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My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

My letter to Santa ATTENTION: YULETIDE BUREAU WISH DEPARTMENT

 

December 24, 2021 | Tabitha Andrews Orth 

My letter to Santa.
ATTENTION: YULETIDE BUREAU 
WISH DEPARTMENT

Dear Santa,
As you know, I have given great thought to my Christmas wish this year.  I hope I have made your ‘NICE LIST”, as my wish is in the form of a favor.

Since I am awake, due to insomnia from the IV Solumedrol infusion I received yesterday afternoon, I thought it was a good time to write you. I guess I didn’t need to tell you that, since you see me when I am sleeping, and you know when I am awake. LOL:)

Sometimes the lessons we need to learn to grow our soul and faith come in the most unexpected forms. I have realized the truth of this and am doing my best to rely on my faith, to celebrate the love and support I receive and do my best to face my adversity.

Santa, I have to be totally naked in my honesty here. As Head Elf, embodied with the Spirit of -now here’s my word finding problem rearing its head… It means to love without qualifying or judging. I can’t remember how to say it Santa, but you get me. How could anyone ever imagine they would get a disease where your immune system attacks your brain?!

I mean if my brain isn’t working right, nothing works right. That’s a hard thing Santa.  We are talking TOUGH challenge here.

Santa, you know this. You have been watching. I don’t have to explain because you have lived it with me. Yeah, I’m totally on the “NICE LIST”.

Sorry, attention span problems, back to my favor. 
But… I have practiced daily random acts of kindness for many years…attention span problems again and paranoia I might not make the cut for that ‘Nice List’.

Santa, this disease has created personality changes that are not the heart of me. It has taken ME away before with episodes of psychosis twice now. I am inside. In my heart, buried deep, I am ME. People were not able to see ME when my brain disease flared in this way, but YOU could!
Sorry, I will try to stay on topic. Attention Span problems, but YOU know this.

Okay, I forgot what I was going to say… LOL. Wait, I’ll reread this and write my main point on a sticky note so I can remember my wish. Why am I telling you that? YOU see me. YOU realize everything.
Ok. Wait just one minute for me Santa. Yes. I am back. Got it. My Christmas Wish. (That is what I wrote on the sticky note. Oops, sorry, forgot. Don’t need to tell you that.)

I am having a problem with worry for my future. I have now faced FEAR.  I have stared it down. I have survived. I get scared sometimes. YOU know I do. My faith and spiritual beliefs sustain me; through them, I draw my courage and do my best to surrender my fear. 

This brings me to that favor I mentioned. My Christmas Wish. 
Gosh, Santa, I need your help.

By the way, Jim says guys like peanut butter cookies and oatmeal raisin the best, so we will be leaving those out in their usual place by the Cocoa. Feel free to use the copper pot I leave out for you to warm up your Cocoa in case you find yourself running late.   Oh, the carrots for the Reindeer are grown by local farmers- I am adding some lovely fresh pears this year…
But, you know that too… Sorry…..
What was I going to say? Oh, right! My Christmas Wish:)

You’ll find an envelope by your nighttime snack. I’ve enclosed all my fears and worries inside. Could you kindly take them from me? That is my Christmas Wish. 

And when you take your leave, by the way, I LOVE that time suspension magic you do- very cool!

When your laughter rings out within and around our home, can you add a bit of Christmas Spirit Magic to infuse your laughter, Joy, and that kind of love I can’t remember the name of …within our home, our hearts and my brain where my fears and worries once were?  By adding your love and laughter to mine Santa, well, that’s a powerful infusion. Exactly what is needed to receive the appreciation of each day.

My family and I laugh a lot Santa. YOU know. My laughter helps my husband, Jim and our son, Matthew. Their laughter helps me. And so, it goes….

P.S.
Should you find other letters written by people with Autoimmune Encephalitis, whether they be drawings by children, a single word, or simply the breath of fear blown inside an envelope and sealed, my wish is that you take these too and leave your laughter.

Oh, make yourself at home as long as you would like Santa. The weather across the Northern Hemisphere is projected to be quite cold. There are extra blankets in the hall closet. Help yourself. When you are working your way across the Southern Hemisphere and get to Sarah’s house in New Zealand since it is Summer there and you won’t need them any longer, you can leave the blankets with her and she’ll get them back to me on her next visit;) 
Pleasant trip and God speed. 
Best Wishes,
Tabitha 

Tabithas letter to Santa facebook Post 300x251 - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

(Originally written December 17, 2014)


IAES invites you to spread awareness of Autoimmune Encephalitis by sharing this delightful musical spoof utilizing the classic holiday music from ‘Rudolf the Red-Nosed Reindeer’. Learn who identified the most common type of autoimmune encephalitis of them all and where that magical moment occurred.



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


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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 premier organization leading in these vital roles.

guidestar platinum logo 300x300 1 e1605914935941 - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

 

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

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.

Trivia Playing cards 3 FB 500x419 - The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis

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.

AE Warrior Store 300x200 - The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis

why zebra - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT
My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

My letter to Santa ATTENTION: YULETIDE BUREAU WISH DEPARTMENT

 

December 24, 2020 | Tabitha Andrews Orth 

My letter to Santa.
ATTENTION: YULETIDE BUREAU 
WISH DEPARTMENT

Dear Santa,
As you know, I have given great thought to my Christmas wish this year.  I hope I have made your ‘NICE LIST”, as my wish is in the form of a favor.

Since I am awake, due to insomnia from the IV Solumedrol infusion I received yesterday afternoon, I thought it was a good time to write you. I guess I didn’t need to tell you that, since you see me when I am sleeping, and you know when I am awake. LOL:)

Sometimes the lessons we need to learn to grow our soul and faith come in the most unexpected forms. I have realized the truth of this and am doing my best to rely on my faith, to celebrate the love and support I receive and do my best to face my adversity.

Santa, I have to be totally naked in my honesty here. As Head Elf, embodied with the Spirit of -now here’s my word finding problem rearing its head… It means to love without qualifying or judging. I can’t remember how to say it Santa, but you get me. How could anyone ever imagine they would get a disease where your immune system attacks your brain?!

I mean if my brain isn’t working right, nothing works right. That’s a hard thing Santa.  We are talking TOUGH challenge here.

Santa, you know this. You have been watching. I don’t have to explain because you have lived it with me. Yeah, I’m totally on the “NICE LIST”.

Sorry, attention span problems, back to my favor. 
But… I have practiced daily random acts of kindness for many years…attention span problems again and paranoia I might not make the cut for that ‘Nice List’.

Santa, this disease has created personality changes that are not the heart of me. It has taken ME away before with episodes of psychosis twice now. I am inside. In my heart, buried deep, I am ME. People were not able to see ME when my brain disease flared in this way, but YOU could!
Sorry, I will try to stay on topic. Attention Span problems, but YOU know this.

Okay, I forgot what I was going to say… LOL. Wait, I’ll reread this and write my main point on a sticky note so I can remember my wish. Why am I telling you that? YOU see me. YOU realize everything.
Ok. Wait just one minute for me Santa. Yes. I am back. Got it. My Christmas Wish. (That is what I wrote on the sticky note. Oops, sorry, forgot. Don’t need to tell you that.)

I am having a problem with worry for my future. I have now faced FEAR.  I have stared it down. I have survived. I get scared sometimes. YOU know I do. My faith and spiritual beliefs sustain me; through them, I draw my courage and do my best to surrender my fear. 

This brings me to that favor I mentioned. My Christmas Wish. 
Gosh, Santa, I need your help.

By the way, Jim says guys like peanut butter cookies and oatmeal raisin the best, so we will be leaving those out in their usual place by the Cocoa. Feel free to use the copper pot I leave out for you to warm up your Cocoa in case you find yourself running late.   Oh, the carrots for the Reindeer are grown by local farmers- I am adding some lovely fresh pears this year…
But, you know that too… Sorry…..
What was I going to say? Oh, right! My Christmas Wish:)

You’ll find an envelope by your nighttime snack. I’ve enclosed all my fears and worries inside. Could you kindly take them from me? That is my Christmas Wish. 

And when you take your leave, by the way, I LOVE that time suspension magic you do- very cool!

When your laughter rings out within and around our home, can you add a bit of Christmas Spirit Magic to infuse your laughter, Joy, and that kind of love I can’t remember the name of …within our home, our hearts and my brain where my fears and worries once were?  By adding your love and laughter to mine Santa, well, that’s a powerful infusion. Exactly what is needed to receive the appreciation of each day.

My family and I laugh a lot Santa. YOU know. My laughter helps my husband, Jim and our son, Matthew. Their laughter helps me. And so, it goes….

P.S.
Should you find other letters written by people with Autoimmune Encephalitis, whether they be drawings by children, a single word, or simply the breath of fear blown inside an envelope and sealed, my wish is that you take these too and leave your laughter.

Oh, make yourself at home as long as you would like Santa. The weather across the Northern Hemisphere is projected to be quite cold. There are extra blankets in the hall closet. Help yourself. When you are working your way across the Southern Hemisphere and get to Sarah’s house in New Zealand since it is Summer there and you won’t need them any longer, you can leave the blankets with her and she’ll get them back to me on her next visit;) 
Pleasant trip and God speed. 
Best Wishes,
Tabitha 

Tabithas letter to Santa facebook Post 300x251 - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

(Originally written December 17, 2014)


IAES invites you to spread awareness of Autoimmune Encephalitis by sharing this delightful musical spoof utilizing the classic holiday music from ‘Rudolf the Red-Nosed Reindeer’. Learn who identified the most common type of autoimmune encephalitis of them all and where that magical moment occurred.



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


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 premier organization leading in these vital roles.

guidestar platinum logo 300x300 1 e1605914935941 - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

 

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

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.

Trivia Playing cards 3 FB 500x419 - The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis

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.

AE Warrior Store 300x200 - The Real-World Challenges of Surviving an Autoimmune Encephalitis Diagnosis

why zebra - My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT
Thanks Dad!

Thanks Dad!

June-21-2020 | Leslie Holland

My dad and my brain go back more than a few decades. When I was two, he convinced my eldest sister she could teach me to read, which I insisted upon in order to move on from coloring books. It wasn’t that I was bored with Dad reading Dr. Seuss to us before bed, it was a fascination with the stories that I wanted MORE.

Among my rather large family—including more than a dozen cousins, with whom our young parents raised more like a collective of siblings—I was given the nickname “The Brain.” I took no offense at the name (until adolescence finally kicked in); rather, I relished reading in a corner when the rest were doing anything else.

It paid off, too. I was immediately admitted to the public school’s advanced program, while my four siblings attended the local parochial school. Dad generally drove us to school and I was the final drop-off, during which time he encouraged my academic achievement in multiple ways, whether quizzing me on spelling words or asking me to solve math problems in my head.

Dad was in advertising, heavily involved in political campaigns, which also set the stage for my career in public relations and marketing—including a stint as a registered lobbyist for the Health Insurance Association of America. As a matter of course, the last three people who hired me asked in the interviews whether I was his daughter. My answer weighed heavily in my favor.

Fast-forward to November 2018… During dinner with my boyfriend, I said something so out of course with our conversation, that Tim was stunned. The next day I had no recollection of it and he insisted I see my internist, who knows me well enough that I was sent immediately for an MRI. Limbic encephalitis was rampant and the obvious diagnosis. (I’ve had epilepsy for nearly 30 years, due to an entirely separate and resolved diagnosis, so all seizure activity was well-controlled, making my memory issues the trigger in my case.)

Upon receiving this new diagnosis, I communicated it to my family. My sister, Suzanne (the one who taught me to read), is now a bioethicist in Washington state, and she arranged for me to be seen at Mayo Clinic for a consult with Dr. Sean Pittock, who agreed out of professional courtesy. Immediately upon meeting me during my office visit, he admitted me and began my steroid treatment.

During this time and since, Suzanne, Dad and I have been in daily text communication. Sometimes it’s related to my encephalitis, though often it’s about our beloved University of Louisville Cardinals, the Chicago Cubs, Thoroughbred racing, or the political antics of the moment. Never has Dad wavered in his commitment to my wellness.

Once it became apparent that working at a job I once loved was no longer possible because of my significant memory issues, he has continued to nudge me along intellectually. He’s convinced “The Brain” will overcome and achieve in new ways. There are days I’m not yet there with his belief, but more often than not, I’m still the kid who’s being quizzed in the car on the way to school.  Happy Father’s Day, Dad!

Terry Holland Leslie Hollands Dad fathers day blog - Thanks Dad!Terry Holland

She Didn’t Stand Alone, She Had Her Father

June-21-2020 | Mari Wagner Davis

 

She did not stand alone, but who stood behind her, the most potent moral force of her life was the love of her father.  Harper Lee.

Father’s Day is a day we celebrate our fathers. We may take them out to dinner, send a card, or make a special phone call. There have likely been times when we have argued or butted heads with our fathers. Times when they have come to our rescue. Times when they have chewed us out for doing something goofy. I didn’t really realize how important my parents, my father is, in my life until I had seizures and was diagnosed with autoimmune encephalitis.

Growing up, my Father’s goal was to make sure we were competent, independent adults. We had to learn to read a map, change our oil, when we were five he helped us open our own bank accounts and made sure we put money in them to save. One thing he couldn’t protect me from or prepare me for was autoimmune encephalitis. But as always, my parents have stood behind me, loving me, supporting me, at times holding me when I cried from the frustration of not being who I was before and celebrating the small goals I have achieved as I have inched along in my recovery.

Events one doesn’t conceive of celebrating such as when I completed day rehab, passed my special driving test so I could resume driving, and when I was able to stay up all day without a nap. (Autoimmune encephalitis makes you celebrate the small things).my Father celebrated with me.

So, when we feel alone, know that whether we still have our Fathers physically with us or not, they stand behind us, cheering us on.

Happy Father’s Day DadMari Davis Father 225x300 - Thanks Dad!Willard Wagner

 

seal - Thanks Dad!

 

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

 

 

 

trivia fb 300x251 - Thanks Dad!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’s 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.

 

 

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

 

why zebra - Thanks Dad!

 

I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

June-10-2020 | Gary Walters

December 2019

Gary Walters 2 281x500 - I’m Looking Forward to Living the Rest of My Life after Autoimmune EncephalitisThe 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.

 

Gary Walters 3 300x300 - I’m Looking Forward to Living the Rest of My Life after Autoimmune EncephalitisEventually, 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.

 

Gary Walters 1 300x300 - I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

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.

 

seal - I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

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

 

 

 

trivia fb 300x251 - I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

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.

 

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

 

why zebra - I’m Looking Forward to Living the Rest of My Life after Autoimmune Encephalitis

 

 

Serious Illness Changes Course of Lawyer’s Life, Career, and Practice

Serious Illness Changes Course of Lawyer’s Life, Career, and Practice

April 15-2020 | Jackie M. Stebbins

Jackie M. Stebbins 1 300x234 - Serious Illness Changes Course of Lawyer’s Life, Career, and PracticeLawyers have two great fears in life: 1) missing a deadline and 2) getting sick. Missing a deadline is scary and keeps us awake at night, but getting sick is about the worst. A simple cold or the stomach flu can require us to reschedule a deposition which could possibly change the course of the case and other deadlines, and it feels like all the dominos then start to fall. We’re humans, so we’re going to get sick, but we’re also lawyers, so we can plan for everything. Or wait, can we? I am a lawyer and I fell deathly ill while in private practice. It was unexpected, there was no accordingly tailored action plan, and it was worse than I ever could have imagined. In late May 2018, I was diagnosed with autoimmune encephalitis (AE) and it has forever altered the course of my life. My disease is a sneaky assailant. It took my body and it took my mind before I, or anyone around me, could get a serious hold on what was going on.

I suffered from insomnia for quite a while before I decided to take it seriously, but only because I was afraid it would start affecting my work. All signs pointed towards depression and anxiety. Lawyers become depressed from their workload and stress, that’s a fact. Who was I to believe I was any different, especially because of the hours I was working and the nature of the cases I was handling as a family law and criminal defense attorney. I didn’t want to admit to any mental health issues, but the slow and serious deterioration of my health finally made me admit and accept it. I was prescribed antidepressants to sleep and continued to plow through work. I told myself I’d take time off in the summer, I just had to make it through my busy spring. By the end of April, I was still an insomniac, my jaws were clenched, my hands were shaking, and my ears rang. In early May, I left work for a week’s break. Earlier that day, I had what I now know was a serious anxiety attack at my desk, and I knew I couldn’t stay at work any longer. I believed I needed time for my medication to kick in, which would hopefully allow me to sleep. On the surface, I told myself I would return to work very soon, but a deep down dark thought told me I’d never return to my office as I nearly collapsed out the door that day. Only six days after I left work, I checked myself into the psychiatric ward. My decline was obvious. I wasn’t very communicative, I stared a lot, I couldn’t sleep during the day or night, I no longer believed I was fit to drive with my children, and my body stopped working while I tried to swim or bike. I suffered from paranoia and confusion and hallucinated with prescribed sleeping medication. During my 48-hours in the ward, my mind started to slip and things, like knowing the date and reading a clock, became a challenge. I struggled to read and write. I cried and exhibited serious tremors. Something told me I didn’t belong in the ward, but I was desperate for help to sleep and to feel better.

From the time I left work until my time in the ward, my memory isn’t great. Once I left the ward, I nearly ceased to exist as a person and my memory is bare. My life during that time has been pieced together through records and my family’s recollections. For six days after I left the ward, my husband of 10 years took care of me like a child and wondered if I had dementia or was possessed, because of my cognitive impairment and strange behavior. At a follow-up behavioral appointment, my nurse practitioner immediately believed I had a neurological condition and expedited my referral to a local neurologist. Her astute thinking absolutely saved my life. However, the word “neurological” led my husband and family to think the worst. They believed I had a brain tumor. My neurologist’s diagnosis the next day was also life-saving for me. He believed I had AE but ordered more testing to rule out other conditions. Over the course of three days, I failed a neurological examination with flying colors, had an MRI image of my brain that was of poor quality because I shook so badly and could not lie still, and I underwent a spinal tap. I have only a few memories of the testing the first day and I don’t remember much after. My family cried for days and I was oblivious to everything. I repeatedly asked the same questions about what was going on but was fairly easily reassured and was compliant. My mother repeated over and over, “Thank God she doesn’t understand what’s going on.” Just 20 days prior, I had successfully defended an order to show cause hearing, but could not draw the face of a clock during my neurological exam. During the early morning hours, the day after the spinal tap, I had a grand mal seizure in bed that broke and dislocated my right shoulder. I was taken to CHI-St. Alexius Hospital in Bismarck, where I spent the next five days. My memories from the hospital are almost nonexistent and the ones I have are skewed. My health was incredibly fragile and there was serious discussion of having the Mayo Clinic’s airplane fly to Bismarck to retrieve me. While I was sick and rendered incompetent, my husband, family, and a few close lawyer friends took over my life and made all of the decisions for my cases, my role in my own law firm, my health care, and whether my husband had to sell our home and move us closer to our family. I had no idea any of this was going on. They all went into crisis mode to fight for my health and so that no one missed a step at the law firm. The thankfulness I have for my Superman husband, my beloved family, and my friends can never be fully explained. My health started to stabilize, so I stayed in the Bismarck hospital. Upon my release, I began a week of IV steroids to treat the AE. By the second day of treatment, my mind rallied, and my family saw the signs of me again. I underwent a CT scan that same week, which revealed blood clots in my right lung and leg, and three broken vertebrae in my back. It was also during that week that I was able to understand I was never mentally ill, but that all my health problems were the AE at work.

I went to the Mayo Clinic in June and my diagnosis and treatment were all verified, which was good news; it was the devil we were coming to understand. But the recovery process was slow from there. I struggled with people diverting my attention, noise, anxiety, fear, personal interactions, any public outings, could not drive, felt broken physically and mentally, was limited in movement, and was shaky and unsteady. I was mostly confined to my home for a year. I had to repair my mind, body, and spirit, and the Superwoman efforts required of me to survive and recover were overwhelming. With the love and support of my husband and family, and my determination to recover for the sake of my children, I rose to the challenge.

I never returned to my law firm after the onset of the AE, and I elected to retire from private practice based upon my health conditions. Starting in fifth grade, I only wanted to be a lawyer and have sacrificed more than I can explain to get to where I was in my career when the AE hit. Feeling like I lost the career I loved and the law firm I was so proud of, felt like the end of me and everything I knew. Although I have lost in unexplainable ways, I have gained an incredible new perspective on life. I have a lot of hope for the future. I also believe I can use the same drive and skills I honed to be a respected lawyer to accomplish the same goal I had as a lawyer: to help others. How I can best do that, only time will tell. I am currently working on a book to describe my experience with AE and I hope it is out for readers to enjoy in 2020.

As I’ve gone through this journey, I believe I’ve learned a few things along the way and hope to offer a little advice. 1) Take your work-life balance seriously. Upon deep soul searching, I know I was working too hard and not enjoying enough around me. I will live with that guilt and lots of “what-ifs” for life. 2) Make sure you surround yourself with colleagues and friends you admire and trust because your professional life requires it if you are ever unexpectedly debilitated. 3) Purchase long-term disability insurance. It is a great investment and was a life-saver for me. But most importantly, understand and read your policy before disaster strikes.

 

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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 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.

Be a part of the solution by supporting IAES.

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A Look Back at Autoimmune Encephalitis Awareness Month

A Look Back at Autoimmune Encephalitis Awareness Month

April-1-2020 | Barbara Layt Vujaklija, RN

Now that frantic February is finally over its time to look back and take stock of what we achieved. World Encephalitis Day (WED) was started by the Encephalitis Society in October of 2013 and has been held every February 22nd annually since then. The purpose is to raise awareness of encephalitis globally. The International Autoimmune Encephalitis Society (IAES) has participated in WED annually since its formation.  This year IAES designated the month of February to be Autoimmune Encephalitis (AE) Awareness month and will continue to exhibit the face of AE every year.

 

So, what did we do to bring attention to AE in February 2020?  IAES President, Tabitha Andrews Orth, put together our 4th annual AE awareness video. This wonderful video features members who were kind enough to send photos of themselves in treatment and before and after shots. The AE Warriors Stepping Forward video was a great success so thanks to all who participated.

 

Our annual Virtual Arts Show 2020 was a beauty to behold. Filled with talent. Drawings and paintings, poems and short stories. It was an outpouring of emotion from the hearts of AE Warriors from all over the world.  If you ever want to know what it feels like to have AE look no further.

 

We rolled out a selection of AE awareness products that provide the support and encouragement of everyone in the AE community’s desires. AE Warrior Gifts, now available year-round, features the Royal Registry certificates for the AE warrior ™ and Caregiver. These certificates recognize the heroism in battling AE by the patient and caregiver for their heroism under fire.  Survival Kits for AE patients, caregivers, doctors, RNs, Kids, and parents were created, and many members gave these out.  Candy bar wrappers with fun encouraging statements were developed for fundraising efforts and are available for free as well as a unique AE Medical Identification card IAES developed.

 

With all these great things going on there was still a magnificent topper to the month. Tabitha and her team rolled out a first of their kind Autoimmune Encephalitis Trivia Playing Cards Deck. These remarkable cards were developed with assistance by Dr. Dalmau and have questions and answers about AE to use for quiz games with families, who want to know more about AE, doubles as a rehabilitation tool or can just be used as playing cards.  The original art on the cards was done by a fellow AE warrior, Julia King.  Complimentary decks are being sent out to AE doctors on our Doctor’s List.  The excited responses IAES is receiving from doctors about AE Trivia playing cards are filled with praise and congratulations in developing the ideal tool for AE patients, families and therapists. AE Trivia playing cards are on sale to the public and all proceeds will go to support AE research.  Well done team.

 

So outreach for the first annual Autoimmune Encephalitis Month was a big success and thanks to all the members, caregivers and volunteers who put in their time and hearts to make it happen.

 

Of course, the culmination of the month is February 22nd and World Encephalitis Day. Many members sent in updates around this date and pictures to show before and after views that we decided to share some of them in case you missed them the first time around. So here are some great pictures and a few quotes to go with them.

Renee Sanchez WED April 1 2020 blog - A Look Back at Autoimmune Encephalitis Awareness Month

 

From Renee Sanchez, “Well today’s the day! Almost exactly 5 months from her first seizure, which would be the last day of work and the beginning of this nightmare for our daughter, she is going back to work!”

From Paula Lee Ramirez, “Happy world encephalitis day

 

Paula Lee Ramirez1 - A Look Back at Autoimmune Encephalitis Awareness MonthPaula Lee Ramirez 2 - A Look Back at Autoimmune Encephalitis Awareness Month

 

From ICU September 2017 to now. Symptoms started in August 12, 2017. I wasn’t admitted and believed I was sick until  September 17,2017. Diagnosed on October 3, 2017 with ANTI-NMDA Receptor encephalitis. I have yet to relapse! ❤️

 

 

 

Lucy May Dawson - A Look Back at Autoimmune Encephalitis Awareness Month

 

Lucy May Dawson, “Today is World Encephalitis Day. I, like 78% of people, had no idea what Encephalitis was until the words fell out of my neurologist’s mouth after I was misdiagnosed as having had a mental breakdown and had spent 3 months in a psychiatric ward…….Regardless, I am one of the lucky ones, and I hope that by continuing to post about encephalitis, someone will one day remember one of my posts when their loved one begins to act strangely, and they will ask their doctor to test for it, and it may just save a life”.

 

Daisy Garuvadoo - A Look Back at Autoimmune Encephalitis Awareness Month

 

Daisy Garuvadoo: World Encephalitis Day today, from being critically ill with Anti-NMDA Receptor Encephalitis in Nov 2017, 2 years ago, having to relearn to walk, speak, to brush her teeth. to get dressed, to read, to write, to learn basic maths, to tell time and to regaining her cognitive abilities to Medical School and thriving. Yesterday, my daughter was honored for her research project related to Intrathecal procedures at her Medical School. INTRATHECAL RITUXIMAB TREATMENT(though the spine) IS WHAT SAVED HER LIFE.

 

Kimberly Anne Thompson - A Look Back at Autoimmune Encephalitis Awareness Month

Kimberly Anne Thompson I beat this. I have no lasting side effects. But awareness is so important… Because Encephalitis often presents with psychiatric symptoms it is often misdiagnosed… I spent six weeks in a Psychiatric Hospital originally diagnosed with having a Nervous Breakdown. I was given meds that only made My condition worse. It wasn’t until I started having Seizures and the Encephalitis began affecting My Heart that I was transferred to ICU and had a Lumbar Puncture which showed I had Anti-NMDA Encephalitis…. It is this delay that kills. I urge all Medical Professionals to consider Encephalitis in Patients that present with Hallucinations for the first time… You could save a life.”

 

Grace Harimate - A Look Back at Autoimmune Encephalitis Awareness MonthGrace Harimate, It is world Encephalitis Awareness day today yesterday in NZ time…. as I contemplated sharing these images and my story, my heart was pounding … I wanted to forget but this ordeal you’ll never forget…I was misdiagnosed and mistreated with bipolar taking the wrong medications and wrong treatment for 6 years from 13y to 18y. Spending most of my teenage years in the mental health ward. But so glad I met the most amazing people in those years…..The hard part was to come when I woke up from a two-year coma… I was fully blind for one-year memory loss still tube fed etc.
I was determined to walk … determined to get my eyesight back determined to get back on that horse determined to get a job … I walked into my 21st blind.

 

These awesome warriors and their brave care givers look at World Encephalitis Day and the new Autoimmune Encephalitis Month as a milestone. Where was I last year and where am I now? Some who have been floundering in a wilderness of lost memories have found their way or at least see a light at the end of their tunnel. Some are still, though making progress, struggling with issues others cannot even begin to imagine. Not only one year at a time but moment to moment. Some have been in comas only to wake up to a new truth, new situation, others are missing months or years of their lives. People they will never see again and new members of family and friends they are only meeting now. Such is the nature of Autoimmune Encephalitis. Your own body turning on you and messing up your brain. Sometimes making you hear, see, taste or smell things that are not there. Trying to make the family and friends in their lives understand the impossible.

March on brave warriors, continue to fight and savor your special month and the day set aside for you every year. May your lives look better next February.

 

Your generous Donations allow IAES to continue our important work and save lives! Due to the coronavirus pandemic, the need for our services has surged exponentially. Some cases of COVID-19 have affected the central nervous system and medical researchers are suspecting the infection could cause ADEM, type of Autoimmune Encephalitis. Donations of any size are vitally important to meet the increased need in our community. If you have the ability to set up a reoccurring monthly donation, it will be greatly appreciated and will immediately begin to help patients and families during this historic time.

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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

 

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Not Stress, Not Lyme, Not Cancer, Not Anti-NMDAr Encephalitis

Not Stress, Not Lyme, Not Cancer, Not Anti-NMDAr Encephalitis

March 3, 2020 | By Mitch Gore

MItch and Jeri Gore Jeri in light blue jacket IMG 5024 scaled - Not Stress, Not Lyme, Not Cancer, Not Anti-NMDAr Encephalitis

Mitch and Jeri Gore (Jeri is wearing blue).

Little things, just little things were first noticed by our immediate family. The need to recite over and over the details of how a decision was made, excessive talkativeness, repetitive conversations. Outbursts of anger over inconsequential events, vocalizations of “yep, yep, yep” and a stutter-step when that happened. Something was not right. Periodic feelings of having a “brain spasm” were added to the mix.

Visits to our personal care physician, referral to a neurologist, MRIs and EEGs all resulted in a conclusion that Jeri was just stressed out. After our son’s wedding, everything would be alright.  It wasn’t. The wedding was a big success but for Jeri, concentrating on the many details was difficult, to say the least. Our college friends noticed immediately that something was not right. “She seems to not be herself, she gets angry easily, what’s up?”

Could it be a recurrence of the chronic Lyme disease from 8 years ago? Off to the holistic physician that successfully treated Lyme disease. The approach there was to test for exposure to environmental toxins and Lyme. Blood was drawn and then we left for a couple of weeks to go to our cottage in Canada. In those two weeks, our Canadian friends also came to us and asked if something was going on. The repetitive conversations continued along with withdrawal from group conversation. A couple of times Jeri blanked out for a few seconds and became worried-looking, really concerning our friends.

Back from the cottage the follow up with the Lyme doctor revealed that she was slightly positive on the Lyme test. (Lyme diagnostics is difficult and subjective – a discussion for another time.)That is probably what was going on. Back on antibiotics and several nutritional supplements – for 24 hours. Then our journey really began.

“Mom’s had a seizure, I’m following the ambulance to the hospital.” Our two adult sons living at home had found Jeri having a seizure shortly after going to bed. Another seizure while in the ER. The workup revealed that her sodium levels were dangerously low. Correction of the hyponatremia began along with MRIs and other scans. Five days as an inpatient to correct the sodium levels and all tests came back negative. No infectious causes, no tumors, must just be a case of spontaneous SAIDH (Syndrome of Inappropriate Antidiuretic Hormone Secretion). It happens, that can be corrected with diuretics and salt pills. Take anti-seizure medicine and follow up with a neurologist.

At this point, the reader needs to know a little about our family. For years Jeri was a researcher in a biochemistry lab and up to a couple of years ago, a practice manager for a busy physician’s office. I’m a Ph.D. biochemist and molecular biologist. One son is a newly trained nurse and one daughter-in-law is a very experienced pediatric intensive care nurse. Jeri’s brother-in-law is an attorney with over 20 years’ experience in large pharma oncology and has a Masters in chemistry.  To say that there were many opinions on what was going on is the understatement of the year. A lot of family knowledge and experience were being tapped, all out of concern, love, and with the best of intentions. Conversations with physician friends and nurses suggested a myriad of causes.  None of us got even close to the cause.

The ‘spells’, absent episodes, memory issues, and cognitive decline all continue and increase in frequency. We were home for a week and then another big seizure puts her back in the ER and again admitted to the hospital. Again, all scans and EEG are normal. The doctors in the regional hospital are again scratching their heads. Despite repeated requests, the hospital physicians and our personal neurologist resisted performing a lumbar puncture. Why? We were told there was simply no indication that would justify such a high-risk procedure. Must be the SIADH. Go back to your local neurologist and nephrologist.

A 72 hour EEG was ordered. This was stopped after 24 hours since we noted so many (~30) ‘spells’ and no further recording was needed. Like two spot EEGs before, nothing showed up. The local neurologist was stumped, calling it a real mystery. We again ask for an LP and testing but were refused on the basis that he didn’t know what to test for. As a shot in the dark, testing was ordered for serum anti-NMDA receptor. Negative results. Sodium levels stay a bit low, but near normal.  Frustration all around.

Escalating the case was now a family priority but we were stymied by the long wait (2 months!) to get into the UPenn neurology group. A week after the last neurology visit another seizure. A couple of days later we hear a crash and see Jeri at the bottom of the stairs having a seizure, this time with a gash to the head. Back to the ER after a very irrational and angry outburst. Another seizure in the ER and another admittance to the local hospital. The next morning during rounds the floor neurologist is in the room and the hospitalist (physician in charge of cases on that floor) pops in to see how things are going. She asks the neurologist if she is going to order an LP and the neurologist says no, there is no need. The matter is dropped. No words can describe our frustration and helplessness at this point. My son and I discussed the situation and decided to go to the hospitalist and ask for her intervention in getting the case moved to UPenn. She agreed but was doubtful that she could get her transferred. To her and our surprise, UPenn neurology agreed right away!

We were just so lucky to have the case taken over by UPenn and a group of physicians that have been integral to the discovery and treatment of autoimmune encephalopathies. Within a week we had a diagnosis of anti-LGI-1 autoimmune disease and a week after that we began immunosuppression therapy via a five-day course of steroid infusions and an infusion of Rituximab.

We’ve been home a week, continuing anti-seizure medicine, oral steroids, salt supplementation, and diuretics. No seizures so far! We are seeing small improvements in cognitive ability and memory. We have a long way to go but are hopeful. The second infusion of Rituximab will happen in about a week.

Lessons learned:

Unfortunately, I think we can consider that knowledge of auto-immune encephalopathies is pretty much non-existent to the average physician and even most neurologists. The resistance to exploit all avenues of testing after repeated presentations to the ER and negative results of conventional diagnostics needs to change. That change will only come with physician education. Also, symptoms can be very, very subtle at first and not consistent from person to person. Like a lot of other neurological conditions, the symptoms are common to many different causes.

Now that the mystery is solved the recovery can begin.  Another Zebra identified.

Mitch Gore, Husband of Jeri.

Your generous Donations allow IAES to continue our important work and save lives!
Learn how you can raise awareness of Autoimmune encephalitis by getting your story published in your local newspaper. 

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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

 

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When Your Brain is on Fire

When Your Brain is on Fire

brain on fire

January-22-2020 | Carolyn Keating, PennNeuroKnow

Imagine you’re a bright twenty-something with a new job and a new relationship.  Everything seems to be going your way until you start becoming paranoid and acting erratically.  Then come the hallucinations and seizures.  You’re admitted to a hospital where you’re (incorrectly) diagnosed with a psychiatric disorder.  You swing from violence into a state of immobility and stupor.  And perhaps even scarier?  You don’t remember any of it.  Sound like a nightmare?  Well, it actually happened to Susannah Calahan, who details her terrifying story first-hand in her 2012 book Brain on Fire: My Month of Madness.

What caused these frightening symptoms?  The answer was a disease that had only been discovered a few years earlier (right here at Penn!): NMDAR encephalitis.  There are four main phases of the disorder.  In the prodromal phase, many but not all patients experience a flu-like illness for up to 3 weeks.  The psychotic phase is accompanied by delusions, auditory and visual hallucinations, depression, paranoia, agitation, and insomnia.  At this stage, most patients are taken to the hospital, where around 40% are misdiagnosed as having a psychiatric disorder like schizophrenia.  As this phase progresses, seizures are very common (although they can occur at any time throughout the illness), as well as involuntary muscle movements like lip-smacking or grimacing, catatonia (muscular rigidity and mental stupor), impaired attention, and memory loss.  The next phase is unresponsiveness, which includes symptoms like the inability to speak, loss of voluntary movement, and sometimes abnormal muscle contractions that cause involuntary writhing movements.  The last phase is the hyperkinetic phase and is characterized by instability of involuntary bodily functions such as breathing, blood pressure, heartbeat, and temperature.  Many patients who breathe too slowly often need to be placed on a ventilator at this stage. The decline to ventilator support can progress very rapidly after several weeks in the psychotic stage, and ultimately patients can be hospitalized for several months with the disease1–3.

What does NMDAR encephalitis actually mean?  This disease is an autoimmune disorder, meaning the body’s immune system mistakenly attacks its own healthy cells.  Normally the body identifies foreign substances by making something called an antibody that recognizes a unique part of the invader, thus targeting it for attack and destruction.  In NMDA encephalitis though, the immune system attacks the brain (that’s where to term encephalitis comes from), specifically a type of neurotransmitter receptor called an NMDA receptor (NMDAR).  These receptors bind the neurotransmitter glutamate, and play an important role in learning, memory, cognition, and behavior.  In fact, the symptoms of NMDAR encephalitis resemble those caused by drugs such as ketamine or PCP that prevent the activation of NMDARs.  For instance, at low doses ketamine and PCP cause paranoia, false perceptions, and impaired attention (like the early stages of NMDAR encephalitis), and at higher doses these drugs cause psychosis, agitation, memory and motor disturbances, and eventually unresponsiveness, catatonia, and coma2.  Several mechanisms have been proposed to explain the symptoms caused by antibodies targeting the NMDAR, but most of the evidence seems to support the idea that the receptors get removed from the cell surface and internalized.  For instance, experiments in the laboratory demonstrate that when animal neurons grown in a dish are exposed to patients’ anti-NMDAR antibodies, the number of NMDARs on the cell surface decreases as the amount of antibodies increase.  When the antibodies are removed, the number of NMDAR receptors on the cell surface returns to baseline within 4 days1.

It’s easy to remove antibodies in a dish, but how do doctors get the body to stop producing antibodies against itself?  Step one is identifying what triggers antibody production in the first case.  Interestingly, NMDAR encephalitis predominantly affects women, and ovarian teratomas (a type of tumor made up of multiple types of tissues, which can include nervous system tissue) are responsible for 50% of cases in young women2.  In patients who have some sort of tumor, removal improves symptoms in 75% of cases.  Interestingly, herpes simplex virus can also cause encephalitis (inflammation of the brain), and about 20% of these patients also develop antibodies against NMDAR2.  Treatment consists of immunotherapy: corticosteroids, IV infusion of immunoglobulins, and/or plasma exchange1, however patients with a viral trigger tend to be less responsive to treatment than those with a teratoma trigger or the 50% of patients with an unknown trigger2.  Once treatments begin improvements in symptoms start within a few weeks, though return to baseline functioning can take up to three years.  Rehabilitation is required for many patients after they leave the hospital.  Deficits in attention, memory, and executive function may linger for years, but luckily over 75% of patients with the disease recover to at or near baseline neurological functioning1.

Doctors and scientists hope to develop new treatments involving immunotherapy combined with small molecules that are able to access the brain to directly combat the effects of anti-NMDAR antibodies, ideally leading to faster control of symptoms and shorter recovery time2.  A brand new animal model of the disease was just described last week that will hopefully lead to more discoveries about how the disease is triggered and potential new therapies4.  And with increased awareness of autoimmune disorders against the brain, doctors will be able to more quickly correctly diagnose patients with this illness and get them the treatment they need.

References:

  1. Venkatesan, A. & Adatia, K. Anti-NMDA-Receptor Encephalitis: From Bench to Clinic. ACS Chem. Neurosci. 8, 2586–2595 (2017).
  2. Dalmau, J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology 87, 2471–2482 (2016).
  3. Dalmau, J. et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 7, 1091–1098 (2008).
  4. Jones, B. E. et al. Anti-NMDA receptor encephalitis in mice induced by active immunization with conformationally-stabilized holoreceptors. bioRxiv 467902 (2018). doi:10.1101/467902

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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

 

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When You Need Help with Your Memory Loss

When You Need Help with Your Memory Loss

January -8-2020 | Mari Wagner Davis, RN

I have been a nurse since 1985. I was working as a nurse case manager when I was at work in December 2017. A co-worker, who I have worked with for years, noticed I was acting strange- laughing inappropriately, busy but not really doing work.  We shared an office and she took my keys out of my purse because I had talked about going home and that idea scared her.

Memory Loss Mari Warger Davis January 2020 500x419 - When You Need Help with Your Memory LossWhat neither of us knew at the time was that she saved my life. Because I couldn’t find my keys, I went back to the unit I worked on, shortly after that I had a seizure. I was hospitalized and after a few days of testing, I was diagnosed with anti-NMDAR encephalitis. Anti-NMDAR stands for anti-N- methyl-D- asparate receptor encephalitis. It is an autoimmune disease where the body creates antibodies against the NMDA receptors in the brain. The antibodies disrupt normal brain signaling and can cause seizures, memory problems, cognitive issues, speech disorders, neuropsychiatric symptoms as well as other symptoms.

I was treated with steroids, IVIG and plasmapheresis. I had inpatient and day therapy rehabilitation. I also did a computer cognitive therapy program. Recovery can be slow. Many patients with autoimmune encephalitis are left with memory problems, cognitive deficits and have problems in situations that are cognitively demanding. Memory continues to be an issue since my diagnosis with AE.

 

I have a calendar and use my phone to keep track of my schedule but what I have found is that people treat me as I was before, that would be great if I had my normal memory, but I don’t. That is part of the problem with having an invisible disability. If you didn’t know what happened to me, I look and can for the most part act “normal”- whatever that is. But in truth, I have trouble remembering previous conversations.

What was easy for me in the past takes far more of my energy than it used to. I find it is difficult to follow a conversation in a group. There is too much information for me to take in at one time. Sometimes in a conversation with several people, it exhausts me, I lose my place, and end up not trying to carry on and be a part of the conversation- it’s just too much for my brain to process. I just give up. I may experience “flooding” where my brain cannot take in so much information at one time.  People in the conversation may notice I get quiet.  They may think that I disagree with them or don’t like the topic.  In truth, I may have lost track of the conversation and have no idea what the topic is.

Things that were easy for me in the past are difficult for me now. For example, recently my sister sent me a text asking me to bring two side dishes to a party to celebrate my Dad’s birthday, I don’t remember having a conversation about it at all. I looked back at my texts and found a message from her. In the past, this would be something I would remember and follow up on. Now, I have no memory of the subject at all.

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There are ways for others to help those of us with Autoimmune Encephalitis become more comfortable in these situations. So, for family members and friends of those with AE these tips may be helpful.

1. When you start a conversation about plans previously made, talk about the previous discussion that was held.  Help by cueing the person. For example, you may say “I messaged you before about dinner on Friday the 12th and wanted to make sure you were still available.”  That reassures me that I did have a conversation about it in the past and reminds me of the topic and date.  It also allows me a chance to confirm it.

2.  If you are asking me to do something, refer back to what had previously happened and provide contact information if you have it, for example, “Last year for the block party you scheduled the Police department to come and register bikes, can you do that again this year? if you can, I will send you the contact information, Let me know by Monday the 8th”

3. If we have made plans, contact me several days or up to a week before to confirm, for example, “I have on my calendar that we were going to the movies on Friday the 10th at 7 pm, will that still work for you?”

4. If we are going someplace where there will be people I may have only met once before, take the time to reintroduce me to them. My husband will usually tell me, “you met them before at the game, but this is John and Mary”

5. When my husband leaves for work and I am still asleep, he leaves a note telling me that he left for work. He knows that if I am not aware, I won’t be sure of the day of the week and may look around the house for him and be anxious about where he is.

Letting family and loved ones know that incorporating these types of supports helps us remain social and successful is truly appreciated.  It can keep us from becoming overwhelmed or “shutting down” and allows us to participate fully with more confidence. Trying to stay socially involved is important. Taking a little extra time to do these things will help us to recall previous discussions and allow us to feel more comfortable.

As time goes on, and friends and family learn the types of difficulties their loved one is experiencing, it will be easier to anticipate situations that they may have trouble managing.   Coming up with solutions by troubleshooting these situations is the best support you can provide.

 

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My letter to Santa ATTENTION: YULETIDE BUREAU  WISH DEPARTMENT

My letter to Santa ATTENTION: YULETIDE BUREAU WISH DEPARTMENT

 

December 23, 2019 | Tabitha Andrews Orth 

My letter to Santa.
ATTENTION: YULETIDE BUREAU 
WISH DEPARTMENT

Dear Santa,
As you know, I have given great thought to my Christmas wish this year.  I hope I have made your ‘NICE LIST”, as my wish is in the form of a favor.

Since I am awake, due to insomnia from the IV Solumedrol infusion I received yesterday afternoon, I thought it was a good time to write you. I guess I didn’t need to tell you that, since you see me when I am sleeping, and you know when I am awake. LOL:)

Sometimes the lessons we need to learn to grow our soul and faith come in the most unexpected forms. I have realized the truth of this and am doing my best to rely on my faith, to celebrate the love and support I receive and do my best to face my adversity.

Santa, I have to be totally naked in my honesty here. As Head Elf, embodied with the Spirit of -now here’s my word finding problem rearing its head… It means to love without qualifying or judging. I can’t remember how to say it Santa, but you get me. How could anyone ever imagine they would get a disease where your immune system attacks your brain?!

I mean if my brain isn’t working right, nothing works right. That’s a hard thing Santa.  We are talking TOUGH challenge here.

Santa, you know this. You have been watching. I don’t have to explain because you have lived it with me. Yeah, I’m totally on the “NICE LIST”.

Sorry, attention span problems, back to my favor. 
But… I have practiced daily random acts of kindness for many years…attention span problems again and paranoia I might not make the cut for that ‘Nice List’.

Santa, this disease has created personality changes that are not the heart of me. It has taken ME away before with episodes of psychosis twice now. I am inside. In my heart, buried deep, I am ME. People were not able to see ME when my brain disease flared in this way, but YOU could!
Sorry, I will try to stay on topic. Attention Span problems, but YOU know this.

Okay, I forgot what I was going to say… LOL. Wait, I’ll reread this and write my main point on a sticky note so I can remember my wish. Why am I telling you that? YOU see me. YOU realize everything.
Ok. Wait just one minute for me Santa. Yes. I am back. Got it. My Christmas Wish. (That is what I wrote on the sticky note. Oops, sorry, forgot. Don’t need to tell you that.)

I am having a problem with worry for my future. I have now faced FEAR.  I have stared it down. I have survived. I get scared sometimes. YOU know I do. My faith and spiritual beliefs sustain me; through them, I draw my courage and do my best to surrender my fear. 

This brings me to that favor I mentioned. My Christmas Wish. 
Gosh, Santa, I need your help.

By the way, Jim says guys like peanut butter cookies and oatmeal raisin the best, so we will be leaving those out in their usual place by the Cocoa. Feel free to use the copper pot I leave out for you to warm up your Cocoa in case you find yourself running late.   Oh, the carrots for the Reindeer are grown by local farmers- I am adding some lovely fresh pears this year…
But, you know that too… Sorry…..
What was I going to say? Oh, right! My Christmas Wish:)

You’ll find an envelope by your nighttime snack. I’ve enclosed all my fears and worries inside. Could you kindly take them from me? That is my Christmas Wish. 

And when you take your leave, by the way, I LOVE that time suspension magic you do- very cool!

When your laughter rings out within and around our home, can you add a bit of Christmas Spirit Magic to infuse your laughter, Joy, and that kind of love I can’t remember the name of …within our home, our hearts and my brain where my fears and worries once were?  By adding your love and laughter to mine Santa, well, that’s a powerful infusion. Exactly what is needed to receive the appreciation of each day.

My family and I laugh a lot Santa. YOU know. My laughter helps my husband, Jim and our son, Matthew. Their laughter helps me. And so, it goes….

P.S.
Should you find other letters written by people with Autoimmune Encephalitis, whether they be drawings by children, a single word, or simply the breath of fear blown inside an envelope and sealed, my wish is that you take these too and leave your laughter.

Oh, make yourself at home as long as you would like Santa. The weather across the Northern Hemisphere is projected to be quite cold. There are extra blankets in the hall closet. Help yourself. When you are working your way across the Southern Hemisphere and get to Sarah’s house in New Zealand since it is Summer there and you won’t need them any longer, you can leave the blankets with her and she’ll get them back to me on her next visit;) 
Pleasant trip and God speed. 
Best Wishes,
Tabitha 

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(Originally written December 17, 2014)


IAES invites you to spread awareness of Autoimmune Encephalitis by sharing this delightful musical spoof utilizing the classic holiday music from ‘Rudolf the Red-Nosed Reindeer’. Learn who identified the most common type of autoimmune encephalitis of them all and where that magical moment occurred.



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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 premier organization leading in these vital roles.

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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

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How does Sleep Affect the Blood-brain Barrier?

How does Sleep Affect the Blood-brain Barrier?

December-11-2019 | Sarah Reitz, PennNeuroKnow

IAES PNK Partnership logo 300x251 - How does Sleep Affect the Blood-brain Barrier?Autoimmune encephalitis (AE) is the name for a group of conditions that occur when the body’s immune system mistakes its own healthy brain cells for invaders, leading to brain inflammation that ultimately triggers a number of other symptoms. Normally, the body’s immune system has only limited access to the brain, as it is protected by the blood-brain barrier (BBB). When this barrier is healthy, it can prevent an immune system attack by blocking immune cells and antibodies targeting brain cells from actually entering the brain. Like any fortress, however, the BBB isn’t completely impenetrable. Given that AE symptoms occur when immune cells or antibodies manage to cross the BBB, researchers think that the weakening of this barrier plays a critical role in AE and may even be a target for future therapies to reduce or prevent AE symptoms. But what causes the BBB to weaken, allowing cells, antibodies, and other molecules to invade the brain?

 

The blood-brain barrier: gatekeeper of the brain

Before discussing how the BBB becomes impaired, we need to understand how the healthy BBB functions. The BBB is often referred to as a “gateway”, made up of tightly joined endothelial cells that surround the blood vessels in the brain and spinal cord1. Outside of the brain, the endothelial cells lining blood vessels have small spaces between them, allowing for the exchange of substances between the blood and the surrounding tissue. However, the endothelial cells of the BBB are connected to each other by proteins called tight junction proteins, which squeeze the cells tightly together, blocking larger cells and molecules from freely flowing between the blood supply and the brain (Figure 1).

PNK Sleep and BBB 300x290 - How does Sleep Affect the Blood-brain Barrier?

Figure 1: The blood-brain barrier is made up of endothelial cells that are tightly connected to each other by tight junction proteins (purple). These tight connections prevent unwanted substances from traveling between the blood and the brain. Different types of transporter proteins (yellow & blue) shuttle only certain types of molecules between the blood and brain.

The BBB is selectively permeable, meaning it allows only certain substances to enter and leave the brain. One way that molecules can cross the BBB is by endocytosis, a process where the endothelial cells uses its cell membrane to take in a molecule on one side (say, the side facing the blood) and pass it through to the other side (facing the brain) where it is released1. The endothelial cells of the BBB also express a variety of transporter proteins, which actively move molecules between the blood and the brain (Figure 1). Additionally, small, fat-soluble molecules can cross the BBB without any help from endocytosis or transporter proteins, giving the brain access to important nutrients and energy sources1.

BBB permeability changes across the day

The permeability of the BBB is not always the same, however. Research has shown that its permeability actually changes depending on the time of day2. Like many cells in the body, BBB cells are controlled by circadian rhythms, biological processes that cycle roughly every 24 hours. These rhythms are driven by a molecular “clock” within each cell, and cells across the body are synchronized by the “master clock” located in the brain.

What do these rhythms mean for BBB permeability though? Interestingly, research in both flies and mice shows that the amount of hormones, inflammatory proteins, and other molecules that cross from the blood into the brain fluctuates across the day, with peak BBB permeability occurring at night2.

Sleep loss impairs BBB function

Circadian rhythms aren’t the only daily process that affects the integrity of the BBB. Sleep—or more appropriately, lack of sleep—is also known to affect the BBB’s protection of the brain. This relationship between sleep and the BBB is increasingly important as sleep restriction becomes more and more common in our modern society.

Sleep loss is also highly relevant to the AE community. One study found that 73% of AE patients surveyed reported sleep disturbances, including gasping/snoring and insomnia. Even further, patients with AE had decreased total sleep time and increased fragmentation of sleep compared to people without AE3. But how exactly does sleep loss affect the BBB?

Blood Brain Barrier after full sleep 300x71 - How does Sleep Affect the Blood-brain Barrier?

Figure 2: After periods of sleep loss, the blood-brain barrier is negatively affected in many ways (left). Inflammatory signaling caused by TNFα and IL-6 increases, leading to the breakdown of tight junction proteins (purple). This causes gaps between endothelial cells, allowing unwanted immune cells and antibodies to enter the brain. After sleep loss, the endothelial cells also make fewer of the transporter proteins (blue and yellow) that are required to shuttle necessary molecules between the brain and blood.

Multiple studies have now shown that sleep restriction weakens the BBB. One reason is due to the increase in inflammatory signaling that results from extended periods of wakefulness. Increases in inflammatory proteins, like TNFα and IL-6, are known to break down the tight junction proteins that keep the endothelial cells tightly joined together2 (Figure 2). Sleep-deprived mice and rats showed decreased numbers of tight junction proteins, leading to increased BBB permeability.

In addition to weakened tight junctions between endothelial cells, sleep loss also increases permeability by enhancing the rate of endocytosis across the BBB2, meaning that the endothelial cells shuttle more molecules from the blood into the brain. Relevant to AE, this increased permeability means that more immune cells and antibodies can enter the brain after sleep loss compared to after a full night’s sleep.

While these results are a bit frightening, there is good news. All of the damage to the BBB caused by sleep loss returns to normal after getting enough sleep! One study found that even an extra 1-2 hours of sleep following sleep loss restored BBB function in most brain areas4. Given even more time to sleep, the BBB throughout the brain returned to normal function5. These results suggest that treating the sleep disorders commonly associated with AE may help strengthen the BBB, increasing the brain’s protection against the immune system’s cells and antibodies and improving long-term outcomes for patients.

The BBB as a treatment target

Given that AE is caused by immune cells and antibodies infiltrating and attacking the brain, researchers are now looking at the BBB as a potential therapeutic target1. Treatments that strengthen the BBB will hopefully reduce the number of immune cells and antibodies that make it into the brain, and may also increase the effectiveness of some AE medications, such as anti-inflammatory drugs or immune-suppressants. Because these AE medications are specifically designed to cross a healthy BBB and access the brain, strengthening a weakened BBB will protect against molecules that aren’t supposed to be in the brain, while still allowing the necessary medication in.

The known circadian effects on BBB permeability can also be used in determining when to give medication that needs to cross the BBB. Medication can be given at the time of day when BBB permeability is highest to increase the amount of drug that makes it into the brain. In fact, this has already been studied with anti-seizure medication in epilepsy. For instance, in both flies and humans, when medication was given at night during peak BBB permeability, it was most effective at controlling seizures6,7.

This new strategy of “chronotherapeutic” dosing schedules has the potential to improve the efficacy of medication in many diseases. By administering drugs when it is easiest for them to enter the brain, doctors may be able to see results at lower doses of the drug, potentially reducing the risk of harmful side effects. As we continue to learn more about the BBB, scientists may identify even more ways to improve BBB health in the many disease states where it is compromised.

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Image References:  Figure 1 and 2 created with BioRender.com

References:

  1. Platt MP, Agalliu D, Cutforth T (2017) Hello from the other side: How autoantibodies circumvent the blood-brain barrier in autoimmune encephalitis. Front. Immunol. 8:442. doi: 10.3389/fimmu.2017.00442
  2. Cuddapah VA, Zhang SL, Sehgal A (2019) Regulation of the blood-brain barrier by circadian rhythms and sleep. Trends Neurosci 42(7):500-510. doi: 10.1016/j.tins.2019.05.001
  3. Blattner MS, de Bruin GS, Bucelli RC, Day GS (2019) Sleep disturbances are common in patients with autoimmune encephalitis. J Neurol 266(4):1007-1015. doi: 10.1007/s00415-019-09230-2
  4. Gomex-Gonzalez B, et al. (2013) Rem sleep loss and recovery regulates blood-brain barrier function. Curr. Neurovasc. Res. 10, 197-207
  5. He J, Hsuchou H, He Y, Kastin AJ, Wang Y, &Pan W (2014) Sleep restriction impairs blood-brain barrier function. J Neurosci 34(44)14697-14706. doi: 10.1523/jneurosci.2111-14.2014
  6. Zhang SL, Yue Z, Arnold DM, Artiushin G, Sehgal A (2018) A circadian clock in the blood-brain barrier regulates xenobiotic efflux. Cell 173(1):130-139. doi: 10.1016/j.cell.2018.02.017
  7. Yegnanarayan R, et al. (2006) Chronotherapeutic dose schedule of phenytoin and carbamazepine in epoleptic patients. Chronobiol. Int 23, 1035-1046

 

 

 

The “Immune” in Autoimmune Encephalitis: The Role of T and B Cells

The “Immune” in Autoimmune Encephalitis: The Role of T and B Cells

Nov-27-2019 | Carolyn Keating, PennNeuroKnow

The Immune System: An Explainer

When we catch a cold, get an infection, or otherwise become sick, our bodies use a natural defense mechanism called the immune system to fight off what’s attacking us.  The immune system has two ways of responding1.  The first, called innate immunity, involves physical and chemical barriers like the skin and saliva, as well as many different types of cells that “eat” and destroy whatever is causing the trouble.  While this innate response happens very quickly, then the downside is that it’s not very specific, and the immune cells can damage healthy parts of the body while trying to gobble up the foreign invaders.  In order to specifically target particular offenders, the body uses its second way of responding: the adaptive immune system.  This response can take days or weeks to develop but is also able to remember what the foreign invader looked like, so if it attacks again a targeted reaction can occur faster than the first time.  To acquire this immunity against a particular foreign substance, the body uses two types of cells that act in different ways: T cells (which develop in an organ called the thymus, that’s where the “T” comes from) and B cells (which mature in the bone marrow, hence the “B”).

 

These two cell types are able to attack so specifically because each one recognizes a particular structure, called an antigen, on a foreign substance.  For instance, one T cell might recognize a certain part of an influenza virus, while another could recognize a specific part of a bacterium; the same situation also holds true for B cells.  The T and B cells travel around between different lymphoid tissues (organs like the spleen, tonsils, and lymph nodes, the last which are spread throughout the body) until they encounter their particular antigen.  Once activated by their antigen, the T and B cells leave the lymph tissues and work in different ways to fight off the foreign invader (Figure 1).

types of T and B cells PNK - The “Immune” in Autoimmune Encephalitis: The Role of T and B Cells

Types of T and B cells

T cells come in many varieties, but the two major types are cytotoxic and helper.  Cytotoxic T cells (sometimes referred to as CD8+ T cells due to a particular identifier on their surface) travel to the disease site to search for cells that also bear the antigen that activated them, and destroy them.  Helper T cells (sometimes referred to as CD4+ T cells), as the name might suggest, help activate other parts of the immune system.  There are many subtypes of helper T cells that activate different types of responses; for instance, some promote the cytotoxic T cell response, while others activate B cells. Another kind of CD4+ T cell called regulatory cells actually tells the immune system, not to attack2.

 

Unlike T cells, B cells do not destroy their target.  Instead, once they are activated by their antigen and T helper cells, they mature into plasma cells that produce antibodies, proteins that recognize the same antigen as the B cell.  These antibodies essentially enhance the innate immune system and act in several ways, including neutralizing toxins, signaling to other immune cells that a cell should be attacked and destroyed, or activating complement.  Complement is a group of proteins (not cells) that make up yet another arm of the immune system.  These complement proteins can recruit immune cells or directly kill foreign cells themselves1.

 

T and B Cells in Autoimmune Encephalitis

So what happens in autoimmune encephalitis (AE)?  In this and other autoimmune diseases, the body mistakenly recognizes part of itself as a foreign invader and mounts an attack. Scientists believe that AE starts when a tumor or virus causes proteins from neurons to be exposed to the immune system. The proteins get picked up by immune cells outside the brain that go on to activate T and B cells in lymphoid tissue. These activated cells then make their way into the brain where they cause AE3,4.  Which cells are responsible for causing the disease depends on what antigen sets off the immune response.

In cases where the antigen comes from inside a cell, cytotoxic T cells are the culprits.  When proteins from inside neurons like Hu, Yo, or Ma2 are the antigens, that usually indicates that the immune system first encountered the proteins in a cancerous tumor, which can express proteins from all sorts of cell types (this cancer association is why these antibodies and diseases are called “onconeural,” or “paraneoplastic”).  Cytotoxic T cells fighting the tumor can make their way into the brain and kill neurons5.  This cell death is likely part of the reason why patients with these diseases have poor recovery.  Antibodies from B cells that have matured into plasma cells can also be produced in response to the tumor, but they do not contribute to AE symptoms6.

Antibodies do have a strong role in producing AE symptoms when the antigen comes from the outside surface of a neuron, like the NMDA receptor for instance.  These antibodies can still be formed in reaction to a tumor, but this is less common.  Research on NMDAR encephalitis, in particular, has revealed the presence of B cells and antibody-secreting plasma cells in the brain7,8.  Because the antibodies have access to the surface proteins they target, they can bind to them and interfere with their function.  In the case of NMDAR encephalitis, it’s thought that the antibodies cause the receptors, which normally are exposed to the outside of the cell, to be taken back inside so that they can’t function properly.  Once the antibodies are gone the receptors can return to the cell surface, reversing many of the symptoms9.  Unlike diseases in which the antibodies target intracellular proteins, in NMDAR encephalitis there are few to no cytotoxic T cells in the brain or neuronal death5,7,8.  But while there are little to no cytotoxic T cells, there have been reports of helper T cells around blood vessels in the brain, including one type called Th17 that act to enhance the immune response10.

 

In other cases of encephalitis with antibodies again a cell surface protein, such as LGI1, CASPR2, or GABA receptors, the precise immune reaction is less certain and in some ways seems to be a little different from NMDAR encephalitis.  B cells and plasma cells are still found in the brain, and antibodies also play a major role in causing symptoms5,11.  For instance, antibodies against the GABAB receptor block it from functioning, while antibodies against LGI1 can disrupt interactions between proteins and lead to a decrease in AMPA receptors12.  The involvement of T cells is unclear and may vary depending on the disease-causing antibody. For example, cytotoxic and helper T cells have been found in the brain of anti-GABAB receptor patients11, while few T cells were found in anti-VGKC-complex patients5.  In addition, scientists sometimes observe signs of complement, the protein arm of the immune system that can kill cells5,6.  In line with the presence of cytotoxic T cells and complement, neuronal loss is sometimes reported5,13.

 

Overall, the type of immune response the body produces appears to be dependent on the specific antigen. In general, diseases with antibodies that target intracellular proteins like Hu, Yo, or Ma2 involve cytotoxic T cells that kill neurons.  In contrast, diseases with antibodies that target cell surface proteins like NMDAR, LGI1, and GABAR involve B cells in symptom production. In this second category, the role of T cells and complement may vary depending on the particular antigen.

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References

  1. Parkin, J. & Cohen, B. An overview of the immune system. Lancet 357, 1777–1789 (2001).
  2. Corthay, A. How do regulatory T cells work? Scand. J. Immunol. 70, 326–336 (2009).
  3. Venkatesan, A. & Adatia, K. Anti-NMDA-Receptor Encephalitis: From Bench to Clinic. ACS Chem. Neurosci. 8, 2586–2595 (2017).
  4. Dalmau, J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology 87, 2471–2482 (2016).
  5. Bien, C. G. et al. Immunopathology of autoantibody-associated encephalitides: Clues for pathogenesis. Brain 135, 1622–1638 (2012).
  6. 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).
  7. Martinez-Hernandez, E. et al. Analysis of complement and plasma cells in the brain of patients with anti-NMDAR encephalitis. Neurology 77, 589–593 (2011).
  8. Tüzün, E. et al. Evidence for antibody-mediated pathogenesis in anti-NMDAR encephalitis associated with ovarian teratoma. Acta Neuropathol. 118, 737–743 (2009).
  9. Dalmau, J. et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 7, 1091–1098 (2008).
  10. Zeng, C. et al. Th17 cells were recruited and accumulated in the cerebrospinal fluid and correlated with the poor prognosis of anti-NMDAR encephalitis. Acta Biochim. Biophys. Sin. (Shanghai). 50, 1266–1273 (2018).
  11. Golombeck, K. S. et al. Evidence of a pathogenic role for CD8 + T cells in anti-GABA B receptor limbic encephalitis. Neurol. Neuroimmunol. NeuroInflammation 3, 1–8 (2016).
  12. Dalmau, J. & Graus, F. Antibody-mediated encephalitis. N. Engl. J. Med. 378, 840–851 (2018).
  13. Shin, Y.-W. et al. Treatment strategies for autoimmune encephalitis. Ther. Adv. Neurol. Disord. 11, 1–19 (2018).

 

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Through my eyes: Watching my Dad’s AE journey

Through my eyes: Watching my Dad’s AE journey

November-13-2019 | Ryan J Dinero

Ryan J Dinero father died of AE 1 e1573603671514 375x500 - Through my eyes: Watching my Dad’s AE journeyIt’s been a little over two years since I got the phone call from my Mom. I was a sophomore in college and was entirely unprepared for the conversation that was to follow. She explained that my dad had a minor seizure while at work and was being transported via ambulance to the hospital. This news came as a total surprise to me because my dad was in seemingly perfect health – no drinking, no smoking, and he exercised frequently. While I could tell that my mom was obviously shaken, in the moments after that phone call I was able to convince myself that everything was fine, that he would get a prescription and return home the same day unscathed. Like many others who have experienced the grueling battle against autoimmune encephalitis I couldn’t have been more wrong about what was to follow. So began my dad’s battle with the disease.

Its hard to explain to people who haven’t ever heard of AE that it’s not a simple disease to diagnose. In my dad’s case, it took two separate stints in the hospital to finally get an accurate diagnosis. After the first incident he was admitted into the ICU where he lost control of his body almost entirely for several days until the steroid medications began to reduce the swelling in his brain. The neurologists at our local hospital were perplexed as to what the cause of his symptoms could be. They ran a series of tests that ruled out everything from a stroke, a bacterial infection, as well as a multitude of other conditions that I can’t begin to remember. After two weeks in the hospital his facilities began to come back as the medicine did its job, and we stayed hopeful that it was somehow an isolated incident not to be repeated. He was released and began resting at the house as he recovered. It couldn’t have been more than two weeks later that I was woken up by my mom’s screaming in the early hours of the morning. As I ran the stairs I prepared myself for what I was about to see, filled with adrenaline and terror that even two years later keeps me awake in the night. When I reached the living room, I discovered my Dad on the couch having a full-on seizure, something that I had only previously seen in movies. Knowing little to nothing about what strategies to employ to help, I opted to pick up the phone and dial 911, and just as I heard the phone ringing I heard my Dad’s voice from the other room express that the seizure was over and that he felt fine. I hesitantly told the operator that things were fine, spent a few minutes making sure my Dad was responsive and lucid, and somehow returned to sleep. Less than an hour later I was awoken by the same screams, and this time I knew that another hospital visit was imminent. Thankfully, the doctors explained that a small reduction in the seizure medication was to blame and they sent us home that same day.

Over the next month my Dad returned to his normal self slowly but surely. His previous hospital stays were characterized by extremely abnormal behavior that the doctors attributed to his brain swelling. Things that he would have normally never said or done became commonplace while he was in the hospital. He forgot names, places, and other details. After the doctors adjusted his medicine he became more lucid. He was able to laugh at jokes and even bust out some of his own signature humor. While things were obviously different, namely his strength and ability to remain energetic throughout the day, things felt normal. They remained that way until Christmas Day, when another minor seizure sent us to the Houston Medical Center. The hospital was almost completely understaffed and made for a less than ideal celebration of Christmas. This visit was the most serious of the many that we experienced, as we called Houston Methodist our second home for over a month while doctors scrambled to properly diagnose my Dad’s symptoms. The team of neurologists there were incredible given the circumstances and after weeks of uncertainty and guessing, they were able to finally conclude that my Dad was suffering from autoimmune encephalitis caused by an antibody that was unknown.

Ryan J Dinero father died of AE 2 500x333 - Through my eyes: Watching my Dad’s AE journeyFor my family, a diagnosis was everything we had wanted; we could finally begin a treatment plan that was focused, instead of one that had no timeline or endgame in sight. By this time however, my Dad’s health had seriously taken a toll. As a result of losing his ability to walk he was bedridden for most of the month. It was also around this time in the hospital that something seemed to be off with him mentally, he was saying things that weren’t necessarily true and overall had developed a very bad attitude that led to frequent outbursts.

After another lengthy stint at a physical therapy center, Dad was finally able to return home. My mom worked tirelessly with him every day on his occupational therapy, encouraging him and try and stay positive. Things were okay again for a few months, but eventually took a dark turn as my Dad began having suicidal thoughts and began exhibiting symptoms of psychosis, leading him to believe things about the world that weren’t true. He was constantly worried about money and convinced himself that the police were looking for him and had a warrant for his arrest. He left the physical therapy facility in February and in April he attempted to take his life for the first time. Thankfully he was unsuccessful, but he did serious damage to his arm as a result and had to be admitted to a psychiatric hospital for a few weeks and had to undergo more physical therapy for his arm.

It was during his stint in the psychiatric hospital that I first reached out to the folks at the International Autoimmune Encephalitis Society where I received tons of resources about how to get Dad released from the psychiatric unit. Once he was released, it became apparent that IVIG treatments were the right treatment, but we were denied a total of 3 times by our insurance provider on the grounds that the treatment was not FDA approved and did not actually treat AE. Thanks to the hours of phone conversation with members of the IAES, I was able to eventually overturn the decision and Dad began receiving IVIG treatments shortly after. When we returned home, I could tell that he was tired of living, that his spirit was drained, and he was ready to give up.

Despite utilizing every available resource at our disposal, he succeeded in taking his life in late September of 2018, just three days before his 51st birthday.

It’s hard to describe my thoughts and feelings throughout the past two years because the only way I can think of it is as a never-ending roller coaster, constantly up and down with no end in sight, until there finally was one.

Ryan J Dinero Father Johnny 9 20 2018 500x375 - Through my eyes: Watching my Dad’s AE journeyI would be failing to honor my Dad’s legacy if I failed to mention that he was an incredible man, the best man I think I’ll ever have the pleasure of knowing. That said, for me, the experience was agonizing and easily the worst thing I’ve ever had to endure. It felt like some days would never end, and it was exhausting trying to rationalize with someone’s who’s brain would not let them experience reality the way it ought to. The countless hours spent sleeping on uncomfortable hospital furniture, the excruciating hours spent at physical therapy being a cheerleader, and the anxiety of waiting for the doctor to step in the room are just a few poignant memories that come to mind when I think about that period of my life. Some of the happier memories involve performing daily miracles: like making my Dad laugh at a dumb joke or getting him excited enough about The Beatles to get him to sing along in the car.

 

This whole experience has shaped me and impacted me in ways that I cannot begin to describe. While my Dad’s battle with AE ended almost a year ago on September 20th, I continue to wage my own battle every day dealing with the repercussions of this deadly disease. It’s still hard to get out of bed some days because everything is a reminder that I’m here and he isn’t. I can’t help but think about what I could have done different, how I could have made his life a little better than I tried. I think these feelings are natural now that he’s gone. As much as I try to not let myself dwell on them they are always in the back of my mind. The whole-time Dad was sick I was in school three hours away, so many weekends were spent back home at the side of a hospital bed while I struggled through my sophomore and junior years of college. I somehow managed to stay in school and keep my grades up, and I’m looking forward to graduating this coming year.

For the first time in a very long time I can confidently say that I am genuinely happy and hope that by writing a little bit of my story that someone out there, whether it be a caretaker, a friend, a family member, or even someone who hasn’t heard of AE would be encouraged and inspired.

To all the caretakers out there – keep going and don’t let up. Use the resources that IAES has to provide the best care to your loved one. I can confidently say that if it weren’t for this organization my Dad would never have received IVIG treatments, which were instrumental in his recovery.

If you’re reading this, and can relate in any way, I’d love to hear from you about your experience. Much love to everyone.

Ryan

Contact Ryan at ryandinero@icloud.com

 

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

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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

 

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Introducing the IAES and PennNeuroKnow Partnership

Introducing the IAES and PennNeuroKnow Partnership

IAES and PNK announce partnership in autoimmune encephalitis and neuroschience education

October 16-2019 | Carolyn Keating and Sarah Reitz

Hello AE Community!

 

Our names are Carolyn and Sarah, and we are happy to announce the partnership between IAES and our blog, PennNeuroKnow (PNK).  We are working with IAES to learn about topics that patients and families in the AE community have trouble understanding, in order to create handouts and blog posts that explain these issues in a way that’s easy to digest.  We’re excited to begin this alliance and to introduce our team to the AE community.

PNK is a blog we founded in early 2018 to dive into the complex field of neuroscience and simplify it so that anyone can understand.  Including the two of us, we have 6 writers creating weekly articles ranging from general topics like how the brain produces curiosity, to breaking down specific journal articles on subjects like how the bacteria in your gut may be linked to depression.  All of us are PhD students in the University of Pennsylvania’s Neuroscience Graduate Group who are committed to better communicating science.  We know that scientific studies are sometimes difficult to both access and understand, so we want to use our training as scientists to share our passion for neuroscience and make our field more accessible to everyone.

We were first introduced to IAES in July 2019, when Carolyn wrote about NMDAR encephalitis in a blog post called When Your Brain is on Fire.  IAES saw the post and shared it on their Facebook page, giving the article much greater reach than we normally experience.  The amount of positive feedback we have received from the AE community has been overwhelming, and we are truly grateful to have been able to help so many people understand the science behind the disease that has affected themselves or a loved one.  Now thanks to IAES President Tabitha Orth reaching out to us about forming a partnership, we are excited to produce more easy-to-read articles on complex topics important to the AE community.

All of our writers are looking forward to learning more about AE and the issues that are difficult for patients and families to grasp.  Already we are hard at work learning and writing about how AE relates to the immune system, memory loss, and FDG-PET scans, just to name a few topics.  We hope that we can use our strengths as neuroscientists to help translate complicated subjects and journal articles into something everyone can understand, and are excited to contribute to this wonderful community. We want to make sure we are writing about topics that are most important to you and your family members, so please do not hesitate to reach out to either Tabitha or us with topics you would like to learn more about!

Get In Touch with IAES

Get In Touch with PNK

E-mail Sarah and Carolyn Directly at PNK 

seal - Introducing the IAES and PennNeuroKnow Partnership

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

 

 

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 

Why the Zebra

 

IAES Turns 3. Meet Our New Board of Directors

IAES Turns 3. Meet Our New Board of Directors

September 5th, 2019

The IAES Board of Directors is a group of committed volunteers. No board member receives compensation. Therefore, we are especially grateful to have the expertise and talents of these dedicated professionals. Our annual election results bring us some familiar faces, who are now in new positions, and some new faces. All of whom are fully dedicated to serving the worldwide autoimmune encephalitis community and advancing our mission.

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Tabitha Andrew Orth, President

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Dr. Daria Muir, Secretary

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Gene Desotell, Vice President

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Oksana Sweeney, C.P.A, Treasurer

 

Dear AE Family,

Who would have imagined in 2016 that IAES would change the direction of the AE community?  Yet that is the praise we are hearing from Doctors, patients, caregivers and families from all over the world.

As the community’s only Family/Patient centered non-profit, IAES offers services that were never available before.  IAES provides guidance from when a person first becomes ill all the way through to their recovery/rehabilitation. You find a family you never knew you had with IAES. We take your hand and become your constant BFF resource no matter what challenge you find yourself faced with.

Our free pro-active guide, the ‘First Aid Tool Kit’, first aid tool kit 225x300 - IAES Turns 3. Meet Our New Board of Directorshas become the ‘go to’ guide in the AE community.

IAES provides information that overturns treatment denials, medical over billing, educational support in understanding treatments, the disease process and what to expect. Guidance to improve relationships with your medical team, resources that affect every avenue of this journey.

Doctors around the world utilize our website to accurately diagnose and treat their patients. A team of doctors in Russia contacted us to thank us for the work we do and reported that the information they were able to access on our website saved their patients’ lives.

Our collaboration with top researchers in Autoimmune Encephalitis continues to widen. The increase in awareness in the medical community has resulted in a much higher rate of patients getting diagnosed faster.  The daily gratitude we receive for our services just might give you a ‘swelled head’, (pun intended), but our work has only just begun!

Celebrate our birthday by becoming an IAES Angel.

When you become an #IAESANGEL, International Autoimmune Encephalitis Society will send you an “I am an IAES Angel” badge and profile frame to place on your Facebook page or Website.  As badges ‘take flight’ heralding IAES has been ‘touched by an angel’, others will take notice and they too may find their wings. Together, we will create a future where AE is eradicated from this world and only referenced in medical history books.

https://autoimmune-encephalitis.org/iaes-angel/

Click image. (note: To become an IAES Angel, select “I would like to designate this donation to a specific fund”, and then select: “I would like to become an IAES Angel”.

 

With Gratitude,

Tabitha Andrews Orth, President,

International Autoimmune Encephalitis Society

E-mail: Tabitha.Orth@autoimmune-encephalitis.org

 

 

 

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

Why the zebra 2 - IAES Turns 3. Meet Our New Board of Directors

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.

CONTACT US


352-527-2470

IAES@AUTOIMMUNE-ENCEPHALITIS.ORG

Autoimmune Encephalitis Trivia Playing Cards

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