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March 29, 2023 | Written by Dr. Robb Wesselingh. Edited by Dr Mastura Monif, Dr Loretta Piccenna, Ms Tiffany Rushen, Ms Amanda Wells (consumer representative) Ms Sasha Ermichina (consumer representative), and Ms. Michelle Mykytowycz.
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A message from IAES Blog Staff:
It is our honor and pleasure to present to all of you an overview of Peripheral monocytes and soluble biomarkers in 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:
https://www.monash.edu/medicine/autoimmune-encephalitis
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Source: R Wesselingh, S Griffith, J Broadley, D Tarlinton, K Buzzard, U Seneviratne, H Butzkueven, TJ O’Brien, M Monif, Peripheral monocytes and soluble biomarkers in autoimmune encephalitis, Journal of Autoimmunity, 2023; 135 https://doi.org/10.1016/j.jaut.2023.103000
Autoimmune encephalitis (AE) is a condition in which inflammation occurs in various regions of the brain. In AE a person’s immune system produces antibodies (proteins) that mistakenly targets components of the person’s own neurons (nerve structures). This can result in inflammation and nerve tissue damage. As a result, a person with AE can present with different neurological symptoms including seizures (sudden, uncontrolled electrical disturbances in the brain) and memory problems. There are different types of AE based on which protein the immune system is mistakenly targeting. Two of the most common types of AE are:
While we know antibodies play a key role in the disease, we do not know what changes occur in other parts of the immune system during the course of AE.
The innate immune system is a part of the immune system that acts as a broad first line of defence against foreign invaders to the body like viruses and bacteria. This system can often start or increase inflammation in the body as a protective mechanism. Monocytes are a major type of cell in the innate immune system that drive this response. Monocytes can alert and activate other parts of the immune system through release of small signalling proteins. These small signalling proteins can be released into the blood and tissues and are called cytokines. In AE it is unknown whether the innate immune system or monocytes play a role in the disease.
For this research, we set out to find out answers to following –
We recruited 40 people with AE and 28 healthy volunteers who provided blood samples. These blood samples were evaluated in the laboratory for:
These findings were then compared between people with AE and the healthy volunteers to see if there were any differences. We also compared these findings between people with different types and severities of AE.
This research showed that there is ongoing inflammation in the blood of people with AE. Also, monocytes and the innate immune system may play a role in the disease.
The research could help clinicians to –
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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:
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.
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:
https://www.monash.edu/medicine/autoimmune-encephalitis
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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.
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
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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:
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.
October 28, 2022 | Written by Dr. Nabil Seery. 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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Source: Seery N, Butzkueven H, O’Brien TJ, Monif. M. Rare Antibody-Mediated and Seronegative Autoimmune Encephalitis: an Update. Autoimmunity Rev. 2022 May 18;21(7);103118. https://doi.org/10.1016/j.autrev.2022.103118
■ Autoimmune encephalitis (AE) is a form of autoimmune disease whereby immune cells in the body inappropriately target components of the nervous system. This causes dysfunction of nerve cells, and in some cases death of these cells, and further produces different clinical symptoms that are reversible. Such symptoms include (but are not limited to) cognitive symptoms, such as difficulties with memory and language, seizures, movement disorders, and psychiatric symptoms.
■ Antibodies are central to the diagnosis of many subtypes of autoimmune encephalitis. Generally, antibodies are proteins produced by the immune system to fight infections. In a proportion of patients with autoimmune encephalitis there can be an abnormal expression of antibodies, where, rather than targeting foreign molecules (e.g. viruses, bacteria), they mistakenly target self-proteins on nerve endings or self-proteins inside the nerve cell or neuron. In up to half of cases, an antibody is not detectable using current available tests or assays. This group of cases is called “seronegative” autoimmune encephalitis, i.e. denoting a lack of antibodies in the serum (a component of a patient’s blood) or cerebrospinal fluid (a clear fluid the surrounds the brain and spinal cord, obtained via a lumbar puncture, a procedure involving a fine needle being inserted in the lower back). ‘Seronegative’ autoimmune encephalitis most likely represents a broader collection of disorders.
■ Over the last two decades, antibody-mediated subtypes of autoimmune encephalitis continue to be discovered, with over ten such forms now recognised. Further, following the respective discovery of such new forms of autoimmune encephalitis, disease mechanisms and clinical features have been revealed. However, seronegative autoimmune encephalitis remains less well characterised, possibly in part to because of its heterogeneous nature – meaning that a variety of diseases forms may be included by the definition.
■ The purpose of our review was to explore advances regarding five rare antibody-mediated forms of autoimmune encephalitis, namely, anti-g-aminobutyric acid B (GABAB) receptor-, anti-a-amino-3hydroxy-5-methyl-4-isoxazolepropinoic receptor- (AMPAR), anti-GABAA receptor-and anti-dipeptidyl-peptidase-like protein-6 (DPPX) encephalitis and IgLON5 disease.
■ We also summarise current research and challenges in relation to ‘seronegative’ autoimmune encephalitis. For a detailed discussion of anti- NMDA autoimmune encephalitis, anti-LGI1 and anti-CASPR2 autoimmune encephalitis refer to (Contemporary advances in anti-NMDAR antibody (Ab)-mediated encephalitis -PubMed (nih.gov) (1) and Contemporary advances in antibody-mediated encephalitis: anti-LGI1 and anti-Caspr2 antibody (Ab)-mediated encephalitides -PubMed (nih.gov)) (2).
■ GABAB, AMPAR and GABAA autoimmune encephalitis have common and distinguishing clinical features. These three forms of autoimmune encephalitis are diagnosed by the presence of antibodies found in the blood or cerebrospinal fluid of suspected patients. All three are relatively rare, compared to some other antibody-mediated forms of autoimmune encephalitis such as anti-N-methyl-D-aspartate receptor (NMDAR) and anti-leucine-rich gliomainactivated 1 (LGI1) Ab-mediated encephalitis. GABAA encephalitis in particular is exceedingly rare, with approximately fifty cases reported overall as at a few years ago.
■In these diseases, antibodies target the GABAB, AMPAR and GABAA receptors (proteins present on nerve cell endings), causing neuronal dysfunction. GABAB and GABAA receptors both attract an inhibitory neurotransmitter called GABA. A neurotransmitter is a signalling molecule that helps with communication and transmission of impulses between neurons, and inhibitory neurotransmitters reduce the likelihood a given neuron will generate an electrical signal called an action potential.
■ Seizures in these diseases are a main feature, and may be particularly non-responsive to conventional anti-seizure treatment. Furthermore, cognitive and psychiatric symptoms are common in all three of these subtypes of autoimmune encephalitis. GABAB and AMPAR subtypes may have similar findings identified on MRI imaging of the brain, with inflammation and swelling seen in part of the brain called the mesial temporal lobe. The mesial temporal lobe is an area of the brain important for memory, emotion and behaviour.
■ The diagnosis of autoimmune encephalitis invariably necessitates that clinicians investigate for the possibility of a tumour (e.g. lung cancer, thyroid cancer, breast cancer) that may have triggered the disease. Treating the tumour or cancer where feasible and as promptly as possible has been linked to improvements in autoimmune encephalitis symptoms. Similarly, the presence of neurological symptoms, if preceding a cancer diagnosis, may allow for this to be facilitated more quickly than might have been the case otherwise, which may help afford a better chance of more effectively treating the underlying cancer.
■ In approximately half of patients diagnosed with GABAB encephalitis, an underlying tumour is found, most often small-cell lung cancer. In AMPAR encephalitis, almost two-thirds of patients have an underlying tumour, with thymus tumours and lung cancer most common. In GABAA encephalitis, approximately one third of patients have also been shown to have an underlying tumour.
■ DPPX encephalitis and IgLON5 disease are two rare and somewhat clinically unique forms of autoimmune encephalitis. In DPPX encephalitis, patients commonly present with profound weight loss or diarrhoea and have features of central-nervous system hyperexcitability. This is a state where the brain has increased responsiveness to a variety of external stimuli. In DPPX encephalitis, features attributed to CNS hyperexcitability include myoclonus, or rapid, involuntary muscle jerks, and tremor. IgLON5 disease on the other hand also has unique clinical features, such as a variety of sleep disturbances.
■ Seronegative autoimmune encephalitis overall requires further study and description to identify potential antibodies which may be the cause. Seronegative limbic encephalitis is a form of seronegative autoimmune encephalitis, where the limbic structures in the brain are affected. In this subset of the disease inflammation is observed in the mesial temporal lobes using Magnetic Resonance Imaging (MRI). Seronegative limbic encephalitis is typically seen in older patients, with conventional antibody testing not revealing an antibody. Patients typically have memory impairment, with or without psychiatric symptoms and seizures, and are treated with medications that lower effects of the immune system, as in other forms of autoimmune encephalitis.
■ These findings are intended to help researchers and clinicians better understand seronegative and rare forms of autoimmune encephalitis. By bringing this information together, it can assist with improving diagnosis and assisting with early treatment by clinicians.
■ It should be noted that antibody-related forms of autoimmune encephalitis are usually diagnosed as “possible autoimmune encephalitis” prior to the availability of antibody results, which can take up to a period of weeks. A diagnosis of autoimmune encephalitis is based on broad criteria involving consideration of a patient’s symptoms and test results, including MRI, electroencephalogram (EEG – a measure of the electrical activity of the brain) and cerebrospinal fluid biopsy results, combined with the exclusion other diseases, for example, viruses that could mimic the observed symptoms.
■ Prompt diagnosis of autoimmune encephalitis, and prompt exclusion of other causes such as viral encephalitis is very important, as there is a growing body of evidence indicating that earlier initiation of immune-lowering treatment for autoimmune encephalitis may be able to facilitate better recovery.
■ The seronegative form of autoimmune encephalitis can represent a large proportion of autoimmune encephalitis patients overall so its understanding is crucial for improvements in clinical care.
■ Regarding very rare subtypes of autoimmune encephalitis, an understanding of the characteristic features of these rare entities is crucial in forming a diagnostic workup plan. Further, awareness of the features of some of these rarer subtypes can ensure prompt and accurate investigation of underlying tumours. Knowledge of rarer subtypes may also be able to inform clinicians and patients about the possible outcomes of these conditions to inform day to day discussions with patients and their caregivers.
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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:
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.
October 12, 2022 | Written by Dr. Robb Wesselingh. 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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Publication:
Source – Wesselingh, R., Broadley, J., Buzzard, K., Tarlinton, D., Seneviratne, U., Kyndt, C., Stankovich, J., Sanlippo, P., Nesbitt, C., D’Souza, W., Macdonell, R., Butzkueven, H., O’Brien, T. J., & Monif, M. (2022). Prevalence, risk factors, and prognosis of drugresistant epilepsy in autoimmune encephalitis. Epilepsy & behavior: E&B, 132, 108729. Advance online publication. https://doi.org/10.1016/j.yebeh.2022.108729
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Seizures (or sudden, uncontrolled electrical disturbances in the brain) are a common initial neurological symptom that occurs in people with autoimmune encephalitis. In autoimmune encephalitis a person’s immune system mistakenly targets different proteins in their brain causing damage and inflammation. For some people, the seizures can progress to very severe and ongoing seizures called status epilepticus, requiring treatment to stop them happening. While some patients will stop having seizures after immune system suppressing treatment, others will continue to have seizures that do not respond, even to increasing amounts of anti-seizure medications. This is known clinically as treatment- or drug-resistant epilepsy. Drug-resistant epilepsy has a significant impact on the quality of life of people with autoimmune encephalitis. We currently do not know why some patients with autoimmune encephalitis develop drug-resistant epilepsy whilst others do not.
It is important for doctors to be able to predict how and why people with autoimmune encephalitis develop drug-resistant epilepsy because it is a disabling complication that may be preventable. For this research, we wanted to find out answers to following questions –
We looked through the medical records of seven hospitals in Victoria (Australia) for people who met the diagnosis of autoimmune encephalitis and had an EEG when they first became unwell. Two hundred and eight patients were identified and selected for analysis. We then collected available data from 69 patients of their symptoms, seizures, treatment, and whether they developed drug-resistant epilepsy at 12 months after their initial illness.
We analysed EEGs from patients to find any brain wave irregularities or signatures (called EEG biomarkers) that were more common in those with autoimmune encephalitis who developed drug-resistant epilepsy than those that did not develop drug-resistant epilepsy. Finally, we combined all the factors and created a tool that doctors can use to predict an individual’s risk of developing drug-resistant epilepsy after autoimmune encephalitis.
Figure 1: This figure shows a summary of our findings with 208 patients with autoimmune encephalitis, 16% had severe form of seizures (SE; status epilepticus), 75% of patients had 1 or more seizures, and 25% did not have seizures at their initial admission. Then after 12 months follow up, 16% of patients who completed follow up, had DRE (drug resistant epilepsy), and 33% of the patients were on anti-seizure medications (ASM) and 48% did not require ASMs.
The research could help clinicians to –
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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:
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.
April 15, 2018 | Devon Frakes Fults
Preamble ~
Female warriors in history who engaged in war are found throughout mythology and folklore. A mythological figure does not always mean a fictional one, but rather, someone of whom stories have been told that have entered the cultural heritage of a people as mortal heroines.
Some women warriors are documented in the written record and as such form part of history. However, to be considered a warrior, the woman in question must have belonged to some sort of recognized legion. In this case, the autoimmune encephalitis community. Today we document the story of Hadley. A little warrior whose battle over insurmountable odds lead her to victory. Hadley’s story is not a legend. Hadley’s story is legendary. Therefore, we enter it into the cultural heritage of our people, the AE community, and mark Hadley into our history as a mortal present-day heroine.
Just over two years ago we woke up to what we thought was a normal Monday and found ourselves by days end standing in the Texas Children’s emergency room with our three year old daughter Hadley in a coma and near death.
I laid down next to my perfectly healthy daughter on Sunday evening and didn’t wake up to the same girl next day. At some point during the night, her immune system launched a wicked attack on her brain. We would later be given a name for this incurable beast, Autoimmune encephalitis. Hadley awoke with a headache and mild vomiting. By early afternoon she would be hallucinating and unable to see. By the time we made the 45-minute trip to the emergency room, she would be mostly unconscious and no longer know who we were. By the time we would have her transported to Texas Children’s Hospital, her blood pressure had declined to a frightening and life threatening 45/25. Hadley’s glucose level was only 11 and her core temperature had already begun to drop. Hadley had entered coma.
We arrived at Texas Children’s Hospital with her having only minutes to live. Within two days we were told her brain was swelling. We faced saying what we feared were our final goodbyes just before the doctors placed her on a ventilator. Our Hadley was a Warrior. She fought the disease that was wreaking havoc with her brain and awakened from coma. Having come out of the coma, Hadley was challenged to have to learn to walk, talk, and eat again. She had to learn who her Mom and Dad were having lost all memory of her family and who and what was familiar to her. She battled her way back through hallucinations and countless hours of therapy.
Little did we know what the next two years would hold… 30 days inpatient with 9 days in PICU, 10 days in a coma, 17 days on a feeding tube, 8 days on a ventilator, 45 outpatient appts, 1 72 HR EEG, 3 short EEGs, 3 MRIs, 1 MRA, 3 cat scans, 3 lumbar punctures, 2 radioactive thyroid scans, 3 ultrasounds, 1 central line. Too many IVs to possibly count. 20 months of IVIG. 1 5-day steroid burst, 2 ER visits.
We have been battling this beast, Autoimmune Encephalitis, for two years now and Hadley has been declared to be in remission! We have seen tremendous progress. God has been so good! Of course, we are not the same people we were two years ago. You cannot walk a journey like this one and remain unscathed. I’d be lying if I told you we had it all figured out. There are days we still grieve but most days we find ourselves overwhelmed by Gods goodness.
The IAES has been a huge resource for us! They recently walked us through an insurance appeal when our daughter’s insurance denied her life saving IVIG treatment! Within 48 hours I had all I needed in hand to appeal and win! This treatment was vital to Hadley’s care. International Autoimmune Encephalitis Society has been a huge support in helping us advocate for Hadley and help us get her to where she is today: remission.
International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.
Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.
(My daughter’s portrait while in the hospital when asked how she felt)
I wanted to share the story of my daughter’s battle with anti-NMDAr encephalitis, the dark side of anti-NMDAr encephalitis. The side that is often breezed over and not discussed. Yet as a carer and as a mother, this is the side that I believe is the most devastating.
Let me start at the beginning.
My daughter’s anti-NMDAr encephalitis was a little different in that her first symptoms were seizures. Not just your normal seizures – back to back seizures that made her lose consciousness and turned her blue. The kind of seizures that stumped professionals and made the most knowledgeable doubt what we were saying as true. In fact, after over 10 ER visits, and 3 hospital stays, a well-known knowledgeable Neurologist, decided that her seizures were in fact, psychological, the result of stress. They did numerous tests, and all came back with no results – which lead to this conclusion. I know now that seizures are a prominent symptom at the beginning of anti-NMDAr encephalitis in children and teens, the diagnoses that its “all in her head”, was dismissive.
This, of course, led her to spiral. She began to have 30 minute long seizures and was unresponsive in between. She was unable to tell me who I was, or where she lived. She was in fact a walking zombie. Finally, after driving her 5 hours away to yet another hospital, someone listened.
But this wasn’t entirely straight-forward. It took her to start showing signs of aggression and self-harm for them to really see that something else was going on. In between seizures (that showed up on the EEG as actual seizures) and long spans of catatonia, she would be running through the hospital halls. Grabbing forks and threatening children, trying to drink the bleach that was used to clean the toilets and repeating words over, and over and over again. She was finally admitted into PICU where they sedated her and treated her for suspected anti-NMDAr encephalitis. The doctors told us she might not make it.
Thankfully, the official diagnoses came through and she began treatment. They saved her life. As the treatment began to take effect, she would show signs of being with us. Unable to talk or walk it took weeks for her to finally begin to come back to us; that is where the true darkness began however. As soon as she was able to find her feet, she would run, fast, slam into doors with full force and knock herself out. She would run and try to leap over the guardrail on the 3rd story – showing no signs of fear, only complete determination to end it all. She would put her whole hand down her throat and try choking herself to death, or stick her hands in her eyes and try to poke them out. She would grab any cord she could and wrap it around herself hard. And then there was the constant thrashing her head repeatedly and with force on whatever surface she could find until her head was left black and blue.
You see, my daughter was just 13 when this all began. A sweet, innocent young lady who I believe had absolutely no idea what she was doing. She wasn’t there at all during these attempts, she did not flinch, not one bit when she was hurt. And boy did she hurt herself. It took nurses and security guards to tackle her to stop her. It took sedation and love to finally get her through it.
Fast forward one and a half years and she is back at school 3-4 days a week. She suffers cognitive fatigue and random seizures. It’s a miracle. But as her carer I feel I can never forget and must be on constant guard. She remembers none of what she did – for this I am so thankful. She is back with us and full of life. She remembers not a single moment of the bad moments, and whilst we breathe a sigh of relief it will be forever in the back of our minds. Today, I watched her walk to the bus stop, and thought to myself – “If she turns right she will catch the bus and be safe at school, if she turns left she is going to jump off the cliff and end it all”
International Autoimmune Encephalitis Society (IAES) is a Family/Patient centered organization that assists members from getting a diagnosis through to recovery and the many challenges experienced in their journey.
Driven by the knowledge that “Education is Power”, International Autoimmune Encephalitis Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones, empowering them to be strong self-advocates and advocates that will lead them to best outcomes and recovery. We are the premiere organization leading in these vital roles.
February 20, 2018 | Kristy Caldwell (Haley’s Mom)
This is the story of 19 year old Haley Bounds
Haley has always been a healthy child; never having anything but the common cold. Out of the “blue” the first part of December 2017 mild seizures began. Haley was also having headaches, confusion, memory deficits, labile mood, sleep disturbance, paranoid thoughts, and hallucinations. Doctors told us at first that this was due to dehydration and/or she had epilepsy.
On December 27, 2017 Haley had her first Grand Mal Seizure and was admitted into our local hospital. Haley started to lose speech, movement, etc. and seizures continued. The Doctors continued to lean towards epilepsy, but despite seizure medications, Haley got worse. She lost all speech, movement, and slipped into a comatose state. A second MRI was performed and had changed from the first MRI; showing infection and swelling. Haley was put on a ventilator and was transferred to a Dallas Texas hospital. After many tests, scans, etc. Haley was diagnosed with anti-NMDA receptor Encephalitis. The lumbar puncture was positive for NMDAr in spinal fluid.
Haley has had all first line treatments: IV steroids, plasma exchange (seven treatments) and IVIG. Her doctor’s advanced her to second line treatments of rituximab, and recently cyclophosphamide, a chemotherapy drug. Seizures have continued with an average of two seizures a day. Haley is not doing better. Haley had a tracheotomy performed. We are on Day 55 of her hospital stay where she is cared for in a seizure monitoring room. We have a meeting with her team of Doctors this week to discuss next steps in her treatment plan. Haley is 17 weeks pregnant, so her case is even more complicated.
We continue to pray and have faith. Your prayer are greatly appreciated. You can follow Haley’s story on Facebook under, “Prayers for Haley”
https://m.facebook.com/Prayers-for-Haley-324873681348375/
Thank you and blessings.
Kristy Caldwell- Haley’s Mom
anti-NMDAr autoimmune encephalitis was identified in 2007 by Dr. Josef Dalmau at UPENN. It is the most common type of autoimmune encephalitis and occurs more frequently than infectious encephalitis. AE is treatable.
The initial presentation of NMDAr encephalitis appears to evolve from a frequent, but not invariable, prodromal stage, to a clinical presentation with behavioral and personality changes and psychiatric features, including psychosis, cognitive dysfunction, and seizures, often occurring within the first few days of symptom onset. These are followed days to weeks later by subcortical features, such as movement disorder/dyskinesia, autonomic dysfunction, central hypoventilation, and reduced consciousness . Finally the patients recover, often showing previous features in reverse order.
Recovery is often protracted with long hospital admissions. One remarkable feature of NMDAR Antibody encephalitis is the prolonged recovery, with progressive improvements in cognitive domains noted for months and even years following the end of treatment. Early treatment is associated with best outcomes.
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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 premiere organization leading in these vital roles.
Our website is not a substitute for independent professional medical advice. Nothing contained on our website is intended to be used as medical advice. No content is intended to be used to diagnose, treat, cure or prevent any disease, nor should it be used for therapeutic purposes or as a substitute for your own health professional's advice. Although THE INTERNATIONAL AUTOIMMUNE ENCEPHALITIS SOCIETY provides a great deal of information about AUTOIMMUNE ENCEPHALITIS, all content is provided for informational purposes only. The International Autoimmune Encephalitis Society cannot provide medical advice.
International Autoimmune Encephalitis Society is a charitable non-profit 501(c)(3) organization founded in 2016 by Tabitha Andrews Orth, Gene Desotell and Anji Hogan-Fesler. Tax ID# 81-3752344. Donations raised directly supports research, patients, families and caregivers impacted by autoimmune encephalitis and to educating healthcare communities around the world. Financial statement will be made available upon request.
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