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.
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.
Little things, just little things were first noticed by our immediate family. The need to recite over and over the details of how a decision was made, excessive talkativeness, repetitive conversations. Outbursts of anger over inconsequential events, vocalizations of “yep, yep, yep” and a stutter-step when that happened. Something was not right. Periodic feelings of having a “brain spasm” were added to the mix.
Visits to our personal care physician, referral to a neurologist, MRIs and EEGs all resulted in a conclusion that Jeri was just stressed out. After our son’s wedding, everything would be alright. It wasn’t. The wedding was a big success but for Jeri, concentrating on the many details was difficult, to say the least. Our college friends noticed immediately that something was not right. “She seems to not be herself, she gets angry easily, what’s up?”
Could it be a recurrence of the chronic Lyme disease from 8 years ago? Off to the holistic physician that successfully treated Lyme disease. The approach there was to test for exposure to environmental toxins and Lyme. Blood was drawn and then we left for a couple of weeks to go to our cottage in Canada. In those two weeks, our Canadian friends also came to us and asked if something was going on. The repetitive conversations continued along with withdrawal from group conversation. A couple of times Jeri blanked out for a few seconds and became worried-looking, really concerning our friends.
Back from the cottage the follow up with the Lyme doctor revealed that she was slightly positive on the Lyme test. (Lyme diagnostics is difficult and subjective – a discussion for another time.)That is probably what was going on. Back on antibiotics and several nutritional supplements – for 24 hours. Then our journey really began.
“Mom’s had a seizure, I’m following the ambulance to the hospital.” Our two adult sons living at home had found Jeri having a seizure shortly after going to bed. Another seizure while in the ER. The workup revealed that her sodium levels were dangerously low. Correction of the hyponatremia began along with MRIs and other scans. Five days as an inpatient to correct the sodium levels and all tests came back negative. No infectious causes, no tumors, must just be a case of spontaneous SAIDH (Syndrome of Inappropriate Antidiuretic Hormone Secretion). It happens, that can be corrected with diuretics and salt pills. Take anti-seizure medicine and follow up with a neurologist.
At this point, the reader needs to know a little about our family. For years Jeri was a researcher in a biochemistry lab and up to a couple of years ago, a practice manager for a busy physician’s office. I’m a Ph.D. biochemist and molecular biologist. One son is a newly trained nurse and one daughter-in-law is a very experienced pediatric intensive care nurse. Jeri’s brother-in-law is an attorney with over 20 years’ experience in large pharma oncology and has a Masters in chemistry. To say that there were many opinions on what was going on is the understatement of the year. A lot of family knowledge and experience were being tapped, all out of concern, love, and with the best of intentions. Conversations with physician friends and nurses suggested a myriad of causes. None of us got even close to the cause.
The ‘spells’, absent episodes, memory issues, and cognitive decline all continue and increase in frequency. We were home for a week and then another big seizure puts her back in the ER and again admitted to the hospital. Again, all scans and EEG are normal. The doctors in the regional hospital are again scratching their heads. Despite repeated requests, the hospital physicians and our personal neurologist resisted performing a lumbar puncture. Why? We were told there was simply no indication that would justify such a high-risk procedure. Must be the SIADH. Go back to your local neurologist and nephrologist.
A 72 hour EEG was ordered. This was stopped after 24 hours since we noted so many (~30) ‘spells’ and no further recording was needed. Like two spot EEGs before, nothing showed up. The local neurologist was stumped, calling it a real mystery. We again ask for an LP and testing but were refused on the basis that he didn’t know what to test for. As a shot in the dark, testing was ordered for serum anti-NMDA receptor. Negative results. Sodium levels stay a bit low, but near normal. Frustration all around.
Escalating the case was now a family priority but we were stymied by the long wait (2 months!) to get into the UPenn neurology group. A week after the last neurology visit another seizure. A couple of days later we hear a crash and see Jeri at the bottom of the stairs having a seizure, this time with a gash to the head. Back to the ER after a very irrational and angry outburst. Another seizure in the ER and another admittance to the local hospital. The next morning during rounds the floor neurologist is in the room and the hospitalist (physician in charge of cases on that floor) pops in to see how things are going. She asks the neurologist if she is going to order an LP and the neurologist says no, there is no need. The matter is dropped. No words can describe our frustration and helplessness at this point. My son and I discussed the situation and decided to go to the hospitalist and ask for her intervention in getting the case moved to UPenn. She agreed but was doubtful that she could get her transferred. To her and our surprise, UPenn neurology agreed right away!
We were just so lucky to have the case taken over by UPenn and a group of physicians that have been integral to the discovery and treatment of autoimmune encephalopathies. Within a week we had a diagnosis of anti-LGI-1 autoimmune disease and a week after that we began immunosuppression therapy via a five-day course of steroid infusions and an infusion of Rituximab.
We’ve been home a week, continuing anti-seizure medicine, oral steroids, salt supplementation, and diuretics. No seizures so far! We are seeing small improvements in cognitive ability and memory. We have a long way to go but are hopeful. The second infusion of Rituximab will happen in about a week.
Unfortunately, I think we can consider that knowledge of auto-immune encephalopathies is pretty much non-existent to the average physician and even most neurologists. The resistance to exploit all avenues of testing after repeated presentations to the ER and negative results of conventional diagnostics needs to change. That change will only come with physician education. Also, symptoms can be very, very subtle at first and not consistent from person to person. Like a lot of other neurological conditions, the symptoms are common to many different causes.
Now that the mystery is solved the recovery can begin. Another Zebra identified.
Become an Advocate by sharing your story. It may result in an accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
I am one of the lucky ones. But, in a way, I think I inadvertently helped make my own luck by contacting the Mayo Clinic when I did. A little over two and a half years ago, my wife and I flew from our home outside of Kansas City, MO to the San Francisco Bay Area to visit family. When we returned and got off the plane in late May, my memory was almost completely gone except for a few instances from my childhood which, since I was 68 at the time, was a long, long time ago.
I started having seizures. At first, just a couple of times a day, gradually increasing to 20 or more a day. I had frequent feelings of nausea and incredible weakness (I couldn’t walk even an 8th of a block without being completely exhausted.) I contacted my physician and he tried to schedule me with the neurology department. They subsequently referred me to another neurological testing group, but scheduling with them was delayed while they were awaiting insurance approval. In retrospect, this may have been another stroke of luck, because, with my symptoms getting worse, I finally contacted the Mayo Clinic in Rochester on a Thursday or Friday in July. Apparently I used the right buzz words because they responded right away and said, “Can you come in on Monday?” Even though they were an out of network provider on my insurance plan, my wife and I jumped in the car and drove up there.
After being run through a battery of tests and being seen by several doctors, I was diagnosed with LGI1 Autoimmune Encephalitis, and placed under the care of Dr. Eoan Flanagan who began treating me with high dosage prednisone – this was about the first of August. I had my last known seizure in late October, though most of the other symptoms have persisted. At this point, I’m certainly not cured, but am adjusting to a new reality.
My memory is still very spotty. For example, as I am writing this, we are once again in California visiting family. While talking to my daughter, who just recently moved back here from Kansas City, I mentioned that I hadn’t been back to California since this had happened and that I was leery about flying even though I knew intellectually that flying had nothing to do with causing the disease.
My wife gently reminded me that yes, we had flown out here just last year. I have no memory of that. I lived in this area a large portion of my life, but as we drive around, it’s like I’m in a foreign country. I don’t recognize much of anything I see. The funny thing about my memory loss, though, is that I remember people, just not places or events. I’m currently in the process of being weaned off the prednisone and replacing it with Rituxan (I had my first Rituxan treatment last August.)
I have no idea what the future will hold, whether I’ll get my memory back or not. But I’ve determined to not let that impede my enjoyment of life while it happens. It’s hard sometimes when I’m with friends or family when the conversation turns to reminiscing about a past which I can’t remember, but I know there’s nothing I can do about that. My new motto is “Live in the Moment.”
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 toIAES@autoimmune-encephalitis.org
Hi, my name is Kerry Jones and I’ve been diagnosed with LGI-1 autoimmune encephalitis by the Mayo Clinic in Rochester MN. I was seen by the Mayo Clinic only after trying to get appointments with local doctors, to discover that I wouldn’t have been able to see the specialists for some months. So, we contacted the Mayo Clinic and they got us in in just a few days. We saw three different doctors there over the course of the next few days, and they were very thorough at coming up with a diagnosis.
My main symptom was primarily memory loss of time and place. I still remembered people and faces, just not events and experiences.
Initially I was started on Prednisone infusions by IV. They were given daily for five days and then weekly for several weeks, then it was going to taper to every other week. However, I was finding that I felt good the day of, and a couple of days after, the infusion but was feeling really bad by the end of the week. I switched to daily pills and have been on those since. They’ve evened out the ups and downs, though I do still have some of both.
Earlier on, I would find that I’d just start crying for no apparent reason. I had frequent dizzy spells, and many emotional swings, along with several minor seizures each day. I haven’t had a seizure now for about a month and the other symptoms also come less frequently. There are still big gaps in my memory but if I work really hard, I can call up some memories that seemed to have been lost. But it’s odd. I was talking to my wife the other day about trying to remember where we lived in California. I could remember the last place we’d lived there, but none before that. But after concentrating I was able to remember the place I thought we’d lived in before that. I remembered the place alright, but we’d lived somewhere else in between the two but I have no recollection of that place!
Spending time with my Grandson at the beach is my last memory before becoming ill with Limbic Encephalitis
As I progress in my recovery, I seem to be able to remember recent stuff and some older stuff, but mostly I’m just trying to observe what is happening with my memory. Early on, I would get quite distressed when we’d go somewhere, I’d been to many times and fine I could not remember it all. Now, I’m just looking at it as a new experience. I still have dizzy spells on getting up, and I get very winded with very little effort, but do feel that I’m getting better.
My recent appointment at Mayo, in late December, was when we planned to start tapering me off the Prednisone.
It’s been a long ride, but, at least for me, I think I can see some light at the end of the tunnel, and hopefully you all will too with proper care.
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.
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