The staff at IAES is proud to share with you a letter by none other than our Blog Division Head, Jeri Gore! Her compassionate, strong, and thoughtful spirit shines through these words addressed to her mom in this heartfelt and insightful piece. We learn not only of AE’s far-reaching impacts on families, but of the joy and opportunity that can await AE Warriors after surviving the worst parts of the illness.
We hope you enjoy this opportunity to learn more about Jeri’s LGI1 story and what drives her to be such a hard-working, dedicated member of IAES!
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“If we accept the science of the law of Conservation of Energy, “Energy cannot be created or destroyed, it can only be changed from one form to another.” ― Einstein
In other words, the total amount of energy in the universe never changes, although it may change from one form to another. Energy never disappears, but it does change form. It isn’t a far leap in realizing that love, the most powerful form of energy, is eternal.
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Dear Mom,
It has been a while since I have written you a letter. I apologize. There is a lot to tell you and talk about. I suppose the emotion of it all made it tough for me. Still, no excuse, you are my Mom and I love and miss you dearly and always will.
I know you probably know ALL about what is going in our lives but, boy oh boy, do we need to have a chat! The grandkids are doing great, and all are busy. Another is going to be getting married, one went off to college and your eldest grandson and his wife are going to have a baby boy this coming November. Just think of it, you would be a great grandmother. I know, you are so excited about this! Bro and I are doing fine as are Mitch and Marina. Keeping track of the kids and their lives is fun and exciting. They would have you laughing as they always did.
We made it up to the cottage in Canada this summer after the long 18 months or so of Covid restrictions and we could not be happier being here in the place that you loved best next to sunny south Florida.
I was standing near the stone we placed in your honor, in front of the cottage facing the lake and I figured it was high time I told you all about me. To talk about the disease, I have. To tell you about the organization I try and do my best to be a part of, and to help to try to ensure that there will come a day when no one needs to write to their Mom about this disease. To help ensure Autoimmune Encephalitis is a term, a disease, that is known about and easily diagnosed and treated.
Mom, I have Autoimmune Encephalitis anti-LGI1. Big word I know. Strange as heck disease for sure. In 2018 and more into 2019 I started having odd symptoms. My brain felt foggy. Sometimes I would be looking at someone and just have a blank look in my eyes. Sometimes I was mean. At times I would repeat things. Sometimes I walked kind of funny and made weird noises. It was like I had dementia. I then had seizures. Lots of seizures. It was super scary for everyone around me. I was in the hospital a lot and I was finally taken to a great hospital in Philadelphia near where we live. A wonderful neurologist at that hospital knew all about Autoimmune Encephalitis and has taken good care of me. I am doing well now. I try and participate in as many clinical trials as I can to help further research and find out more about AE. I joined an organization called IAES or the International Autoimmune Encephalitis Society to help, in my small way, to get the word out about AE. No one needs to go thru what so many with AE do. No families and friends need to go thru what your grandchildren, Mitch, Bro, Marina and all of our friends did before I was diagnosed. I know, I know you would tell me NOT to pack 25lbs in a 5lb. bag. You said that to me all the time. But, Mom, for this, for AE, I must. There has been some research that leads scientists to believe that for some folks there may be a genetic link to the type of AE I have. I have to do everything I can to ensure others get this info and to get this information for your grandchildren and generations to come. I must do this for you.
Mom, you passed away ten years ago. We were told and we believed you had dementia. You were 81 years old. You started showing symptoms of neurological issues 5/6 years prior to when you died (right around the time of this last family picture taken in 2007). You were relatively young, and those symptoms came out of the blue. I know hindsight is always 20/20 but I cannot say, in my heart, that you did not have some form of AE.
You simply started showing signs of dementia-like behavior. This is one of the things many with AE have and show. We will never know.
We will miss you always. And I will do my damnedest to make sure no one ever needs to write a letter like this to their Mom in the future.
Mom, thanks for the chat. I promise not to wait this long to write and talk again. I hope you are doing great. I hope you are smiling down at us and have that twinkle in your eye. I would give anything to see it in person and to hold your hand. I’ll be sure to write about the upcoming baby and wedding and all the goings on with a young college kid. You would be laughing like crazy with everything that goes on with the other grands as well.
Your generous Donationsallow 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.
Although, our paths are never straight and not without curve balls thrown at us at every turn, this story certainly highlights the utter joy we can all feel upon receiving very good news regarding our health! This news and outcome only come about with AWARENESS! Here is to you Mr. Jones…. a Survivor and Thriver!!
I cried with relief and joy over having learned from my Doctor that the testing for the antiLGI1 autoimmune encephalitis antibodies which attacked my brain a little over two years ago were no longer present in my bloodstream or spinal fluid. Sure, I am likely to have residual difficulties with cognitive function and memory, but I at least feel I’m finally on the road to recovery. I cried because I know I’m one of the lucky ones.
I cried in sorrow for those who have been on the AE journey longer than me and are not progressing as well. While I can never fully understand the pain and anguish they are going through, I do have an inkling because of my experiences of the past two years.
I cried in sympathy for those who are just starting out on their journey. I cried for them and their wondering how and why this happened to them, for their confusion and fear about what lies in store.
I cried with love for our families and friends who we drag through this disease with us. For their loving desire to comfort and help us while not knowing what to do or how to do it.
I cried in empathy for our doctors who know that every case is different, and that medicine is not an exact science which can provide an easy solution for every case, yet they work tirelessly to try to help us each as best they can.
Yes, I cried last night, albeit mostly silently.
Yet, as I look out the window this morning on a rainy day, I’m able to marvel at the beauty in the gray sky, the green in the trees and grasses and the sounds of thunder reminding me of the power of nature. On other days, I wonder at the beauty of the cardinals, orioles, goldfinches, blue jays and other birds which bring such bright colors into the world. I try to savor these moments and bask in them, if only for a moment, for I find they bring me great solace.
Your generous Donationsallow 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.
Limbic encephalitis is a type of autoimmune encephalitis (AE) that targets the brain’s limbic system. The limbic system is a group of brain structures that underlie memory and emotion (Fig. 1). The term limbic encephalitis is slightly misleading, however. The disease does not affect all areas of the limbic system and frequently involves non-limbic regions as well1. The classification, however, can be useful to categorize several specific types of encephalitides that target similar regions of the brain and thus result in common symptoms, even though they may arise from different antibodies and underlying causes. Some of the more common types of AE that fall into this category are caused by antibodies against LGI1, the GABAB receptor, and the AMPA receptor.
The major brain structures of the limbic system include the amygdala and the hippocampus (Fig. 1). The amygdala is critical in regulating emotion while the hippocampus is primarily responsible for creating new memories. Regardless of the root cause, the different types of limbic encephalitides disproportionally affect these regions1. This is likely because these regions contain higher levels of the proteins that the antibodies target. Even when doctors cannot identify the antibody that is causing encephalitis, scientists can determine which areas of the brain have high levels of antibody activity. By exposing rodent brains to the cerebrospinal fluid (CSF) from patients containing the antibody, the scientists see that binding of the antibody to neurons is much higher in the hippocampus, for example, than other areas1.
Figure 1. The Limbic System. The temporal lobes (green, left) are located on the sides of the head, behind the ears. The image to the right shows the inside of the brain. The temporal lobe houses the hippocampus (blue, right), and the amygdala (purple, right) which are two of the major brain structures that make up the limbic system.
While the symptoms and progression of limbic encephalitis vary widely, there are several commonly experienced symptoms due to the similarities in affected brain regions. Patients typically become irritable, depressed, and have trouble sleeping. These signs may rapidly give way to seizures, hallucination, and severe short-term memory loss1. As the disease progresses and begins to involve other parts of the nervous system, symptoms vary even more widely based on which antibody is present. For example, patients with antibodies against an intracellular protein called Hu experience loss of sensation and even loss of reflexes due to spinal cord neuron damage2.
What causes limbic encephalitis?
There are two main causes of limbic encephalitis: viruses and an autoimmune response. An infection with a virus such as the herpes-simplex virus (HSV) can cause a disease called viral encephalitis1,3. In this case, it is the virus itself that attacks the cells in the limbic system. Thus, while it is a type of limbic encephalitis, it is not an autoimmune disease since it is a foreign agent that is attacking the brain rather than the body’s own antibodies. Viral infections can, however, trigger a patient’s own immune system to attack the brain, resulting in autoimmune encephalitis3.
Non-viral causes result from an autoimmune response involving either cytotoxic T-cells or antibodies. Cytotoxic T-cells arise as a result of a cancerous tumor. In limbic encephalitis, these T-cells target proteins inside neurons (common proteins targeted are Hu and Ma2)2,4. In contrast, limbic encephalitis caused by antibodies rather than cytotoxic T-cells may develop in response to cancerous tumors or benign tumors. In fact, many cases of limbic encephalitis are not associated with tumors at all5. These antibodies target proteins on the surface of neurons like the GABAB receptor, the AMPA receptor or, in the case of LGI1 limbic encephalitis, the voltage gated potassium channel complex5. In either case, neuronal damage is found in limbic regions, explaining why similar symptoms may be observed with these seemingly distinct diseases1,5.
Diagnosis and treatment
When patients present with symptoms indicating a possible diagnosis of limbic encephalitis, there are several diagnostic tests that are typically performed to confirm the diagnosis. An electroencephalogram (EEG) is administered to measure electrical brain activity. EEG electrodes are placed throughout the scalp, allowing doctors to pick up seizure-like activity in the brain and often isolate where in the brain the seizures originate. EEGs from patients with limbic encephalitis frequently suggest involvement of the temporal lobe1. The temporal lobe houses the amygdala and hippocampus and is therefore often the source of seizures in limbic encephalitis. A magnetic resonance imaging (MRI) scan is also performed which gives doctors an image of the brain. Differences in contrast can indicate that the blood brain barrier is compromised in the temporal lobe, giving the antibodies access to neural tissue1. Finally, doctors can take samples of patients’ CSF, which may have increased immune cells and other markers of inflammation1. However, the findings of any one of these diagnostic tests can be normal which can make diagnosis challenging. Therefore, the results from all tests are considered when making a diagnosis.
Despite the devastating effects autoimmune limbic encephalitis may have on patients, many people are able to fully recover following treatment, though long-term recovery depends on the specific type of encephalitis1,5. The treatment involves removal of the tumor or other growths that initiated the antibody or T-cell production. In cases where antibodies against cell-surface proteins were present, removing the root cause along with a course of steroids and immunotherapy to restore the immune system can be an extremely successful treatment. In cases where cytotoxic T-cells attack intracellular proteins, patients often continue to experience symptoms even after removal of the tumor2. A variety of T-cell therapies can be tested to see if any lead to improvement in individual patients1. The hope is that future and ongoing research on treatment-resistant types of limbic encephalitis will guide individualized care and improve patient outcomes.
References:
Erdem, T. & Dalmau, J. Limbic Encephalitis and Variants: Classification, Diagnosis and Treatment. The Neurologist 13, 261-271 (2007).
Dalmau, J., Graus, F., Rosenblum, M.K., & Posner, J.B. Anti-Hu–associated Paraneoplastic Encephalomyelitis/Sensory Neuronopathy. A Clinical Study of 71 Patients. Medicine (Baltimore) 72, 59‐72 (1992).
Your generous Donationsallow 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.
Little things, just little things were first noticed by our immediate family. The need to recite over and over the details of how a decision was made, excessive talkativeness, repetitive conversations. Outbursts of anger over inconsequential events, vocalizations of “yep, yep, yep” and a stutter-step when that happened. Something was not right. Periodic feelings of having a “brain spasm” were added to the mix.
Visits to our personal care physician, referral to a neurologist, MRIs and EEGs all resulted in a conclusion that Jeri was just stressed out. After our son’s wedding, everything would be alright. It wasn’t. The wedding was a big success but for Jeri, concentrating on the many details was difficult, to say the least. Our college friends noticed immediately that something was not right. “She seems to not be herself, she gets angry easily, what’s up?”
Could it be a recurrence of the chronic Lyme disease from 8 years ago? Off to the holistic physician that successfully treated Lyme disease. The approach there was to test for exposure to environmental toxins and Lyme. Blood was drawn and then we left for a couple of weeks to go to our cottage in Canada. In those two weeks, our Canadian friends also came to us and asked if something was going on. The repetitive conversations continued along with withdrawal from group conversation. A couple of times Jeri blanked out for a few seconds and became worried-looking, really concerning our friends.
Back from the cottage the follow up with the Lyme doctor revealed that she was slightly positive on the Lyme test. (Lyme diagnostics is difficult and subjective – a discussion for another time.)That is probably what was going on. Back on antibiotics and several nutritional supplements – for 24 hours. Then our journey really began.
“Mom’s had a seizure, I’m following the ambulance to the hospital.” Our two adult sons living at home had found Jeri having a seizure shortly after going to bed. Another seizure while in the ER. The workup revealed that her sodium levels were dangerously low. Correction of the hyponatremia began along with MRIs and other scans. Five days as an inpatient to correct the sodium levels and all tests came back negative. No infectious causes, no tumors, must just be a case of spontaneous SAIDH (Syndrome of Inappropriate Antidiuretic Hormone Secretion). It happens, that can be corrected with diuretics and salt pills. Take anti-seizure medicine and follow up with a neurologist.
At this point, the reader needs to know a little about our family. For years Jeri was a researcher in a biochemistry lab and up to a couple of years ago, a practice manager for a busy physician’s office. I’m a Ph.D. biochemist and molecular biologist. One son is a newly trained nurse and one daughter-in-law is a very experienced pediatric intensive care nurse. Jeri’s brother-in-law is an attorney with over 20 years’ experience in large pharma oncology and has a Masters in chemistry. To say that there were many opinions on what was going on is the understatement of the year. A lot of family knowledge and experience were being tapped, all out of concern, love, and with the best of intentions. Conversations with physician friends and nurses suggested a myriad of causes. None of us got even close to the cause.
The ‘spells’, absent episodes, memory issues, and cognitive decline all continue and increase in frequency. We were home for a week and then another big seizure puts her back in the ER and again admitted to the hospital. Again, all scans and EEG are normal. The doctors in the regional hospital are again scratching their heads. Despite repeated requests, the hospital physicians and our personal neurologist resisted performing a lumbar puncture. Why? We were told there was simply no indication that would justify such a high-risk procedure. Must be the SIADH. Go back to your local neurologist and nephrologist.
A 72 hour EEG was ordered. This was stopped after 24 hours since we noted so many (~30) ‘spells’ and no further recording was needed. Like two spot EEGs before, nothing showed up. The local neurologist was stumped, calling it a real mystery. We again ask for an LP and testing but were refused on the basis that he didn’t know what to test for. As a shot in the dark, testing was ordered for serum anti-NMDA receptor. Negative results. Sodium levels stay a bit low, but near normal. Frustration all around.
Escalating the case was now a family priority but we were stymied by the long wait (2 months!) to get into the UPenn neurology group. A week after the last neurology visit another seizure. A couple of days later we hear a crash and see Jeri at the bottom of the stairs having a seizure, this time with a gash to the head. Back to the ER after a very irrational and angry outburst. Another seizure in the ER and another admittance to the local hospital. The next morning during rounds the floor neurologist is in the room and the hospitalist (physician in charge of cases on that floor) pops in to see how things are going. She asks the neurologist if she is going to order an LP and the neurologist says no, there is no need. The matter is dropped. No words can describe our frustration and helplessness at this point. My son and I discussed the situation and decided to go to the hospitalist and ask for her intervention in getting the case moved to UPenn. She agreed but was doubtful that she could get her transferred. To her and our surprise, UPenn neurology agreed right away!
We were just so lucky to have the case taken over by UPenn and a group of physicians that have been integral to the discovery and treatment of autoimmune encephalopathies. Within a week we had a diagnosis of anti-LGI-1 autoimmune disease and a week after that we began immunosuppression therapy via a five-day course of steroid infusions and an infusion of Rituximab.
We’ve been home a week, continuing anti-seizure medicine, oral steroids, salt supplementation, and diuretics. No seizures so far! We are seeing small improvements in cognitive ability and memory. We have a long way to go but are hopeful. The second infusion of Rituximab will happen in about a week.
Lessons learned:
Unfortunately, I think we can consider that knowledge of auto-immune encephalopathies is pretty much non-existent to the average physician and even most neurologists. The resistance to exploit all avenues of testing after repeated presentations to the ER and negative results of conventional diagnostics needs to change. That change will only come with physician education. Also, symptoms can be very, very subtle at first and not consistent from person to person. Like a lot of other neurological conditions, the symptoms are common to many different causes.
Now that the mystery is solved the recovery can begin. Another Zebra identified.
Become an Advocate by sharing your story. It may result in an accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
It was the day after Labor Day, Tuesday, September 6, 2018. I was sitting in the emergency room At El Camino Hospital in Mountain View, CA, watching the chaos that was now surrounding my husband who is lying down on the bed just a few feet away from me. I knew something was very wrong with my husband, but now reality is setting in that things are about to change forever.
Let’s rewind for just moment to three weeks before September 6th when my husband had gone to see his regular doctor due to some abnormal body and nerve pains. His doctor thought the body pains might be fibromyalgia since they were lasting so long, but I really wasn’t buying that. It was certainly strange, but nothing to panic about so we made an appointment with a neurologist for a nerve test a few weeks out which just happened to be on September 6th.
It wasn’t until September 1st, the Thursday before Labor Day, that the body aches and pains stopped, and things started to shift. He came home early from work on Thursday. He was sweating and pale. I asked him why he was home early. He said his boss sent him home because he wasn’t completing thoughts during their conversations. Weird I thought, and he looked awful, so I told him to go take a nap. Having a high-level corporate job with a growing company can be overwhelming at times and it has caused him to get sick in the past so perhaps it was just stress induced. Friday was a vacation day and he seemed OK, but then Friday night we went out to dinner. When he looked at me from across the table it was like he was looking through me. The only way I can describe it is that he looked like a serial killer. I realize that’s a horrible thing to say but that was truly what he looked like. It was downright spooky.
Then that evening he started to sit straight up on the edge of our couch (which he never does) and that night and through the weekend he was sleeping really stiff in bed, like a mummy. Then the cognitive issues set in on Saturday and he started to decline rapidly. By Sunday he couldn’t complete a sentence. He would start to speak, and the last words of a sentence would just not be there.
Then there was the dog incident. Every time out dog goes potty outside she asks for a treat by sitting and staring at us until she gets one. Standard behavior she has done just about every night of her life. So, when she was staring at him because she wanted a treat for using her pee pad outside, he turned to me with a totally confused look and said “why is the dog staring at me?”. Now I’m starting to freak out a bit.
By Sunday he became really lethargic, could barely start a sentence no less complete one, lost interest in just about everything, and couldn’t recognize objects I would show him. We already had an appointment with a neurologist on Tuesday, so I thought that was our best chance of getting the right care rather than going to the emergency room on Sunday. Since Monday was a holiday, I didn’t think the right doctors would be there, so I decided to wait. By Tuesday morning he could not cognitively function at all. I was guiding him to the car to get him to the doctor.
Once we arrived, I told the technician who was supposed to start the nerve test to get the neurologist in the room asap because something was very, very wrong with him. She tried to tell me I didn’t have an appointment for that. Clearly, she didn’t know who she was dealing with. I told her to go get the doctor NOW! Then a nurse came in and tried to tell me the same thing. I lost it. I started to yell and cry and demanded she get the neurologist immediately. Once the neurologist came in it took her less a minute of questions and observation before she said “get to the emergency room right now!”
Unbeknownst to us at the time, she happened to study AE as an undergraduate at UCSF and knew the symptoms. She also told us exactly who to ask for at the ER and the hospital was less than 10 minutes away.
Almost immediately upon our arrive we were whisked into a room and were being seen by their neurosurgeon who specialized in AE. She explained to me what she thought was going on. Autoimmune what???? She told me what AE was and all the treatment and tests to follow.
Due to the quick onset she was worried from the get-go. From there it was a testing frenzy. MRI, Spinal Tap with samples going to Stanford and the Mayo Clinic, CT Scan and tons of blood work. The urgency was obvious.
As I sat their dumbfounded all I could think about was 1) how do I explain this to his family, and 2) is my husband going to be a vegetable? That probably sounds like a horrible thing for a wife to say but that was where my head was at. He was the strong one, always totally sure of himself, always knowing what he is doing and where he was going in life. It was just infused into his personality to make sure our future was solid. Seeing him so helpless on that hospital table I couldn’t help but think if everything we dreamed about for the future would now be gone or in serious question.
You might be saying to yourself at this point “geez she was awfully selfish. It’s all about me, me, me me!” You would be correct. I was being completely selfish and needed to grow up.It was time to put on my big girl pants. I now had someone who needed to rely on me now, so I better step up. I can count on one hand how many times I had been in a hospital, and now I was about to spend 10 days there. It was 8 am to 8pm, taking it day by day, hoping for the best but getting ready to deal with the worst.
The MRI was negative, but the initial spinal tap results showed an elevated white blood cell count. She wanted to start treatment immediately but needed the results from Stanford to make sure it wasn’t AE with a viral or bacterial infection. The standard protocol, which is IV steroids, cannot be used on a viral or bacterial infection. She called Stanford and bugged them for the results (they are only about 20 miles away) and it was luckily neither of those.
Due to her extensive knowledge of AE she determined from the symptoms and elevated white blood cell count that it was Autoimmune Encephalitis. His antibodies were also sent to the Mayo Clinic to identify what type of antibodies he had but she was not going to wait 10 days for those results to start treatment. Whatever antibody it was the initial treatment was the same, so she got started immediately.
This was the best of all circumstances one could ask for. It’s why it’s so important for people to see an AE neurosurgeon as quickly as possible so they can get the right diagnosis and treatment. Any additional time spent with a wrong diagnosis can lead to so many more years of recovery due to more extensive brain injury occurring.
So, we started with the 5 days of IV steroids and then 3 days of IVIG treatments to follow. All in the hospital, thank god, since I don’t think I could have taken him home in the state he was in. He was really scared, and I was doing my best keep him in good spirits and to keep myself together at the same time.
During all of this I am getting phone calls and texts from friends, family and coworkers asking how he was and what the hell is autoimmune encephalitis. Fair questions, since I had certainly not heard of it before this happened. As he started to get a little better, he didn’t want to talk to anyone or have any calls because he couldn’t form complete sentences yet and his voice was very weak. He also didn’t want any company which was difficult since everyone wanted to see him.
Then there were the memory issues. He had a really hard time with names and retaining them in the beginning. From day 1 to day 4 he only could verbally remember my name and, of all people, his boss. Nobody else. He recognized some people but could not say their names. I remember sitting in bed with him and watching a Tom Cruise movie. He recognized Tom Cruise as someone he should know but did not know his name.
He couldn’t understand how to use his cell phone until day 6 or 7 and then I had to show him how to use it. His reading, motor skills, and mathematical skills didn’t seem to be too affected. (I know, totally weird. Don’t try to figure it out or your own brain might explode. 🙂
He improved dramatically every day that went by. The zombie staring finally went away about day 6 in the hospital. He could talk in full sentences, although his voice was still hoarse and not very loud, and he started remembering most routine things in his life.
The tests came back from Mayo and his antibodies were not identified as any of the ones known at this time. (They were actually donated to UCSF in hopes of identification someday.) After 10 days in the hospital we went home.
The first few weeks were interesting. Some things came right back, others, not so much. His long-term memory for the last 10-15 years seems to be gone. That’s been the hardest thing since we have only been married 12 years. Things come back kinda like dreams but only if I show him pictures or tell him stories. I showed him our wedding video and he doesn’t have much recall which was a bit disappointing, but understandable.
Once home, I asked him to start a daily journal to keep track of his progress. He showed me his first entry. It was in cursive. He hasn’t written in cursive since he was a kid. I asked him why he was writing like that and his response was “you have to write a journal in cursive”. Huh? He was dead serious when he said it so I just went with it. I couldn’t read the thing since the writing was terrible, but eventually it got better. It took him quite some time to get back to his normal printing which is actually quite neat.
Getting his signature back was also quite a struggle. This was a weird one. He couldn’t sign checks because he couldn’t get his signature right. He was so frustrated, and it would stress him out so much that he would shake when he tried to do it. I felt horrible for him. I signed all the checks until he got comfortable again signing things. I had him practice on some paper where there was no stress to get it right. He couldn’t understand why it was happening and I had no answer for him except that he had to give it time. It eventually came back but it did take a few months. There seemed to be no rhyme or reason as to why some things came back quickly while others took months. That’s when I realized that the brain is not something we were ever going to figure out and we just had to go with the flow.
It was the higher brain functions that took the longest to come back. Work was the last to be addressed. He initially took a three month leave of absence in hopes of returning in November. His boss and team have been fantastic through this. Super supportive. They all truly care about him and had made it perfectly clear that his job was in no jeopardy. He was to get well and have no worries about work. Such a great group of people we really couldn’t have asked for anything better.
For some reason a lot of his work knowledge from his current company came back which is within the last 5 years. However, conversations and presentations he had given were gone from his memory. Any get togethers he had, whether it be for work or otherwise were also forgotten. But information and learned materials seemed to be there. I think most of it was actually relearning a lot of his work skills which is really amazing considering the high brain function required for that. After about a month at home, I had him slowly go through old emails he had sent to people so he had a feel for what he used to do and how he did it. Only a few minutes a day at first and then we built up from there. In the beginning he got tired very easily, so I had to force him to take lots of naps. I had to watch him very carefully since I just wasn’t sure at first what he was going to be capable of doing without putting undo pressure on his brain.
I mentioned above we had to go with the flow when it came to this entire process. My husband is not a “go with the flow” kinda guy. He is someone who wants to know how to get from point A to point B. He wants a schedule and to know what he needs to do to accomplish the goal. I had to explain to him that the brain does not work that way. There is no exact path. It will tell you when and how your recovery will go. If you push it you will only go backwards. Wow, did that piss him off. So frustrating for him. The man who always has a plan couldn’t really have one.
Then there are the emotional issues. This one really threw me for a loop and has been the hardest to adjust to. My husband is an introvert. Quiet, stable, hard to read, and doesn’t show a lot of emotion. Tends to keep things inside, which is probably why he does so well in corporate America. Very few people don’t like him because he is so even tempered. So it was a shock when we got home and there was a significant change in his personality, particularly when it came to his emotions.
First there were the panic attacks. I have never seen him have one, ever. The first one was when he couldn’t remember his password to Quicken, which had all of our financial data on it. He lost his freakin’ mind!! I hardly knew what to do because it was such a strange behavior coming from him. So, after the initial 5 seconds of total shock, I realized I had to be the voice of reason which, if you knew me, would be the funniest thing in the world. HE was always MY voice of reason.
It was seriously like walking someone off a ledge. I had to sit him down and tell him it wasn’t the end of the world and that we would call customer support and figure out how to get into his files. I also told him to take deep slow breaths. In and out. In and out. This had worked for me in the past so thought I would give it a try. Sure enough it helped. Thank goodness because I’m not sure what I would have done next. There would be more of those meltdowns to come and I got better and better at handing them. Talk about role reversal? Wow. I was the meltdown queen! Not anymore. I’ve had to keep those big girl pants on even today because we are in no way at the end of this battle.
Then there was the crying. When we first got home he cried at every little slightly sad thing. I was totally confused. He has cried maybe twice since we had been married, but about 30 times in the first two weeks we were home. It took pretty much nothing to trigger him and he was not even sure why he did it most of the time. He always cried when something sad was, say, on tv, but it was not sad enough to invoke tears, normally. We kinda turned it into a joke. Every time he cried I laughed and asked why he is crying. He would say he didn’t know and then we would both start cracking up. Hey, if you can’t laugh how else are you going to heal, right? He doesn’t do it much anymore, but we went through a lot of tissues those first few weeks.
Some of the personality changes have been a good thing. His emotions are now much more on the outside than the inside. He may cry more but he laughs more too which is great. Just the other night we laughed at something so stupid, but we laughed so hard we were in tears. We never did that before. We also talk a lot more. You really must. Once you go through something like this you either get closer or it tears you apart. The former is definitely true for us.
As a caregiver, this whole experience has taken a big emotional toll. I’m dealing with someone I know, but now need to get to know all over again. I sometimes find myself standing in the middle of a room, thinking how much things have changed, yet little by little realizing we have come to a new sense a normalcy.
In the hospital things were easier. I wasn’t responsible for this person getting by day to day. I could leave the room to have a good cry, but once home that wasn’t an option. Life as I knew it would never be the same. AE patients feel that way too but it’s different.
From a treatment perspective we were very lucky to have gotten the care we did in the time-frame that we did. However, he is still in recovery, not “cured”, and every day I see the person who used to be confident about everything and never question himself, to someone who is constantly questioning everything.
I’m on edge a lot watching for things to go wrong. You can’t help doing that as a loved one. You dissect every little thing and wonder constantly if this is the way things will be for the rest of our lives.
He is back to work full time now. Although the doctor gave us approval to have him start back to work in January, I was the one who had to decide how it would be done since I knew the stress level and high level brain function required to do what he does. I had to watch him start back slowly and hold him back when he wanted to do more. Not fun.
He started for a few hours and then worked his way up to the full 8 hour day. However, he comes home every day at 12:30pm, takes a nap, and then works the rest of the day from home. I don’t think he will ever go back to work like he was before. That required no less than a 50 hour work week which is normal to sustain a job at his level.
He’s already talking about having to give up the position he has now, which he worked so very hard to achieve, and take a different job within his company that requires less of a workload. He has already had to decrease his workload significantly. It’s a huge ego hit even though the company is doing their best to help him by making changes and reducing his team.
Then there are the financial concerns. He wants to retire earlier than he had planned before just in case something goes awry. We’ve met with our financial adviser several times just to work out “worst case” scenarios” because with this disease you just never know.
So here I am, not only wondering what our future holds, but having to tell him everything will be OK when I am not sure that it is. I try not to show him how stressed I am because that just makes him more stressed and gets us nowhere.
It’s hard for people to understand what I am going through. I don’t want to worry his parents, so I tell them just as much as I think they need to know. My friends, when I tell them about what happened to Gary, say “Holy cow, that must have been terrifying for you!”. My response; Yea, it was, and still is.
So, the journey continues. Where it ends, who knows. But we will take it together and conquer each challenge as it comes. For all you caregivers out there who want to put your fist through the wall and scream “why me?” I give you the cheesiest line ever with a twist. When life gives you lemons, make lemonade. BUT, remember this….you can only make lemonade if you have sugar or it tastes bitter. Your strength and resilience is the sugar. Without it, all you have left is a bitter taste in your mouth. So, hang in there and make some sweet lemonade. ? You’re stronger than you think.
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It was a beautiful summer’s day on July 5, 2017. The temperature was perfect and there was not a cloud in the sky. My adorable young nephews, charming brother-in-law Joseph, and his lovely wife had all left the day before. My husband Peter had left to fly back to Houston, Texas where we live, and my eldest son Jack was at work nearby in the General Store in the small town of Dorset, Ontario. My two younger sons Andrew and Luke had just made smoothies and we had walked down the steps to sit on the lower deck and contemplate an imminent boat ride. What a perfect day at the cottage! As I settled into a Muskoka chair and we discussed the fun we’d had the last few days, I looked out over the sparkling lake and suddenly saw flashing lights in the sky and a strange pulsating movement. “I don’t feel very well”, I remarked to my boys. The next thing I remember is waking up lying on the deck. I could see the legs of paramedics and firemen around me and hear my friend Karen’s voice calmly repeating, “Sweetie, it’s ok, you’ve had a seizure”. This was my first grand mal seizure.
My boys will tell you that, after what I now know was an aura, I shook very violently, holding my arms stiff and straight out in front of me, my eyes slightly rolled back and staring vacantly at the sky, I slid out of the Muskoka chair to the deck (no easy feat if you’ve ever been in one of these deep, sloped chairs!). Andrew (15) had the presence of mind to cradle my head as I fell to the deck, then shouted to Luke (11) to call for help. Luke ran the 50 feet up the cottage and grabbed the cordless phone and called 911. He ran back down to the deck as the call went through and they both gave the dispatcher a description of what happened and our address. Then these smart boys called Karen, a school teacher and my summer bestie, who has a cottage at the other end of the lake. She immediately jumped in a neighbor’s boat and came speeding up the lake to help. It took 20 minutes for the emergency services to arrive and I was unconscious that entire time. When I regained consciousness I had no idea what had happened. I was transported to the small, local hospital in Huntsville, Ontario where I was given fluids, told that I was probably just dehydrated and sent home. It would take the next seven months of becoming progressively more ill, to finally receive an accurate diagnosis of LGI1 Autoimmune Encephalitis.
Looking back from where I am now on this journey, I know that I started having symptoms back in September of 2016. I started experiencing numbness and tingling in my forehead that spread under my eyes, as if I was wearing a mask. The neurologist at the time prescribed medicine for migraines and told me to take it when I felt a migraine coming on. I never took it as I never had a migraine. In late May 2017, I also started having numbness and tingling down my left side, affecting my left arm and left leg. I would have moments where I would be walking or talking or eating and I had to pause to let a strange feeling kind of wash over me. As a family, we started calling these moments “episodes” and I would just pause what I was doing for a few seconds until it passed. I’m a public health nurse, and I wouldn’t know until months later that these “episodes” were actually Partial Focal Seizures. That would be discovered, when, shortly after the Grand Mal Seizure and the diagnosis of dehydration in July 2017, my husband Peter knew in his heart this wasn’t right and flew me back to Houston from Canada for further investigation. A 24 hour ambulatory EEG showed that I was having over 40 partial focal seizures a day. I was diagnosed with Epilepsy and was started on the seizure medication named Vimpat. Fycompa was added a few months later.
My seizures decreased to about 8-10 a day but still, they persisted. Some days I would sleep for 20 hours a day. I hadn’t been able to drive since the Grand Mal seizure and a new school year was starting for my boys. Jack (17) had just received his driver’s license and was suddenly the main driver for me and his brothers to school, activities and appointments. Thankfully they were old enough to help with dinner, laundry, and chores and getting themselves to school and their homework finished. A myriad of friends jumped in to help drive me to appointments and brought meals and groceries when my husband was travelling for work and I was unable.
In September an MRI suggested Limbic Encephalitis and a Lumbar Puncture was done with samples sent to the Mayo Clinic for testing. My neurologist started to treat based on my symptoms and I received four days of IVIG in October 2017. My last known seizure was October 25th, 2017. Hurray! We were finally onto something and finally some relief from the constant assault on my brain. Unfortunately, it wasn’t to last. Leading up to a scheduled booster IVIG infusion in November, symptoms of numbness, tingling, weakness and fatigue were returning. My teeth ached and the tip of my tongue was all pins and needles. The IVIG in November gave me no relief.
The results eventually came back from the Mayo clinic, but unfortunately, the doctor did not collect enough CSF (cerebrospinal fluid) for the Mayo Clinic to run the full antibody panel, so the test was inconclusive. My neurologist ordered a blood test to check antibodies and on December 6(the day after my 46th birthday), I was told that I had tested positive for Voltage Gated Potassium Channel Antibodies (VGKC). We were devastated by the diagnosis but also relieved to finally have one. Over the next few days, I experienced an even more rapid decline. I experienced gait changes and was now limping and dragging my left foot, I had trouble choosing the right word when talking or texting (aphasia), my fatigue had come back with a vengeance, and my short term memory was abysmal and driving my children and husband around the bend as they had to repeat and remind me over and over of things.
I was admitted to Houston Methodist Hospital on December 11, 2017. I was admitted through the ER and will never forget the young, bearded, strawberry blond ER Doctor who, after reading my history and diagnosis, came into my curtained cubicle, plopped down in the chair and said “WOW”. Not very reassuring and only mildly terrifying. My husband Peter arrived to the ER late that evening. I had said goodbye to him early in the morning as he took a short business trip. He flew back urgently when he learned that I was to be admitted. In the ER just before midnight, I asked him to help me up to the toilet. He was shocked to see my physical deterioration as I leaned heavily on him for support, no longer able to walk unassisted. That night I began a five day course of IV steroids and was admitted to the Neurology unit on the 10th Floor. December 11th marks the beginning of one of the most challenging years of my life.
Early on the third day, Physical Therapy came and performed and short assessment. They recommended that I start using a walker. I tried to convince them that I didn’t need one, that I wouldn’t be sick for long. They insisted I be issued with one so that I was less of a fall risk. I depended on that walker for the next three months. The disease had affected not only my memory, but also my motor function. That week I endured multiple needle sticks as my veins kept failing due to the assault of the steroid infusions on my veins and my IV had to be reinserted a number of times. I was sent for another Lumbar Puncture, this time with sufficient fluid drawn to be sent to the Mayo clinic for antibody testing. As I lay in the bed, multiple doctors, residents and medical students visited me on rounds, most of them unable to hide their shocked, worried expressions.
I was discharged on December 17, 2017. My mum had flown in from Toronto, Canada to help look after me. It was my son Luke’s first ever Houston Youth Symphony Concert and I was determined not to miss him playing the violin. Clothes and a hat were brought to the hospital for me and the nurses gave me a bag full of surgical masks that I would wear in public to help prevent infection due to my weak and immunosuppressed state. Discharge went smoothly, the nurses all knew how important this was to me and I was driven by Peter, with my mum Lynda and my boys to the concert. We planned to arrive just as it was starting (friends had taken Luke early for dress rehearsal) and sit at the back, hoping to not be noticed but still able to hear the beautiful music these children had prepared over the last four months. It was possibly the most difficult walk I have ever made. The ramp into the Middle School where the concert was held was so steep and so long to me. The walk to the auditorium seemed interminable, even though I was able to see if from the entrance. The concert was outstanding and enjoyed it with tears rolling down my cheeks, proud of my young son and grateful to be alive. That night, finally back at home, Peter and the kids set off on vacation to spend Christmas with family in British Columbia – it had been planned for months. This is the way I wanted it as I did not want my kids robbed of the magic of Christmas. I knew that Auntie Laurie and their cousins would be good for their souls and they would be showered with love and reassurance that their mum would be ok.
My mum Lynda, a retired nurse, got to work getting me settled at home and getting my house adapted to allow me to move around safely with a walker. In the two weeks that she looked after me, she had to help shower me as I was too weak to stand in the shower or get in the bath. She shopped, cooked, cleaned, drove me to appointments and cared for me 24/7. I’ll never forget her love and care or her worried expression. Living in Toronto, she’d had no idea how sick I had become. On December 26 I had my first assessment at TIRR, the Texas Institute of Rehabilitation and Research, whose mission it is to “improve the lives of people who have sustained central nervous system damage through injury or disease”. Jeffrey conducted my new patient assessment that day with patience and incredible kindness. I was an emotional wreck as it was finally sinking in that it was going to take a lot of work to regain what I had lost. That day I burst into tears when I was even unable to walk with the walker for 2 minutes.
Thanks to the remarkable staff at TIRR, I would spend the next 6 months attending five hours of therapy a week, gradually reducing to two hours a week by May 2018. The therapy was very physically challenging and I spent many hours with Michelle doing physical therapy on land, Charlotte in the pool, Amy for Speech Therapy and Imelda for Occupational Therapy. I will be forever grateful to my friends in Houston that quickly organized a driving sign-up sheet so that I never had to worry about how I would get to and from my many therapy sessions. It was often the highlight of my day to catch-up with friends who were driving me. Friends also stepped in and began delivering lovely home cooked meals to our doorstep three or four times a week for five months.
I began seeing a skilled Naturopath once a week who I still see and continues to perform acupuncture, make chiropractic adjustments and suggest nutritional supplements. With his guidance, I adopted a modified Autoimmune Protocol Anti-inflammatory diet, which I continue to follow and may never change. I began practicing daily meditations. Peter and I began contemplating getting a second opinion. Everything we were learning about AE indicated that early and aggressive treatment led to the best outcomes. Were we doing enough only with the hours of therapies each week? We were very fortunate to have my brother in law Joseph, a practicing neurologist and my father in law, a retired neurologist on our team. They have both been excellent counselors, educators and a source of great encouragement through all of this chaos. The problem was they both live in North Carolina and we live in Texas.
Thankfully, Joseph was able to get us an appointment to see the highly regarded Dr. Christopher Eckstein at Duke University Medical Center in the next few weeks. Friends stepped in to look after our 3 children and my husband and I made the trek to North Carolina. One of the only pictures I have of this devastating period of my life is the photo of me and my 87 year old father in law “racing” our walkers down the hall on that trip.
We knew we had made the right decision as soon as Dr. Eckstein completed the most thorough neurological assessment I have had to date. He and his team spent hours with us, taking an extremely detailed history. We also saw Dr. Saurabh Sinha who assessed my response to the seizure medications in an effort to get a full picture of what was going on. Labs were drawn and sent for antibody screening and a few weeks later Dr. Shah, Dr. Eckstein’s Fellow called me to tell me that I had tested positive for LGI1 Antibodies. LGI1 Autoimmune Encephalitis is known to affect 0.83 in a million people. I have never been so relieved and terrified in the same moment. Relieved to finally have a diagnosis but terrified as to what it all meant. Dr. Shah patiently listened to me sobbing until I collected myself and then explained what she and Dr. Eckstein recommended next.
I was to begin infusions of Rituximab (Rituxan) every 6 months. Back in Houston, after consulting with Dr. Eckstein, my neurologist concurred with this new diagnosis and agreed to implement the recommendations. So far I have had two infusions of Rituximab in the outpatient ward and aside from causing some moderate headache for a day or two and extreme fatigue that lasts about two weeks, I have suffered no serious side effects. As I understand, the Rituximab works to destroy the LGi1 antibodies that are attacking my brain. Repeat Infusions are needed (every six months in my case) to teach my body to stop making these antibodies. Hopefully, one day I will no longer need these costly infusions but current research indicates that the relapse rate is high and we just don’t know how long I’ll need treatment. Some people ask about the long term side effects of the treatment. I’ve never asked the question because, to be honest, the alternative of not treating this disease is pretty grim. We are just grateful that I have a diagnosis and treatment plan, good health insurance, and a local neurologist who, so far, has agreed to implement Dr. Eckstein’s recommendations.
It is now January 2019 and I am proud of the huge gains I have made over the last year. I no longer use a walker or a cane and recently started walking about 2 miles about 5 times a week. I continue to go to TIRR for Physical Therapy twice a week to work on regaining the strength that I have lost. I now weigh 106 Lbs. when I used to weight about 125 Lbs. before becoming ill. I know I’ve lost weight through elimination of sugar, gluten, red meat etc. from my diet but I’ve also lost muscle mass. My legs were unrecognizable to me in December 2017. I continue to see my beloved Naturopath, Dr. Joe Lindley. I rely on my friends, children (now two of them drive) and my husband to do most of the driving, although I do drive in a bubble between my home, WholeFoods and my children’s school (about 7 miles end to end).
This stage continues to be challenging, but in different ways than 2018. Now my disease is largely invisible, except to my family and closest friends. I suffer from Brain Fatigue and have to be very careful to not over commit or over extend myself daily. I have become good about saying “that doesn’t work for me” when managing my commitments. I get headaches more frequently than I ever did and I still battle fatigue. When I am physically tired, Brain Fatigue affects me by causing aphasia and short term memory issues as well as gait changes where I begin to limp and drag my left leg again. I tire very easily driving and even being a passenger in a car can be exhausting for my brain. Being in a room where multiple conversations are occurring is very challenging for me as it is difficult to filter out the extraneous noise and focus my attention. But most of these effects aren’t outwardly visible and is what makes it challenging.
Still, I will be forever grateful for my ultimate diagnosis and recovery to date. I will be forever grateful that my children called 911 and my husband and family never gave up hope and advocated tirelessly on my behalf for diagnosis, treatment and aggressive rehabilitation. And lastly, I will be eternally grateful that I never, even in my darkest days, gave up hope for recovery. I somehow always believed that this wasn’t going to be the end of my story.
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Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society cannot provide medical advice.
International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.
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