as a result of SARS-CoV-2 viral infection could be one reason for the increased risk of developing AE after COVID-19.

Accidental autoimmunity:

While we want an immune system that can recognize and react to foreign invaders (e.g., SARS-CoV-2, tumor cells, etc.), it is just as important that our own cells don’t get caught up in the crossfire. Fortunately, our immune system has evolved to both quickly and accurately distinguish outsiders from the body itself. However, sometimes in the face of viral infections that cause extreme inflammation, this protective, self-recognition feature goes awry and the body begins to produce antibodies that accidentally target its own tissue (“autoantibodies”). This autoantibody-induced self destruction is called autoimmunity.⁴It is possible that SARS-CoV-2 viral infections induce AE through an autoimmune process that generates antibodies targeting brain cells.

What implications might this have for AE research?

AE is notoriously rare and frequently misdiagnosed.¹⁵Evidence for a link between SARS-CoV-2 infections and the development of AE means more of the scientific spotlight is being given to AE. This increased awareness could make physicians more likely to explore AE as a possible diagnosis, decreasing the time patients spend in limbo waiting for answers and treatment. Perhaps more importantly, in scientific research, money and resources flow where attention goes. This could mean more funding for AE research and more AE clinical trials. Hopefully, this will lead to a better understanding not only of the relationship between COVID-19 and AE, but AE and autoimmunity more broadly.

A final note: It’s important to remember that getting infected or re-infected with COVID-19 doesn’t mean you will end up with AE. While there have been a fair number of case reports of post-COVID AE, it is still a rare outcome. Moreover, it is very difficult to establish any sort of causal link between SARS-CoV-2 infection and the later development of a disease. In most cases it is impossible to know whether some of these patients would have developed AE even without exposure to COVID-19. Nonetheless, the best path forward is to be aware of ongoing research and continue preventive measures, like wearing a mask in high-risk situations and making sure you stay up to date on COVID-19 vaccinations.

Work cited:

WHO Coronavirus (COVID-19) Dashboard. (n.d.). Retrieved May 1, 2023, from https://covid19.who.int
Nearly One in Five American Adults Who Have Had COVID-19 Still Have “Long COVID.” (2022, June 22).https://www.cdc.gov/nchs/pressroom/nchs_press_releases/2022/20220622.htm
Lledó, G. M., Sellares, J., Brotons, C., Sans, M., Antón, J. D., Blanco, J., Bassat, Q., Sarukhan, A., Miró, J. M., & de Sanjosé, S. (2022). Post-acute COVID-19 syndrome: A new tsunami requiring a universal case definition. Clinical Microbiology and Infection, 28(3), 315–318. https://doi.org/10.1016/j.cmi.2021.11.015
Stoian, A., Stoian, M., Bajko, Z., Maier, S., Andone, S., Cioflinc, R. A., Motataianu, A., Barcutean, L., & Balasa, R. (2022). Autoimmune Encephalitis in COVID-19 Infection: Our Experience and Systematic Review of the Literature. Biomedicines, 10(4), 774. https://doi.org/10.3390/biomedicines10040774
Nabizadeh, F., Balabandian, M., Sodeifian, F., Rezaei, N., Rostami, M. R., & Naser Moghadasi, A. (2022). Autoimmune encephalitis associated with COVID-19: A systematic review. Multiple Sclerosis and Related Disorders, 62, 103795.https://doi.org/10.1016/j.msard.2022.103795
Payus, A. O., Jeffree, M. S., Ohn, M. H., Tan, H. J., Ibrahim, A., Chia, Y. K., & Raymond, A. A. (2022). Immune-mediated neurological syndrome in SARS-CoV-2 infection: A review of literature on autoimmune encephalitis in COVID-19. Neurological Sciences, 43(3), 1533–1547. https://doi.org/10.1007/s10072-021-05785-z
Armangue, T., Spatola, M., Vlagea, A., Mattozzi, S., Cárceles-Cordon, M., Martinez-Heras, E., Llufriu, S., Muchart, J., Erro, M. E., Abraira, L., Moris, G., Monros-Giménez, L., Corral-Corral, Í., Montejo, C., Toledo, M., Bataller, L., Secondi, G., Ariño, H., Martínez-Hernández, E., … Zabalza, A. (2018). Frequency, symptoms, risk factors, and outcomes of autoimmune encephalitis after herpes simplex encephalitis: A prospective observational study and retrospective analysis. The Lancet Neurology, 17(9), 760–772. https://doi.org/10.1016/S1474-4422(18)30244-8
Saffari, P., Aliakbar, R., Haritounian, A., Mughnetsyan, R., Do, C., Jacobs, J., Hoffer, J., Arieli, R., Liu, A. K., Saffari, P., Aliakbar, R., Haritounian, A., Mughnetsyan, R., Do, C., Jacobs, J., Hoffer, J., Arieli, R., & Liu, A. K. (2023). A Sharp Rise in Autoimmune Encephalitis in the COVID-19 Era: A Case Series. Cureus, 15(2). https://doi.org/10.7759/cureus.34658
Mekheal, E., Mekheal, M., Roman, S., Mikhael, D., Mekheal, N., Manickam, R., Mekheal, E., Mekheal, M., Roman, S., Mikhael, D., Mekheal, N., & Manickam, R. (2022). A Case Report of Autoimmune Encephalitis: Could Post-COVID-19 Autoimmunity Become a Lethal Health Issue? Cureus, 14(6). https://doi.org/10.7759/cureus.25910
Kim, E. Y., & Moudgil, K. D. (2008). Regulation of autoimmune inflammation by pro-inflammatory cytokines. Immunology Letters, 120(1), 1–5. https://doi.org/10.1016/j.imlet.2008.07.008
Byun, J.-I., Lee, S.-T., Moon, J., Jung, K.-H., Sunwoo, J.-S., Lim, J.-A., Kim, T.-J., Shin, Y.-W., Lee, K.-J., Jun, J.-S., Lee, H. S., Lee, W.-J., Kim, Y.-S., Kim, S., Jeon, D., Park, K.-I., Jung, K.-Y., Kim, M., Chu, K., & Lee, S. K. (2016). Distinct intrathecal interleukin-17/interleukin-6 activation in anti-N-methyl-d-aspartate receptor encephalitis. Journal of Neuroimmunology, 297, 141–147. https://doi.org/10.1016/j.jneuroim.2016.05.023
Liu, J., Li, S., Liu, J., Liang, B., Wang, X., Wang, H., Li, W., Tong, Q., Yi, J., Zhao, L., Xiong, L., Guo, C., Tian, J., Luo, J., Yao, J., Pang, R., Shen, H., Peng, C., Liu, T., … Zheng, X. (2020). Longitudinal characteristics of lymphocyte responses and cytokine profiles in the peripheral blood of SARS-CoV-2 infected patients. EBioMedicine, 55, 102763.https://doi.org/10.1016/j.ebiom.2020.102763
Liu, J., Liu, L., Kang, W., Peng, G., Yu, D., Ma, Q., Li, Y., Zhao, Y., Li, L., Dai, F., & Wang, J. (2020). Cytokines/Chemokines: Potential Biomarkers for Non-paraneoplastic Anti-N-Methyl-D-Aspartate Receptor Encephalitis. Frontiers in Neurology, 11.https://www.frontiersin.org/articles/10.3389/fneur.2020.582296
Byun, J.-I., Lee, S.-T., Moon, J., Jung, K.-H., Sunwoo, J.-S., Lim, J.-A., Kim, T.-J., Shin, Y.-W., Lee, K.-J., Jun, J.-S., Lee, H. S., Lee, W.-J., Kim, Y.-S., Kim, S., Jeon, D., Park, K.-I., Jung, K.-Y., Kim, M., Chu, K., & Lee, S. K. (2016). Distinct intrathecal interleukin-17/interleukin-6 activation in anti-N-methyl-d-aspartate receptor encephalitis. Journal of Neuroimmunology, 297, 141–147. https://doi.org/10.1016/j.jneuroim.2016.05.023
Lancaster, E. (2016). The Diagnosis and Treatment of Autoimmune Encephalitis. Journal of Clinical Neurology (Seoul, Korea), 12(1), 1–13. https://doi.org/10.3988/jcn.2016.12.1.1

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

image - A link between COVID-19 and autoimmune encephalitis?

IVIG Side Effects: When to Seek Medical Attention

by Alanna | Aug 8, 2023 | Special Series

August 8, 2023 | By Cindy Berry, RN, BSN. Reprinted with permission from IG Living

Introduction from the IAES Blog Team:

It is with great pleasure that IAES presents to you an article reprinted with permission by IG Living Magazine.

The IG (or Immune Globulin) community not only produces an online magazine but also a podcast and offers other resources for all those taking IG, interested in having IG as a part of their medication regime and for all those interested in IG in general. For further resources from IG Living feel free to peruse at their content at: https://www.igliving.com/magazine/subscribe.aspx

For many AE Warriors, IVIG is a staple in our treatment toolbox.

IVIG infusions are something most with AE have had at one time or another as a treatment option.

Many with AE, our caregivers and loved ones have been curious about the possible side effects of IVIG. What should we expect, how concerned should we be, when to seek medical intervention? IG Living has done a wonderful job in answering many of our IVIG side effects questions. We hope you gain as much information as we have, and we thank IG Living for let us republish this wonderful article.

——

This IGL blog is sponsored by NuFACTOR Specialty Pharmacy.

Understanding the most common, mild side effects of immune globulin (IG) therapy is important when setting proper expectations during treatment. It is also important to recognize when unexpected side effects occur, and what to do about them.

It’s necessary to take measures to minimize side effects when receiving IG therapy . These measures include staying well-hydrated, taking pre-medications as ordered and listening to your body. But, even when diligently taking these measures, unexpected side effects sometimes occur. With the exception of anaphylaxis, most of these side effects generally occur after an infusion, and they are usually considered either moderate or severe. In every instance, they need to be evaluated by a physician, and in some cases, medical intervention is necessary.

Moderate side effects are those that usually affect your daily activities such as going to work, sleeping well, eating and even showering. The most common reported moderate side effect is a headache lasting more than 24 hours with a pain rate of 6 to 8 on a scale of 1 to 10. This means taking medications such as Tylenol or Advil does not help alleviate symptoms. Sometimes, this headache can progress into a more serious headache called aseptic meningitis.

Aseptic meningitis occurs when the IG drug has caused irritation of the meninges in the brain, resulting in symptoms that present like meningitis. This unexpected side effect can occur during an infusion or after an infusion. Patients experience an excruciating headache, as well as neck pain and stiffness, and generally, patients will have severe sensitivity to light. Vomiting is also very common. If these symptoms present, the patient should go to the emergency room for evaluation. Usually, IV hydration, IV steroids, IV antiemetics and IV pain medication are given to help alleviate symptoms. With proper medical intervention, patients usually feel better within 24 to 48 hours.

Renal dysfunction is another unexpected side effect that can be caused by IG therapy. This side effect is more common in patients who are over the age of 65, and who have pre-existing conditions such as hypertension and diabetes. Patients should pay particular attention to any changes in urination, including color changes (dark or amber colored urine can signify a change in kidney function) and a decrease in urine output. If either or both symptoms are experienced, a physician should be notified, and the patient should be evaluated immediately. Since renal dysfunction is a potential serious adverse event, it is important to have periodic renal testing, which is easily accomplished with blood work ordered by a physician.

Thrombolytic events, or clot formation, have been reported in very few cases. Although this is a very uncommon side effect, it is important to be aware of the signs and symptoms. Patients at greatest risk include those with a history of thrombotic events, history of diabetes, advanced age, multiple cardiovascular risk factors, impaired cardiac output and long periods of immobilization. If a clot is formed, this usually occurs after an infusion. Symptoms of a possible thrombolytic event include severe chest pain and difficulty breathing, which could be an indication of a pulmonary embolism or possible myocardial infarction. If severe chest pain is experienced at any time, immediate attention is needed, and 911 should be called.

The final, most serious side effect that is unlikely to occur is anaphylaxis. It is the least-likely serious side effect that can occur. Anaphylaxis usually occurs within the first 15 to 30 minutes of an infusion. It is characterized by a sudden onset of any of the following symptoms: difficulty breathing (chest tightness, bronchospasms, wheezing), changes in the gastrointestinal system (severe cramps, vomiting, diarrhea), cardiovascular changes (low pulse rate, high pule rate, hypotension/shock, chest pain) or skin changes (hives, angioedema, rash). If anaphylaxis is suspected, 911 should be called immediately. If it occurs during your infusion, your nurse will administer emergency medications to help control the symptoms. Medical attention is required and necessary, and 911 should be called despite the administration of emergency medications.

Although the list of unexpected side effects may seem scary, it is important to remember that while most patients will experience mild side effects, they do not typically experience serious ones. In any event, it is always important to understand them and to have your physician’s number ready. Always inform your healthcare team of any changes in response to IG therapy.

Immune Globulin Therapy Side Effects When receiving IG therapy – either by IV administration or subcutaneous administration, it is important to understand the difference between side effects that are expected and side effects that are not expected. Since side effects may have an onset after drug administration, it is important for the patient and/or caregiver to identify when to seek medical attention.

—

Click here or the image below to subscribe to our mailing list :

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

A Mighty Miracle

by Alanna | Jul 26, 2023 | AE Warrior Personal Stories

July 26, 2023 | By Rebecca Jablon

Introduction from the IAES Blog Team:

It is our honor and pleasure to bring to you the story of a young boy’s journey into the uncertain and terrifying world of being diagnosed with Autoimmune Encephalitis from the heart of a mother. A mother who thru faith, resilience, determination and, above all, love found help across the miles and a fierce desire to assist others and raise awareness!

——-

Whether you are a parent or grandparent to a child who has been thrown into the world of autoimmune encephalitis, or into the world of rare disabilities ( or a physician, therapist, special education teacher, or social worker…) I was moved to publish my story “To Add a Miracle” to provide you with further insight and strength. I wrote with absolute candor and honesty, sprinkled with a bit of humor, in an attempt to accurately portray the emotional rollercoaster that we have experienced.

Our son, Yehuda’s, steep fall into the world of autoimmune encephalitis began just days after the holiday of Hanukkah, the Holiday of Miracles, four- years ago, at the age of five. He was born just days before the Holiday of Hanukkah. As I approached the Hanukkah season this year and Yehuda’s ninth birthday, I suddenly felt a strong drive to sit down. And to write. And to write more. Perhaps this is not a coincidence.

At the beginning of our journey/ FALL into the unknown, the International Autoimmune Encephalitis Society stood as one of the lights illuminating our absolute confusion and darkness. The brave volunteers, often facing current or past struggles of their own, held out lights of information, direction, medical advice, and most importantly glimmers of hope, for a family struggling far away in Israel, where knowledge of autoimmune encephalitis in the medical world is even more limited. At the time, I was able to connect with only one mother living here in Israel, who was able to hold my hand and guide me on our journey. I gathered additional armor and strength from the mothers whom I could reach out to through the AE website. I was ready to grab onto anything and anyone who understood. I was desperate. Who or What had overnight stolen our son’s words, skills, and identity?

How can a mother accept an unknown or rare diagnosis?

How can she grapple with an experimental and even further unknown treatment plan?

How can a mother not throw up her hands in total despair when top neurologists eventually throw up their hands?

How can she survive when she screams out, yet no one can answer,

“What suddenly happened to my five- year old son?”

While many books have been written highlighting the challenges of raising a child with disabilities, as you all are painfully aware, autoimmune encephalitis is a recently discovered and often misunderstood illness with a shocking onset. My writing of To Add a Miracle was fueled by my intense desire to spread awareness and hope, to strengthen mothers, fathers, and families, as they bravely journey toward recovery or increased acceptance and strength.

I will never forget when my then five-year-old Yehuda desperately called out to me as I left his bedroom one night, marking the beginning of our descent into the unknown,

“Imma (Hebrew for Mother), my brain is broken. If I die, will you…”

Total confusion, darkness, and piles of despair. I wish that at the sudden onset of Yehuda’s illness, I had known about, and did not have to wait to discover the collective voices of the International Autoimmune Encephalitis Society, to call out to me, and to hear my cries… to help me to not feel totally alone in my struggles.

It is my hope and prayer that my book will provide you with an additional dose of strength in order to navigate the bumpy ride, that is our lives with special needs children. It is my dream to be able to continue to help other parents, using all of the tools, both medical and sometimes emotional, that I have gathered upon our journey with Yehuda. As I describe in the book, there is nothing that gives me more comfort than seeing another child and family progress and advance, even if that particular treatment did not advance Yehuda.

Someone recently asked me, “So what do you mean by adding a miracle?” Please order and delve into my book to find out. There is no quick answer.

Thank you to all of the administrators and volunteers of the International Autoimmune Encephalitis Society, for allowing me to take part in their holy work in my attempt to spread awareness of autoimmune encephalitis, through the writing of my book.

To Add A Miracle on Amazon

Book Description:

To Add A Miracle details with raw honesty, sprinkled with moments of humor and laughter, the dark and light shadows of the Jablon family’s journey; the story also highlights the tremendous strength of Yehuda’s siblings and selected “messengers of miracles” along the way.

With no filters, the story tells the author’s emotional journey as a mother in distress, facing piles of despair, culminating in a greater acceptance of the unacceptable, and a powerful recognition of the miracles that Yehuda has added to her family’s life.

While many books have been written highlighting the challenges of raising a child with disabilities, autoimmune encephalitis is a recently discovered and often misunderstood illness with a shocking onset.

The writing of To Add A Miracle was fueled by the author’s intense desire to spread awareness and hope, to strengthen mothers, fathers, families, and medical practitioners, as they bravely journey toward recovery or increased acceptance and strength.

Rebecca Jablon, the author of To Add A Miracle, tells the story of her sudden and dramatic fall into the world of autoimmune encephalitis, and resulting diagnosis of autism for her son, Yehuda.

How can a mother accept an unknown or rare diagnosis?
How can she grapple with an experimental and even further unknown treatment plan?
How can a mother not throw up her hands in total despair when top neurologists eventually throw up their hands?
How can she survive when she screams out, yet no one can answer, “What suddenly happened, overnight, to my five-year-old son?!!”

No stranger to the world of rare illnesses, Yehuda’s sudden overnight descent into the unknown rocked the author’s family. Who or What had stolen her son’s words, skills, and identity?

Click here or the image below to subscribe to our mailing list :

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Who do you become when you are sleepy?

by Alanna | Jul 11, 2023 | PennNeuroKnow

July 12, 2023 | by Lindsay Ejoh, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

For all of us with AE, sleep can be an ongoing issue whether it be too little, too much, interrupted sleep cycles and everything in-between! Sleep issues often go hand in hand with an AE diagnosis. PNK author Lindsay Ejoh wrote this piece for the PNK weekly series and graciously gave IAES permission to publish it in our monthly series. We hope you find this as informative as we have!

——-

Introduction

Sleep experts recommend that most adults get 7-9 hours of good-quality sleep each day^1,2 to avoid the myriad of issues that can occur when the brain and body are sleep-deprived. We all know what it is like to be tired. We may feel cranky and sluggish, as well as physically and mentally exhausted. We may also face issues with memory and attention³, emotional regulation, and diminished sex drive^4,11. It is hard to feel like yourself when sleep-deprived- so what occurs in the brain during sleep deprivation, and how does it affect our daily lives?

Memory

As a child, I remember learning to trick my mother, a sleep-deprived emergency room nurse that worked the night shift, by asking her for permission to do things while she was coming home from work in the mornings, half-asleep. When I’d approach her in bed to ask for permission to go on a sleepover across town or to eat food we were saving for an occasion, she would always say yes. Eventually, she caught on, and warned me against waiting until she was sleepy to get my way, but the reason it worked at first is because sleep deprivation impacts decision-making⁵.

It also affects short-term memory, so as a result, my mother would never remember giving her approval. Long-term memory is affected as well, as sleep is very important for consolidation, or storage of memories. This is also why you may not remember everything you studied after cramming for an exam all night.

Reaction time

Being awake is not the same thing as being alert. When we are sleepy, we tend to have slow reaction times, or time to respond to a change in our environment. This can have devastating effects for those who operate cars and other heavy machinery while sleepy and can be dangerous for people who work with under these conditions. Sleep deprivation can make you 70% more likely to get into work-place accidents, which happen at higher rates in people with insomnia⁶. Additionally, missing just a couple hours of sleep can substantially increase the risk of having a car accident⁷. It may seem in the moment like you can stay awake while driving, but as explained in a previous NeuroKnow article, going 24 hours without sleep can be just as dangerous as driving drunk.

Changes in the brain

Sleep deprivation impacts many regions of your brain, but two are of notable importance: amygdala and prefrontal cortex.

Amygdala

Scientists can measure brain activity by taking functional magnetic resonance imaging (fMRI) scans. Using this method, researchers found that sleep deprivation leads to a hyperactive amygdala³. The amygdala is critical for emotional regulation, and its dysfunction may be related mood issues that occur from sleep deprivation. A single sleepless night can trigger a 30% increase in anxiety levels⁹, due to the loss of ability to regulate emotions or deal with stress, and people with anxiety disorders also have hyperactive amygdalae when faced with unpleasant changes in their environment¹⁰. In other words, sleep deprivation causes disruption in emotional centers in the brain, which is linked to increased anxiety.

Prefrontal Cortex

Another brain region with altered activity during sleep deprivation is the prefrontal cortex, which is important for rational thinking and decision-making³. This region has decreased activity during sleep deprivation, and these activity patterns are associated with impaired judgment, a common symptom of sleep deprivation.

Chronic sleep deprivation and sleep apnea

Most of us have experienced sleep-deprivation in our lives, but for some, it is the norm. People who suffer from inadequate sleep for a prolonged period of time (weeks to years) are in a state of chronic sleep deprivation⁶. Many people wake up in the mornings feeling symptoms of sleep deprivation despite getting a long night of sleep, which may be indicative of a sleep disorder known as sleep apnea. Patients with sleep apnea wake up over a hundred times throughout the night, due to difficulty breathing¹². A research lab in Australia found that sleep apnea patients have altered brain activity during wakefulness¹³. Certain parts of their brains “go offline” briefly, despite being awake, and brain activity resembles that of a sleeping person^14. Sleep disorder patients aren’t the only ones that experience this- it can occur from other forms of sleep deprivation. When sleep intrudes into the waking brain, this can lead to errors in tasks like driving. Despite being abnormal for humans, this brain activity phenomenon is not uncommon in the animal kingdom. Some animals like seals and dolphins sleep with half of their brains “awake” while the other halves are “asleep.”

Conclusion

Neuroscientists are working to understand the neurobiological consequences of sleep deprivation, so that we can inform and treat people who must continue to perform daily tasks despite running on little sleep. Though harmful for the brain, sleep deprivation is a normal part of daily life for 30-40% of US adults¹⁵, including parents of newborns, procrastinating college students, night-shift workers, military and medical personnel, sleep disorder patients, and many others. We live in a sleep-deprived society, where people are often celebrated for trading rest for productivity. I encourage you to take this as your sign to go to bed early tonight- you are not yourself when you’re sleepy!

References

Watson NF, Badr MS, Belenky G, et al. Recommended amount of sleep for a healthy adult: a joint consensus statement of the American Academy of Sleep Medicine and Sleep Research Society. Sleep. 2015;38(6):843–844.
Consensus Conference Panel, Watson, N. F., Badr, M. S., Belenky, G., Bliwise, D. L., Buxton, O. M., Buysse, D., Dinges, D. F., Gangwisch, J., Grandner, M. A., Kushida, C., Malhotra, R. K., Martin, J. L., Patel, S. R., Quan, S. F., Tasali, E., Non-Participating Observers, Twery, M., Croft, J. B., Maher, E., … Heald, J. L. (2015). Recommended Amount of Sleep for a Healthy Adult: A Joint Consensus Statement of the American Academy of Sleep Medicine and Sleep Research Society. Journal of clinical sleep medicine : JCSM : official publication of the American Academy of Sleep Medicine, 11(6), 591–592. https://doi.org/10.5664/jcsm.4758
Krause, A. J., Simon, E. B., Mander, B. A., Greer, S. M., Saletin, J. M., Goldstein-Piekarski, A. N., & Walker, M. P. (2017). The sleep-deprived human brain. Nature reviews. Neuroscience, 18(7), 404–418. https://doi.org/10.1038/nrn.2017.55
Chen, K. F., Liang, S. J., Lin, C. L., Liao, W. C., & Kao, C. H. (2016). Sleep disorders increase risk of subsequent erectile dysfunction in individuals without sleep apnea: a nationwide population-base cohort study. Sleep medicine, 17, 64–68. https://doi.org/10.1016/j.sleep.2015.05.018
Rasch, B., & Born, J. (2013). About sleep’s role in memory. Physiological reviews, 93(2), 681–766. https://doi.org/10.1152/physrev.00032.2012
Suni, E. (2023, April 5). The relationship between sleep and workplace accidents. Sleep Foundation. Retrieved April 26, 2023, from https://www.sleepfoundation.org/excessive-sleepiness/workplace-accidents#references-197012
AAA. (2016, December 6). Missing 1-2 hours of sleep doubles crash risk: Study reveals the dangers of getting less than 7 hours of sleep. ScienceDaily. Retrieved April 26, 2023 from www.sciencedaily.com/releases/2016/12/161206110235.htm
Wong, M. M., Robertson, G. C., & Dyson, R. B. (2015). Prospective relationship between poor sleep and substance-related problems in a national sample of adolescents. Alcoholism, clinical and experimental research, 39(2), 355–362. https://doi.org/10.1111/acer.12618
Ben Simon, E., Rossi, A., Harvey, A. G., & Walker, M. P. (2020). Overanxious and underslept. Nature human behaviour, 4(1), 100–110. https://doi.org/10.1038/s41562-019-0754-8
Etkin, A., & Wager, T. D. (2007). Functional neuroimaging of anxiety: a meta-analysis of emotional processing in PTSD, social anxiety disorder, and specific phobia. The American journal of psychiatry, 164(10), 1476–1488. https://doi.org/10.1176/appi.ajp.2007.07030504
Sleep Center of Middle Tennessee. (2022, June 22). Sleep deprivation and its effects on the brain. Sleep Centers of Middle Tennessee. Retrieved April 26, 2023, from https://sleepcenterinfo.com/blog/sleep-deprivation-effects-on-brain/
Slowik, J. M., Sankari, A., & Collen, J. F. (2022). Obstructive Sleep Apnea. In StatPearls. StatPearls Publishing.
Hung, C. S., Sarasso, S., Ferrarelli, F., Riedner, B., Ghilardi, M. F., Cirelli, C., & Tononi, G. (2013). Local experience-dependent changes in the wake EEG after prolonged wakefulness. Sleep, 36(1), 59–72. https://doi.org/10.5665/sleep.2302
Mannix, L. (2019, January 27). Your brain could be sleeping … even while you’re awake. The Sydney Morning Herald. Retrieved April 26, 2023, from https://www.smh.com.au/national/your-brain-could-be-sleeping-even-while-you-re-awake-20190124-p50tgg.html
Centers for Disease Control and Prevention. (2022, November 2). Adults – sleep and sleep disorders. Centers for Disease Control and Prevention. Retrieved April 26, 2023, from https://www.cdc.gov/sleep/data-and-statistics/adults.html

Cover image by Karollyne Videira Hubert on Unsplash

References

Zeng, J. & James, L. C. Intracellular antibody immunity and its applications. PLOS Pathog. 16, e1008657 (2020).
CDC. COVID-19 and Your Health. Centers for Disease Control and Preventionhttps://www.cdc.gov/coronavirus/2019-ncov/your-health/about-covid-19/antibodies.html (2020).
Elkon, K. & Casali, P. Nature and functions of autoantibodies. Nat. Clin. Pract. Rheumatol. 4, 491–498 (2008).
Hermetter, C., Fazekas, F. & Hochmeister, S. Systematic Review: Syndromes, Early Diagnosis, and Treatment in Autoimmune Encephalitis. Front. Neurol. 9, 706 (2018).
Graus, F. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 15, 391–404 (2016).
Anti-NMDA receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-nmda-receptor-encephalitis/.
Anti-AMPAR encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-ampar-encephalitis/.
Anti-GABAA receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-gabaa-receptor-encephalitis/.
Anti-GABAB receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-gabab-receptor-encephalitis/.
LGI1-antibody encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/lgi1-antibody-encephalitis/.
CASPR2-antibody encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/caspr2-antibody-encephalitis/.
Malter, M. P., Helmstaedter, C., Urbach, H., Vincent, A. & Bien, C. G. Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann. Neurol. 67, 470–478 (2010).
Voltz, R. & Eichen, J. A Serologic Marker of Paraneoplastic Limbic and Brain-Stem Encephalitis in Patients with Testicular Cancer. N. Engl. J. Med. (1999).
Graus, F. & Dalmau, J. Paraneoplastic neurological syndromes in the era of immune-checkpoint inhibitors. Nat. Rev. Clin. Oncol. 16, 535–548 (2019).
Lancaster, E. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. (2011).

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

image - Who do you become when you are sleepy?

You’re alive, you’re breathing, and growing older is a privilege

by Alanna | Jun 27, 2023 | AE Warrior Personal Stories

June 28, 2023 | By M Ledferd

Introduction from the IAES Blog Team:

The staff at IAES brings to you the inspirational thoughts and feelings of a mighty AE Warrior shortly after his AE journey began. M Ledferd has put into words with heartfelt elegance the way we have all felt at one point or another on our journey. His gift with prose has brought to life our deepest feelings and resonated with our hearts and minds. We hope you enjoy this as much as we have! Thank you M!

——-

For those AE survivors, let gratitude carry us. For the caretakers, bless you. For those still struggling, please keep fighting the good fight. We are all here for you.

When I first awoke in the hospital, I felt like a 90-year-old man. A weak, tired old man with no autonomy. A man that could barely move or speak. A man at the end of his life.

Coming in and out of consciousness, I had a lot to think about. (Because I had nothing else to do.)

I realized that people generally saw their lives in stages: a beginning, middle and end. With a middle age that seemed to go on forever. That’s because we have no idea when the end is near. It’s hard to gauge and probably doesn’t even exist in most people’s minds. We simply can’t fathom it. It’s so unknown and far away. But as sure as the sky is blue it eventually reaches us all.

We’re all so different yet all the same. We go from rambunctious, fearless little kids with endless curiosities, to busy, hard-working adults, generating income so that hopefully one day we can retire, where the hours stretch on for days (just like it did when we were little kids).

In retirement, we are sold that we can do anything we want. From reading books to painting, to just chilling on the beach, or seeing the world by cruise ship, or just slow swinging on a porch with an old cat in our lap. I dunno, it’s different for everyone. What I do know is that I had obviously miscalculated a long middle for a short end. Crazy how that happens.

Laying there, motionless, with the chirps and beeps of hospital equipment, the days and nights blurred together. I didn’t know what day it was. But it didn’t matter as time had no relevance. I realized I has spent so much of my life working hard and saving up for a future that would never come. I felt stupid. Decades of grinding, all for what.

I tried to stay positive. To look on the bright side of things. To reflect. I had my fair share of adventures and vacations. My fair share of accomplishments, of friendships. I once took a 3-month solo motorcycle trip across the USA (remember that?). Damn, that was cool.

I got to see the world and was even beginning a new family with my wonderful spouse. I regretted not being able to raise my daughter until she was at least 20. Let me live another 20 years, I said, so I can instill in her self-reliance, self-discipline, curiosity, and grit. To let her know that anything is possible. But I knew that all would eventually be ok. My wife is a warrior with a great big supportive family. And I mean, there’s nothing I can do about it now.

I had a lot of feelings but above all I was calm and grateful. I was grateful I got to experience most of what life had to offer. The exciting parts. The sad parts. The whole gamut of human experience from birth to baby—which is more than anyone is guaranteed. Being in that dark, desolate place in my mind. That place where I had no external voice, I still had gratitude. But it was a resigned gratitude. One with plenty of I-couldas, I-wouldas, and I shouldas.

Coming back into consciousness, hearing the same high-pitched, rhythmic beeping from the heart-monitoring machine, I knew I had been there for a very long time. I felt like I wasn’t getting any better. Every day was just like the last. Groundhog Day. I felt like, maybe, I would be in the hospital forever in that state. Even if I hadn’t died I felt like a ghost. To be seen but not to see. To be touched but not to touch. A fly on the wall of a busy hospital with ears instead of eyes. A vegetable frozen in time, with tubes and wires coming out of everywhere.

Then just like that, like some kind of reverse “Benjamin Button” disease, I was blasted back into reality, back to my 40-year-old body. I had aches and pains all over, shed a lot of tears, but, damn, it felt good to sit up on my own, to just breathe again. It had been 23 days, with 16 of them in the ICU. I would spend the next 11 days relearning everything. How to walk. How old my daughter was. How to use my phone. But I was back.

Today (April 12, 2023) marks the 100th day of leaving the hospital. Though I’ve been back probably half a dozen times since, they have all been for check-ups, bone density scans, MRI’s, physical therapy, and all ending with my favorite part—going home.

I am not sure what the point of this post is. I guess it’s for you to envision yourself where I was. A dead man with no future, with the woulda, coulda, shouldas. To put yourself there and see if you would change anything when you were granted your wish. To realize that most everything that stresses you out right now probably doesn’t even matter.

You’re alive, you’re breathing, and growing older is a privilege. Don’t waste it with your head down.

Click here or the image below to subscribe to our mailing list :

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

What are intracellular and extracellular antibodies and what do the differences mean for patients with autoimmune encephalitis?

by Alanna | Jun 13, 2023 | PennNeuroKnow

June 14, 2023 | by Catrina Hacker, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

——-

Introduction

There are many subtypes of autoimmune encephalitis (AE) that vary in their causes, the symptoms that patients experience, and what treatments are most effective. One of several factors that distinguish these different subtypes of AE is whether they involve intracellular or extracellular antibodies. In this post we will explore exactly what these terms mean and how they contribute to the differences between types of AE.

N Engl J Med 2018;378:840-51. DOI: 10.1056/NEJMra1708712 Copyright © 2018 Massachusetts Medical Society

What are intracellular and extracellular antibodies?

When a virus or bacteria enters our body, our immune system mounts an attack to destroy the foreign invader and protect us from harm. If our immune system is like an army ready for battle, then antibodies are like the scouts sent ahead of the battalion, patrolling for signs of a threat. Just like security personnel might scan ID badges to determine who is allowed in a building, each antibody is tasked with looking for a particular feature of something that the body has deemed harmful, called an antigen¹. You might have heard antibodies discussed in reference to COVID-19, where infection with COVID-19 or vaccination can cause your body to produce antibodies that recognize features of the COVID-19 virus². When antibodies are already present in the body, they can recognize the newly-arrived COVID-19 virus and mount an attack more quickly, helping to avoid a more serious infection.

This ability to quickly mount a defense against a threat before getting too sick is what makes antibodies an important part of our body’s immune system army. However, antibodies are only helpful if they recognize and defend against foreign substances that are harmful. Unfortunately, this isn’t the case in AE. Patients with AE have antibodies that bind to proteins found in their own cells, called autoantibodies (the prefix “auto” means self, so autoantibodies are antibodies that bind the body’s own proteins)³. Autoantibodies trigger the body’s immune system to attack itself, leading to the many symptoms of AE.

Each antibody can recognize only a small part of a whole cell, and there are many different parts of a cell that an antibody can recognize. What distinguishes extracellular from intracellular antibodies is whether their antigen (the ID badge they’re looking for) is inside or outside of the cell^1,4. Extracellular antibodies recognize antigens that are on the outer surface of the cell (“extra” meaning outside). Conversely, intracellular antibodies recognize antigens that are inside the cell (“intra” meaning inside). The intracellular antibodies inside the cell trigger a different set of immune reactions than the extracellular antibodies outside of the cell.

Which kinds of AE involve intracellular versus extracellular antibodies?

Subtypes of AE are distinguished by what kind of autoantibody a patient has⁴, which is why they are typically named after the antigen that the autoantibody recognizes. For example, patients with anti-NMDAR AE have antibodies that recognize NMDA receptors. Types of AE associated with antigens outside the cell involve extracellular antibodies and types of AE associated with antigens inside the cell involve intracellular antibodies.

Many of the most common subtypes of AE involve extracellular antibodies^4,5. Most are associated with antibodies that recognize a kind of protein that sits on the surface of the cell called a receptor. Receptors recognize and bind specific molecules and send signals that tell the cell how to respond. The receptors on neurons, a type of brain cell, are especially important because one neuron communicates with another by releasing molecules that can be recognized by the other neuron’s receptors. When antibodies bind the receptors, they activate an immune response and disrupt the ability of those receptors to participate in neural signaling. This leads to the many neurological symptoms of AE. Subtypes with these kinds of antibodies include anti-NMDAR AE⁶, anti-AMPAR AE⁷, anti-mGLUR5 antibody encephalitis^4,5, GlyR antibody encephalitis⁴, anti-GABA_aAE⁸, and anti-GABA_b AE⁹. Several other extracellular antibodies associated with AE have antigens that sit on the cell’s surface and help with neuronal signaling but aren’t receptors themselves. Subtypes of AE with these kinds of antibodies include LGI1-antibody encephalitis¹⁰, CASPR2-antibody encephalitis¹¹, and DPPX-antibody encephalitis^4,5.

Subtypes of AE associated with intracellular antibodies are less common^4,5. One example is GAD-antibody encephalitis¹². Patients with this form of AE have antibodies that target Glutamic Acid Decarboxylase (GAD), a protein found inside the cell that is needed to synthesize GABA, a special type of molecule that is necessary for some kinds of neural signaling. Other subtypes of AE that target intracellular proteins are anti-Hu encephalitis⁵, and Ma2-antibody encephalitis¹³.

How are subtypes of AE associated with intracellular antibodies different from subtypes of AE associated with extracellular antibodies?

One big distinction is that most subtypes of AE associated with intracellular antibodies are also associated with tumors⁴. These subtypes of AE are called paraneoplastic. Paraneoplastic AE can occur when tumor cells express proteins on their surface that are normally expressed elsewhere. Sometimes this includes proteins that are normally found inside healthy neurons. To recognize and fight the tumor, the body’s immune system creates antibodies that recognize these proteins. These antibodies don’t distinguish the proteins found in the tumor cells from the healthy proteins found in neurons, so when they reach the brain, they also bind the naturally-occurring proteins in neurons and trigger the immune response responsible for the symptoms of AE¹⁴.

Patients with subtypes of AE associated with intracellular antibodies also tend to have poorer outcomes and respond worse to immunotherapy than patients with subtypes associated with extracellular antibodies^4,15. This is because many of the symptoms of AE associated with extracellular antibodies are thought to result from the antibodies disrupting the normal function of the cell-surface proteins that they target. Conversely, the presence of intracellular autoantibodies typically accompanies an immune response against neurons more broadly that results in neuronal death. This means that successful treatment can often reverse symptoms of AE resulting from extracellular antibodies, as limiting the action of the antibodies allows the neurons to function normally, whereas even after treatment, symptoms do not typically reverse in subtypes of AE associated with intracellular antibodies, as many neurons have already died. For patients with paraneoplastic AE, removing the tumor is also an important step toward relieving symptoms¹⁵.

Despite general differences in outcomes for subtypes of AE associated with extracellular and intracellular antibodies, early detection and treatment are key to successful outcomes for all subtypes of AE⁴. Determining which type of AE a patient has can have an important impact on how doctors choose to treat and manage the disease. This distinction is also important for researchers developing new treatments and possible cures, as approaches that might work for one type of AE may not work for others. Determining which patients will be most receptive to a particular new treatment leads to better outcomes for clinical trials, which means more treatment options for all patients.

Download PDF

References

Zeng, J. & James, L. C. Intracellular antibody immunity and its applications. PLOS Pathog. 16, e1008657 (2020).
CDC. COVID-19 and Your Health. Centers for Disease Control and Preventionhttps://www.cdc.gov/coronavirus/2019-ncov/your-health/about-covid-19/antibodies.html (2020).
Elkon, K. & Casali, P. Nature and functions of autoantibodies. Nat. Clin. Pract. Rheumatol. 4, 491–498 (2008).
Hermetter, C., Fazekas, F. & Hochmeister, S. Systematic Review: Syndromes, Early Diagnosis, and Treatment in Autoimmune Encephalitis. Front. Neurol. 9, 706 (2018).
Graus, F. et al. A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol. 15, 391–404 (2016).
Anti-NMDA receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-nmda-receptor-encephalitis/.
Anti-AMPAR encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-ampar-encephalitis/.
Anti-GABAA receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-gabaa-receptor-encephalitis/.
Anti-GABAB receptor encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/anti-gabab-receptor-encephalitis/.
LGI1-antibody encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/lgi1-antibody-encephalitis/.
CASPR2-antibody encephalitis. Autoimmune Encephalitis Alliance https://aealliance.org/ae-types/caspr2-antibody-encephalitis/.
Malter, M. P., Helmstaedter, C., Urbach, H., Vincent, A. & Bien, C. G. Antibodies to glutamic acid decarboxylase define a form of limbic encephalitis. Ann. Neurol. 67, 470–478 (2010).
Voltz, R. & Eichen, J. A Serologic Marker of Paraneoplastic Limbic and Brain-Stem Encephalitis in Patients with Testicular Cancer. N. Engl. J. Med. (1999).
Graus, F. & Dalmau, J. Paraneoplastic neurological syndromes in the era of immune-checkpoint inhibitors. Nat. Rev. Clin. Oncol. 16, 535–548 (2019).
Lancaster, E. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. (2011).

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

image - What are intracellular and extracellular antibodies and what do the differences mean for patients with autoimmune encephalitis?

Your Potential Self is Infinite

by Alanna | May 30, 2023 | AE Warrior Personal Stories

May 31, 2023 | By Lisa Lauter. Reposted with permission from Lisa Lauter Journey to Health

A message from IAES Blog Staff:

The entire staff at IAES is very excited to share with all of you a blog published by one of our own members from her own website.

This is an anniversary blog of sorts. Almost 4 years ago to the day IAES published the first blog about Lisa that includes a video clip regarding her AE(LGI1) diagnosis and journey.

Four fast and eventful years have passed. Lisa is doing well along her AE journey, has had her share of ups and downs, has moved, has an active website and has written a book published that is soon to launch. Please feel free to follow Lisa, read her blogs and celebrate her book launch @ https://www.lisalauter.com/

———

Life is Marked by Journeys

What started as a journey north from Texas to hunker down during the pandemic in a little mountain town in British Columbia, evolved into a permanent move back to Canada and the realization of a dream to build our forever home on the edge of a lake, surrounded by mountains. I have to keep pinching myself to believe that’s it’s real, but I wouldn’t be here without supportive friends and family.

The Shady Ladies

I have a group chat with a circle of women who are some of my closest friends. We call ourselves the Shady Ladies – sounds a lot more dodgy than it is. We support each other daily by giving random advice on everything from politics, to fashion, to children, to aging parents, to what the heck should I make for dinner. They are my tribe and they got me through some really dark days. When helping me decide what to write today, one of them said write about “how to cope with multiple projects without losing your mind!” (There may have been a well placed expletive in that sentence). I immediately chirped back, “great idea, but you’re assuming I haven’t lost mine yet!”. Another one said, use a couple of inspirational quotes – the kind you get on tea bags! Hence, today’s blog.

Pace Yourself

If you’ve been following me for a few months, you know that I had a setback recently that threw me for a loop, bringing back symptoms of numbness, tingling and gait changes, and slowing down my cognitive function. It reminded me of the importance of listening to my body and giving it what it needs (sleep, good nutrition, positive mindset, exercise), and learning that sometimes, something’s gotta give.

But here we are! Almost at the finish line and about to move into our new home. The busy-ness isn’t over yet. We still have to move out of the rental we’ve been in for three years, and unpack a truck load worth of stuff and memories that have been in storage all this time. (Pace yourself, Lisa, make space for the daily walks and meditation, eat well and rest.)

Taking Chances

Sometimes you have to take chances to realize dreams. Like my journey to health, we put one step in front of the other, made a ton of lists, and day-by-day checked things off. Sometimes I don’t know how I did it. My camera reel is full of screen shot reminders and my desk and kitchen are plastered with sticky notes and endless lists. Sure, we make mistakes along the way and it’s not always easy, but we try to laugh and remember the big picture. It may sound cliché, but don’t sweat the small stuff. Keep your eye on the prize and have another cup of tea (herbal of course). You never know where you might end up.

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Why are brain injuries so misunderstood?

by Alanna | May 24, 2023 | AE Warrior Personal Stories

May 24, 2023 | By Emerson Jane Browne. Reposted with permission from Dancing Upside Down

A message from IAES Blog Staff:

The staff at IAES is excited to bring to you a blog from author Emerson Jane Browne.

Emerson, a traumatic brain injury (TBI) survivor herself, wrote this very insightful blog about a subject we have all wondered about and tried to understand. Why are brain injuries so complex and misunderstood? We hope you enjoy her insight and thoughts as much as we have.

To find out more about Emerson please visit her website ‘Dancing Upside Down’ at https://www.dancingupsidedown.com/.

——

Why are brain injuries so misunderstood?

We’ve all heard it. “What the heck? You look fine to me!”

Or “It’s been months! Get over this brain injury stuff! Move on already!”

#1 Blame it on Hollywood!

Movies and television have spread so much misinformation about brain injuries it is practically criminal!!

Hollywood has convinced people that if you have a brain injury you have to look it! You have to drag one foot, or have speech problems, or in some manner “look” disabled.

Nothing could be further from the truth! The majority of brain injuries are not visible at all. You try to express how much you are struggling and the response is “You look great!”

And that is the second reason:

#2 You do look great! You look normal.

People are used to thinking of injury in terms of a broken bone. Subconsciously they are looking for the cast, the bandage . . . the visual evidence of your injury. And you don’t have it so their brains draw the conclusion that you must not be injured.

The interesting thing about this, is it is an automatic processing in their own brain and they are not even aware of it.

For example, we learn very early in our life to equate a glowing red burner or flame on a stove as “Hot”. We learn it so well that it becomes automatic. Each time you see a stove you do not laboriously have to think through or reason out that a glowing burner means hot.

The exact same thing is going on in people’s heads in relation to equating injury with visual cues. Their brains have learned to equate the lack of obvious signs of injury with “non-injured”.

And that leads right into the third reason that brain injuries are so misunderstood:

#3 Brains control everything about us!

Until a person’s brain is injured, we do not realize how much we took our brain for granted.

All the little automatic things – from knowing how to add two numbers to knowing what a paintbrush is for or how to read, or when you get to the store remembering to look at a grocery list you just made . . . All the even more important things like who we are, our own sense of identity, the “me” that you have always counted on from the inside . . . all of that is automatic … until it no longer is.

It is dreadfully hard to explain that to someone who has not experienced it because they really don’t get that our brains are essentially us.

Since you still look like you and sound like you, it is extremely hard for another person to grasp how deeply you feel and know you are no longer the you that you were.

And it is even more difficult for someone to grasp how big of a deal that is, and how lost you feel. If you aren’t you on the inside then who are you? And are you always going to be like this? Are you ever going to get you back?

And that brings us to the fourth reason brain injuries are so misunderstood:

#4 Brains take a very long time to heal and remap!

We are used to the healing we have seen all our life – the cut that heals in a week, the broken bone that heals in six, the bad sprain that heals in ten. Heck, after open-heart surgery most people are able to return to work in about eight weeks!

But think about your brain. It took years to develop! That is what the majority of childhood and young adulthood is about – developing the brain. Then, if you are an adult, you have had even more years of refining your neural network.

The brain is a very complex neural network of connections. The brain strengthens and speeds up the neural pathways we use the most. A brain injury disturbs those delicate intricate pathways and connections.

Healing and regrowing of neurons is slow.

Sadly nerve tissue is some of the slowest growing tissue in our body – likely because of how complex it is. Then, in addition, remapping all the intricate connections takes an even longer time.

So how can I get people to understand my brain injury?

Show them this article.

I am not saying that because I authored this article. It is the reverse. I authored the article specifically so you can show it to friends and family.

Show them the High IQ TBI article – there is a lot of information in there no matter what someone’s IQ.

Communicate

Talk to the people you need to educate. And understand that you do not need to educate everyone. Energy is limited when you have a brain injury. Choose the people who you need to have understand what you are going through.

And choose people who want to understand. You will find that those are the people who really care about you.

Use the term “brain holes”.

I find using the term “brain holes” works well. Yes, it is not a medical term. But naming a problem a “brain hole” is creates a visual image which makes it easier for people to understand.

Use Before and After Examples

Do your best to use examples of how things used to work/feel in comparison to how they now work/feel. Again, using descriptions that people can relate to helps a lot.

For instance, I used the analogy of going from a sleek car with a smooth automatic transmission to suddenly finding myself in a clunker with a standard transmission with a clutch that keeps slipping.

Some Never Will. Let them Go.

I wish I did not have to say that!

But sadly, letting go of friends and even family is one of the biggest heartbreaks of having a TBI.

Some people will not want to try to understand. They are adamant in their beliefs and opinions that you are “faking it to get attention”; that “you need to just get over it”; that you are “not really injured” because you “look fine”. Some people will get tired of trying to understand. They will get impatient with the process. Most people cannot truly grasp or understand how extremely slow and incremental brain healing and remapping is.

They may have supported you and been understanding for a while but somewhere along the line they paste a label on you. And they may never bother to come back around to see if anything changed.

Lastly: Blog

Yes, I am serious. Just do a real simple one. Ask a friend to help you set it up. It is dead-easy on WordPress.com or Google Blogger. (Use a pen name or just your initials because it will free you up to not worry about what you say or any perfection issues. Plus, no one will find it if they google your name.)

Write. Just write. Just write what is going on for you.

Writing will help YOU get a grasp on what is going on, which will then make it easier for you to explain it to others. Writing will also help you be more compassionate with yourself. Interestingly, as you become more compassionate with yourself, others will become more compassionate with you too!

Plus, for those who are truly trying to understand and be supportive, sometimes reading what you have written is the best way to help them grasp what it is like for you on the inside.

Question: What problems are you having being understood? What is working to help others understand?

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Honoring Mothers in the Autoimmune Encephalitis Community

by Alanna | May 12, 2023 | Holiday Stories

May 13, 2023 |by Tabitha Orth, IAES President

Honoring Mother’s in the Autoimmune Encephalitis Community:

Happy Mother’s Day from International Autoimmune Encephalitis Society!

—-

How this piece by Erma Bombeck came to me will always be fresh in my memory. There was a synchronicity to it that touched my heart. Our son Matthew is now 32 years old. When he was four, I was fortunate enough to be accepted into a very elite advocacy training program for people with disabilities called Partners in Policymaking. Raising a child with autism is hard. Attending a yearlong program that met for a 3-day weekend each month was challenging for Jim and me as Matthew required 24/7 care and without back-up, that meant Jim would only get a few hours of sleep a night during those periods.

On this weekend, I was to give a presentation. A break was scheduled right afterward. So, people got up to mingle after my presentation. The room was packed with a few hundred people milling about. As I stepped off the stage, I noticed a woman weaving her way through the crowd toward me. She wore a brown hat and was about a foot shorter than my 5’4”, I vividly recall. She came up to me and handed me a piece of paper, smiled, nodded her head to gesture that she was pleased and that she had completed her task, and then wordlessly turned around and wove her way back through the crowd. I remember following that hat with my eyes wondering at her silent retreat.

Then I looked down at the paper she had given me and saw its age. Three of the edges were dusty brown with the remainder a greyish white that told me it had been kept in a book smaller than the size of the page for many years. The paper itself was from an old-fashioned mimeograph machine from the 1960’s. The texture had that slick feel I recalled from my days in elementary school. This dated the paper back more than thirty years. The crisp edges had worn away years ago as they were now softened, curled and brittle enough with cracked tears in the mimeograph paper all a witness to its age. The print was faded with wavy lined sentences from the imprecise printing of the drum of the machine.

This is what it said.

Choosing “Special” Mothers

Most women become mothers by accident, some by choice, a few by social pressures and a couple by habit.

This year nearly 660,000 women will become mothers of a child with a disability. Did you ever wonder how mothers of children with disabilities are chosen?

Somehow, I visualize God hovering over earth selecting his instruments for propagation with great care and deliberation. As he observes, he instructs his angels to make notes in a giant ledger.

“Armstrong, Beth, son. Patron saint: Matthew. Forrest, Marjorie, daughter, Patron Saint: Cecilia.”

“Rudledge, Carrie, twins, Patron Saint…Give her Gerard. He’s used to profanity.”

Finally, he passes a name to an angel and smiles. “Give her a disabled Child.” The angel is curious. “Why this one, God? She’s so happy.”

“Exactly,” smiles God. “Could I give a disabled child to a mother who does not know laughter? That would be cruel.”

“But, has she patience?” asks the angel.

“I don’t want her to have too much patience, or she will drown in a sea of self-pity and despair. Once the shock and resentment wear off, she’ll handle it.”

“I watched her today. She has that feeling of self and independence that is so rare and so necessary in a mother. You see, the child I’m going to give her has his own world. She has to make it live in her world and that’s not going to be easy.”

“But, Lord, I don’t think she even believes in you.”

God smiles, “No matter. I can fix that. This one is perfect, she has just enough selfishness.”

The angel gasps. “Selfishness, is that a virtue?”

God nods. “If she can’t separate herself from the child occasionally, she’ll never survive. Yes, here is a woman whom I will bless with a child less than perfect. She doesn’t realize it yet, but she is to be envied. She will never take for granted a spoken word. She will never consider a step ordinary. When her child says ‘Momma’ for the first time, she will be present at a miracle and know it. When she describes what a sunset makes her feel like to her child, she will see it as few people ever see my creations.”

“I will permit her to see clearly the things I see…ignorance, cruelty, prejudice…and allow her to rise above these. She will never be alone. I will be at her side every minute of every day of her life because she is doing my work as surely as she is by my side.”

“And what about her Paton Saint?” asks the angel, his pen poised in midair.

God smiles. “A mirror will suffice.”

By: Erma Bombeck

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Treatments for Autoimmune Encephalitis

by Alanna | Apr 26, 2023 | PennNeuroKnow

April 26, 2023 | by Sophie Liebergall, PennNeuroKnow and IAES Collaboration

A message from IAES Blog Staff:

The staff at IAES is proud to present to all of you another wonderful article/blog from the amazing team at PennNeuroKnow. Since 2019 IAES has been extremely lucky to be in partnership with the PennNeuroKnow(PNK) team to help us all better understand complex medical issues related to AE and neurology in general. The talented PNK team continues to keep us up-to-date and help clarify the complexities we face each day along our AE journey, and we are eternally grateful! You can find out much more about this stellar group at: https://pennneuroknow.com/

——-

Introduction

Though it can be challenging for doctors to correctly identify and diagnose autoimmune encephalitis (AE), once patients do indeed receive a proper diagnosis there are treatment options that can go a long way in alleviating their symptoms sending them down the road to recovery. A recent study reports that 94% of patients with AE have significant improvement in or complete resolution of symptoms in the first few years after their diagnosis.^1,2 One important key to success is promptly starting treatment which both reduces the likelihood of long-term symptoms and prevents relapses.

The job of your body’s immune system is to find and eliminate invaders, like bacteria and viruses, that may be harmful. But in the case of AE, the immune system mistakes the brain as an invader and mounts an attack, leading to inflammation in the brain.³ This inflammation is what causes the symptoms of AE, like hallucinations, memory problems, and seizures. Therefore, all current medical treatments for AE are aimed at decreasing inflammation.⁴ But even if the ultimate goal is always to reduce brain inflammation, there may be slight variations in the choice of therapies depending on the type of AE and the patient’s unique medical history.

Physicians divide the treatments for AE into first-line and second-line therapies. First-line therapies are treatments that doctors generally prescribe first when a patient is diagnosed with AE. Second-line therapies are treatments that doctors reach for when the first-line therapies didn’t work, or if there are lingering symptoms following initial improvement with first-line therapies.

In this article, we’ll walk through some of the common treatments for AE, why doctors may or may not choose them for a given patient, and how these treatments are thought to reduce AE symptoms.

First-Line Treatments

Steroids

If you or a loved one has been diagnosed with AE, you’re probably familiar with steroids, the medicine that doctors often use first when treating AE. When many people hear the term “steroids,” they think of Barry Bonds or other professional athletes who have used performance-enhancing drugs to get an edge on the competition. But in reality, “steroids” is an umbrella term used to describe a group of chemicals that share a similar shape. Whereas athletes looking to circumvent the rules use steroids called anabolic steroids, doctors treating AE prescribe steroids called glucocorticoids.⁴

Though doctors can administer glucocorticoids to a patient as a pill or in an IV, we actually make glucocorticoids naturally in our bodies all the time! Our homemade glucocorticoids are essential for a wide range of our bodily functions – from controlling how our body manages sugars and fats, to telling our brain to be alert to our surroundings, to damping down inflamation.⁵ When prescribing glucocorticoids to patients with AE, doctors try to take advantage of the anti-inflammatory properties of these chemicals.

How exactly do glucocorticoids put the brakes on inflammation? They act quickly and powerfully at the source of inflammation: the cells of your immune system (Figure 1).⁵ Once they breach the walls of an immune cell, glucocorticoids enter the nucleus, which serves as the control center of a cell. It’s in the nucleus that the cell writes out the instructions for making the proteins that it needs to mount an immune attack. By breaching this nucleus control center, glucocorticoids can override the machinery that the cell uses to write these instructions. This ultimately prevents the immune cells from causing inflammation.

glucocorticoid mechanism summary V1 500x358 - Treatments for Autoimmune Encephalitis

Figure 1. How do glucocorticoids treat AE? Glucocorticoids enter the nucleus of an immune cell, where they override the messages that the cell writes as it tries to make inflammatory proteins.

Unfortunately, glucocorticoids don’t just interfere with the instructions that immune cells use for making inflammatory proteins. They also interfere with the instructions that many other kinds of cells in the body rely on for carrying out their own important functions.⁶ For example, glucocorticoids can affect the instructions that the cells in your bones use to tell themselves to grow and retain their strength. This can lead to the weakening of your bones, which is a common side effect of glucocorticoids.⁷ Other side effects include problems with your body’s metabolism, like the redistribution of body fat, as well problems with your skin, like impaired wound healing.⁶ When patients with AE are first diagnosed, they are often very sick, so very high doses of glucocorticoids may be required to stabilize their condition.⁸ But as AE symptoms improve, doctors try to slowly reduce the amount of glucocorticoids that a patient is prescribed to prevent some of the harmful and uncomfortable side effects of these powerful medications.

Plasma Exchange (PLEX)

Rather than targeting the inner workings of the immune cells, other treatments for AE target the proteins that are made by the immune cells. One type of protein that immune cells make is called an antibody. Antibodiesselectively stick to invaders and flag them for destruction by other cells in the immune system.⁹ But in the case of AE, the body accidentally makes antibodies against its own proteins in the brain. When the immune system sees these flags, it mistakenly attacks the healthy brain.

Plasma exchange (PLEX) is a therapy that tries to remove these antibodies that erroneously tell the immune system to attack the brain.¹⁰ Antibodies are generally transported in the plasma, which is the liquid-y component of blood. During PLEX therapy, tubes are placed in your veins so that your blood can pass through a machine as it is pumped around your body (Figure 2). This machine acts like a coffee filter, separating the liquid part of your blood (the coffee) from the blood cells (the grounds). Because the liquid part of your blood contains the harmful antibodies, the liquid is discarded and replaced with the plasma of a healthy donor. This healthy plasma is then recombined with your own blood cells that were trapped in the coffee filter, and sent back into your body through another tube.

PLEX summary V2 500x279 - Treatments for Autoimmune Encephalitis

Figure 2. What happens during plasma exchange (PLEX) therapy? Whole blood is removed from the patient’s vein, then separated into its plasma and red blood cell components. The patient’s plasma is discarded and replaced with donor plasma, which is recombined with the patient’s red blood cells and returned to the patient’s blood stream.

PLEX is generally safe and effective, and it can be especially useful for patients who are particularly vulnerable to the side effects of glucocorticoids.⁸ However, a major downside of PLEX is that it requires the placement of the tubes that are used to remove and return the blood to the body for the duration of the treatment. These tubes can be a source of infection or bleeding, and can make it logistically challenging for patients to receive PLEX if they aren’t already in the hospital.

Intravenous Immune Globulin (IVIG)

Intravenous immune globulin (IVIG) is another AE treatment that tries to interfere with the antibodies that mistakenly target a patient’s healthy brain in AE. Our blood contains thousands of different antibodies, most of which are designed to target the foreign invaders that we have encountered during our lifetimes. IVIG is the result of taking the blood of thousands of different people, extracting the antibodies from that blood, and then combining the antibodies of all of the different donors.¹¹ This creates a very concentrated slushy of thousands and thousands of antibodies that target all sorts of different proteins. When IVIG is administered to a patient, these antibodies then enter their bloodstream and circulate with the rest of the patient’s blood.

IVIG summary V1 500x323 - Treatments for Autoimmune Encephalitis

Figure 3. What is intravenous immune globulin (IVIG) therapy? The antibody-containing serum of thousands of donors is combined and then administered to the patient through an IV.

Given that AE is caused by a rogue antibody, it may seem crazy that doctors would give patients many more highly concentrated antibodies to treat AE. But, IVIG is very effective with minimal side effects beyond an increased risk of blood clots in some patients.¹² So how does it work? Doctors think that IVIG overwhelms the immune system by flooding it with so many antibodies that the AE-causing antibodies just get swept up in the rush. In other words, the immune system may be so distracted by the onslaught of other antibodies that it forgets about the antibody that was driving the AE symptoms.¹¹

Second-Line Treatments

Rituximab

If the first-line therapies don’t provide sufficient relief for a patient with AE, the most common second-line therapy is a drug called rituximab.⁸ Rituximab, which was initially designed to treat cancer, is, itself, an antibody.¹³ But, interestingly, its job is to “tag” the cells in the body’s own immune system that make other antibodies. This causes the body’s immune system to kill its own antibody-producing cells, ultimately halting the production of antibodies.

This means that rituximab can stop the immune system from making the harmful brain-targeting autoantibodies that cause AE symptoms. But Rituximab doesn’t just suppress the production of the AE-causing antibodies – it suppresses the production of all antibodies, including those necessary for fighting infections. This can leave patients vulnerable to bacterial and viral invaders that they would normally be able to fight off. Additionally, rituximab is known to cause other side effects like fevers, fatigue, and nausea.¹³Nevertheless, rituximab has been shown to be an effective at restoring functioning for patients with AE who need additional treatment on top of first-line therapies.¹⁴

Cyclophosphamide

Cyclophosphamide is another cancer drug that has been repurposed as a second-line agent in the treatment of AE.⁸ Cyclophosphamide works by entering the nucleus of a cell and attaching chemical “decorations” to the cell’s DNA.¹⁵ These “decorations” confuse the machinery that a cell uses to duplicate its DNA, which impairs the ability of a cell to replicate itself. Thus, cyclophosphamide can significantly impair the function of cells that rely on frequent replication to do their job, like immune cells.

Cyclophosphamide is very good at killing the immune cells that cause inflammation, which makes it a useful treatment for AE. The side effects of cyclophosphamide, however, can include nausea and hair loss, as well as more dangerous conditions such as bladder injuries and problems with fertility.¹⁶ Because of this, cyclophosphamide is generally recommended for patients whose symptoms aren’t eliminated by first-line therapies or rituximab.

Symptomatic Treatment of AE

The immune-targeting therapies for AE aim at eliminating the source of a patient’s symptoms. But oftentimes it can be beneficial to provide patients with additional therapies that can help alleviate the symptoms themselves. For example, the brain inflammation associated with AE can cause patients to experience seizures.¹⁷ Seizures are uncontrolled bursts of electrical activity in the brain. Depending on where a seizure starts and spreads, this electrical activity can result in phenomena ranging from the experience of strange sensations to full-body convulsions.¹⁸ Many patients with AE may be prescribed anti-seizures medications, which act to quiet down the electrical activity in the brain and decrease the likelihood of the uncontrolled activity of a seizure.

Medical therapies targeting inflammation dramatically reduce symptoms in the majority of patients diagnosed with AE. Some patients, however, will continue to have symptoms even after treatment, and some may be resistant to treatment altogether. We are still early in our research efforts to try to understand how and why people get AE. And as we deepen our understanding of this complex disorder, hopefully we can work towards developing more treatments specifically targeting the underlying causes of AE that are more effective with fewer side effects.

Download PDF Handout

References

Titulaer, M. J. et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. The Lancet Neurology 12, 157–165 (2013).
Abboud, H. et al. Residual symptoms and long-term outcomes after all-cause autoimmune encephalitis in adults. Journal of the Neurological Sciences 434, 120124 (2022).
Dalmau, J. & Rosenfeld, M. R. Paraneoplastic and autoimmune encephalitis.
Lancaster, E. The Diagnosis and Treatment of Autoimmune Encephalitis. J Clin Neurol 12, 1–13 (2016).
Ramamoorthy, S. & Cidlowski, J. A. Corticosteroids-Mechanisms of Action in Health and Disease. Rheum Dis Clin North Am 42, 15–31 (2016).
Hodgens, A. & Sharman, T. Corticosteroids. in StatPearls (StatPearls Publishing, 2022).
Briot, K. & Roux, C. Glucocorticoid-induced osteoporosis. RMD Open 1, e000014 (2015).
Abboud, H. et al. Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management. J Neurol Neurosurg Psychiatry 92, 757–768 (2021).
Janeway, C. A., Travers, P., Walport, M. & Shlomchik, M. J. Immunobiology. (Garland Science, 2001).
Bobati, S. S. & Naik, K. R. Therapeutic Plasma Exchange – An Emerging Treatment Modality in Patients with Neurologic and Non-Neurologic Diseases. J Clin Diagn Res 11, EC35–EC37 (2017).
Patient education: Intravenous immune globulin (IVIG) (Beyond the Basics) – UpToDate. https://www.uptodate.com/contents/intravenous-immune-globulin-ivig-beyond-the-basics.
Lee, S. et al. The safety and efficacy of intravenous immunoglobulin in autoimmune encephalitis. Ann Clin Transl Neurol 9, 610–621 (2022).
Hanif, N. & Anwer, F. Rituximab. in StatPearls (StatPearls Publishing, 2022).
Thaler, F. S. et al. Rituximab Treatment and Long-term Outcome of Patients With Autoimmune Encephalitis: Real-world Evidence From the GENERATE Registry. Neurology – Neuroimmunology Neuroinflammation 8, (2021).
Ahlmann, M. & Hempel, G. The effect of cyclophosphamide on the immune system: implications for clinical cancer therapy. Cancer Chemother Pharmacol 78, 661–671 (2016).
Ogino, M. H. & Tadi, P. Cyclophosphamide. in StatPearls (StatPearls Publishing, 2022).
Davis, R. & Dalmau, J. Autoimmunity, Seizures, and Status Epilepticus. Epilepsia 54, 46–49 (2013).
Turek, G. & Skjei, K. Seizure semiology, localization, and the 2017 ILAE seizure classification. Epilepsy & Behavior 126, 108455 (2022).
Feyissa, A. M., López Chiriboga, A. S. & Britton, J. W. Antiepileptic drug therapy in patients with autoimmune epilepsy. Neurol Neuroimmunol Neuroinflamm 4, e353 (2017).

All figures made with biorender.com.

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Be a part of the solution by supporting IAES with a donation today.

image - Treatments for Autoimmune Encephalitis

Friendships After A Brain Injury

by Alanna | Apr 11, 2023 | AE Warrior Personal Stories

April 12, 2023 | By Emerson Jane Browne. Reposted with permission from Dancing Upside Down

A message from IAES Blog Staff:

The staff at IAES is excited to bring to you a blog from author Emerson Jane Browne.

Emerson, a traumatic brain injury (TBI) survivor herself, wrote this very insightful blog about a subject we have all encountered. Along our own autoimmune encephalitis journeys, as we have changed, so have our friendships. We hope you enjoy her insight and thoughts as much as we have.

To find out more about Emerson please visit her website ‘Dancing Upside Down’ at https://www.dancingupsidedown.com/.

——

The 4 Categories of Friendships

Essentially friends of most brain injury survivors fall into four categories with a small amount of overlap:

Friends who knew you before your brain injury;
Close friends who are seeing you through the brain injury and recovery;
Friends who met you or got to know you during the brain injury and recovery;
Friends who are getting to know you after the brain injury and recovery.

NOTE: Though I will use the word “Friends” and “Friend” throughout this article you can add in the word “Family Members” because family members will and do fall into the same categories discussed here.

Before Brain Injury Friendships

Before Friends are the ones who knew us at work, through our hobbies, in our neighborhood, at our place of worship, etc.

They knew you as a capable, clear thinker. They knew you as a friend who was fun to be around. They knew you as someone who could get things done.

And especially they knew you as a friend who was like them.

“Like Me” Before Friendships

Many of the Before Friends just cannot handle the changes they see in you from the brain injury. Some friends split fast. Others try to hang on but end up drifting away.

Who are those people? Yes they really were your friends, but the basis of the friendship was that you were similar to each other – the like them factor.

We are different now. It is not that these people wish us ill. It is that we are no longer like them so the like them bond is broken.

“True” Before Friendships

And then there are the Before Friends who stick around. They hang with you through thick and thin. Your friendship becomes deeper and richer.

These “true friends” live the adage of “That which doesn’t kill a friendship makes it stronger”.

They will be some of your best supporters over the long haul because they can remind you of who you were and cheer you on (and razz you) to get back there.

Sometimes those “true friends” are the ones who have known you the longest. But often it is surprising who turns out to be one of the caring friends who sticks with you.

Through Brain Injury & Recovery Friendships

The key thing that makes someone a Through Friend is that they are with you; physically with you, seeing you often. Or they are at least on the phone or Skype with you very frequently.

They have to be close enough that they are able to experience and notice the small improvements; to watch you change over time.

“Through friends” may be family. They may be people who knew you pre brain injury. Or they may be people you have met since the brain injury.

These friends and family are close enough to see you grow into your “new self”. They adjust to the incremental changes along with you. They know and understand when you are able to take on new responsibilities.

This is a critical distinction from the During group below. The During Friends know you during the same period as the Through Friends but they are not close enough to really be able to grock the brain injury healing process.

During Brain Injury & Recovery Friendships

During Friends are ones who did not know you pre-brain injury, or at least did not know you well. They get to know you while you are injured and in the recovery period.

The key thing about this group is they are not close enough to you to understand much about your brain injury (or brain injuries in general) in the first place. And they are not close enough to see and realize the changes that are occurring as you heal and recover.

During friends become friends with you thinking “this is the way she/he is”.

I think the like them factor crops up again in the During Friend category. The basis of the friendship is, once again, that there are interests you share in common, or community – like a place of worship or club. They are friends that you see socially. Friends that you may work with in your recovery period.

During Friends can also be attracted to your disability. They can be people who like helping other people. They can be people who feel good about being “more together” than you. You can explain to the brain injury to your During Friends. You can comment about it frequently. But they just will not get it on the level that the Through Friends do. They cannot fully understand that you were very different before the brain injury or that you are incrementally becoming a new you.

After Brain Injury & Recovery Friendships

Brain injury recover is a very slow process. The healing changes are small but cumulative. They build on each other. Recovery takes years! But it happens. The more you stretch and grow, the more your brain remaps.

The “After Recovery Friends” are friends who get to know you after you are well into your recovery. They know you as you are now; the new you.

Friendships at Risk after Brain Injury Recovery

So which of the above friendships is most at risk as you become a new, capable you? As you regain the skill and talent you had pre-injury?

The During Friends!

They became friends with you thinking “this is the way she/he is”. They are not close enough to you to see and celebrate the small changes. And they did not know you pre-accident!

Your changing upsets the balance with During Friends.

Think of a mobile hanging in perfect balance. If you change one item on a mobile all the pieces move and jerk around until a new balance is restored.

The balance in your friendships with your during friends is similar to a mobile. If you change and become someone different from who your during friends expect then you to be upset the whole balance that they have come to count on! People do not like their balance upset so they try to push you back into “your place” in the friendship mobile.

I don’t think they mean to try to keep you small or injured. They just never knew you pre-injury so did not know you could change as much as you have!

And I also do not think that having them be naysayers is necessarily the end of the friendship. But I do think that the friendship will either adjust over time or end.

You cannot play small to please them.

My experience of Before & Through Friendships

In my case the Before Friend category is especially marked because I moved to a different state soon after I had the “main event” brain injury.

Most of my adult, professional life was spent in the Denver/Boulder area of Colorado. I moved to Seattle and suffered an additional brain injury a week after arriving. Though I have stayed in touch with friends from Colorado, they were not in a place to move into the Through Friends category.

Since returning to Washington, I have been graced with developing a few very close friendships and reconnecting with an old friend and deepening our friendship. Plus, recovering from the brain injury has also strengthened my relationship with my two sisters.

These people are my Through Friends. They have seen me through all the years of recovery. They totally get how much I have healed and how much I am back to full-force capability.

It is the reaction of other friends – my During Friends – that caused me to write this article.

New Job and Friends’ Reactions

I recently was hired into an interim position at an executive level. I am very capable of doing the job and extremely excited about it, even though it is only interim.

My Through Friends are rejoicing with me and cheering me on. They know I am ready to make this step.

My Before Friends are happy for me too. Since I moved right after my brain injury, most of my pre-injury friends never knew how bad off my brain was. They knew I could not work for a while, but for me to be hired at an executive level seems normal to them. I was working at that level in Colorado too.

My During Friends are people who I consider good friends. Most of them are social friends I see often. I thought they understood how how hard I have been working at recovery. I thought they would be supportive and excited about my new position.

But that isn’t the case. It is not that they do not wish me well. I think they do.

But their reaction to my new job is more like a “You’ve got to be kidding me” shock response and a “Who do you think you are!?!” look or maybe the look is even “You can’t do that! Why on earth did they hire you?” I also think some of them do not believe it is a real job.

In hindsight, I understand.

Since they did not know me pre-injury, I don’t think they understood how injured I was when they met me. Therefore, they did not look for, nor see the changes that were occurring as I healed. And since they had no idea what I had been like pre-injury, they had no idea who I was working to reclaim.

I told them what was going on for and with me. I thought they had understood. Now I realize that they thought it was ridiculous that I did not have a “regular job” for so long. They totally did not get what I was working on (both healing wise and work wise). I do not feel I have to entirely give up on my During Friends. They are good friends who I enjoy socially. However, I do feel I need to “encapsulate” them.

It is not important to me that they someday see me in a different light. But it is important that their wishing to put me back into a small box is unsuccessful in how I view myself and how I operate in the world. I cannot play small to please them.

Read more from Emerson Jane Browne on her website: https://www.dancingupsidedown.com/

friendship brain injury 500x333 - Friendships After A Brain Injury

Thanks to Nazka @ morguefile.com for the generous sharing of your photos

Your generous Donations allow IAES to continue our important work and save lives!

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 those 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.

Be a part of the solution by supporting IAES with a donation today.

Peripheral monocytes and soluble biomarkers in autoimmune encephalitis

by Alanna | Mar 29, 2023 | Australian Autoimmune Encephalitis Consortium

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.

——

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

—-—-

Peripheral monocytes and soluble biomarkers in autoimmune encephalitis

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

Introduction

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:

NMDAR AE – antibodies targeting a brain protein called N-methyl-D-aspartate receptor or NMDAR and
LGI-1 AE – antibodies targeting a brain protein called leucine-rich, glioma inactivated-1 or LGI-1

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 –

Are monocytes in people with AE different than in healthy people?
Is there other evidence of inflammation in the blood of people with AE?
Does the level of inflammation in AE determine disease severity?
Are the inflammatory changes the same in different types of AE?

How we did this work

We recruited 40 people with AE and 28 healthy volunteers who provided blood samples. These blood samples were evaluated in the laboratory for:

Characteristics of the monocytes (whether they show signs of being active and more inflammatory), and
Levels of different cytokines in the blood that may show increased activity of the immune system and increased inflammation

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.

What were the interesting things we found

We found that a certain type of monocyte known to play a key role in inflammation in other diseases are increased in number in people with AE compared with healthy volunteers

We also identified that certain cytokines (IL-6, TNF-a) that are important in starting and maintaining inflammation are also increased in people with AE compared with healthy volunteers

These changes were present in both severe and mild AE but were much stronger in people with LGI-1 antibody associated AE.

What do these findings mean?

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 –

Identify new treatments that target monocytes and the innate immune system
Use the inflammatory changes identified as a way to diagnose and monitor the disease.

———-

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:

Download Plain Language Summary

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow IAES to continue our important work and save lives!

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.

Your generous Donations allow IAES to continue our important work and save lives!

Your generous Donations allow IAES to continue our important work and save lives!

Introduction

Your generous Donations allow IAES to continue our important work and save lives!

Introduction

What is the connection between COVID-19 and AE?

How can SARS-CoV-2 infection lead to AE?

What implications might this have for AE research?

Your generous Donations allow IAES to continue our important work and save lives!

Your generous Donations allow IAES to continue our important work and save lives!

Your generous Donations allow IAES to continue our important work and save lives!

Introduction

Your generous Donations allow IAES to continue our important work and save lives!

Your generous Donations allow IAES to continue our important work and save lives!

Introduction

What are intracellular and extracellular antibodies?

Which kinds of AE involve intracellular versus extracellular antibodies?

How are subtypes of AE associated with intracellular antibodies different from subtypes of AE associated with extracellular antibodies?

Your generous Donations allow IAES to continue our important work and save lives!

Life is Marked by Journeys

The Shady Ladies

Pace Yourself

Taking Chances

Your generous Donations allow IAES to continue our important work and save lives!

Why are brain injuries so misunderstood?

#1 Blame it on Hollywood!

#2 You do look great! You look normal.

#3 Brains control everything about us!

#4 Brains take a very long time to heal and remap!

So how can I get people to understand my brain injury?

Show them this article.

Use the term “brain holes”.

Use Before and After Examples

Some Never Will. Let them Go.

Lastly: Blog

Your generous Donations allow IAES to continue our important work and save lives!

Choosing “Special” Mothers

Your generous Donations allow IAES to continue our important work and save lives!

Introduction

First-Line Treatments

Steroids

Plasma Exchange (PLEX)

Intravenous Immune Globulin (IVIG)

Second-Line Treatments

Rituximab

Cyclophosphamide

Symptomatic Treatment of AE

References

Your generous Donations allow IAES to continue our important work and save lives!

The 4 Categories of Friendships

Before Brain Injury Friendships

“Like Me” Before Friendships

“True” Before Friendships

Through Brain Injury & Recovery Friendships

During Brain Injury & Recovery Friendships

After Brain Injury & Recovery Friendships

Friendships at Risk after Brain Injury Recovery

My experience of Before & Through Friendships

New Job and Friends’ Reactions

Your generous Donations allow IAES to continue our important work and save lives!

Peripheral monocytes and soluble biomarkers in autoimmune encephalitis

Introduction

How we did this work

What were the interesting things we found

What do these findings mean?

Your generous Donations allow IAES to continue our important work and save lives!

guidestar

Blog Categories

Recent Posts

Recent Comments

Archives

CONTACT US

352-527-2470

IAES@AUTOIMMUNE-ENCEPHALITIS.ORG

Quick Link