In May of 2020, I was a healthy, vibrant, smart, loving, pre-med student at Penn State, nicknamed the ‘mayor’ of my large friend network … until I fell acutely ill. Within 7 days of hospital admission, for what seemed like some type of stress induced altered mental status, I became catatonic. My mom watched in horror as her daughter’s light rapidly dimmed; losing a piece of what defined me each day. By day 7, I no longer spoke or appeared to recognize anyone, my heart rate became unstable, I could no longer walk or eat, and my kidneys began to fail. I was rushed to the University of Rochester Medical Center (URMC) with a preliminary diagnosis of Anti-NMDAr Autoimmune Encephalitis (AE) – an illness my family had never heard of, but one unfortunately they would get to know very well over the next six months of my horrific journey.
Full disclosure — this part of my story comes from my mom; I don’t remember anything from my admission until fuzzy memories of my time in inpatient rehab 5 months later. By the time I reached URMC I was catatonic, seizures were starting to take over. My mother described the surreal experience as ‘seeing our beautiful Rachie rapidly falling through the AE ‘looking glass’ – a tumbling, bouncing, free fall which lasted for days, weeks, eventually resulting with me being placed on life support for months’.
Timing is everything, right?! It was during COVID lockdown, visitation was not allowed. My family was no longer able to be by my side. For 42 days, physicians and nurses cared for me while in a medically induced coma; trying to quell the relentless seizures. My mom sat on the virtual sidelines, distraught, making decisions regarding PIC lines, arterial lines, sedation, feeding tubes and a tracheostomy – decisions she never imagined she’d be making for her child, who outside of a tonsillectomy, was the picture of health. Without the communication, compassion, trust and love I received from the URMC team, my mother said she wasn’t so sure she would have been able to get through those first 42 days. They were her eyes, ears, and most importantly, heart.
When restrictions finally lifted and my parents were able to be by my side, they witnessed firsthand what a cruel, relentless illness AE was. There is no official playbook. My mom, a person who prefers order and predictability, was at a loss watching the team trying to control an illness that twisted and turned, never letting up. AE laughed at us and didn’t care how desperately we wanted it to release its grip. During this acute phase, one of my Neuro-intensivists explained his view on Anti-NMDAr AE (after treating a handful of cases each year) to my mom – ‘It comes out of nowhere, attacks HARD, stays as long as it pleases, eventually leaves, rarely returns. Our job is to treat Rachael with what we know works and keep her alive until it leaves”, and they did just that. I received steroids, plasma pheresis, IVIG, Rituxan and when things still seemed to be stalled, Cytoxan. I was also placed on a strict KETO diet, which in some pediatric patients has been proven effective in reducing/eliminating certain type of seizure activity.
By mid-August of 2020, my mom tells me, “Our ‘Alice’ ended her free fall and SLOWLY started to reemerge!”. Day by day, small pieces of me started coming back. Eventually, the ICU staff took me out to the children’s garden at URMC (safety protocols in place (!)) to see my brother Andrew for the first time in 4 months (pictured)!
My family and team celebrated each breath on my own, swallow of water, baby step, new word, and smile! When it was finally time to leave my nurturing Neuro-ICU ‘nest’, the team through me a dance party — sending me off to inpatient rehab in style! Since I have no memory of that time, I’m thankful it was recorded! Taylor Swift’s ‘Shake it Up’ will forever rock as my survival song 😊!
My dance party send off!
I was released from the hospital in October 2020, just short of 6 months. While my journey was far from over, I was on my way! I still needed to be weaned (carefully) from 8 different anti-seizure medications, regain my cognitive functions and physical strength and dexterity. Through the work of an amazing rehab team and sheer determination, I made my way back. By the summer of 2021, I was taking college calculus II and preparing for my college return in the fall.
But here’s the best part of my story! In December 2021, I spent a week in the URMC Neuro-ICU, only this time NOT as a patient but rather to shadow the amazing physicians, nurses and professionals to learn firsthand the world of intensive care neurology. As a result of this shadowing, I’m sure they hope I choose Neurology as my specialty (maybe?!), but FAR more was gained from this experience! Rarely do providers, nurses, and staff get to see the results of their amazing efforts, and a recovered AE patient rarely gets to come back to see their heroes in action — Life came full circle.
I finished my fall semester (Deans List!) and as I write this, and I’ve returned for my final semester at Penn State. I’ll begin studying for the MCATS with the goal of medical school in 2023 (maybe URMC? 😊). Autoimmune Encephalitis was a cruel thief who tried to steal me and subsequently a year of my life, but as the Mad Hatter said to Alice, ‘If you knew time as well as I do, you wouldn’t talk about wasting it’. I’m not about to waste it!!
My mom reminds me often; I have a story to tell! I think it’s a story of a young woman falling through the looking glass — finding beauty, love, compassion, and competence all along her journey. However, her name isn’t Alice, it’s Rachael.
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.
Larissa was born after a normal, healthy pregnancy on May 29, 2007. The next 5 years were seemingly “normal” as she hit all of her milestones on time and ahead of most of them. She was potty trained by 2, reading Dr. Seuss books by memory at 19 months! She was classic first child doing everything early. At 19 months after moving into our house where we would live the next 5 years in September 2008, I took her to her scheduled doctors appointment for her checkup and to get the scheduled vaccines. On the way home from the doctor Larissa projectile puked, which she had never done before. She was completely breastfed and had never even spit up. After checking her out she seemed fine and I blamed it on her drinking her bottle too fast in the hot car. The next 6 months she continued to vomit intermittently projectile and then would return to fine and playing every time. At age 2, after trying to cut back on her milk and other diet changes thinking it was something she was eating, I took her to her pediatrician about these vomiting spells. After Conducting some classic GI test he didn’t find anything but presumed it was some type of reflux and sent us on to a Gi specialist. She was 3 by the time we got into the GI specialist. All the while she was still progressing fine, growing, eating , playing ….The GI specialist conducted a few other tests over the next year and started her on ranitidine for a presumed ulcer and Prevacid for the puking to protect her esophagus.
In March of 2012 right before she was turning 5, she did a GI scope and found no ulcer, proceeded to order an MRI to check hormone secretions in her brain from her hormone glands since we noticed she was also breast budding and these vomiting spells seemed to by cyclic. Larissa would have days at this point where it seemed like a vertigo where she would be sick like the flu puking all day every time she would try to move another nauseous spell would hit. She never had a fever. Then like a light switch was flipped off she would wake up and be back to her normal self and only puking once or twice a day.
The MRI was conducted April 2012. The next morning at 6 am I will never forget that call. The G I specialist called to tell me the findings of the MRI. I thought it was weird he was calling me himself and not a nurse. He told me there was a lesion in Larissa’s brain in the right temporal lobe and that sometimes when children vomit it is actually some type of seizure. Everything froze for me in that moment. I cried. I prayed. I couldn’t believe something so serious was going on with my baby. I kept hoping this puking thing was something with an easy fix and nothing serious, but it was getting worse and I knew in the back of my mind something was not right.
In May of 2012 Larissa was referred to a neurologist due to the MRI findings and the possibility of her having seizures. The neurologist immediately started her on trileptal just by our description of spells of nausea.
In July of 2012 an overnight EEG was conducted in the hospital. It identified that Larissa was having up to 20-30 seizures per hour! The seizures were the cause of her spells of nausea. The next year there were several hospital trips where she would be having these spells all day every day.Aware that seizures were the cause, we would go back to the hospital. It seemed as if countless seizure medications were tried. She was put on phenobarbital (which she was allergic to), onfi, Keppra and Vimpat. All had the same effect. They would help a little and then their effectiveness would wear off.
The next year in March of 2013, after another hospital stay where her seizure frequency reached 30 per hour all day, no sleep barely eating, it was determined Larissa was a candidate for brain surgery. They told me she would have an 80% chance of being seizure free after surgery.As scary as it was, I agreed. I wanted to do anything to help her get better.
During this time Larissa was still able to thrive and play and progress. She loved swimming and going down the big slide at the Mooresville pool. She knew how to read and write. She loved the library and singing.
The surgery was performed on June 28, 2013 and lasted 12 hours.Afterward, the brain surgeon told us there was much more scar tissue than she had anticipated. She removed almost the entire tail of her amygdala and part of her hippocampus. Larissa recovered well. She was seemingly seizure free for about a month until episodes of nausea slowly crept back. She had started kindergarten in August and was one of the only kids in her class who could count to 100, write her name and read. Despite her intelligence, she had a lot of behavior problems and low attention span. She had an attention span of about a minute and would have a lot of outburst of pinching, biting or screaming or rolling around on the floor. Because of her challenges, she was in the student support center a lot and was cut down to half day attendance.
In October I noticed she would fumble with her pencil a lot. She was right handed but was using her left hand instead. I mentioned this to the neurologist thinking it might be some type of brain dominance issue since her surgery. They didn’t know. By December 2013 I figured out why she was dropping her pencil. She had a twitch in her right hand and foot.Every few seconds they would twitch, which also caused her walking gait to be out of alinement as her right foot was thrown outward.
After 6 months, and with no new findings of seizure activity on a comparative EEG, and having weaned her onfi, to eliminate the possibility that she had developed a movement disorder caused by the medication, the neurologist thought her movements were “behavioral”. Larissa’s twitching had escalated to such a point that she wasn’t able to walk or eat anymore. Her condition was so severe she had to be pulled around in a wagon at school. By May 2014 they decided it was no longer safe for her to attend school. One year almost to the day of her brain surgery, in July of 2014, she was admitted to the hospital once more. It was during this hospital admission another EEG was performed that found she was having a constant partial seizure coming from the left side of her brain now.
Before her surgery the previous year, after several tests, I was assured that there was NOTHING on her left side and no seizures coming from there. This was why they had such high confidence the surgery would be successful. They were WRONG. She was now having the same type of seizures, and much worse coming from the left side of her brain. Whatever the cause, it had progressed. One of her neurologists thought her condition was caused by brain inflammation of some sort and mentioned autoimmune epilepsy. He started her on Solumedrol and IVIG in late 2014.
She experienced great improvement after her first IVIG treatment. She went from spending a great deal of time in a stroller to coming home and playing and jumping on the trampoline. It seemed the IVIG was a magic cure. but it was short lived. Larissa’s seizures came back the next day. Larissa continued IVIG with solumedrol pulses for the next 6 months. She had hit a plateau. She wasn’t declining. She was walking but was still having daily seizures. At this point her treating neurologist felt he needed to be seen by a specialist as she felt Larissa’s case was beyond her expertise.
The new neurologist immediate stopped the IVIG feeling it wasn’t warranted. She questioned the treatment with a laugh in her voice indicating she felt it was not medically necessary. June 2015 was her last IVIG treatment. After stopping IVIG, a VNS was placed in August of 2015. In October of 2015 a swallow study was conducted and confirmed she was aspirating when she drank or ate. A g-tube was then placed. The g-tube was so hard on my little girl. She loved to eat. As a parent, I was beyond frustrated in trying to get my child healthy.
By 2017 her health declined, and her education suffered because of it. She was now behind her classmates. While she was able to retain learned skills from kindergarten she was not able learn anything new. In hindsight I wonder if the IVIG had been continued, would she have had this downhill slide? It was such a slippery slow slope. I didn’t connect the two until I was able to look back over time. She was having 20-40 seizures a day. Her doctor would adjust her seizure medication or try another medication trying to find the right combination that could arrest them.One of her neurologist’s considered “presumed Rasmussen encephalitis” as a differential diagnosis in her chart noting she “didn’t know what else it could be”. She explained to me that the treatment for Rasmussens was removal of the half of the brain affected and Larissa had already had surgery on the other side, so she was just stuck basically. We were told there was no treatment. It is what it is. All we could do was try to manage her seizures with seizure medications.
All the while I kept asking her what else it could be because the hallmark with Rasmussen’s encephalitis is atrophy on the side of brain affected, and this had been going on for years and she had no atrophy.She would again tell me there was nothing else it could be!
Finally, in November of 2017 I requested a referral for Larissa to be seen by a neurologist, Dr. Grenier at Cincinnati Children’s Hospital, who specialized in treating Rasmussen’s patients. In March we received confirmation that it was not Rasmussen’s. Dr. Grenier explained that her MRI finding showed she should still be walking. He noted brain inflammation on both sides of her brain on the MRI done prior to her surgery in 2012 and felt that the surgery had been unwarranted. While it didn’t hurt her or cause anything to worsen, he felt based on her MRI that the surgery would not have been beneficial. He was determined to arrive at an accurate diagnosis. He ruled out mitochondira disorders and genetic disorders and ordered antibody testing that were sent off to the Mayo Clinic.
On May 8th, 2018, ten years after our journey began, we found out what was wrong with our daughter. It was treatable. She had Anna-1 autoimmune encephalitis. Larissa was admitted into the hospital for what would be a 6 week stay. Anna-1 has a cancer association. All testing for cancer was negative. They started her on 10 rounds of plasmapheresis. Her MRI showed that there was no brain cell death. So, if we can get the inflammation down, she would regain full movement in her right and walk again. We noticed some improvements with the plex (plasmapharesis), her next treatment was Cytoxan, a chemotherapy treatment that can impact the inside of the brain cell where they Anna-1 antibody had taken up residence. She received her first round of 6 Cytoxan treatments in the hospital before finally coming home. After each treatment we noticed positive improvement. Her seizures were less frequent, and she was more alert.
Each Cytoxan infusion required an overnight hospital stay.
Our journey had taken us to many different types of doctors. Now we were with doctors who specialized in autoimmune neurology. To date, when I asked what could cause her AE, I was met with an unclear answer. Infections and tumors where the only thing known to cause antibody mediated AE per the research up until that point. The doctor who was treating Larissa at the time was relocating and referred us to Dr. Michael Sweeney in Louisville, who happens to be on the Medical Advisory Board of IAES.
At our first appointment, Dr. Sweeney said he had been studying Larissa’s chart trying to figure out what could have triggered her disease process. He felt it was a vaccine reaction. He went on to tell me about a whole vaccine court set up for people like Larissa and that it is fairly easy to file and that I should file a claim for Larissa. I tried and there is a 3 year time limit from the time the vaccine is given, even if it takes years to get the correct diagnosis. He also started seeing my younger daughter Layla who has autism and determined her “autism” was caused by her vaccines that can cause encephalomyelitis followed by onset of regression. ADEM is a type of AE that has a known trigger of vaccination. He has given both of my daughters medical vaccine exemptions. Due to a family vulnerability, and this is specific to my children, so please don’t jump to any conclusions, both Dr. Sweeney and our pediatrician agree that in their circumstances vaccines should be avoided.
After Cytoxan, Larissa underwent tocilizumab infusions. We continued noticing improvement. After 6 months of treatment Larissa was moved to an actemra pen. An injection I can do for her at home every 2 weeks. In November 2019 IVIG added because she seemed to have plateaued with tocilizumab.
Larissa has continued to improve. When her school saw her on a zoom conference call last March they were ecstatic at how alert and physically strong she was. She still has a ways to go. She is not yet walking or able to color with either hand. We can have IVIG home infusions monthly, with a home infusion nurse attending.
It has been a long journey. I had a dream recently. I was on the phone telling Dr. Sweeney that I was concerned because Larissa wasn’t walking yet. Upon hearing my end of the conversation, Larissa got up off the coach and began walking around the house smiling proudly. “Look at me!” She called out with joy! I take my dream as a divine motivation to never give up hope. What seems to be the impossible will be possible.
My daughter Larissa has Anna-1 antibody positive autoimmune encephalitis and is now 13 years old.
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 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.
Jan. 1, 2020 – It was a new year, and based on the progress that he’d made I emailed Dr. Tomatore regarding this progress and questioning, through my research, exactly what tests results were reviewed, what tests were done and how exactly the diagnosis of CJD was made? Dr. Tomatore never responded to this email, nor did he an acknowledge receiving it.
For the next two weeks, the medical team was re-evaluating Reggie’s care and treatment plan based on the progress he was making and with different symptoms. During this time new MRIs, blood tests, and EEG were ordered to determine what exactly was going on.
I then got a call from the resident in charge of Reginald’s care for that time. Upon review of: the EEG, which showed NO EVIDENCE of the preliminary things they saw earlier; and the MRI, which showed evidence of clearing up the previous spots; and the blood tests, that were still to be determined, they were moving off the CJD diagnosis and agreeing with me that this this was AE. They were also moving forward finally with the next line of treatment, rituximab, and he was set to receive that treatment.
Rituximab had been denied by insurance from the first of December based on the CJD diagnosis, but now it was approved based on the correct diagnosis. There was also a family meeting around this time, at which they recommended sub-acute rehab for Reggie, asking me for facilities that I may suggest in the area for him to go, and the team moved forward with sending referrals.
On Jan. 20, the blood test results came back and the CASPR2 antibody was identified as the cause of his AE. Reggie then went through a full-body CT scan as the creation of the antibody is known to fight off cancer. The results of the CT scan showed no traces of tumors or cancer anywhere, and the team would still try to move him to a sub-acute rehab. Two days later, the nurse case manager called to tell me that he had been accepted to a facility in Lanham, MD. There was concern on my part because I did some research and I was uncomfortable with reports on quality of care by that facility. Then there was a snafu with both time of discharge and dispense of medicine, which he never received.
Upon arriving at the subpar sub-acute center, as Reggie was settling in I was talking to the nurse—there was only one of her for 13-plus patients—he fell twice against a cabinet with metal handles. Prior to his being admitted here, I had let them know he had to be restrained in both his bed and chair with a waist belt, as he attempts to get up and falls. They denied I said that, letting me know they would have never accepted him under those conditions.
Ultimately, I read the reports provided and they only stated that Reggie was agitated at times and would get up, which was a blatant untruth. As a result of two falls over 24 hours, they called 911 and he was transferred back to the PG ER.
Upon arrival at PG, Reggie was evaluated by the attending physician, who was familiar with him from his original admission. I informed this doctor of the AE diagnosis and what caused him to be brought back to the hospital. He let me know he would evaluate him and try to work with me regarding my requests moving forward. Reggie then checked out fine from the falls and that they had no medical reason to admit him.
Knowing the situation however, the physician would try to complete a doctor-to-doctor transfer for him from PG to the attending doctor at Georgetown. While the doctor was doing this, two social workers told me Reggie had three options:
He could be discharged to me and go home.
I could transport him to Georgetown ER.
He could be taken back to the sub-acute facility, as I hadn’t given them a chance.
I was opposed to all options and I was not signing for his discharge, so I got a CareFirst case manager to talk with the social workers. They came back to me with the case manager on the line and it was determined that Reggie would return to the sub-acute facility, be evaluated by their medical staff and nurses, so they could determine he’d be readmitted to PG’s rehab. I agreed and he was medically transported back to the facility that evening.
Upon Reggie’s arrival for the evaluation, I was taken into the conference room to meet with their team. They told me they could not do an evaluation, as promised, and could not recommend acute rehabs for a patient. Also, they informed me that they could not keep Reggie, as they could not ensure his safety, as by law they cannot do any form of restraint. They advised for me to take him back to Georgetown ER on my own and that I not him leave there.
I replied that I was unable to take a medically fragile, immunocompromised patient, on a cold night in my car. They then agreed to have Reggie transported back to Georgetown in a van, but without medical personnel, to the ER at Georgetown. I got on the phone again and informed the case worker all of this, and the violation of the agreement worked out. The van then arrived and did not have a wheelchair in which he could be transported. The staff said that if he used their wheelchair, I needed to bring it back the next day. I agreed and he was prepared for transport. Just as he was wheeled out, they told me that the van had left and they could not contact the driver to come back.
I informed the staff that as a last resort I would take Reggie to Medstar Georgetown in my car as HE COULD NOT STAY HERE ONE MORE MOMENT because this facility WOULD NOT BE A PLACE I WOULD WANT ANY LOVED ONE! Also, I informed them that if Reggie, because of his compromised immune system, got as much as a sniffle and this caused his recovery to be extended, I would be suing the facility. He was placed in my car and I began the took him to Georgetown. All this time, Reggie wasn’t provided with the crucial medication that was prescribed for his condition.
As I was transporting him to Georgetown ER, I called ahead to inform them of the situation. I also called the supervisor of case managers and the case worker so that everyone was fully aware. During three conversations with the supervisor, I was told the sub-acute rehab hadn’t known about Reggie’s needing restraints on his waist and an alarm on the bed, prior to or when making the referral.
He was admitted to the ER, where he stayed in a room for two days, despite needing an individual room because of his agitation and need of a sitter. I was also informed at this time that referrals would be made to Encompass Health in Virginia or Capital Region Physical Therapy at PG for acute rehabs. The director for Encompass called and let me know they would turn him down, but would re-evaluate if Reggie required only an alarm on the bed as an alert.
Jan. 24-Feb. 5 – Reggie received a room assignment in the post-surgical unit. I was impressed with the care as he was receiving PT and OT every day, and he was walking and progressing with less agitation. They were actually working with Reggie to devise a plan for him not to be restrained by the waist belt, and he was right outside the nurses’ station and they available to assist him immediately.
The social worker and the rehab physician for the unit, though, had no clue that Reggie was a returning patient to the hospital until I told them. The rehab doctor actually offended me by saying, “I would suggest or recommend a sub-acute rehab because that would get the most bang for your buck from insurance.” This was based on getting things out of insurance and not on the patient’s quality of life. The last time rehab was suggested and tried, Reggie went to acute rehab then to sub-acute rehab, both of which were cleared by insurance, and this would have been a total of 10-12 weeks or less, if they would have done it correctly?
Feb. 5 – Reggie moved back to the neurology unit and the care varied as they attempted to get him to be less agitated so he’d be accepted by acute care rehabs. There was no family meeting regarding this plan or any plans moving forward once Reggie was readmitted to Georgetown. When he was there previously, he received PT, OT and speech daily, and the nursing staff was walking him and getting him up to the bathroom daily, as he showed the interest. This no longer happened in the neurology unit. Most everything that Reggie did and attempted to do was prompted by me when I visited, or when I showed the nurses who agreed to do it and tried when I was not able to be there.
Feb. 15-22 – While visiting him in the evenings over two weekends I assisted him in eating of his meals. I then noticed he was not chewing as well as he had been previously. I inquired of the nurse and tech if this is what they had experienced during the previous meals, and they confirmed it.
I later noted that Reggie’s bottom row of dentures were not in his mouth because someone had removed them and placed them in a green cup. I alerted the nurse of this on Feb. 16, as my concern was that this could have caused choking or other problems with his lungs. Her response was that she did not know he had a bottom row of dentures, but said she’d write it in his record chart. Imagine my surprise when I came in the next weekend and discovered the same thing was happening and his bottom dentures were removed again; when I was assured that all would be informed? So, I took the action of writing on the chart, in his room to ensure dentures are in before eating, and that he has both top and bottom.
Feb. 27 – I was contacted at 2 pm by the attending physician that Reggie had experienced a fall while trying to get out of his chair. Because the footrest was in the up position, he got stuck and fell. Though the chair’s alarm sounded, the nursing and tech staff didn’t arrive in the room, and he fell on his knee and head. It was our experience that the nursing and tech staff were inconsistent in arriving in the room after Reggie or I hit the call button. All staff were aware that if Reggie tried to move or grabbed himself, he was letting them know he needed to use the bathroom and attempts should be made to get him up. The connections within his brain are being encouraged to promote independence. Right now, though, he needed assistance because I didn’t want the habit to form of just urinating or having a bowel movement on himself while trying to go as he normally would.
Mar. 2 – A meeting with the medical director and others of Georgetown was held regarding “next steps” and where they were planning to send him. The doctors gave me a great deal of information and I respectfully allowed them to speak. But they contradicted previous statements from other doctors. I stopped them from talking and said, “Reggie will not be going anywhere except Medstar National Rehabilitation Neurorehabilitation Acute Rehab or Encompass Health and Rehabilitation in Virginia!” If they proposed anything else, they would hear from a team of lawyers that I had contacted with all the prior information. Within the next hour they had a new PT physician evaluate Reggie and he was then set to move to Medstar NRH on Mar. 5.
Mar. 5-Apr. 1 – Reggie was admitted to Medstar NRH and made great progress there receiving an hour everyday of speech, OT and PT. I was there daily until I wasn’t allowed to because of COVID-19. Reggie finished his 30 days in acute rehab and was transitioned to sub-acute rehab at Largo Nursing and Rehabilitation Center.
Apr. 1-28 – Reggie was admitted and continued sub-acute rehabilitation at Largo Rehabilitation and Nursing Center and even though this facility was just a 10-minute drive from our house I could not visit him, because of the Covid-19. I was able to talk on the phone or Skype with him daily. This absolutely killed me, as I knew what we had gone through in the past and I wanted to be with him to ensure the maximum was being done. As we were battling AE, limited knowledge of healthcare workers and therapists have of both it and COVID-19 caused concern.
Apr. 28-Jun. 28 – Reggie was discharged from the sub-acute rehab for home-based rehab through the Medstar VNA. He received 10 sessions of PT and OT and 14 sessions of Speech throughout this time, and he improved every day.
Jul. 27 – Reggie was taken to Georgetown because he had a two-minute seizure at home. It was determined this “breakthrough” episode was caused by Reggie’s not following his medication protocol, i.e. not taking anti-seizure medications as prescribed. Even before his bout with AE, Reggie was non-compliant with medications and argumentative about taking anything, even vitamins. While in the hospital, he completed his second round of rituximab infusion.
Aug. 13- Oct. – Reggie is now an outpatient who receives OT and speech therapy at least twice weekly. He is making great strides and progress and I know will continue to recover with these therapies. I am impressed by these therapists, as they are trained in the field and have a neurological background. The OT and the speech therapists work together to help Reggie with his aphasia and apraxia toward reaching his stated goals. At the time of this writing, I am hopeful and joyful of what is going to be a better than before recovery!
Conclusion – This is the entire saga to this point with Reginald Johnson. As you can see, we have been through much that is both heart-wrenching and frustrating. At times, we not only have had to fight this disease, we have to fight the medical community so that they would not stop searching, treating, and taking proper care of him.
Reggie appears to get better every day. I have faith that full recovery is possible and will happen. I wrote this so all who choose to read it can be helped and assisted in any way. More importantly, understand to fight for your loved ones—especially when they cannot fight for themselves!
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.
October 28, 2020 | By Dr. Robert Larry Reese-Johnson
Oct. 3 – This was the date in 2019 that this all began for my husband, Reginald Johnson-Reese, and me. I arrived home from work around 5 pm, and saw Reggie on the couch. We had a normal conversation as every evening. I then went upstairs to change and went to place my clothes in the washing machine.
The laundry room was in total disarray, as if someone had fallen within the room. I asked Reggie what happened and he couldn’t remember. He said he had passed out and fallen, but couldn’t recall when, nor for how long. He didn’t want to go to the hospital, had no injuries and seemed to be holding normal conversations. Knowing what I know now, this was what I believe to be his original seizure that sparked the entire saga that I am documenting here.
Oct. 5 – Reggie complained this entire day that someone was talking in the background of his phone conversations through the earpiece he was using. He went so far as to call his mobile carrier, asking whether it could possibly be an app running in the background or an issue with the phone. I now know this was another symptom of autoimmune encephalitis (AE).
Oct. 6 – Reggie and I were on our way to church. He was unusually quiet and not making comments about my driving. When I asked Reggie where we were going, he answered me in “gibberish.” I then asked him who I was, and he answered again in the same “gibberish.”
Immediately, I took him to the nearest hospital, where we walked in together to the Emergency Room (ER). He was admitted to the University of Maryland Capital Region Healthcare at Prince George’s Hospital Center (PG), for symptoms of what they diagnosed as a stroke, though he never had all the symptoms of stroke, nothing other than confusion and not speaking correctly. He was treated with tissue plasminogen activator (TPA) medications, to which he had an allergic reaction and then small seizures.
Oct. 7-16 – Reggie continued to receive inpatient treatment for what was believed to be a stroke. During this stay he needed a feeding tube to provide nutrition and calories for him. The initial tube did not remain in place and twice it had to be repositioned and reset.
Oct. 16-Nov. 1 – Reggie was admitted to and received acute rehabilitation treatment from the physical therapy unit at PG, progressing from what they still believed to be a stroke. Later data provided to me on three different occasions while at Georgetown Hospital, indicated Reggie had no current nor previous evidence of a stroke, based on MRIs from that facility’s images. As a result, Reggie was misdiagnosed and treated for a medical condition that he did not have any evidence of having.
Nov. 1 – I received an afternoon call and request for a joint conference with the treatment team and social workers from PG. Reggie and I were advised that he had received clearance to be admitted to a sub-acute rehab center, Capital Region Future Care. While on the call, Reginald determined that he did not want to go there, against the team’s medical advice. He was allowed to make this decision, despite the fact that he was cognitively unable to process what was being said to him, per the facility’s speech and language pathologist, who had diagnosed him with Wernicke’s Aphasia which impacts his memory and cognitive processing abilities.
I had informed the attending doctor on Oct. 29 that the doctors, nurses and therapy staff would need to prepare Reggie for the next level of care, i.e. that he would not be coming home because he had another step in the process. They obviously did not have these conversations.
At 7 pm, under the direction of the social worker and supervisor, the hospital made the recommendation of discharging Reggie for home care, regardless of his readiness. They knew the Visiting Nurses Association (VNA) could not come to evaluate him at this late hour, and they informed me of that. I told them that I could not take off from my job to provide him with care on a full-time basis without VNA coming to the home daily.
Regardless, PG allowed Reggie, without the ability to read or understand what he was reading, to discharge himself. He was experiencing agitation and bits of rage at the time directed at me as I would attempt to assist him. The supervisor of social work said to me, “Mr. Johnson has to go because he cannot stay as the insurance company is not paying for a bed here past midnight…”
Nov. 2-4 – Reggie was at home with me, during which he was unable to read his medications, had two separate anger episodes in which he attacked and argued with me. I called the VNA to come for an initial evaluation on three different occasions on Saturday and Sunday, left messages and got no response to my phone calls. When Reggie threatened me on Monday morning, I called the attending doctor on his discharge papers and the VNA and was advised to contact 911.
The ambulance and police arrived and the EMT confirmed that Reggie could not determine the correct date and year, remember what the situation was, nor that he had previously attacked me. Reggie was given the option to allow me to drive him back to PG’s ER, which we did. He remained there, in a bed in the hallway for about 15 hours, before being transferred to his own room. He was disoriented, agitated, combative with me, and cognitively unable to process.
Nov. 4-8 – Reggie was readmitted to PG for review of medications and the symptoms he was experiencing. During this time no further MRIs were ordered, nor were any other tests done to determine whether he was experiencing the typical symptoms of a stroke, which could have determined something more significant was going on with him.
Nov. 8-10 – He was discharged that afternoon again to receive home care from the VNA. I called them twice that day, three times the next day, and twice again the following day. Again, no one responded to my call to come to do an evaluation or schedule an evaluation of him until the third day, while we were on our way to church. I let the caller know we would be back by 1 pm and they could come then. I was informed that this would not happen and that they were unsure if they could come on Monday.
While at church, Reggie experienced a seizure towards and was transported by ambulance to Medstar University of Georgetown Hospital (Georgetown) for assistance. There, he was treated with anti-seizure medications and had images taken through MRI and CT scans.
Nov. 11-13 – Because the doctors were concerned when Reggie’s level of
speech didn’t return to his baseline, he received care in the Neurology ICU unit, because the doctors were concerned that at this time Reginald did not return to the level of speech that was his baseline prior to the seizure. That was an indicator that he was suffering from something else other than a stroke.
Nov. 15 – I was informed by Dr. Mayson, a neurologist and stroke specialist, that Reggie never had a stroke, because the MRIs from PG and the ones obtained from Georgetown showed no evidence of dead areas of the brain—the major sign of a stroke. This should have been discovered by the neurologists at PG and upon that information, if they did not know what Reggie was experiencing he should have been medically transported to a facility in the area prepared to treat other possibilities.
Georgetown immediately began to treat Reggie for AE, a diagnosis that from the onset of symptoms requires an aggressive treatment course, and we know now that is actually the disease that Reginald had for six weeks. His misdiagnosis and receiving treatment for the wrong ailment may have caused further relapses and a continuation of the disease to the progression that it has now. This has also had an impact on his time of recovery and long-term effects as his life proceeds.
As a result, I filed an online complaint with the Maryland Joint Commission on Health and the attorney general’s office for investigation of the above.
Nov, 14-15 – After review of both the PG and current MRIs, it was determined that there was no evidence of stroke activity within Reggie’s brain. This is when the neurologist believed that Reginald may be suffering from AE, and began treatments with five days of steroids and IVIG. They also performed a procedure in which a tube was inserted through which five infusions of plasmapheresis could be administered. The attending neurologist conducted a lumbar puncture to obtain cerebral spinal fluid for testing, as all other tests were coming back negative for all other diseases or cancers.
Nov. 20- Dec. 1 – Reggie was to receive five plasmapheresis infusions over the course of 10 days. I was informed by the technician that was doing the treatments Nov. 20-21, but that he could not have three treatment days in a row. Therefore, he would have one on Nov. 23, then again on Nov. 25 and 27.
When I arrived at the facility on Nov. 25, I was told that he was completed his treatments. Naturally, I questioned what I was told and said he would need at least one more treatment, maybe two. The nurse responded, showing me on the computer that they had recorded the treatments. I questioned the dates because I had not seen them there that day. I was ignored and they attempted to reassure me that he was done with those treatments.
On Nov. 23, Reggie had surgery to insert a GI tube for feeding as he was not eating on his own. He was up from recovery by noon. I was with him from early that morning until the evening. When I was leaving, I told the nurses’ station that he was bleeding into the bag attached to his stomach tube and the tube attached to his nose; I was informed that this was normal clearing or the stomach contents. I questioned this as it was now just seven hours’ post op and this was not the case for the entirety of the day.
At 5 am the following morning, I got a call from the head of the surgery team that my permission was needed for emergency surgery to replace the tube and reset it as Reginald had internal bleeding overnight (which I was not informed about by the floor nurses that night) as the tube had come loose from the abdominal wall. I let them know I’d told the nurses as to my concern prior to leaving and was ignored or told not to worry.
After the second surgery was completed, the surgeon came to the room and explained the person performing the first surgery placed only two staples to secure the tube, thinking it would stay with that way, despite given Reggie’s level of agitation and movement during the surgery. Four staples were placed in during the second surgery as that is what that surgeon customarily does.
In advance, I had let the medical team know he is severely agitated with tubal restriction, and that this tube remaining in place was a problem at the first hospital, but I was ignored. The lead team surgeon stated to me that I should not worry and that what happened with Reggie would be a topic of their next team meeting. I was furious!
His final night, he was moved overnight to the ICU because his oxygen level dropped to a dangerous level. Once more, they neglected to tell me that this had happened.
Dec. 3-5 – Reggie was moved twice more because he was no longer in need of IVIG treatment. At this time, the plan was discussed with me by his attending team was to obtain another lumbar puncture, conduct more MRIs, and proceed to Rituximab treatments, as Reggie was now 12-15 days from his last plasmapheresis treatment, and there had been little or no change.
Dec. 11 – I was called for a meeting with Dr. Tomatore, the medical team and a palliative care doctor. During this meeting, they told me Reggie’s diagnosis was Creutzfeldt-Jakob disease (CJD), fatal without cure and no treatment. It was explained that he would not get better, but would progressively get worse and most likely quickly decline to the point of dying. They added his overall life expectancy was a year or less, and that more than likely he would contract an infection, and this would take his life. We were advised to change his resuscitation orders from resuscitate to do not resuscitate as there was no hope of Reginald surviving this disease, so we complied.
This devastated my family and me, and I also told that because Reggie would need a nursing home and/or hospice care that I would need to work with the social workers to attain Medicare and/or Medicaid for the long-term care for Reggie. This was heart-wrenching because the date was right after our anniversary, Reggie’s birthday, Thanksgiving and within weeks of Christmas.
Dec. 12-13 – After researching CJD, I sent an email to Dr. Tomatore in which I requested a second opinion, including the exact version of CJD that Reginald they’d diagnosed. My question was regarding the signs I was seeing that weren’t
consistent with what I was reading from CJD. I also asked for an explanation of all the tests and images used to determine that this was CJD, as I understood that the most conclusive and newest tests used to determine CJD came back negative, but they were ruling that out because it had a 10 percent false-positive rate. Dr. Tomatore responded that they were excellent questions and requested my phone number to call me, which was within the email. I never received a call.
Dec. 16 – Reggie’s sisters and I met with Dr. Mayson and the team to review the the MRIs taken prior to the treatments, the tests and additional information related to care he received before being transported to Georgetown. Dr. Mayson explained that the MRIs conducted prior to treatments or the anti-seizure medications demonstrated the areas of the brain that were impacted were the outside portions of primarily the frontal temporal lobe.
He also said the CSF tests came back with three of the four tests positive for the protein for CJD, and these tests were 85 percent accurate. The team again stated that there was nothing more they could do except keeping Reggie comfortable, and that I should look into long-term or hospice care because CJD takes an aggressive downhill path to death.
Dec. 11-27 – Reggie was still under the care of the neurology team and the palliative care doctor, and they were treating only his symptoms. They didn’t conduct tests for images, fluid or blood after the November treatments were completed. It felt the team was no longer concerned for him, and needed him out of the hospital to make space for someone that was not going to die.
A few days before Christmas, the medical and social work teams came into Reggie’s room to tell us he was denied both Medicare and Medicaid. What happened was they submitted incorrect income to receive the benefit: my income wasn’t as much as they indicated, nor was he receiving a pension. With the information at hand, they recommended discharging him for either hospice or home health care, to be paid for by me or us.
I was insulted and livid because the figures that I provided them with were misinterpreted by both them and the disability office. I reiterated the correct information for them to resubmit for review: Reggie doesn’t have a pension; we do not have assets; nor do I make a salary that is able to pay our monthly bills as well as pay for a hospice or long-term care. As a result, I refused signing on for discharged anywhere without a review of the corrected information.
Shortly after Christmas, Reggie began speaking his name, interacting with the medical team and nurses, and the palliative care doctor. He was showing signs of progress not in alignment with the CJD diagnosis! On Dec. 28, Dr. Mayson examined him, stating he was more interactive than he had ever seen him and added, “Well, Mr. Reese, medicine is not an exact science.”
Right before the new year, Reggie wasn’t in his room when I arrived around noon. I inquired at the nurses’ station as to where he was, hopeful he’d been taken for more tests based on the improvement. They scrambled to his room as they had just left it, and he was in the bed. They opened the bathroom door and we saw Reggie on the floor. He had gotten up from the bed on his own and fell, as he had either walked or crawled to the bathroom. He told me they said I was coming and he wanted to take a shower. After changing and cleaning Reggie, they had unfortunately failed to set the alarm on the bed, nor did they set the wait-belt restraint that he had to ensure he not get up by himself.
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.
Laura Martin is a 20-year-old college student from Winchester, Kentucky, right outside of Lexington. She was a Kentucky Governor’s Scholar and student at Transylvania University, as well as a standout goalie for the university’s women’s soccer program.
In August 2019, Laura’s future was on the rise. But in a matter of a few days, she and her family were soon facing their lowest moment. Her health suddenly declined. And no one knew if this promising student and gifted athlete would make it past 20 years old.
As she was getting ready for her sophomore year and second season with the women’s soccer team, Laura started behaving differently. Her friends began to notice she wasn’t herself. They told her family, who also noticed a drastic change in Laura’s behavior.
Laura wasn’t sleeping and started having intense paranoid delusions, acting in a way that was completely unrecognizable to her friends and family.
“I didn’t even know who this girl was,” Keri Martin, Laura’s mother, said.
Laura’s condition continued to decline. On Aug. 28, 2019, her family decided to take her to the Emergency Department at a Lexington hospital close to where they are from. When the family arrived, they were told that Laura was suffering from mental illness. Doctors took Laura into the behavioral health unit to be placed on a 72-hour hold.
With no family history of mental illness, Keri requested neurological testing for her daughter. But Laura’s doctors were dismissive of her request, and released her from the hospital after seven days without any solution to her symptoms.
“I thought this was how life was going to be for me now,” Laura said. “Coming back from the hospital was hard. Not being in school and missing my friends was difficult.”
Although Laura seemed better when she returned home, she immediately reverted back to behaviors that frightened her family. After having a poor experience at another healthcare system, Keri and James, Laura’s father, didn’t know what to do. They had many sleepless nights as they tried to take care of their daughter. With help from other family members, they watched Laura around the clock as she got worse day by day.
Laura wasn’t able to sleep and couldn’t walk, write or remember who her family members were. She was losing her cognitive ability at an alarming rate and she could no longer take care of herself for basic tasks.
“As a family, we were broken and at the bottom. There was nothing that could be worse than this,” Keri said.
Keri fought to get Laura in to see a neurologist in Lexington, only to be once again rejected by another physician. The family was told that Laura should be sent to a behavioral health unit. Keri felt that if they sent Laura back to a psychiatric facility, she would die there.
“We didn’t know what to do or where to go,” Keri said.
Running out of time and answers, Laura’s parents decided to bring her to Cincinnati to UC Health, home to the region’s No. 1 preferred provider for neuroscience care.
Immediately upon arrival at University of Cincinnati Medical Center on Oct. 9, 2019, the Martin family knew this experience would be different. Clinicians compassionately spoke to the family and quickly arranged for Laura to have a private room in the neurological unit.
Laura’s treatment when she arrived at UC Medical Center was led by Jordan Bonomo, MD, UC Health neurologist, associate professor in the Department of Emergency Medicine and director for Neurocritical Care Fellowship at the UC College of Medicine.
An experienced group of residents and nurses made Laura’s family feel at ease, leaving them with a glimmer of hope for the first time in months. One resident involved with Laura’s care even told her family that she would advocate for her.
Another resident, Laura DiDomenico, MD, remained in the unit with Laura even after her rotation ended so she could see her treatment through. Laura’s entire care team was united and committed to finding out what was wrong and how to save her life.
“It was an entirely different experience from the moment we walked through the doors of the Emergency Department,” Keri said.
Laura’s Recovery after having “Brain on Fire”
Laura had an EEG that revealed she was suffering from many small seizures, leading to her unusual behavior. The seizures were part of a neurological disease called Autoimmune Encephalitis, which refers to a group of conditions that occur when the body’s immune system mistakenly attacks healthy brain cells, leading to inflammation of the brain. This condition is often referred to as “brain on fire.”
Her family suspected this was the reason for Laura’s rapid health decline, but it wasn’t until they came to UC Health when this was confirmed. No other healthcare system in Lexington would even consider offering Laura neurological testing.
Over the next 12 days at UC Medical Center, Laura’s care team worked tirelessly to find a way to improve her condition. Joseph Broderick, MD, director of the UC Gardner Neuroscience Institute and professor in the Department of Neurology and Rehabilitation Medicine at the UC College of Medicine, took over Laura’s care on her third day in the hospital.
“Inflammatory disorders of the brain can be devastating and challenging to diagnose,” Dr. Broderick said. “But patients can also respond dramatically to the correct treatment.”
In order to improve her condition, Laura started Intravenous Immunoglobin (IVIG) treatment, which is used to treat various autoimmune diseases. The first couple days were difficult for Laura and her family, but as time wore on, her condition gradually improved.
By Oct. 21, 2019, Laura had completed her IVIG treatment and was well enough to return home. Laura’s treatment included IV steroids, and to this day, she continues using oral steroids. Both of these have contributed to her improved condition.
After discharge, she continued to improve further and subsequently went back to work while waiting to restart college. “Laura is a walking miracle,” Keri said.
“It’s gratifying to see such a positive response from Laura. We are very proud of our treatment team who made the diagnosis and started her on the appropriate therapy,” Dr. Broderick said.
Post-hospital treatment, Laura is seen by Aram Zabeti, MD, director of the Waddell Center for Multiple Sclerosis at the UC Gardner Neuroscience Institute and associate professor in the Department of Neurology and Rehabilitation Medicine at the UC College of Medicine.
“As the region’s academic healthcare system, we are proud of our ability to diagnose and treat rare diseases such as Autoimmune Encephalitis,” Dr. Zabeti said. “Early intervention in Laura’s devastating disease saved her education, productivity, family and even her life.”
Handling the COVID-19 Pandemic
When the COVID-19 global pandemic began, Laura’s family made sure to keep a close eye on her to protect her from possible infection due to her compromised immune system. Laura continued her post-hospital steroid therapy during the pandemic. Unfortunately, she wasn’t able to continue her part-time job she recently started, as the store she worked at temporarily closed to prevent the spread of COVID-19.
With the help of Dr. Zabeti, Laura successfully completed her steroid therapy and is no longer considered immunocompromised. She will be able to return to her part-time job when the store reopens.
Going from a healthy, successful college student-athlete to a neurological patient was something Laura could never have expected. She and her family went through adversity, frustration and fear along the way. But now, Laura is able to return to her life she had to give up prior to her diagnosis.
She’s able to sleep, walk, drive and work. Her next goal is to return to college in August 2020 and complete her bachelor’s degree in English with a minor in Secondary Education.
“It’s amazing to know that I have a condition that I can live with, that’s also treatable,” Laura said.
After being written off by other healthcare systems, Laura and her family found hope at UC Health. They know where to go in the future if anything happens.
“Everyone went out of their way to help Laura and all of us,” Keri said. “UC Health gave us our daughter back.”
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.
She was diagnosed with Anti-NMDA Receptor Encephalitis on November 5, 2019. She was 13 years old then, and turned 14 on March 15 this year.
Our healthy daughter was showing the exact opposite of herself. She was very healthy and athletic. She has a blackbelt in Taekwondo and was bagging gold medals left and right in combat tournament.
All the standard symptom of encephalitis were there. It was very hard to understand and we were very shocked to digest the sudden alteration of our lives.
The early recovery period was the toughest. The IAES Facebook group witnessed how scared and frustrated we were as her parents. She didn’t sleep for 2-3 days. And when she did sleep it was only for 5-6 hour at a times. Often she had tantrums. Crying, shouting and there were times that she just snapped and hit anyone in our house.
Today, is June 1, 2020. Almost 3 weeks that she has been showing very good behaviors. She sleeps on time now and for a good 8-10 hours. Our house is filled with laughter again. She has become funny again. She loves playing with her dolls again. Art, music and movies are her pastimes. Unlike before when she could not sit for more than 5 minutes. She takes her medicine and milk by herself now. She can now take care of herself in the bathroom.
This illness is too much and no one deserves it. Many people helped us during the hard times. And we cannot thank them enough, especially those who prayed for us. Who helped us to understand her situation. And of course, the IAES Facebook group. It helped us know more and understand more. And I thank God for the everyday miracle.
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.
August 4, 2019, was the day that changed our lives forever—one that we call the perfect storm. A few weeks prior to this day, my daughter Brynn had turned two, with a baby sister born a few months before.
We noticed some weird behavioral changes as I returned to work from my 12-week maternity leave. Brynn is my first child, so my thoughts were immediately, “Wow. Two-year-olds are tough.” I kept asking my coworkers and friends that had been through this, whether this was normal, and they assured me that it was simply the “Terrible Twos.” They promised me she would get used to my working again. They even commented, “Wait till she turns three… it gets even worse!”
Brynn had been a very mild-tempered, funny, happy-go-lucky child that I really never had any behavior issue with. Developmentally, she was ahead of schedule: speaking full sentences; knowing numbers and colors; and speaking and understanding a good amount of the Spanish language.
As a week went by, she started not sleeping through the night, tantrums started becoming inconsolable, and exhibition of impulsive behavior. Starting on a Monday she vomited and her appetite changed. My husband, observing very subtle movements like Brynn cocking her wrists outward. He took me aside on Wednesday and said, “It’s almost like she is autistic. Additionally, her behavior towards her cousins changed. I remember my sister and I laughing under our breaths because she was in a RAGE yelling and screaming at them! By Thursday, my mother-in-law reported to me that she was barely eating. She had even turned down a Popsicle in the bathtub which she looks forward to every night!
It is truly the hardest thing as a parent to decide when to call the doctor, you never want to miss anything, but at the same time, you don’t want to be one of those parents that take your kid in for every little thing. My husband and I decided that since I had the day off on Friday, It would be a good idea to just take her in to make sure everything was ok. Throughout all of this, she never spiked a fever!
By Friday morning, she was looking pretty rough. Little did I know, this was the last day I would hear her sweet little voice for seven weeks. At the pediatrician’s office, I explained what was going on. They did the full exam including looking in her mouth. As a dental hygienist, it’s normal for me to observe her mouth regularly, and I had seen nothing weird. Her nurse practitioner stopped and said, “Do you see this little white dot in her mouth? This is very common. Your child has Hand-Foot-Mouth disease.”
She further explained that this is why she wasn’t eating because it is very painful. She also stated to me that it would probably get worse before it got better, and this virus has to run its course. She instructed us to make sure she was drinking fluids and not to let her get dehydrated. On Saturday we laid low, gave her some Pedialyte and began syringe-feeding her liquids and had to bribe her to get a couple drips down. The next morning, I ran a quick errand and my husband stayed home with Brynn. As I was sitting in the parking lot, my husband FaceTimes me, saying, “She is unresponsive,” as he lightly moved her face from side to side, calling her name, all without her making eye contact.
I have never shoved groceries in my car so fast. I instructed my husband to get dressed and I will be home ASAP and we will be taking her somewhere! I immediately got on the phone with my best friend who happens to be the nurse practitioner at the local pediatric urgent care clinic. She said, “Come on in and we will check her out… she is probably just dehydrated. We will give her some fluids and see how she does.” When I got home, Brynn had perked up as if nothing was wrong and I questioned my thought process. We chose to proceed, thinking, “Better safe than sorry!”
We arrived at the clinic and the nurse practitioner agreed that she didn’t seem normal and started an IV immediately. Upon observation, the doctor noticed her eyes were rolling back in her head and noticed unusual movements. They called an ambulance right away due to the fact it looked like seizure activity. I was taken back by how fast everyone was moving and thinking, “OMG, is this really happening! What is going on? Our worse nightmare was coming true. Something is wrong and we have no idea!” We were rushed to the local hospital and there they did a MRI, spinal tap and drew some blood. Everything came back normal, except a herpes 6 virus (a/k/a, Roseola which every kid under the age of five comes in contact with and fights off) which was wonderful, except no one could explain what was going on. The doctor did state that it seemed neurological, but he couldn’t put his finger on it exactly.
From here started a seven-week journey to the world of a medical nightmare with Autoimmune Encephalitis. We only stayed at the first hospital for two nights, due to the lack of attention my daughter was getting. They initially placed her on EEG machines to watch her closely for seizures, which she had none! They treated her for the herpes with a high doses of Ganciclovir, saying it was to risky to do steroids. Getting a doctor to answer any questions or return to our room was a nightmare. There was no infectious disease doctor or rheumatologist, and getting a hold of a neurologist was a chore.
The second day I kept hearing meningitis/encephalitis, though no one said this to me directly, nor could explain anything. At this point, I had not had a meal or slept. I felt like no one was doing anything and I was up all night researching on Google different signs and symptoms. I got wind from one of the doctors that a transfer could be in place if I wanted, to which and I replied, “Yes, let’s get outta here!” This was the best decision I ever made.
The next day we were transferred to Arnold Palmer Children’s Hospital in Orlando where a plethora of doctors asked all the right questions. They ran about 100 different test to different labs, and I could tell they were really working toward a diagnosis. No one had a clue until a Rheumatologist who had previously worked for a doctor that had a lot of experience with patients with AE. She strongly suspected the NMDA Autoimmune Encephalitis but we realized for the test it takes over a week to confirm because it has to be sent to the Mayo Clinic.
They were actually very confident with this diagnosis that they started with very high dose of Solu-Medrol and IVIG for a couple of days. Brynn at this point was in bad shape. They attached ” No-No’s to her arms so she could not pull the feeding tube out. Her eyes were rolling back in her head, she couldn’t sit up, and had this movement disorder which is very common with AE. There were days she went without sleep and then days all she did was sleep! It was so scary to watch your child go through this as you helplessly sit on the sidelines, praying the doctors and nurses are doing what’s best for your child. Then, the diagnosis came back positive for NMDA Autoimmune Encephalitis, at which point the doctors decided to treat it aggressively with five rounds of plasmapheresis and two rounds of a chemotherapeutic drug called Rituxan. We spent three weeks in the ICU and another two weeks on a stable floor weaning her off the drugs, and beginning PT, OT, speech and music therapy. Brynn had reverted back to a baby: sticking things in her mouth; moaning to try and communicate; and crawling on the hospital floors. Around week five, she became a two-person job, changing diapers and keeping her from getting tangled around all the cords to which she was attached.
To make this whole experience even more challenging, Hurricane Dorian was headed to our hometown of Melbourne, and we had a three-month-old infant being passed around the family, driving freshly pumped milk back-and-forth. I fully believe the only way we made it through this terrible nightmare was the support we had from our friends and family. Truly, we would not have been able to do it without them. Kudos to the Ronald McDonald house that housed my family as they came to visit to stay long weekends so I got to see my infant.
Daily, we saw subtle changes. Brynn became stronger and actually started to walk before we changed hospitals. That was the best day ever seeing her up walking, though she was very unsteady. Little did we know, this now presented us with new challenges as she became more difficult to keep in a bed or hospital room.
Our last stop was we being accepted into a rehab facility across town at another children’s hospital. Here she learned how to eat and bathe, and she started saying words again the very last night we were there! What a relief my kiddo was coming back to us!!! Doctors to this day can’t tell us where we will be in the long run or how long it will take her to fully recover, but we have high hopes with positivity and a heck of a support system that works with her everyday to be all she can be.
That concluded our seven weeks and we finally got to come home. I was a little nervous because we had so much help and proper tools to keep her safe at the hospital. We continued at home with occupational therapy and speech therapy until the end of the year. Brynn was back! Doctors would like to still remain on monthly IVIG treatments for one complete year and supplement with Rituxan as needed. All of them are amazed by how fast she snapped back from this disease. I can honestly say that it was because of a fast aggressive treatment, attentive doctors, and a strong support system full of love, patience and positivity that pulled this little girl back. Life is way better than we had projected at this point. The monthly IVIG treatment is rough as my daughter hates getting the IV. Hopefully we can look back at this and say, “Wow, that was just a bump in the road.” Until then, we will just keep our heads up and plug along!
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.
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.
As the name suggests, autoimmune encephalitis (AE) is a group of diseases in which the body’s immune system attacks the brain. To treat it, there are a variety of therapies that target different aspects of the immune system. The goal of these immunotherapies is to reduce brain inflammation and the resulting symptoms, as well as maintain these improvements by preventing relapses1.
Immunotherapy is most successful in patients with antibodies against cell-surface proteins (such as NMDR, LGI1, and Caspr2). These diseases tend to be caused by B cells and autoantibodies. In contrast, when antibodies are directed against molecules inside of cells (such as Hu, Ma, or GAD65) the disease is usually mediated by T cells, and these patients typically do not respond as well to immunotherapy. It should also be noted that removal of any disease-associated tumors, such as the ovarian teratomas frequently seen in NMDAR encephalitis or tumors seen in patients with intracellular antigens, should be an early treatment priority as removal quickly produces improvements2. However, there are currently no standardized treatment guidelines; at present, different regimens are used based on the patient’s particular condition and clinical status, as well as the opinion of their doctor.
First-line Treatments
The first treatment for most patients is typically steroids, also calledcorticosteroids. Corticosteroids act to broadly inhibit inflammation in multiple ways, which results in the depletion of mainly T cells. They offer the additional benefit of restoring the blood-brain barrier (BBB), which can be impaired in
AE. However, corticosteroids aren’t perfect. They have many side effects, and can aggravate or even induce psychiatric symptoms associated with AE such as depression, insomnia, agitation, and psychosis. What’s more, corticosteroids do not target B cells, the cells that go on to produce the antibodies that cause many of the symptoms of AE3.
Two other first-line therapies do target autoantibodies. One is administration of intravenous immunoglobulin (IVIg). IVIg is a blood product prepared from the serum of more than 1,000 donors that contains a broad range of antibodies. Some of these antibodies target a patient’s autoantibodies and neutralize them, along with other pro-inflammatory aspects of the immune system3. The other first-line treatment targeting autoantibodies is plasma exchange (PLEX, also called plasmapheresis). PLEX “cleans” the blood of autoantibodies by replacing the liquid plasma portion of a patient’s blood with that of a donor. PLEX also changes T and B cells in favorable ways. A more refined form of PLEX called immunoadsorption has also been used to treat AE, and selectively removes antibodies from the blood, instead of all the other components that are also in the plasma3. However, both PLEX and immunoadsorption only remove antibodies from the blood, not from the brain; although decreasing antibodies in the blood can lead to a decrease in the brain4. Furthermore, all first-line treatments but especially PLEX require a good deal of patient compliance, which can limit their use if the patient is agitated or displays other behavioral problems5.
Different subtypes of AE respond differently to treatment. For instance, patients with LGI1 antibodies who are diagnosed early are often responsive to corticosteroids alone. In contrast, only about 50% of patients with NMDAR antibodies are responsive to first-line treatments, and the remaining require second-line therapies6.
Second-line Treatments
There are two main second-line immunotherapies for AE. The first is a drug that destroys B cells called rituximab. Rituximab is actually an antibody that targets B cells, which normally go on to become antibody-producing cells. It is expected to work particularly well in patients with LGI1 and Caspr2 autoantibodies. However, because B cells can cross into the brain and become antibody-producing cells, but rituximab cannot cross the BBB, its effects may be limited3.
The other second-line treatment is a chemotherapy drug called cyclophosphamide. Cyclophosphamide directly prevents T and B cells from multiplying, but it affects the ability of many other cells to multiply as well. For that reason, it has some potentially serious side effects including infertility, and instead rituximab is usually the preferred second-line therapy3.
Alternative Treatments
Sometimes second-line treatments are also not effective at treating AE. When that happens, options include re-administration of first-line therapies, extended use of second-line therapies, or use of other non-steroid (steroid-sparing) drugs to suppress the immune system. For instance, the steroid-sparing drug mycophenolate mofetil prevents T and B cells from multiplying and has a better side-effect profile than cyclophosphamide3.
Other alternative treatments are also available. One option interrupts the inflammatory effects of a molecule called interleukin-6 (IL-6). Normally, when IL-6 binds to its receptors on immune cells, it causes B cells to multiply and mature into antibody-producing cells, and causes pro-inflammatory T cells to mature. The antibody drug tocilizumab targets the IL-6 receptor and prevents these inflammatory processes. A molecule related to IL-6, IL-2, is also a target. Instead of inhibiting this molecule, giving patients low doses of IL-2 activates a “good” type of T cell called regulatory T cells that help the body shut down autoimmune responses. Another option, bortezomib, directly targets antibody-producing cells, instead of their immature B cell precursors3.
Maintenance Treatments
Even if AE is successfully treated, sometimes the disease can relapse. Relapses could be caused by some antibody-producing cells that can survive for many months, which are not targeted by treatments. Many of the therapies described above, including the first-line treatments, steroid-sparing agents, and rituximab, have been used as maintenance therapy to try and prevent this from occurring. However, the length of time patients should continue to receive treatment is unknown, and can range from 6 months to several years depending on the patient’s condition and doctor’s opinion3.
In addition to immunotherapy, other important aspects of treatment include supportive care (particularly while in the hospital); treatment of symptoms such as seizures, spasms, and psychiatric issues; and rehabilitation1. While responses to tumor removal and immunotherapy are often seen within a few weeks, it may take years for patients to return to normal7. As more is discovered about which aspects of the immune system are involved in each subtype of AE, hopefully more directed treatments will become available.
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.
1. López-Chiriboga, A. S. & Flanagan, E. P. Diagnostic and Therapeutic Approach to Autoimmune Neurologic Disorders. Semin. Neurol.38, 392–402 (2018).
Seki, M. et al. Neurological response to early removal of ovarian teratoma in anti-NMDAR encephalitis. J. Neurol. Neurosurg. Psychiatry79, 324–326 (2008).
Shin, Y.-W. et al. Treatment strategies for autoimmune encephalitis. Ther. Adv. Neurol. Disord.11, 1–19 (2018).
Fassbender, C., Klingel, R. & Köhler, W. Immunoadsorption for autoimmune encephalitis. Atheroscler. Suppl.30, 257–263 (2017).
Damato, V., Balint, B., Kienzler, A. K. & Irani, S. R. The clinical features, underlying immunology, and treatment of autoantibody-mediated movement disorders. Mov. Disord.33, 1376–1389 (2018).
Varley, J., Taylor, J. & Irani, S. R. Autoantibody-mediated diseases of the CNS: Structure, dysfunction and therapy. Neuropharmacology132, 71–82 (2018).
Venkatesan, A. & Adatia, K. Anti-NMDA-Receptor Encephalitis: From Bench to Clinic. ACS Chem. Neurosci.8, 2586–2595 (2017).
The last two years of my life seem to have disappeared from my memory. Gone. Holidays, weddings, a funeral, birthday parties, Christmas and New Year. Friends and family keep telling me things I’ve done and the way I’ve behaved, some of which are very embarrassing to hear. It’s just not me.
This is the result of the disease Autoimmune Encephalitis (AE), the symptoms of which I was clearly exhibiting without my even realizing. Hallucinations, involuntary movements (which developed to 20-30 movements over four or five minutes), insomnia, loss of inhibition, memory loss and finally seizures, none of which I can remember. Apparently, I had two massive seizures at work (a Secondary School where I teach Physical Education), one of which resulted in the police being called due to my resisting all help. I’m told this was the last of a number of smaller seizures as the disease continued to develop and take hold.
The last seizure resulted in hospitalization for more than two months and being seen by a number of doctors and specialists who put me through a whole host of MRI scans, EEG’s, blood plasma analysis and other tests I don’t recall. I was eventually transferred to another hospital, where a specialist identified AE straight away, where I stayed and was treated for more than three weeks.
Lots of my friends and family traveled to see me, none of which I can remember and feel very guilty about admitting to. I do have a memory of very small flashes of shouting inappropriate comments at some female staff and having to apologize the next day and some very small flashes of walking around on other wards in my underwear looking for my ward. Again, it’s very embarrassing and most of it I can’t fully remember.
I’ve been told of other embarrassing events I performed, which eventually resulted in my being placed in a room by myself and given sedatives to help me “relax.” I was given all sorts of medicines, went through a whole host of tests and discussions with specialists on how to treat and control this very rare disease, and no one knew the right answer. It was a case of controlling the disease from worsening and monitoring my condition very closely.
Eventually, I was transferred back to my local hospital and continued to have a wide range of tests, blood samples were taken every morning to be analyzed and, after three more weeks, I was released to go home with a cannular inserted into a vein in my wrist with 24 tablets to be taken during the morning and 12 at night. I also received IVIG treatment to boost my immune system every morning at the local hospital.
I am now awaiting a decision from my consultant regarding the next steps. He has to speak with specialists in London as he is also unsure about what to do. I’ve been signed off work until after Christmas, which I also find frustrating as I feel back to 100%, but deep down I know that is the right decision.
I hope this article gives other sufferers of this very rare disease assurance there are other people out there experiencing similar symptoms of this confusing and inexplicable disease for which hopefully leads to further research and ultimately a successful pathway to complete recovery.
May 2020
Since I wrote the above piece back in late December 2019, I’m feeling back to 100%. The doctors, specialists, occupational health therapists are all very surprised how quickly I seem to have fully recovered, and it’s all down to the support and treatment I’ve had.
It’s now early May, I’m still taking a large dose of meds including Lamotrigine, Levetiracetam, Prednisone, Adcal etc, which to be honest it’s now a case of a gulp of water and down the hatch. It’s now about 10 tablets in the morning and eight at night — massively down from 24 and 12.
I went back to work in early January on a carefully monitored and phased return, building back to full-time before the dreaded COVID-19 hit. I’m not going to lie, going back to work has been tough. The school I work at has moved on, which has is expected in the 12-18 months I’ve been in and out (mostly out). Different students. Different staff. Different expectations. It’s been hard for me, but harder and more frustrating for the brilliant people I’m lucky enough to work with.
When I got back into work I tried to pick up where I left off. It didn’t work, because my expectations of myself are so high. I wanted to know why, how, what had happened to this, that, and the other. I tried to rush back too quickly, and it didn’t work. I wasn’t listening or asking for help enough.
I thought I could pick up where I left off, but I couldn’t. My brain needed time to click back into action. Those billions of neurons needed time. Time, I thought I didn’t have. Fortunately, my employers have been incredibly understanding. They’ve supported me so much, something for which I am eternally grateful.
This is where I know I’m so lucky. I’ve read so many stories of people that are struck with different forms of encephalitis and other life-changing illnesses where they haven’t recovered enough to be able to return to work, or their employers haven’t been as understanding. At times, I feel guilty reading their stories.
As I write this I now only see my specialist twice a year and my meds are being reduced every 10 days. I’m back into full-time work in my original role and physically fit again, running at least three times a week, with lots of sport and the gym. I seem to have a “small” gap in my memory of about 12-18 months, but it could have been a lot worse.
So, it is very possible to make a completely successful and long-lasting recovery from AE and other forms of the disease. I read so many stories of people who have suffered from this terrible illness and are still suffering, so I wanted to write this to let people know there are success stories and you can — with the correct treatment and support network around you — return to full health and look forward to living the rest of your life.
Become an Advocate by sharing your story. It may result in someone receiving an accurate diagnosis who is suffering right now and 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.
We received the dreaded letter of denial from our insurance company 7 days before my daughter was due for her IVIG treatment. I immediately turned to IAES for guidance. I was instructed to go to their website and read the page about “How to overturn a treatment denial”and then ask them follow up questions to verify I fully understood what needed to be done. I read every word. Then, I read it again.
If I was going to advocate for my child, Grace, I had to know and understand exactly what steps to take! The very first thing I did was double check what diagnostic code the ordering doctor used. International Autoimmune Encephalitis Society’s website makes it easy because they give you the correct code in their information. I compared it with the code the doctor used. There it was. Just as the website reported that the most common reason for receiving a denial was that the wrong diagnostic code is used, the coding for Grace’s diagnosis was not for autoimmune encephalitis. The doctor or a staff member in their office had made a mistake and used an incorrect code. Having identified the problem, I now knew that it could be corrected.
I immediately called the doctor’s office to make them aware of the situation. But I was faced with the next challenge. They had received the same denial letter from our insurance company prior to us receiving ours and instead of double checking their codes, they wrote an immediate appeal and attached 45 pages of notes stating WHY my daughter needed the treatment. They informed me we would know in 72 hours if it went through.
I checked back in with IAES and updated them on the situation. I was quickly informed that the doctor’s office or whoever was in charge of doing the prior authorizations in the doctor’s office were making ANOTHER mistake by appealing the denial. “You don’t want to appeal a denial when the wrong diagnostic code is used! A NEW prior authorization with the correct code must be submitted.” They explained.
So, I got back on the phone with the doctor’s office and gave this information to them. The answer I received from them was, “Well, the doctor wrote the appeal and gave 45 pages of evidence and it should take 72 hours before we know if they will accept it.” I again insisted that they should not appeal due to the incorrect code. She said, “oh ok, I’ll just let them know about the new code.”
Oh boy, I started getting a little irritated. To top it off, I had been told the doctor had left town for two weeks. Clearly, I was getting nowhere in getting the office staff to understand what actions needed to be taken. I knew that their efforts would result in a denial of the appeal. So, I decided to contact the hospital nurses in the infusion center that are in charge of submitting prior authorizations. I went over all the information with the nurse. She agreed a new prior authorization was needed and would contact the insurance company to get it done. Thinking I was out of the woods, I relaxed a little.
The next day the infusion nurse called to let me know the insurance company told her she could NOT submit a new authorization for 6 months. Ahh! What?! I knew this simply was not true and I demanded she contact them again. She said she would try.
I updated IAES and we decided it would be easier to go over the situation on the telephone. I learned that the insurance company appeared to be under the impression the patient was being given a different diagnosis and not addressing this as a clerical error.
“If you need a lifesaving treatment, and pay good money for health insurance, do you really thing the insurance company is going to tell their customer: I’m sorry but due to a clerical error we can not give you your lifesaving treatment? I’m afraid your life will have to be cut short?” She explained that, “Most infusion nurses are really wonderful and want to help, but there are times when you will sense that you have to be pushy. Be confident, you are informed now. If you have to, be pushy. At times, it’s appropriate.”
I followed up with the infusion nurse the next day. I was being pushy, and I was learning that with that and my sense of confidence I was getting the job done. We only had 2 days left until my daughter’s treatment was scheduled. I was feeling the urgency of getting this accomplished. At this point, the insurance company accepted the new prior authorization and the nurse told me she marked it as “urgent” and “high priority”. At the end of the day we should have an answer.
– – But no… that’s not what happened. The nurse called to check the status of the authorization and the insurance had NOT put it as high priority. She said it wouldn’t get looked at for another day or two or maybe up to 14 days. This was not acceptable. The insurance company had already made one mistake by telling us we had to wait 6 months, now they don’t push it through when marked urgent?
While problem solving on the telephone with IAES, I was told that when a clerical error is made and a delay created from their mistake a new prior authorization with the correct code could be faxed to the insurance company and the person who submitted the corrected prior authorization could then ‘walk it through’ explaining to the insurance company that it was their clerical mistake. That in situations like this, the correction could be made in a matter of hours or by end of day. In fact, I was advised to give them a time limit as to when I expected to have it approved and let them know I expected a call back telling me the mistake has been corrected.
So, I called member service on the back of my insurance card and told them I need to make a member complaint and asked to speak directly to a member services supervisor. This is what got the ball rolling. They were apologetic, offered as much assistance as I needed, and they even got the authorization labeled high priority. I wrote down the supervisor’s name and direct contact number. I told her I would call in the morning and make sure it went through.
Friday morning: the day of Grace’s infusion. The Nurse at infusion center called to let me know the prior authorization had NOT gone through and was still pending review. I put my big girl pants on and called the supervisor and said, “My daughters treatment is scheduled to take place in 45 mins. THAT’s how long you have to get the authorization through or I’m going above your head.”
My daughter and I showed up at the appointment, waited about 15 minutes and the nurse walked over to tell us the authorization was approved. Hallelujah!!
International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.
Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.
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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