January 25, 2023 | Written by Dr.Hannah Ford. Edited by Dr Mastura Monif, Dr Loretta Piccenna, Ms Sarah Griffith, Ms Tiffany Rushen, Ms Amanda Wells (consumer representative) and Ms Sasha Ermichina (consumer representative).
A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of typical psychiatric manifestations of autoimmune encephalitis This overview is by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif, who is a member of IAES’ Medical Advisory Board.
We are proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project as we work closely with them to best support AE patients, caregivers, and their families.
You can find out more about the Australian Autoimmune Encephalitis Consortium and its efforts to help those with AE and their families via the following link:
Psychiatric Manifestations of Autoimmune Encephalitis
WHY WE DID THIS WORK
Autoimmune encephalitis is a disorder in which antibodies accidentally created by the immune system attack parts of the brain. This can lead to inflammation and nerve damage.
Psychiatric problems are common in autoimmune encephalitis and can imitate mental health conditions, for example psychotic illnesses like schizophrenia. It is important to separate patients with AE from those with mental illness as treatments are very different.
There are different subtypes of AE. Some cases are due to the presence of detectable auto antibodies (a protein targeting the person’s own nerve endings) which is known as ‘sero positive’ AE. In ‘sero negative’ AE, there is no detectable antibody when using currently available techniques for detection.
Within the ‘sero positive’ group are different AE categories depending on the type of antibody. We discuss this further in the next section.
Anti-NMDAR encephalitis
The most common type of AE and typically occurs in young women. Psychiatric problems are the presenting feature in most patients and many are seen first by a psychiatrist. Symptoms start abruptly and progress rapidly over days to weeks.
Common features include psychosis (disruption of person’s thoughts and perceptions that can make it difficult for them to understand what is real versus what is not real). They can present with hallucinations (seeing or hearing things that are not there) and paranoia (false beliefs; for example, believing that people are out there to get you, or having unfounded mistrust of others), agitation, and elevated mood. Occasionally, anti NMDAR encephalitis patients can present with catatonia (complete lack of movement or lack of communication).Almost 90% of patients develop other related neurologic features (including seizures, abnormal movements and speech, and drowsiness) within a month, however some may have only psychiatric problems without neurologic signs.
Anti-LGI1 encephalitis
The second most common AE and typically affects older males.Seizures are usually the first symptom and often occur before patients develop psychiatric and/or memory problems. These seizures can be very brief, i.e. seconds long, and subtle (face and arm twitching known as “faciobrachial dystonic seizures”), but can be very frequent (up to 100s of times per day).
Memory difficulties develop slowly over months and may be accompanied by disinhibition (actions or words that might seem inappropriate or rude or inconsiderate).
They can also present with compulsive behaviors (performing an action persistently repetitively), including excessive eating, cleaning and hoarding.
Psychotic symptoms such as hallucinations and paranoia can occur but are less common, and usually are not an early or major feature.
Anti-CASPR encephalitis
Involves confusion, memory difficulties and ‘slow’ thinking which may be associated with depressed mood. Memory problems can slowly worsen over 12 months or longer in a proportion of patients. These individuals may appear like they have dementia.
Psychotic symptoms such as hallucinations, delusions and paranoia can occur as inflammation of the brain worsens, and usually develop with other neurologic symptoms including seizures, unsteadiness and abnormal jerking and twitching movements.
Anti-AMPAR encephalitis
Typically presents with short-term memory problems, confusion and behavioral changes which get worse over weeks to months.
Psychotic symptoms are variable (20-90% of patients) and may be associated with manic (abnormally elevated or extreme in mood, emotions, energy or activity levels) and aggressive behavior.
Seizures are rare. This type of encephalitis is frequently associated with cancer.
Anti-GABA-A encephalitis
Most commonly presents with seizures.
Memory loss and confusion develop slowly over weeks to months and are associated with personality and behavioral changes in approximately half of patients.
Features of psychosis with hallucinations and paranoia are uncommon but can occur later in severe cases.
Anti-GABA-B encephalitis
Also commonly presents with seizures.
Memory difficulties, confusion and abnormal behavior develop with or after seizures start.
Patients often become depressed and/or anxious at a later stage, usually 1 to 2 years after other symptoms have started.
Memory difficulties are slow to improve and may remain even after treatment.
Psychosis is not a feature.
Anti-DPPX encephalitis
Preceded by diarrhea and weight loss for several months, followed by mild, slowly worsening memory and cognitive difficulties associated with depression and anxiety.
Months or even years later patients develop psychotic features including hallucinations, delusions and aggression with neurologic symptoms such as seizures, limb shaking and jerking.
Anti-mGluR5 encephalitis
A rare type of AE with three major features – psychosis, memory problems and drowsiness.
Patients experience headaches, fevers, weight loss and nausea followed by rapid onset of memory problems, slowed thinking and severe psychiatric symptoms including hallucinations, depression, anxiety and major mood swings.
Many different neurologic symptoms can occur, including seizures, abnormal movements and difficulty using the eyes and face.
Anti-Neurexin-3a encephalitis
The disorder develops quickly over several days with headaches, fevers and nausea, followed by confusion and agitation.
Patients then experience severe neurologic symptoms of drowsiness, abnormal movements, seizures and breathing problems.
Diagnosis and treatment
Features (“red flags”) that may indicate AE as a cause of psychiatric presentation are shown in table 1.
Diagnosis of AE is challenging, and is confirmed by identifying the antibody in the blood or fluid from around the brain and spinal cord (cerebrospinal fluid), however these tests are not always available and may take a long time to return. Other test results that indicate AE may be the cause of psychiatric symptoms include high white cells or inflammation in cerebrospinal fluid, abnormal brain imaging on MRI and abnormal brain electrical activity on EEG (electroencephalogram; refer to our previous summary on EEG here: https://autoimmune-encephalitis.org/using-eeg-nmda
Early treatment of AE can lead to partial or full recovery.
Table 1.
Red Flags for Autoimmune Encephalitis in Psychiatric Presentations
· Preceding physical symptoms such as fever, headache, stomach upset and dizziness
· Seizures
· Neurologic symptoms such as abnormal movements, speech difficulties, clumsiness, weakness and changes in sensation
· “Catatonic” features such as abnormal posturing, repeating another person’s speech (echolalia), lack of movement or erratic movements
· Memory problems
· Psychotic symptoms that start rapidly and/or worsen quickly
What do the findings mean?
Each subtype of AE presents with different psychiatric features. Our research can help clinicians identify patients with psychiatric symptoms due to AE rather than a mental illness.
Early consideration of AE as a differential for psychiatric presentations is important as patients respond well to appropriate treatment (immunotherapy), particularly if given early.
Further studies are needed to continue describing the syndromes associated with each subtype. Fast and accurate testing for the diagnosis of AE is an important area for future research.
—-
For more information and resources from Dr. Monif and her group at the Australian Autoimmune Encephalitis Consortium Project, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:
Your generous Donationsallow IAES to continue our important work and save lives!
On June 16 th, 2022, Tabitha Orth, President and Founder of International Autoimmune Encephalitis Society officially became the 7,315 th “point of light”. Recognized for the volunteer work she and IAES has done to spark change and improve the world for those touched by Autoimmune Encephalitis. The award was founded by President George H.W. Bush in 1990.
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE. For this interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
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
(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.
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.
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.
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
(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.
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.
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.
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
(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.
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.
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.
September 26, 2020 | Claudia Lopez-Lloreda, PennNeuroKnow
The plastic brain
In autoimmune encephalitis (AE), the body generates antibodies that mistakenly attack neuronal proteins that are important for brain function. Among the most important proteins targeted in AE are neurotransmitter receptors1. Neurotransmitter receptors function as the “lock” for different neurotransmitters, like dopamine and serotonin, that act as “keys”. Neurotransmitters unlock these receptors and through this allows neurons to communicate with each other.
One defining feature of the nervous system that neurotransmitters play a key role in is brain plasticity. Brain plasticity, also called neuroplasticity, is the ability of the brain to adapt by changing, re-wiring, or making new connections between neurons. This is important because this plasticity in response to lived experiences is what enables behavioral changes, such as learning new things and forming new memories2. Research and anecdotal evidence show that learning and memory are affected in AE, which makes it important to understand what brain plasticity is and how it is affected in disease.
Brain plasticity: How does it work?
At the cellular level, plasticity is seen mainly by the change in strength of the connections between neurons, called synapses. This is known as synaptic plasticity and it can go two ways: synapses can strengthen, known as long-term potentiation (LTP), or they can weaken, known as long-term depression (LTD)2. Importantly, the quantity and function of neurotransmitters and their corresponding receptors are critical for synaptic plasticity. More neurotransmitter molecules and more receptors means that neurons can communicate better and more effectively, which allows for the strengthening of their connection.
Strengthening usually happens when two neurons synchronize their activity. One famous researcher, Donald Hebb, said it conclusively: “Neurons that fire together, wire together.” This usually happens in our brain in response to different experiences, but it can also be studied in the lab. This process is studied by artificially stimulating connections to induce either LTP or LTD2. Using a baseline, scientists then study how different interventions affect whether the connection strengthens or weakens. By doing this, they can see how different changes, such as a generation of autoantibodies, can change these connections.
Can autoantibodies affect plasticity?
Since we know that AE is characterized by autoantibodies against important neuronal proteins—specifically neurotransmitter receptors—scientists wondered whether these autoantibodies could affect synaptic plasticity. One study looked at this by treating mice with antibodies against one specific subunit of the AMPA receptor derived from AE patients3. The AMPA receptor is one of the locks for the neurotransmitter glutamate, which is important in excitatory transmission, the type of communication where neurons activate other neurons. The researchers found that treating mice with autoantibodies led to internalization of the receptor, meaning the cells took the receptor away from its normal location on the outside of the neuron. Inside the neuron, the receptor could no longer exert its function and the neurotransmitter lost its effect.
The mice treated with these human antibodies against AMPA receptors had impaired LTP in a specific pathway of the hippocampus, an area that is critical for the formation of memories3. This means that with autoantibody treatment from AE patients, the strengthening of the synapses did not occur as well as it did when mice were not treated with the antibodies. As a consequence, treating mice with these antibodies affected their learning and memory. The researchers saw that the impairments that mice developed with antibody treatment paralleled the strong memory impairments seen in disease.
Similarly, a group of researchers treated brain slices from mice with fluid derived from the brains of patients with AE4. This fluid had autoantibodies specifically against an important neurotransmitter receptor called the NMDA receptor (NMDAR), another lock for the same neurotransmitter glutamate. Once again, the antibody-rich fluid derived from AE patients impaired LTP4. Injecting fluid from patients with NMDAR encephalitis straight into the brains of live mice also blunted the ability of connections to strengthen5.
However, these studies were done in animals. In humans, studying brain plasticity is a bit trickier, since neurons are deep inside the human brain in humans and artificially activating them is not an easy task. One way it can be done is by pairing two activations. The first activation, called peripheral electrical stimulation, is done by giving a jolt of electrical pulses to peripheral nerves such as those in the hand. At the same time, the researchers non-invasively stimulate the area in the brain that connects with the peripheral nerve by using a technique called transcranial magnetic stimulation. By doing this, they can “look” at what is happening in the brain to see how this artificial paired activation leads to changes in synaptic plasticity.
Studies show that this type of stimulation in humans produces something similar to the plasticity seen in mice and in tissue slices6. One study applied transcranial magnetic stimulation to patients with NMDA encephalitis and found that plasticity was impaired when compared to healthy individuals6. Strikingly, the degree of impairment in synaptic plasticity was associated with disease severity. These studies suggest that the autoantibodies generated in AE can be detrimental to the important function of synaptic plasticity in the brain. Further, impairments in synaptic plasticity could be a contributing factor to the symptoms seen with disease.
What does this mean for recovery?
Brain plasticity is also a mechanism that the brain uses to recover from damage. After injury, the brain can try to find new ways to do things. For example, if an area that controls understanding speech is damaged, the brain can reorganize to change where it gets different speech information from. In this case, the rearrangement of synapses and the alteration of synapse strength could be a way the brain tries to respond to the injury mediated by autoantibodies in AE. As a treatment, activating plasticity has been considered for psychiatric disorders7. Different strategies include medication8 and even exercise, which has been shown to enhance plasticity9. Therefore, it is possible that plasticity could be activated to help patients with AE. However, more research has to be done to further understand how exactly these interventions could change brain plasticity in AE and potentially help people recover from the debilitating symptoms.
Haselmann, H., Mannara, F., Werner, C., Planagumà, J., Miguez-Cabello, F., Schmidl, L., … Geis, C. (2018). Human Autoantibodies against the AMPA Receptor Subunit GluA2 Induce Receptor Reorganization and Memory Dysfunction. Neuron, 100(1). https://doi.org/10.1016/j.neuron.2018.07.048.
Zhang, Q., Tanaka, K., Sun, P., Nakata, M., Yamamoto, R., Sakimura, K., … Kato, N. (2012). Suppression of synaptic plasticity by cerebrospinal fluid from anti-NMDA receptor encephalitis patients. Neurobiology of Disease, 45(1), 610–615. https://doi.org/10.1016/j.nbd.2011.09.019
Würdemann, T., Kersten, M., Tokay, T., Guli, X., Kober, M., Rohde, M., … Kirschstein, T. (2016). Stereotactic injection of cerebrospinal fluid from anti-NMDA receptor encephalitis into rat dentate gyrus impairs NMDA receptor function. Brain Research, 1633, 10–18. https://doi.org/10.1016/j.brainres.2015.12.027
Volz, M. S., Finke, C., Harms, L., Jurek, B., Paul, F., Flöel, A., & Prüss, H. (2016). Altered paired associative stimulation-induced plasticity in NMDAR encephalitis. Annals of Clinical and Translational Neurology, 3(2), 101–113.https://doi.org/10.1002/acn3.277
Uscinska, M., Mattiot, A. P., & Bellino, S. (2019). Treatment-Induced Brain Plasticity in Psychiatric Disorders. Behavioral Neuroscience. https://doi.org/10.5772/intechopen.85448
Nitsche, M. A., Müller-Dahlhaus, F., Paulus, W., & Ziemann, U. (2012). The pharmacology of neuroplasticity induced by non-invasive brain stimulation: building models for the clinical use of CNS active drugs. The Journal of Physiology, 590(19), 4641–4662. https://doi.org/10.1113/jphysiol.2012.232975
Erickson, K. I., Miller, D. L., Weinstein, A. M., Akl, S. L., & Banducci, S. (2012). Physical activity and brain plasticity in late adulthood: a conceptual and comprehensive review. Ageing Research, 3(1), 6. https://doi.org/10.4081/ar.2012.e6
Your generous Donationsallow IAES to continue our important work and save lives!
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
For this interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
Autoimmune encephalitis (AE) is a disorder that can be hard to diagnose. Typically, early symptoms are flu-like, making it difficult to distinguish from many other illnesses. Psychiatric symptoms and behavior changes are often among the first signs of autoimmune encephalitis, especially NMDAR encephalitis, and a majority of patients are seen first by psychiatrists upon entering the emergency room1. Other neurological symptoms of AE, such as seizures and problems with movement and memory typically develop later than the psychiatric symptoms1. However, AE is not usually diagnosed until the appearance of these neurological symptoms since the early psychiatric symptoms are often misdiagnosed as a psychiatric disorder, which leads to a delay in treatment for AE1. Understanding the psychiatric symptoms and behavior changes that often signal the onset of AE can lead to quicker detection, earlier treatment, and better outcomes for patients.
One study of AE found that 77% of patients with anti-NMDAR encephalitis initially came to the hospital due to psychiatric symptoms2. Usually, the psychiatric symptoms caused by AE include agitation, aggression, irritability, hallucinations, delusions, and depressed mood3. The most common symptom was agitation or irritability, appearing in 59% of adults and 66% of children2. Psychotic symptoms such as hallucinations were the second most common2. These psychiatric symptoms are often misdiagnosed as a psychiatric disorder rather than being investigated as early symptoms of AE.
An additional challenge in diagnosing AE from psychiatric symptoms is that the pattern of symptoms often differs between adults and children. Adults are more likely than children to experience psychotic symptoms like hallucinations2. Children, unlike adults, are likely to have temper tantrums as a symptom4. Children also often have some early neurological symptoms like seizures in addition to the behavior changes, while adults usually begin with psychiatric but not neurological symptoms6. The differences in type and timeline of symptoms between children and adults could be explained by different underlying causes of AE. For example, in adults AE is often the result of a tumor, but tumors are usually not the cause of AE in kids7.
How might AE affect behavior?
While the exact mechanisms by which AE causes behavior changes are not well understood, anti-NMDAR encephalitis research provides some potential insights into processes in the brain that might lead to these symptoms. Anti-NMDAR encephalitis, as the name suggests, involves antibodies against a type of neurotransmitter receptor called NMDARs. These NMDARs bind a neurotransmitter called glutamate. Several conditions must be met for NMDARs to become active: glutamate must bind to the NMDAR and the electrical voltage of the cell must reach a certain level8. When NMDARs are active, they allow charged ions to cross the cell membrane, which can then send a signal to other cells. NMDAR activation is involved in processes like learning, memory, and behavior.
In anti-NMDAR encephalitis, antibodies in the immunoglogbulin G (IgG) subclass target the NMDA receptors9. These IgG antibodies bind to part of the NMDA receptor and make it so they are not able to signal as usual. Neurons from rats that were treated with IgG from AE patients had a decreased number of NMDARs on the cell surface. When the antibodies were removed, the NMDARs returned back to normal levels10. This indicates that IgG antibodies can cause removal of NMDARs from the surface of the cell, where they can no longer interact with neurotransmitters. Many NMDARs are found on a type of cell called GABAergic neurons9. These neurons typically suppress activation of nearby neurons and help to regulate levels of activity in the brain. The attack on NMDARs in AE may lead to reduced activity of GABAergic neurons, which in turn causes too much activity in other parts of the brain.
How does this change in glutamate signaling that is mediated by NMDARs relate to psychiatric symptoms? One theory about the mechanisms underlying schizophrenia, a psychiatric disorder characterized by hallucinations and delusions, also includes reduced availability of NMDARs11. The subsequent deactivation of GABAergic neurons is believed to produce too much activity that leads to many of the psychiatric symptoms of schizophrenia. Drugs that block NMDARs, such as ketamine, are known to cause psychosis, agitation, and difficulties with memory11. All of these are also common symptoms of anti-NMDAR encephalitis. The progressive loss of NMDARs due to antibody attack could create these same psychiatric symptoms in people with AE.
How are psychiatric symptoms addressed?
Getting a better handle on understanding and treating the behavioral symptoms of AE requires improved diagnosis and intervention. When someone arrives in the hospital with significant behavioral changes or psychiatric symptoms, it would be beneficial if doctors could screen for and diagnose AE even before some of the more severe neurological symptoms begin to appear.
Many patients receive medications that target the psychiatric symptoms that are later diagnosed as related to AE12. These medications include antipsychotics for people who are having symptoms of psychosis. However, in some cases, antipsychotic medications have been shown to cause adverse effects such as catatonia and coma in AE patients, so doctors need to give these medications with care2. Earlier diagnosis of AE can prevent patients from getting incorrect diagnoses and psychiatric treatments that can actually worsen their AE. Importantly, while medications may help to manage the psychiatric symptoms, they do not target the underlying causes of AE and patients will still need standard treatments like immunotherapy or tumor removal to treat the AE itself.
Typically, once patients receive immunotherapy, the behavior symptoms of AE begin to go away. Most patients recover fully and no longer have any psychiatric symptoms after recovery. However, approximately 30% of patients may have lasting neuropsychiatric deficits after treatment for AE13. In patients who have psychiatric symptoms after immunotherapy, continued use of antipsychotic medications such as clozapine can help to alleviate symptoms14.
In children who recover from AE, behavioral symptoms may continue and pose particular challenges for parents7. Some children were reported to have academic difficulties after recovering from AE15. Parents and caregivers dealing with bad behavior from a child who had AE can learn behavior management techniques to help address these behavioral difficulties. These strategies, as well as early screening of psychiatric symptoms and behavioral changes, could help to improve diagnosis, treatment, and recovery from AE.
References:
Finke, C. (2019). A transdiagnostic pattern of psychiatric symptoms in autoimmune encephalitis. The Lancet Psychiatry. 6 191-193.
Sarkis, R. A., Coffey, M. J., Cooper, J. J., Hassan, I., & Lennox, B. (2019). Anti- N -Methyl-D-Aspartate Receptor Encephalitis: A Review of Psychiatric Phenotypes and Management Considerations: A Report of the American Neuropsychiatric Association Committee on Research. The Journal of Neuropsychiatry and Clinical Neurosciences, 31(2), 137–142.
Al-Diwani, A., Handel, A., Townsend, L., Pollak, T., Leite, M. I., Harrison, P. J., … Irani, S. R. (2019). The psychopathology of NMDAR-antibody encephalitis in adults: a systematic review and phenotypic analysis of individual patient data. The Lancet Psychiatry, 6(3), 235–246.
Brenton, J. N., & Goodkin, H. P. (2016). Antibody-mediated autoimmune encephalitis in childhood. Pediatric Neurology. 60 13-23.
Suthar, R., Saini, A. G., Sankhyan, N., Sahu, J. K., & Singhi, P. (2016). Childhood Anti-NMDA Receptor Encephalitis. Indian Journal of Pediatrics, 83(7), 628–633.
Esposito, S., Principi, N., Calabresi, P., & Rigante, D. (2019). An evolving redefinition of autoimmune encephalitis. Autoimmunity Reviews. 18 155-163.
Armangue, T., Petit-Pedrol, M., & Dalmau, J. (2012). Autoimmune encephalitis in children. Journal of Child Neurology. 27(11) 1460-1469.
Husi, H. (2004). NMDA Receptors, Neural Pathways, and Protein Interaction Databases. International Review of Neurobiology, 61, 49–77.
Dalmau, J., Lancaster, E., Martinez-Hernandez, E., Rosenfeld, M. R., & Balice-Gordon, R. (2011). Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. The Lancet Neurology. 10(1) 63-74.
Dalmau, J., Gleichman, A. J., Hughes, E. G., Rossi, J. E., Peng, X., Lai, M., … Lynch, D. R. (2008). Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. The Lancet Neurology, 7(12), 1091–1098.
Uno, Y., & Coyle, J. T. (2019). Glutamate hypothesis in schizophrenia. Psychiatry and Clinical Neurosciences, 73(5), 204–215.
Kuppuswamy, P. S., Takala, C. R., & Sola, C. L. (2014). Management of psychiatric symptoms in anti-NMDAR encephalitis: A case series, literature review and future directions. General Hospital Psychiatry, 36(4), 388–391.
Liu, X., Zhang, L., Chen, C., Gong, X., Lin, J., An, D., … Hong, Z. (2019). Long‐term cognitive and neuropsychiatric outcomes in patients with anti‐NMDAR encephalitis. Acta Neurologica Scandinavica, 140(6), 414–421.
Yang, P., Li, L., Xia, S., Zhou, B., Zhu, Y., Zhou, G., … Li, F. (2019). Effect of clozapine on anti-N-methyl-D-aspartate receptor encephalitis with psychiatric symptoms: A series of three cases. Frontier 13(315)
Basheer, S., Nagappa, M., Mahadevan, A., Bindu, P. S., Taly, A. B., & Girimaji, S. C. (2017). Neuropsychiatric Manifestations of Pediatric NMDA Receptor Autoimmune Encephalitis. The Primary Care Companion For CNS Disorders, 19(04), 0–0.
Your generous Donationsallow IAES to continue our important work and save lives!
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.
For this interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.
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
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 DadWillard Wagner
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’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.
Lawyers 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.
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.
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.
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
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, “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: 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 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, 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 Donationsallow 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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Little things, just little things were first noticed by our immediate family. The need to recite over and over the details of how a decision was made, excessive talkativeness, repetitive conversations. Outbursts of anger over inconsequential events, vocalizations of “yep, yep, yep” and a stutter-step when that happened. Something was not right. Periodic feelings of having a “brain spasm” were added to the mix.
Visits to our personal care physician, referral to a neurologist, MRIs and EEGs all resulted in a conclusion that Jeri was just stressed out. After our son’s wedding, everything would be alright. It wasn’t. The wedding was a big success but for Jeri, concentrating on the many details was difficult, to say the least. Our college friends noticed immediately that something was not right. “She seems to not be herself, she gets angry easily, what’s up?”
Could it be a recurrence of the chronic Lyme disease from 8 years ago? Off to the holistic physician that successfully treated Lyme disease. The approach there was to test for exposure to environmental toxins and Lyme. Blood was drawn and then we left for a couple of weeks to go to our cottage in Canada. In those two weeks, our Canadian friends also came to us and asked if something was going on. The repetitive conversations continued along with withdrawal from group conversation. A couple of times Jeri blanked out for a few seconds and became worried-looking, really concerning our friends.
Back from the cottage the follow up with the Lyme doctor revealed that she was slightly positive on the Lyme test. (Lyme diagnostics is difficult and subjective – a discussion for another time.)That is probably what was going on. Back on antibiotics and several nutritional supplements – for 24 hours. Then our journey really began.
“Mom’s had a seizure, I’m following the ambulance to the hospital.” Our two adult sons living at home had found Jeri having a seizure shortly after going to bed. Another seizure while in the ER. The workup revealed that her sodium levels were dangerously low. Correction of the hyponatremia began along with MRIs and other scans. Five days as an inpatient to correct the sodium levels and all tests came back negative. No infectious causes, no tumors, must just be a case of spontaneous SAIDH (Syndrome of Inappropriate Antidiuretic Hormone Secretion). It happens, that can be corrected with diuretics and salt pills. Take anti-seizure medicine and follow up with a neurologist.
At this point, the reader needs to know a little about our family. For years Jeri was a researcher in a biochemistry lab and up to a couple of years ago, a practice manager for a busy physician’s office. I’m a Ph.D. biochemist and molecular biologist. One son is a newly trained nurse and one daughter-in-law is a very experienced pediatric intensive care nurse. Jeri’s brother-in-law is an attorney with over 20 years’ experience in large pharma oncology and has a Masters in chemistry. To say that there were many opinions on what was going on is the understatement of the year. A lot of family knowledge and experience were being tapped, all out of concern, love, and with the best of intentions. Conversations with physician friends and nurses suggested a myriad of causes. None of us got even close to the cause.
The ‘spells’, absent episodes, memory issues, and cognitive decline all continue and increase in frequency. We were home for a week and then another big seizure puts her back in the ER and again admitted to the hospital. Again, all scans and EEG are normal. The doctors in the regional hospital are again scratching their heads. Despite repeated requests, the hospital physicians and our personal neurologist resisted performing a lumbar puncture. Why? We were told there was simply no indication that would justify such a high-risk procedure. Must be the SIADH. Go back to your local neurologist and nephrologist.
A 72 hour EEG was ordered. This was stopped after 24 hours since we noted so many (~30) ‘spells’ and no further recording was needed. Like two spot EEGs before, nothing showed up. The local neurologist was stumped, calling it a real mystery. We again ask for an LP and testing but were refused on the basis that he didn’t know what to test for. As a shot in the dark, testing was ordered for serum anti-NMDA receptor. Negative results. Sodium levels stay a bit low, but near normal. Frustration all around.
Escalating the case was now a family priority but we were stymied by the long wait (2 months!) to get into the UPenn neurology group. A week after the last neurology visit another seizure. A couple of days later we hear a crash and see Jeri at the bottom of the stairs having a seizure, this time with a gash to the head. Back to the ER after a very irrational and angry outburst. Another seizure in the ER and another admittance to the local hospital. The next morning during rounds the floor neurologist is in the room and the hospitalist (physician in charge of cases on that floor) pops in to see how things are going. She asks the neurologist if she is going to order an LP and the neurologist says no, there is no need. The matter is dropped. No words can describe our frustration and helplessness at this point. My son and I discussed the situation and decided to go to the hospitalist and ask for her intervention in getting the case moved to UPenn. She agreed but was doubtful that she could get her transferred. To her and our surprise, UPenn neurology agreed right away!
We were just so lucky to have the case taken over by UPenn and a group of physicians that have been integral to the discovery and treatment of autoimmune encephalopathies. Within a week we had a diagnosis of anti-LGI-1 autoimmune disease and a week after that we began immunosuppression therapy via a five-day course of steroid infusions and an infusion of Rituximab.
We’ve been home a week, continuing anti-seizure medicine, oral steroids, salt supplementation, and diuretics. No seizures so far! We are seeing small improvements in cognitive ability and memory. We have a long way to go but are hopeful. The second infusion of Rituximab will happen in about a week.
Lessons learned:
Unfortunately, I think we can consider that knowledge of auto-immune encephalopathies is pretty much non-existent to the average physician and even most neurologists. The resistance to exploit all avenues of testing after repeated presentations to the ER and negative results of conventional diagnostics needs to change. That change will only come with physician education. Also, symptoms can be very, very subtle at first and not consistent from person to person. Like a lot of other neurological conditions, the symptoms are common to many different causes.
Now that the mystery is solved the recovery can begin. Another Zebra identified.
Become an Advocate by sharing your story. It may result in an accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
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:
Venkatesan, A. & Adatia, K. Anti-NMDA-Receptor Encephalitis: From Bench to Clinic. ACS Chem. Neurosci.8, 2586–2595 (2017).
Dalmau, J. NMDA receptor encephalitis and other antibody-mediated disorders of the synapse: The 2016 Cotzias Lecture. Neurology87, 2471–2482 (2016).
Dalmau, J. et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol.7, 1091–1098 (2008).
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 toIAES@autoimmune-encephalitis.org
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.
What 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.
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.
Your generous Donationsallow IAES to continue our important work and save lives!
Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
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
(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.
Become an Advocate by sharing your story. It may result in an accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org
It’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.
For 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.
I 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 Donationsallow 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
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 likehow 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 WhenYour 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!
Your generous Donationsallow 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
Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society cannot provide medical advice.
International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.
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