Cognition in autoimmune encephalitis

by Alanna | Aug 30, 2022 | Australian Autoimmune Encephalitis Consortium

August 31, 2022 | Written by Sarah Griffith. Edited by Dr Mastura Monif, Ms Tiffany Rushen, Dr Loretta Piccenna, 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 how autoimmune encephalitis can affect cognitive abilities. 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. This blog has been facilitated by IAES Support Services coordinator Mari Wagner Davis, with input from IAES volunteers Sasha Ermichina (impacted by GFAP AE) and Amanda Wells (caregiver for her daughter with AE). These IAES representatives provide input from their unique perspectives, helping to educate researchers in the difficulties that patients and families face.

You can find out more about the Australian Autoimmune Encephalitis Consortium and their efforts to help those with AE and their families via the following link:

https://www.monash.edu/medicine/autoimmune-encephalitis

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Cognition in Autoimmune Encephalitis – Summary

Source – Griffith, S., Wesselingh, R., Broadley, J., O’Shea, M., Kyndt, C., Meade, C., Long, B., Seneviratne, U., Reidy, N., Bourke, R., Buzzard, K., D’Souza, W., Macdonell, R., Brodtmann, A., Butzkueven, H., O’Brien, T. J., Alpitsis, R., Malpas, C. B., Monif, M., & Australian Autoimmune Encephalitis Consortium (2022). Psychometric deficits in autoimmune encephalitis: A retrospective study from the Australian Autoimmune Encephalitis Consortium. European journal of neurology, 10.1111/ene.15367. Advance online publication. https://doi.org/10.1111/ene.15367

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Background

Autoimmune Encephalitis is a rare disease that affects different regions of the brain. Patients with this condition can have a variety of cognitive symptoms (for example, memory deficits, slow speed of information processing, attention lapses, word finding difficulties, trouble with following complex commands, difficulties in judgement, difficulties in comprehension of complex tasks, difficulties in forming new memories, and difficulties in understanding tasks or situations). These symptoms can change from one individual to another individual, and can fluctuate over the course of the illness. Also different types of autoimmune encephalitis can have differing presentation of the above symptoms.

Historically (and to this day) clinicians and researchers have used a scale called the modified Rankin Score as a tool to monitor an individual’s function – i.e. how independently they can function in their day to day life. However, this scale is very inaccurate and it does not capture complex and specific issues that might be troublesome for the patient. The modified Rankin Scale also does not reflect the gravity of the individual’s symptoms and does not provide in detail information about specific symptoms and complaints that the individual with autoimmune encephalitis might be experiencing. Importantly, the scale does not measure behavioural, mood or cognitive outcomes in autoimmune encephalitis. Cognitive changes can be associated with long term disease related morbidity and can reduce quality of life. Therefore we set out to gain a better understanding of the cognitive difficulties in autoimmune encephalitis.

What did the researchers do?

We gathered cognitive data from patients previously diagnosed with autoimmune encephalitis (retrospective data) from six hospitals in Victoria (Australia) to inform the analysis. Patients were identified retrospectively through medical records with a search for diagnosis of autoimmune encephalitis with a hospital admission between July 2008 and July 2019. Patients who met this criteria participated in a neuropsychology assessment of their cognitive function (i.e., memory, attention, language, judgement, planning, comprehension, and recall. We collected clinical data about each patient and also information of various clinical investigations (i.e. lumbar puncture results and MRI).

What did the researchers find?

The average age of patients at diagnosis was 49 years old and more than half showing as seropositive autoimmune encephalitis (meaning they had identifiable antibodies in their blood or their cerebrospinal fluid). Of the seropositive group:

nine patients had anti-NMDAR antibodies,
nine anti-Leucine-rich glioma-inactivated 1 antibodies (LGI-1),
seven Voltage-gated potassium channel complex antibodies (unspecified) (VGKC),
three glutamic acid decarboxylase 65- (GAD65) one α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor antibodies (AMPA),
one γ-aminobutyric acid B antibodies (GABA-Band), and
one collapsin response mediator protein 5 antibodies (CRMP5).

Forty two percent of patients had impairments on executive function tests (e.g. a set of tasks that includes working memory, planning, flexible thinking, and ability to remember multiple instructions, and capacity for self-regulation and ordering or prioritizing of tasks). The next most common impairment was on memory tests (e.g. the mental processes involved in acquiring, retaining and recalling information; 40.7% of patients).

For the first-time, we found 29 patterns of cognition among patients with autoimmune encephalitis in our analysis. The four most common patterns of cognition were:

Intact cognition (no cognitive deficits elicited),
Isolated memory deficits,
Executive dysfunction with memory impairment (combination pattern), and
Isolated visuospatial/visuoconstructional impairments (referring to visual or spatial perception of objects.

But, given we found 29 patterns means that cognitive outcomes in patients with autoimmune encephalitis are complex and need further detailed investigation.

What do these findings mean?

Our research highlighted that more detailed and systematic analysis of memory and executive function profiles in patients with autoimmune encephalitis is required. Impairments in memory and executive dysfunction can have huge implications for the patient and their caregivers, and tools that would better characterise and follow these symptoms in autoimmune encephalitis are needed. Understanding memory and executive function impairment in autoimmune encephalitis can help us in devising strategies that would assist patients with day to day function, but also monitor disease trajectory over time and delineate patient’s response to treatment.

We could not predict good cognitive outcome (e.g. having ‘intact’ cognition after autoimmune encephalitis) in patients. We recommend clinicians provide ongoing comprehensive cognitive monitoring in patients with autoimmune encephalitis, and reactive intervention when required. An individualised approach will assist in the management the long-term morbidity of this disease, to minimise the effect on the individual’s quality of life and any damaging psychological outcomes.

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To download a plain language PDF of the paper summarized in this blog, click the button below:

Download Plain Language Summary

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Your generous Donations allow 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.

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

why zebra - Aphasia as a Symptom of Autoimmune Encephalitis

My Journey to Raising Awareness for Anti-NMDAr Autoimmune Encephalitis

by Alanna | Aug 23, 2022 | AE Warrior Personal Stories

June 23, 2022 | By Reyna Felix

Introduction from the IAES Blog Team:

It is with great pleasure that IAES presents the story of one of our mighty Warriors. Reyna Felix, in her own words, explains the events that finally lead to her diagnosis during the beginning of a worldwide pandemic that left her alone without family and friends by her side at the very beginning of her AE journey. Her story was picked up by a few TV news channels and lead to an article written about her struggles in the Barrow Neurological Foundation newsletter. The link to this article is below. The Barrow Neurological Foundation strives to advance neurological research, patient care and provide education to help save lives.

Read Barrow Neurological Foundation Article

My name is Reyna and I am 29 years old. I have been married for 5 years (together for 11), I have worked as a 911 dispatcher for the past 7 years, and I am a dog mom to a fun rescue pup. I love to read, hike, exercise, travel, cook, and learn new things.

I am also a survivor of anti-NMDA receptor Autoimmune Encephalitis. Like most patients, my diagnosis was not easily found. The search for what was wrong with me included weeks of struggle for myself and my family. As you’ll read about in the article, I was dismissed from hospitals or left against medical advice. I spent time in a psychiatric facility, and eventually was correctly diagnosed and began the treatment process which led into the recovery process I am now in. I am missing many months of life from my memory, which is a common symptom of this disease. I received my diagnosis in April 2020 which was at the same time as a pandemic began around the world. This compounded the struggles experienced by my husband and family. Hospitals had restrictions on people accompanying patients in emergency rooms, ICUs, rehabilitation facilities, etc. and I was in no condition to be responsible for myself during the worst of my symptoms. There was about a 40-day period where my husband couldn’t be with me in person which increased the emotional stress of my condition for him. A teratoma was located and removed, I received a few IVIG treatments, and then I received two weeks’ worth of everyday Plasmapheresis which significantly improved my condition. It was during this time that I began to “wake up.” I spent two weeks becoming more alert, learning how to eat on my own, write, speak, walk, etc. during inpatient neurological rehab. I was discharged to return home with restrictions such as 24/7 supervision and outpatient therapies such as speech, physical, and occupational therapy that continued for 3 months. In June 2020, I started rituximab treatments that I continue to receive twice a year.

I continue to work on my mental and physical health, and I receive testing to ensure I am doing well by my doctors such as neuropsychological tests and cancer screenings. In September 2020, a local news channel picked up my story and did a short interview about Autoimmune Encephalitis which led to another news channel completing a story on me for my first World Encephalitis Day in February 2021. My neurologist shared with me that these two stories were shared amongst other doctors and hospitals and contributed to more awareness and for clinical studies needing to take place. More studies and awareness for this condition means that, someday, people will be diagnosed and treated properly which will lead to better outcomes for more people. I am not someone who enjoys attention. I used to keep my life private but all I hope for from opening up about my experience is to help others get proper treatment, raise awareness for medical professionals and other people to understand more about the disease, and to show other patients and families that successful recovery is possible.

Autoimmune Encephalitis came into my life swiftly and had the potential to destroy it, but it has not won. My life looks differently now but I am alive. I have bad days and good days, but life is full of ups and downs that we can move forward through. While a lot of our symptoms and experiences can be similar, I think it’s important to remember that we are each individual so it’s also good to remind yourself to not compare your own personal progress and recovery to other patients.

To all my fellow warriors, we’ve got this. You are loved, you are strong, and you deserve to be happy and alive. To all our caregivers, families, friends, medical professionals, etc., thank you for what you do to help us each day. Additionally, I hold those who have passed from Autoimmune Encephalitis and their families close in my heart.

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

Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.

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

Using Electroencephalogram for quicker diagnosis and prediction of the likely course for patients with Autoimmune Encephalitis

by Alanna | Jul 26, 2022 | Australian Autoimmune Encephalitis Consortium

July 27, 2022 | by Dr. Robb Wesselingh

A message from IAES Blog Staff:

It is our honor & pleasure to present to all of you an overview of the use of an Electroencephalogram or EEG for diagnosis and prediction in the treatment of Autoimmune Encephalitis by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif. The International Autoimmune Encephalitis Society is proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project. Dr. Monif is on the board of directors for IAES and we work closely with them to best support AE patients, caregivers and their families. You can find out more about the team and their efforts to help those with AE and their families via the following link:

https://www.monash.edu/medicine/autoimmune-encephalitis

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Using Electroencephalogram for quicker diagnosis and prediction of the likely course for patients with Autoimmune Encephalitis

Publication:

R Wesselingh, J Broadley, K Buzzard, D Tarlinton, U Seneviratne, C Kyndt, J Stankovich, P Sanfilippo, C Nesbitt, W D’Souza, R Macdonell, H Butzkueven, TJ O’Brien, M Monif, Electroclinical biomarkers of autoimmune encephalitis, Epilepsy & Behaviour, 2022;128: 108571. https://doi.org/10.1016/j.yebeh.2022.108571

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Autoimmune encephalitis (AE) is a brain inflammation disorder caused by antibodies. A person’s immune system mistakenly targets different proteins in their brain causing damage and inflammation. This can result in different neurological symptoms including seizures (sudden, uncontrolled electrical disturbances in the brain) and memory problems. Autoimmune encephalitis can be classified into different subtypes based on the brain protein targeted by the antibodies produced. The most common subtypes are anti-NMDAR autoimmune encephalitis, anti-LGI-1 autoimmune encephalitis and seronegative autoimmune encephalitis (in which there is no identified antibody). While treatment is effective and available, the diagnosis of autoimmune encephalitis is not straightforward. Also, knowing which patients need more intensive treatment is tricky.

Patients thought to have autoimmune encephalitis usually have a few clinical tests to confirm the diagnosis. They include brain magnetic resonance imaging (MRI), an electroencephalogram (EEG), and blood or cerebrospinal fluid tests to analyse the presence of inflammation. The EEG is a procedure that measures brain electrical activity (brain waves) by using electrodes placed on the scalp. It can show different patterns or irregularities depending on the person’s health state. For example, an EEG can show seizure activity, or it can indicate drowsy or comatose states. In some situations, it can also show very subtle changes that could be useful in our understanding of autoimmune encephalitis and guiding management. It is important for patients with suspected autoimmune encephalitis to have a diagnosis as soon as possible because earlier treatment leads to better long-term recovery. But doing multiple clinical tests takes time, some can be invasive or may only be available in certain centres. For this research, we wanted to find out answers to following –

Can we use an EEG to identify different types of Autoimmune Encephalitis?
In the early part the disorder, can the EEG tell us about a person’s likely course in the long-term (outcomes)?

How we did this work

We looked through the medical records of seven hospitals in Victoria, Australia for people who had possible autoimmune encephalitis and had an EEG when they first became unwell. Overall, 208 patients were identified and selected for our analysis. We collected data from 131 patients of their symptoms, seizures, treatment, and their ability to return to normal day-to-day living. Key clinical characteristics of the patients can be seen below:

We analysed EEGs from patients to find any brain wave irregularities or signatures (called biomarkers) that would show different subtypes of autoimmune encephalitis. Other EEGs were analysed that could predict which patients might have impaired functional outcomes in the long term.

What were the interesting things we found

We identified four specific brain wave signatures or biomarkers that were associated with one type of autoimmune encephalitis called anti-NMDAR autoimmune encephalitis.

We also found a disruption of the normal electrical activity of the brain that was more common in patients who had significant functional disability on discharge from hospital.

Large spikes of abnormal electrical activity called periodic discharges were seen in patients who ended up having long-term impacts on their day-to-day functioning.

What do these findings mean?

The brain wave signatures or biomarkers we identified can be useful for clinicians to recognise and use in practice as part of diagnosis and provide targeted treatment.

The research could help clinicians to –

More quickly identify the type of autoimmune encephalitis a patient has and provide a specific treatment strategy, and

Recognise patients with autoimmune encephalitis who are likely to have more long-term functional disability due to their illness.

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For more information and resources on anti-NMDAr encephalitis, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:

Download Plain Language Summary

Click here or the image below to subscribe to our mailing list:

Your generous Donations allow 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.

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

A Happy Ending for Surprise Diagnosis for One Doctor by Another Doctor from a Land Far Far Away

by Alanna | Jun 21, 2022 | AE Warrior Personal Stories

June 23, 2022 | By Mozna Osman

Introduction from the IAES Blog Team:

IAES is proud to present to you the AE story of a doctor that truly exemplifies our international impact! We hope you find this as inspirational as we have.

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I am a family medical doctor living and practicing medicine in Khartoum, Sudan. My story began about three years ago when I realized I was often feeling very tired and had terrible headaches. I decided to go and get my own blood work analyzed to see if there were any obvious issues going on. What I found out was that I had Malaria and a UTI (urinary tract infection). As a doctor, I decided to go ahead and treat myself with the appropriate medications needed for both issues.

I thought I was doing well and, on the mend, but I was not. Not long after this, my son found me, unresponsive, on the floor. I was admitted into the intensive care unit (ICU) of a local hospital and was in a coma for a month. As luck would have it, a visiting doctor from Chicago was asked to review my case. After reviewing my medical chart, he diagnosed me with anti-NMDA Autoimmune Encephalitis. He suggested a course of treatment that included medication and plasmapheresis. I feel very fortunate and blessed to have had my case reviewed by a doctor from so far away.

I am now feeling much but better but I still suffer from memory issues like many patients with AE. My short memory seems more affected by AE than my long-term memory but is getting much better. I am happy to report I am back to practicing medicine and am working at AL-Shaheed Wedatallah Medical Center in Khartoum. I am hoping for a bright, happy, and healthy future.

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

Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.

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

Psychosis or Something More? A Family’s Search for Answers

by Alanna | May 25, 2022 | AE Warrior Personal Stories

May 26, 2022 | By Libya Matney

Introduction from the IAES Blog Team:

The International Autoimmune Encephalitis Society is proud to present to you the AE story of one of our mighty Warriors in her own words, an article written about her AE path in the University of Arkansas newspaper (link below) and beautiful artwork given to her by a wonderful friend and artist depicting her within the struggle of this devastating diagnosis!

As a 21-year-old stay at home mom to a precious little boy, Benjamin, I never thought that something so life-altering would happen to me. My husband and I had spent 9 months trying to conceive our second child. Finally, in June of 2021, we were able to get pregnant. Around the same time, many random things started happening to my body. Nobody understood what was happening. My scalp was burning, I had chronic migraines, confusion, insomnia, OCD tendencies, and I started showing aggression towards my family. All these things seemed to begin to rule my life. I began cleaning my house aggressively and trying to get rid of everything that I own. I would tear things out of the closets and try to reorganize them at the same time. I was exhausted. I was forgetting everything. I began to believe that I couldn’t be left alone to take care of my son. My family and friends began taking turns staying with me during the day. At this point I had been to the doctor several times and had multiple blood tests and two CT scans. Everything came back normal.

On August 19th, 2021, my husband and mother-in-law decided to take me to a hospital in Fayetteville, Arkansas (AR) to see if they could figure out what was happening to me. They performed the same blood tests and scans that I had received previously and, of course, they came back normal. On the way home from the hospital that day, I had what the doctors would call a “psychotic break”. I began kicking and hitting my husband in the car and trying to open the door to get out. My husband and mother-in-law frantically called my mom. She said that she could hear my husband in the background begging me to stop. My mom told them that she was on her way to us and to call 911. When the police and Emergency Medical Services (EMS) arrived, my mom had my doctor on the phone to convince them that something was medically wrong with me and that I needed to be taken to the emergency room (ER). When EMS tried to get me to the gurney I dropped to the ground. They had to lift me onto the gurney and into the ambulance. This was when I was transported to the ER in my hometown, North Arkansas Regional Medical Center (NARMC). The last thing I remember from that fateful day was stopping in a restaurant parking lot and trying to get out of the car. I don’t remember the police or paramedics being there. I don’t remember anything from the rest of that day or the month that followed.

My family has told me that while I was in the ER, I kept trying to leave. I would fight the medical staff and my family so hard that they had to restrain my arms and legs to keep me from getting out of bed or hurting myself. During the 4 days that I was in the ER at NARMC, I had a CT scan, an MRI, a spinal tap, and multiple blood tests done. I ended up miscarrying my child the last day that I was there. I was transferred to St. Bernard’s Medical Center in Jonesboro, AR on August 23rd, 2021. During the transfer, I slipped into an unresponsive/catatonic state. A procedure was performed to remove the tissue from the fetus, as well as the same testing that had previously been done in the ER. Other testing that was done consisted of EEGs and ultrasounds. Six days after being transferred I was placed on a ventilator because of having back-to-back seizures and my heart stopping. After two weeks of having no nourishment besides IV fluids, a feeding tube was also placed in my nose. I was in St. Bernard’s for a month before they sent my blood and spinal fluid to Mayo Clinic. An infectious disease doctor diagnosed me with anti-NMDA Receptor Autoimmune Encephalitis and GFAP. A few days before I left St. Bernard’s, I woke up with a feeding tube still in my nose. I did not know the month, day, or what was happening. I couldn’t walk or use the right upper side of my body. Also, due to having been on the ventilator and having the feeding tube, I could not talk. I was given a letterboard to communicate and the first thing that I asked about was my pregnancy. I couldn’t remember miscarrying the baby. On September 22nd, I was transferred to Barnes Jewish Hospital in St. Louis, Mo. Doctors began plasmapheresis (plasma exchange). During the 10 days that I was there I received 5 rounds of it. I had another MRI, EEG, spinal tap, and two ultrasounds during my stay there. Blood and spinal fluid samples were sent to Mayo Clinic to be rechecked. On October 1st, I was moved to Everest Rehabilitation Center in Rogers, AR, where I spent every day in physical, occupational, and speech therapy. I had to relearn how to walk, and I had to regain my physical strength and my voice. My last week at the rehab center, I finally got to see my son, Benjamin, after not seeing him for over a month. They included him in my physical and occupational therapy. On October 19th, two months after being taken to the first hospital, I was released to finally go home. When I got home, I started outpatient physical therapy at Mount Carmel Physical Therapy Center in Harrison, AR. I had physical therapy twice a week for 8 weeks. In January of 2022, I had two rounds of Rituxan infusions.

I am now back home with husband and son, and I can enjoy spending time with my family and friends once again. My life will never be the same after my AE diagnosis. I will always have to keep tabs on my stress levels and watch for signs of relapse. I still have months before I can drive again and am still at risk for seizures. However, I have learned a lot the past year. I’ve been cared for by people that will forever hold a place in my heart. I’ve grown, I’ve changed, and I am thankful for the life and family that God has blessed me with.

Click here or the image below to subscribe to our mailing list :

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

Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.

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

An overview of N-methyl D-aspartate receptor (NMDAR) antibody-associated encephalitis

by Alanna | May 10, 2022 | Australian Autoimmune Encephalitis Consortium

May 11, 2022 | by Seery N, Butzkueven H, O’Brien TJ, Monif M.

A message from IAES Blog Staff:

It is our honor & pleasure to present to all of you an overview of anti-NMDAR Autoimmune Encephalitis by the esteemed team at Monash University in Australia & lead by Dr. Mastura Monif. The International Autoimmune Encephalitis Society is proud to be in collaboration with Dr. Monif and her team in the Australian Autoimmune Encephalitis Consortium Project. Dr. Monif is on the board of directors for IAES and we work closely with them to best support AE patients, caregivers and their families. You can find out more about the team and their efforts to help those with AE and their families via the following link: https://www.monash.edu/medicine/autoimmune-encephalitis

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An overview of N-methyl D-asparate receptor (NMDAR) antibody-associated encephalitis

Contemporary advances in anti-NMDAR antibody (Ab)-mediated encephalitis, Autoimmunity Reviews, April 2022, Volume 21, Issue 4, https://doi.org/10.1016/j.autrev.2022.103057

WHY WE DID THIS WORK:

N-methyl D-aspartate receptor (NMDAR) antibody-associated encephalitis, or anti-NMDAR encephalitis is a type of autoimmune encephalitis. Autoimmune encephalitis occurs when parts of the body’s immune system inappropriately attack certain components of nerve cells in the brain. Anti-NMDAR encephalitis is one of the most common defined types of autoimmune encephalitis. Young people who are on average 21 years of age, and females, experience the illness most commonly. But it can occur in older adults (over 45 years) and in young children (less than 12 years), where a relatively higher number of patients are males.
To make a diagnosis, doctors record key symptoms (e.g. memory issues, personality change, changes to behaviour alteration, or seizures) and clinical features from tests. The detection of antibodies that bind the receptor (NMDAR) from a patient’s blood or cerebrospinal fluid (fluid that cushions the brain and spinal cord) are also used to confirm the diagnosis of anti-NMDAR encephalitis. Antibodies are proteins produced by the immune system that in normal circumstances have a role in fighting infections. In certain autoimmune diseases, antibodies, along with other parts of the immune system, target normal components of a healthy individual.
Anti-NMDAR encephalitis can be treated using medications that selectively reduce components of the immune system, but as the illness is different from person to person, it is challenging to both diagnose and manage. The use of biological markers, or biomarkers (proteins, cells or other characteristics that can generally be detected via a certain test and are association with a particular disease), may be particularly useful for the diagnosis and assessment of how effective treatment is to manage a patient’s illness. Accurate and specific biomarkers for anti-NMDAR encephalitis still do not exist.
We did this update to collect the latest research findings from the field to inform researchers and clinicians who manage this condition and have a keen interest.

WHAT WERE THE THINGS WE DISCOVERED?

The N-methyl D-aspartate receptor (NMDAR) belongs to a group called glutamate-gated ionotropic receptors. These receptors are mostly located in a region of the brain called the hippocampus more so than other regions, and assist in excitatory transmission of nerve cells. In anti-NMDAR encephalitis, antibodies target a part of the receptor called GluN1.Anti-NMDAR antibodies exist in the serum part of blood and the cerebrospinal fluid.
Biomarkers in cerebrospinal fluid that have been identified include B-cell and T-cell chemokines, which are molecules that serve to attract particular white cells to a given part of the body (in particular, CXCL13 and CXCL10), interferon gamma, tumor necrosis factor alpha and interleukins (in particular 6, 7, 10 and 17-A). The levels of certain of these biomarkers were found to also coincide with poor long-term outcomes in patients.

TRIGGERS OF ANTI-NMDAR ENCEPHALITIS:

Two known triggers for anti-NMDAR encephalitis are the presence of an ovarian teratoma (an unusual type of tumour that can express nervous tissue) and less commonly, a viral infection of the brain called HSV-encephalitis.
Other than these two triggers it is less understood what causes the illness. At a molecular level, patient antibodies induce complex changes to the nerves by disrupting the functioning of the receptor. These findings may explain the variety of symptoms that patients experience, and varying and often impressive degrees of recovery.

CLINICAL FEATURES:

The diagnosis of the disease is not always straightforward. Symptoms and other clinical features may overlap with other diseases, and sometimes, at least in the blood, antibodies to the receptor may be seen without the disease itself. A broad range of clinical features exists in anti-NMDAR encephalitis. Patients can experience the following, which may change over time –
- - Changes in behaviour and hallucinations (psychiatric symptoms)
  - Difficulties in mental function (cognitive impairment)
  - Seizures
  - Abnormal movements and
  - Irregularities of the function of the autonomic nervous system (e.g. temperature, heart rate and breathing).
Therefore, detection of certain proteins and molecules in the blood or spinal fluid may assist in the diagnosis of the disease, and its management. A variety of candidates have been explored, but so far, generally remain limited in their use in clinical practice.

TREATMENT:

The cornerstone of treatment is suppression of the immune system. Research has shown that earlier treatment results in a better long-term outcome.
Exciting new treatment options are emerging, but research studies for these treatments are limited to relatively small numbers of patients and their approval for use by government therapeutic agencies is still awaiting. Therefore, we advise clinicians making the decision to use new therapies be made on based on each individual patient’s situation.

WHAT DO THE FINDINGS MEAN?

This research can help clinicians understand how the illness progresses at the cellular and molecular level, its symptoms, the results of supportive tests (e.g. spinal fluid and MRI) and treatment options, and highlight areas of progress and needs in the current research.
Most patients will have some degree of recovery in their illness following appropriate treatment, but some symptoms can persist, particularly issues in mental function. Cognitive rehabilitation and follow up with psychiatry and psychology and in some cases antipsychotic medication, anti-depressants and mood stabilizers maybe warranted.

monif group - An overview of N-methyl D-aspartate receptor (NMDAR) antibody-associated encephalitis

2021 Monif group L-R – Paul Sanfilippo, Nabil Seery, Katrina Kan, Robb Wesselingh, William O’Brien, Tiffany Rushen, Mastura Monif (Group Leader), Tracie Tan, Sher Chim Ting, Sarah Griffith, Andrea Muscat.

For more information and resources on anti-NMDAr encephalitis, visit this link here. To download a plain language PDF of the paper summarized in this blog, click the button below:

Download Plain Language Summary

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Your generous Donations allow 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.

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

Falling through the looking glass…

by Alanna | Mar 8, 2022 | AE Warrior Personal Stories

March 9, 2022 | by Rachael Muggleton

In May of 2020, I was a healthy, vibrant, smart, loving, pre-med student at Penn State, nicknamed the ‘mayor’ of my large friend network … until I fell acutely ill. Within 7 days of hospital admission, for what seemed like some type of stress induced altered mental status, I became catatonic. My mom watched in horror as her daughter’s light rapidly dimmed; losing a piece of what defined me each day. By day 7, I no longer spoke or appeared to recognize anyone, my heart rate became unstable, I could no longer walk or eat, and my kidneys began to fail. I was rushed to the University of Rochester Medical Center (URMC) with a preliminary diagnosis of Anti-NMDAr Autoimmune Encephalitis (AE) – an illness my family had never heard of, but one unfortunately they would get to know very well over the next six months of my horrific journey.

Full disclosure — this part of my story comes from my mom; I don’t remember anything from my admission until fuzzy memories of my time in inpatient rehab 5 months later. By the time I reached URMC I was catatonic, seizures were starting to take over. My mother described the surreal experience as ‘seeing our beautiful Rachie rapidly falling through the AE ‘looking glass’ – a tumbling, bouncing, free fall which lasted for days, weeks, eventually resulting with me being placed on life support for months’.

Timing is everything, right?! It was during COVID lockdown, visitation was not allowed. My family was no longer able to be by my side. For 42 days, physicians and nurses cared for me while in a medically induced coma; trying to quell the relentless seizures. My mom sat on the virtual sidelines, distraught, making decisions regarding PIC lines, arterial lines, sedation, feeding tubes and a tracheostomy – decisions she never imagined she’d be making for her child, who outside of a tonsillectomy, was the picture of health. Without the communication, compassion, trust and love I received from the URMC team, my mother said she wasn’t so sure she would have been able to get through those first 42 days. They were her eyes, ears, and most importantly, heart.

When restrictions finally lifted and my parents were able to be by my side, they witnessed firsthand what a cruel, relentless illness AE was. There is no official playbook. My mom, a person who prefers order and predictability, was at a loss watching the team trying to control an illness that twisted and turned, never letting up. AE laughed at us and didn’t care how desperately we wanted it to release its grip. During this acute phase, one of my Neuro-intensivists explained his view on Anti-NMDAr AE (after treating a handful of cases each year) to my mom – ‘It comes out of nowhere, attacks HARD, stays as long as it pleases, eventually leaves, rarely returns. Our job is to treat Rachael with what we know works and keep her alive until it leaves”, and they did just that. I received steroids, plasma pheresis, IVIG, Rituxan and when things still seemed to be stalled, Cytoxan. I was also placed on a strict KETO diet, which in some pediatric patients has been proven effective in reducing/eliminating certain type of seizure activity.

By mid-August of 2020, my mom tells me, “Our ‘Alice’ ended her free fall and SLOWLY started to reemerge!”. Day by day, small pieces of me started coming back. Eventually, the ICU staff took me out to the children’s garden at URMC (safety protocols in place (!)) to see my brother Andrew for the first time in 4 months (pictured)!

My family and team celebrated each breath on my own, swallow of water, baby step, new word, and smile! When it was finally time to leave my nurturing Neuro-ICU ‘nest’, the team through me a dance party — sending me off to inpatient rehab in style! Since I have no memory of that time, I’m thankful it was recorded! Taylor Swift’s ‘Shake it Up’ will forever rock as my survival song 😊!

IMG 5135 300x225 - Falling through the looking glass...

My dance party send off!

I was released from the hospital in October 2020, just short of 6 months. While my journey was far from over, I was on my way! I still needed to be weaned (carefully) from 8 different anti-seizure medications, regain my cognitive functions and physical strength and dexterity. Through the work of an amazing rehab team and sheer determination, I made my way back. By the summer of 2021, I was taking college calculus II and preparing for my college return in the fall.

But here’s the best part of my story! In December 2021, I spent a week in the URMC Neuro-ICU, only this time NOT as a patient but rather to shadow the amazing physicians, nurses and professionals to learn firsthand the world of intensive care neurology. As a result of this shadowing, I’m sure they hope I choose Neurology as my specialty (maybe?!), but FAR more was gained from this experience! Rarely do providers, nurses, and staff get to see the results of their amazing efforts, and a recovered AE patient rarely gets to come back to see their heroes in action — Life came full circle.

I finished my fall semester (Deans List!) and as I write this, and I’ve returned for my final semester at Penn State. I’ll begin studying for the MCATS with the goal of medical school in 2023 (maybe URMC? 😊). Autoimmune Encephalitis was a cruel thief who tried to steal me and subsequently a year of my life, but as the Mad Hatter said to Alice, ‘If you knew time as well as I do, you wouldn’t talk about wasting it’. I’m not about to waste it!!

My mom reminds me often; I have a story to tell! I think it’s a story of a young woman falling through the looking glass — finding beauty, love, compassion, and competence all along her journey. However, her name isn’t Alice, it’s Rachael.

Never. Lose. Hope ❤️.

August 2021, me, mom, and stepsister Kaitlyn

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

Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.

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

Amy’s Resilience

by Alanna | Mar 9, 2021 | AE Warrior Personal Stories

March 10, 2021 | By Amy Underwood-Crossley

Message from the IAES blog staff:

We are proud to bring to you the story of an AE Warrior and her journey thus far. Here is to an unlikely diagnosis path and those that have stood by her side!

—-

My AE journey began quite unexpectedly in 2015! I was attempting to see a doctor for a consultation for spine issues at the Cleveland Clinic. All scans, x-rays, MRIs etc. were sent there for the consult from Ohio State’s Wexner Medical Center.

The surgeon at the Cleveland Clinic quickly told me he could not fix my spine, but he urged me to see a neurologist as he suspected something else was going on in my body. Something that required many tubes of blood and a lumbar puncture. Quickly, it was determined I have NMDA Autoimmune Encephalitis.

I went thru over a year of treatments that left me still very sick physically and with unrelenting psychiatric symptoms. I, then, contacted folks at IAES who were very helpful in referring me to another specialist. With a new doctor and new treatments, I have had periods of time where my AE seems to be in remission only to rear its ugly head and relapse. I suffer from seizures, tremors, drug induced Parkinson’s disease, mobility issues and lesions in my brain as a result of the AE. I have had many infusions, a complete hysterectomy, ongoing doctor’s visits and, at times, I am better.

Throughout my six-year journey with AE I have had my son and family by my side. My son has been my caretaker. It is an unusual teenage experience for my son, yet he is always here for me. I have hope that by telling my story more people will become aware of AE and I hope over time I will become much better.

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

Become an Advocate by sharing your story. It may result in accurate diagnosis for someone suffering right now who is yet to be correctly identified. Submit your story with two photos to IAES@autoimmune-encephalitis.org

International Autoimmune Encephalitis Society (IAES), home of the AEWarrior®, is the only Family/Patient-centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey. Your donations are greatly appreciated and are the direct result of IAES’ ability to develop the first product in the world to address the needs of patients, Autoimmune Encephalitis Trivia Playing Cards. Every dollar raised allows us to raise awareness and personally help Patients, Families, and Caregivers through their Journey with AE to ensure that the best outcomes can be reached. Your contribution to our mission will help save lives and improve the quality of life for those impacted by AE.

For those interested in face masks, clothing, mugs, and other merchandise, check out our AE Warrior Store! This online shop was born out of the desire for the AE patient to express their personal pride in fighting such a traumatic disease and the natural desire to spread awareness. Join our AE family and help us continue our mission to support patients, families and caregivers while they walk this difficult journey.

Cognition in Autoimmune Encephalitis – Summary

Background

What did the researchers find?

What do these findings mean?

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Using Electroencephalogram for quicker diagnosis and prediction of the likely course for patients with Autoimmune Encephalitis

How we did this work

What were the interesting things we found

What do these findings mean?

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

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

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An overview of N-methyl D-asparate receptor (NMDAR) antibody-associated encephalitis

WHY WE DID THIS WORK:

WHAT WERE THE THINGS WE DISCOVERED?

TRIGGERS OF ANTI-NMDAR ENCEPHALITIS:

CLINICAL FEATURES:

TREATMENT:

WHAT DO THE FINDINGS MEAN?

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

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

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

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