Don’t Be Left Behind. Keep Up With THE HERD!
Supportive assistance guide for your AE Journey~
September 2022 Studies and Clinical Trials
In this Issue~
- *A Letter From IAES Founder and President
- *AE Studies & Clinical Trials: The IGNITE Study, The ExTINGUISH Trial
- *Clinician’s Corner: Medical Text: Autoimmune Encephalitis and Related Disorders of the Nervous System
- *Monthly Zoom Support Groups for Patients & Caregivers: Caregiver Monthly Zoom Support Group, #AETuesdayTries for patients and caregivers
- *AE Trivia Cards: The Single Most Important Educational/Rehabilitation Tool for AE
- *AE Warrior Store: Shop for Your AE Bling and Raise Awareness
- *Shop & Support IAES: Sign up for PayPal’s #GiveAtCheckOut and Amazon Smiles
A Letter From IAES Founder and President
Dear AE Family,
No matter where you are on your AE journey, as a patient or caregiver, there is one unanimous cry we all declare: “Nothing about AE is okay. Things need to get better!” My favorite quote is by Gandhi, “Be the change you wish to see in the world.” That time is now. You can be that change and help make the future brighter for people touched by autoimmune encephalitis.
IAES has been working with three research teams to assist them in furthering our mutual goals. Two teams are working to identify new treatments for AE. The ExTINGUISH Trial is the first-ever Clinical Trial for a promising new drug, Inebilizumab, to treat anti-NMDA receptor encephalitis and the LGI1 Research Survey that can be completed by patients and caregivers where a Pharma company hopes to take your information and apply it to the development of a new treatment for LGI1. Over the past few months, IAES has been working with a research team at Stanford University that hopes to answer the question: Is there a genetic predisposition to AE? This study is open to all AE patients, with active disease or in remission who have an identified antibody. This means that up to 75% of patients with a diagnosis of AE can participate in one or more of these studies and move the ball forward to a brighter future for us all and those not yet impacted by AE.
Whether you are newly diagnosed or have been in our AE community for several years, I wanted to take the opportunity to share just how far we have come, due to the dedication of researchers and organizations like IAES. IAES was incorporated in September 2016, yes we are six years old this month, and we remain the only non-profit organization dedicated to helping AE patients, caregivers, and families through their journey.
In 2005, Dr. Josep Dalmau had two baffling female patients he was caring for at UPENN. He published their case studies in 2007. Dr. Dalmau had accomplished something extraordinary. He had identified a previously unknown disease and named it by the offending antibody he had discovered, anti-NMDAr encephalitis, an antibody-mediated form of encephalitis that was autoimmune. The field of autoimmune encephalitis has grown exponentially since then and now includes a new subspecialty of neurology called ‘autoimmune neurology’. Patients share two important features: they often have a subacute clinical onset that in a few weeks transports a person from an independent, healthy life to one of functional impairment with severe neurological deficits. The other feature is that prompt diagnosis and treatment reverse this downhill clinical course and, frequently, result in good outcomes.
‘Autoimmune encephalitis or encephalitides’ or ‘antibody-mediated encephalitides’ (AE) constitutes a group of diseases with autoantibodies targeting important brain proteins, including neurotransmitter receptors, ion channels, and associated membrane proteins. The spectrum and understanding of AE—an umbrella term for a group of noninfectious, inflammatory central nervous system diseases—have expanded dramatically over the past seventeen years. Since then, 18 autoimmune encephalitides and the corresponding syndromes have been described. In many of these diseases, the cause that triggered the disease has been identified as a tumor or viral infection (HSV). In some types of AE, AE types are named for the identified antibody, a genetic susceptibility linked to distinct human leukocyte antigens (HLA) has been shown.
AE is characterized by abnormal psychiatric behavior or cognitive dysfunction, speech dysfunction, seizures, movement disorder, decreased levels of consciousness, autonomic dysfunction, and central hypoventilation.
The proliferation of new autoantibodies can be overwhelming to non-specialists. However, the great majority of patients have a few distinct syndromes that physicians can learn to recognize. Familiarity with AE syndromes ensures prompt diagnosis and treatment.
Following infectious encephalitis, AE is the second most common cause of encephalitis, with an estimated incidence of approximately 6.5/10,000. Approximately 80%–85% of patients with AE respond favorably to timely immunosuppressive therapies; however, a significant portion of patients with AE progress to critical conditions and often require long-term hospitalization.
Patients may suffer from the poor consequences of severe AE with functional and psychosocial deficits due to delayed diagnosis and treatment. However, most AE patients have some level of lasting deficits regardless. Consequently, the emphasis on timely and effective interventions for severe AE has increased, which may salvage this “critical zone” and consequently prevent disease progression and relapse, facilitating neurological function recovery. Autoimmune Encephalitis has a 7% mortality rate with up to 15% for anti-NMDAr, LGI1, and GABABr.
From the first two cases of AE in 2005 to the present, a great deal has been realized about this group of diseases. Earlier this year, Drs. Dalmau and Graus published the first medical text on AE which is now being used in medical school. Soon we will be experiencing a new landscape filled with medical professionals who are familiar with AE having studied it in medical school and/or more clinicians with a specialty in autoimmune neurology. A great deal has been accomplished in a short period of time. Please join me in creating the change we want to see in the world and participate in one or more of these studies.
You are not alone. We ARE #StrongerTogether.
President and Founder,
International Autoimmune Encephalitis Society
AE Studies and Clinical Trials
The IGNITE Study
Dr. Sergio Muñiz-Castrillo and his research group at Stanford University in California have requested IAES’s help in recruiting autoimmune encephalitis patients for The IGNITE study. This study aims to investigate the genetic predisposition to autoimmune encephalitis and related disorders. Patients must have an identified antibody to participate. The patient may have an active case or be in remission. This exciting study includes patients of all ages and all antibodies. Patients outside of the United States will need the physician who diagnosed them, usually a neurologist, to contact Dr. Muñiz-Castrillo directly. No travel is involved. Saliva and blood kits will be sent to you in the mail and can be returned by mail or dropped off at named participating pharmacies.
Your participation will help uncover that answer and your act of advocacy will eventually enhance lives! The first step to take is to fill out and submit the screening survey below.
Seronegative/antibody-negative patients can play an active advocacy role by distributing the IGNITE study flyer to their diagnosing doctor, and asking that it be posted at the infusion center you use, as well as hospitals and clinics you visit.
Sharing the flyer on your social media platforms will assist us in getting the highest level of participation possible.
The ExTINGUISH Trial
New Medical Text:
Autoimmune Encephalitis and Related Disorders of the Nervous System
Autoimmune encephalitis, diseases where the immune system attacks the brain, have become a fast-moving field of study in recent years. The authors, Drs Josep Dalmau (who collaborated with IAES on AE Trivia Playing Cards), and Francesc Graus, have played pivotal roles in the discovery of these diseases. Here, they provide a comprehensive clinical guide to the differential diagnosis of these disorders, illustrated with over 200 figures, 30 videos and numerous clinical vignettes, many from their own practice. Clinical descriptions are straightforward, emphasizing distinctive diagnostic clues for each disease. The strengths and weaknesses of diagnostic tests and clinical criteria are discussed extensively, as well as the best evidence supporting the use of available treatments. There is an up-to-date description of immunological triggers and comorbidities, and well-illustrated and clearly summarized pathogenic mechanisms and disease models.
Monthly Zoom Support Groups for Patients and Caregivers
Caregiver Monthly Zoom Support Group
The next monthly meeting will take place on October 13th at 6 pm PST/9 pm EST. The IAES Caregiver Support Group takes place on the 2nd Thursday of each month. This is a ‘safe space’ where caregivers can build relationships/friendships with others who are “walking that walk”, share/vent, and receive the support and direction they so richly deserve.
AE Caregivers have a variety of unique challenges that are different from the AE Warrior’s. Often they feel alone and need to be able to share their concerns with others who are on that same journey. Sometimes hearing a chorus of ‘Me Too!’ can have great healing power of its own. Additionally, some of you may qualify for services you are unaware of that could assist or solve a challenge you (or your loved one) are having. As topics are discussed, your hostess, Mari Davis, will direct you to available solutions, services and supports.
Your Hostess, IAES Support Services Coordinator Mari Wagner Davis, has 30 years of experience as an accredited Nurse Case Manager. Mari’s professional career prior to becoming ill with Autoimmune Encephalitis was to locate and arrange for the support services needed for patients when released from the hospital to rehabilitation or home.
The next monthly #AETuesdayTries zoom meet-up will take place September 27th, and will always be the last Tuesday of each month.
“Tuesday Tries” addresses the emotional support and rehabilitation AE patients and caregivers require in recovering from autoimmune encephalitis by building your resilience. The program utilizes science-based exercises from the work of PERMA; Seligman, 2012.
IAES wants to foster your capacity in the belief that better days are ahead! “Tuesday Tries” is about practicing a strengths-based approach by “normalizing the try;” celebrating critical milestones in the recovery and care of AE patients and caregivers in order to build a more resilient network of care.
A workbook organizer, “Growing Your Resilience” has been created to accompany the #AETuesdayTries program and is located on the ‘Apps and Tools for AE Warriors’ page in the section: ‘Building Your Resilience’ on the IAES website under the ‘For Patients’ tab. A link to download the organizer is also provided within your e-mail confirmation when you register.
Join AE patients and caregivers of all ages and stages in their AE journey. Receive insights and encouragement from others and celebrate your critical milestones in recovery and care while building a more resilient personal AE network!
Your facilitator, Tessa McKenzie, IAES’ Chief Resilience Officer, is a life coach and studies “resilience” through her private practice, Envisage Vocation Creation and work with Johns Hopkins University’s Life Design Lab. Join Tessa and other AE patients and caregivers who are ready to “Share Your Try Tuesdays”. Tessa will provide prompts for the reframe of perceived failures as well as peer-to-peer validation of “wins” and opportunities for relationship building.
AE Trivia Playing Cards
The perfect companion for patients, caregivers & therapists
We appreciate the National Organization of Rare Diseases’ (NORD) enthusiastic support in recommending this groundbreaking product.
AE Warrior Store
Shop for Your AE Bling and Raise Awareness
IAES holds the trademark for #AEWarrior, AE Warrior and Autoimmune Encephalitis Warrior and is the creator of these phrases. Proceeds also go to support research that may one day lead to a cure.
Don’t forget to #GiveAtCheckOut if you purchase your gift through PayPal! (See: Shop and Support IAES Through PayPal’s #GiveAtCheckOut program below).
The proceeds of your purchase will immediately support Autoimmune Encephalitis patients, caregivers, and families who are walking this difficult journey. Proceeds also go to support research that may one day lead to a cure.
Shop & Support IAES Through PayPal’s
|Get that great deal online and support IAES at the same time!|
When you shop Amazon Smiles and select IAES as the non-profit you want to support, Amazon will donate .5% of our purchases to the International Autoimmune Encephalitis Society.
This simple act of kindness, will support the work we do and advance our ability to service the community. MakeMeSmile is an extension for Chrome that makes sure you select International Autoimmune Encephalitis Society when you shop online. It’s free to download. And you don’t even need to sign up.
Donations raised are greatly appreciated and directly support research, patients, caregivers, and families through their journey so the best outcomes can be reached. Your contribution to our mission will help save a life and improve the quality of lives for others. Be a part of the solution by supporting IAES.
Driven by the knowledge that “Education is Power”, Int’l AE Society manages an educational support group for patients diagnosed with Autoimmune Encephalitis and their loved ones on Face Book, 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.