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THE HERD July 2023 1st edition

theherd - THE HERD July 2023 1st edition

Don’t Be Left Behind. Keep Up With THE HERD!

Supportive assistance guide for your AE Journey~

July 2023 1st Edition

In this Issue~

  •  *Children’s Corner (for all ages): Pediatric anti-NMDA-receptor autoimmune encephalitis in siblings: Developmental, Electrophysiologic, and Genetic Implications, All About Butterflies Crossword
  • *ABI Rehabilitation: Jigsaw Puzzles
  • *Helpful Tips for the AE Warrior:  National Parks Disability Access Pass, Immunogenetics in Autoimmune Encephalitis Webinar
  • *Most Popular Visuals: LGI1 Autoimmune Encephalitis (AE) Infographic, LGI1 Autoimmmune Encephalitis Journey
  • *Featured AE Article: An Evolving Redefinition of Autoimmune Encephalitis
  • *Video: Acute Disseminated Encephalomyelitis (ADEM)
  • *Clinician’s Corner: Psychiatric Illness or Immune Dysfunction—Brain Perfusion Imaging Providing the Answer in a Case of Anti-NMDAR Encephalitis
  • *Open Access: Pre-existing autoimmunity is associated with increased severity of COVID-19: A retrospective cohort study using data from the National COVID Cohort Collaborative
  • *Monthly Zoom Support Groups for Patients & Caregivers: Caregiver Monthly Zoom Support Group, #AETuesdayTries for patients and caregivers  
  • *AE Studies & Clinical Trial: The IGNITE Study, The ExTINGUISH Trial 
  • *AE Trivia Cards:  The Single Most Important Educational/Rehabilitation Tool for AE 
  • *AE Warrior Store:  Shop for AE Bling and Raise Awareness. Check out our new arrivals
  • *Shop & Support IAES: Sign up for PayPal’s #GiveAtCheckOut

Children’s Corner

Pediatric anti-NMDA-receptor autoimmune encephalitis in siblings: Developmental, Electrophysiologic, and Genetic Implications

Background

Anti-NMDAR encephalitis is a leading cause of autoimmune encephalitis in children. Untreated disease can lead to long-term neurological disability. 

Case report

We present siblings with pediatric-onset anti-NMDAR encephalitis. One was treated early, while the other’s diagnosis and treatment were delayed by several years. Developmental, electrophysiologic, and genetic implications are discussed. 

Conclusion

This is the first report of siblings with NMDARE, a severely debilitating disease, allowing for a direct comparison of early versus late treatment amongst genetically similar individuals with similar disease phenotypes at initial presentation. It provides further support for early recognition and aggressive treatment to improve long-term outcomes and reduce relapse risk. Future studies comparing larger cohorts of early versus late treatment and investigating possible genetic underpinnings of biological disease onset in NMDARE.

All About Butterflies Crossword

When it comes to brain health and rehabilitation from brain injury from our AE, we’re all looking for ways to re-learn and re-train our brain. Research shows doing crossword puzzles regularly can also improve your ability to focus attention on a desired task and improve your executive function and working memory. All these skills improve one’s ability to successfully navigate the challenges of everyday life. Crossword puzzles improve your overall quality of thinking and memory, attention span, organizational skills, and vocabulary while reducing stress.

Other helpful exercises for children are found on the Children’s Corner webpage under the ‘For Patients’ tab on the IAES website. 

ABI Rehabilitation From AE

Jigsaw Puzzles

These puzzles have been created with original art by autoimmune encephalitis patients making them a unique gift for the AE Warrior as well as a collector’s item. Puzzles are a long-time group or individual pastime. They are highly recommended cognitive exercises for anyone with a brain injury or in recovery from AE. Puzzles assist with comprehension, organization, memory, hand-eye coordination, and fine motor skills and provide goal setting, patience, and a sense of achievement.

Helpful Tips for Patients & Families

National Parks Disability Access Pass

Did you know that you can get a National Parks Disability Access Pass? This pass allows free access to National Parks for disabled individuals and their family members, (those in 1 car), to National Parks, and a discount on camping. They are available at National Parks. Go to the National Park Service website for needed information.

Immunogenetics in Autoimmune Encephalitis

The Romanian Association for Autoimmune Encephalitis (RAAE- an organization we supported in getting established), continues its Webinar Series with this exciting event featuring keynote speaker, Sergio Muniz Castrillo, MD, Ph.D. (a member of IAES’ Medical Advisory Board) on the 12th of July, 10 AM PST.  

Sergio Muñiz-Castrillo is a trained neurologist specializing in autoimmune encephalitis and paraneoplastic neurological syndromes. Currently, Dr. Muñiz-Castrillo is a postdoctoral scholar at Stanford University. 

Drs. Emmanuel Mignot and Sergio Muñiz-Castrillo and their research group at Stanford University are currently recruiting patients for the IGNITE study which aims to determine if a genetic predisposition to AE exists. 

Most Popular Visuals

LGI1 Autoimmune Encephalitis (AE) Infographic

This information on the infographic summarizes insights from primary research conducted in 2022, commissioned by UCB and supported by patient organizations, to understand the journeys and needs of people in the LGI1 AE patient community. In total, 13 LGI1 patients and 10 caregivers, from the US and UK, contributed to this research through surveys and qualitative research interviews. Researchers also surveyed and interviewed 32 Neurologists. Researchers collaborated with three patient organizations to develop this output – Detailed data from the study and organization collaboration are not published. Special thanks to the people living with LGI1 AE who contributed to this research and output development.

Partnering patient organizations: International Autoimmune Encephalitis Society (autoimmune-encephalitis.org) Encephalitis Society (www.encephalitis.info) Autoimmune Encephalitis Alliance (aealliance.org).

LGI1 Autoimmune Encephalitis Journey

Featured AE Article

An Evolving Redefinition of Autoimmune Encephalitis

  • Take-home messages

  • The hallmark of autoimmune encephalitis is antibody-mediated central nervous system autoimmunity, which is related to the development of antibodies recognizing neuronal receptors or synaptic proteins as foreign proteins.

  • The pathogenic mechanisms that underlie autoantibody-triggered autoimmune encephalitides are poorly understood and how autoantibodies breach the blood-brain barrier remains obscure for almost all these disorders.

  • Although clinical manifestations of autoimmune encephalitides can significantly vary and different autoantibodies can be associated with similar phenotypes, the global evaluation of clinical symptoms, EEG findings and brain/spinal cord MRI can strongly suggest the diagnosis of a well-defined disorder in a relevant number of cases. However, many of these conditions remain considerably underdiagnosed in children.

  • Various clinical features can herald the onset of encephalitic syndromes, but the dominating modality of onset is characterized by movement and psychiatric disorders.

  • Seizures and movement disorders along with psychosis, confusion or additional behavioural changes are the most relevant initial manifestations in all age groups.

  • There are few reports of pediatric autoimmune encephalitis in the medical literature and wide variations can be noted in both diagnostic procedures and treatments.

  • The general approach to children with autoimmune encephalitis has been driven by global information related to adult patients.

  • A host of autoantibodies has been found in the sera of patients with autoimmune encephalitis, and screening for malignancy is essential to rule out a neoplastic syndrome.

  • Anti-glutamic acid decarboxylase (GAD) 65 encephalitis has been mostly reported in adults, variably presenting as limbic encephalitis, stiff person syndrome, cerebellar ataxia, ocular movement disorders, epilepsy, but also with extralimbic manifestations.

  • Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is the most frequent autoimmune encephalitis in children: several infectious agents have been implicated in its pathogenesis, and its overall outcome is largely favorable.

  • Voltage-gated potassium channels (VGKCs)-a positive group of encephalitides starts as limbic encephalitis with cognitive impairment, seizures, and variable neuropsychiatric symptoms.

  • Facio-brachial dystonic seizures can be considered pathognomonic of anti-LGI1 encephalitis, which prevalently occurs in the elderly.

  • Anti-glycine receptor (GlyR) and anti-γ-aminobutyric acid receptors (GABARs) encephalitides are phenotypically diverse neurobiological entities that have been mostly reported in adults.

  • Future multicenter studies on the pediatric population with autoimmune encephalitis are needed to identify whether the phenotype clusters may direct therapy or predict treatment response and outcome.

Video

Acute Disseminated Encephalomyelitis (ADEM)

In this lecture, Professor Zach Murphy presents on Acute Disseminated Encephalomyelitis (ADEM). The lecture includes detailed diagrams and figures in order to understand the etiology, pathophysiology, clinical features, diagnosis, and treatment of ADEM.

Clinician’s Corner

Psychiatric Illness or Immune Dysfunction—Brain Perfusion Imaging Providing the Answer in a Case of Anti-NMDAR Encephalitis

This case presents a potentially beneficial approach for detecting a rare disease and is an example of SPECT quantification analysis as a missing step in resolving clinical questions. It is limited because it only presents the results of only one patient. However, to our knowledge, it is the only known published case of anti-NMDAR encephalitis caused by pheochromocytoma evaluated with brain perfusion single-photon tomography. It is important to emphasize that while SPECT study analyses have been performed and published, a quantitative comparison of this type has not been described in cases of anti-NMDAR encephalitis. Further studies with quantitative analysis of multiple affected patients would provide a basis for determining specific [99mTc]-HMPAO SPECT brain perfusion patterns. We conclude that SPECT quantitative brain perfusion analysis using the whole brain as a normalization structure improved confidence in visual detection of anti-NMDAR encephalitis and provided unexpected solutions to atypical psychiatric dilemmas. Furthermore, adding quantification in routine clinic use with SPECT/CT can provide more convincing results in departments without PET/CT equipment and can provide better treatment results.

Open Access

Pre-existing autoimmunity is associated with increased severity of COVID-19: A retrospective cohort study using data from the National COVID Cohort Collaborative

Background

Identifying individuals with a higher risk of developing severe COVID-19 outcomes will inform targeted or more intensive clinical monitoring and management. To date, there is mixed evidence regarding the impact of pre-existing autoimmune disease (AID) diagnosis and/or immunosuppressant (IS) exposure on developing severe COVID-19 outcomes.

Methods

A retrospective cohort of adults diagnosed with COVID-19 was created in the National COVID Cohort Collaborative enclave. Two outcomes, life-threatening disease, and hospitalization were evaluated by using logistic regression models with and without adjustment for demographics and comorbidities.

Results

Of the 2,453,799 adults diagnosed with COVID-19, 191,520 (7.81%) had a pre-existing AID diagnosis and 278,095 (11.33%) had a pre-existing IS exposure. Logistic regression models adjusted for demographics and comorbidities demonstrated that individuals with a pre-existing AID (OR = 1.13, 95% CI 1.09 – 1.17; P< 0.001), IS (OR= 1.27, 95% CI 1.24 – 1.30; P< 0.001), or both (OR = 1.35, 95% CI 1.29 – 1.40; P< 0.001) were more likely to have a life-threatening COVID-19 disease. These results were consistent when evaluating hospitalization. A sensitivity analysis evaluating specific IS revealed that TNF inhibitors were protective against life-threatening disease (OR = 0.80, 95% CI 0.66- 0.96; P=0.017) and hospitalization (OR = 0.80, 95% CI 0.73 – 0.89; P< 0.001).

Conclusions

Patients with pre-existing AID, exposure to IS, or both are more likely to have a life-threatening disease or hospitalization. These patients may thus require tailored monitoring and preventative measures to minimize negative consequences of COVID-19.

Monthly Zoom Support Groups For Patients and Caregivers

Caregiver Monthly Zoom Support Group

The next monthly meeting will take place on July 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.

#AETuesdayTries

The next monthly #AETuesdayTries Zoom meet-up will take place on July 25th, 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 Studies and Clinical Trials 

The ExTINGUISH Trial

This is the first-ever Clinical Trial for a promising new drug, Inebilizumab, to treat anti-NMDA receptor encephalitis, and will be funded by the National Institutes of Health (NIH). 

This trial is actively recruiting. International Autoimmune Encephalitis Society is honored to be supporting the ExTINGUISH trial along with Autoimmune Encephalitis Alliance and The anti-NMDA Receptor Encephalitis Foundation. 

 Main Inclusion Criteria (in part)

Enrollment  is limited to 116 newly diagnosed patients with a confirmed diagnosis of anti-NMDAR Encephalitis who are 18 years or older. Patients must have received at least 3 days of methylprednisolone 1000 mg IV or equivalent corticosteroid within 30 days prior to randomization (Day 1). In addition, patients must have received EITHER of the following treatments within 30 days before randomization.

  • IVIg, at a minimum dose of 2 g/kg
  • Plasma exchange or plasmapheresis, with a minimum of 5 treatments. NOTE: These treatments may be provided during the screening period, but must be completed prior to randomization.

 mRS of ≥3 at the screening visit, indicating at least moderate disability.  Ability and willingness to attend study visits and complete the study.

The IGNITE Study

Drs. Emmanuel Mignot and Sergio Muñiz-Castrillo and their 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. 

AE Trivia Playing Cards

The perfect companion  for patients, caregivers & therapists

Doctor Recommended

Created by IAES and in collaboration with Josep Dalmau, M.D., Ph.D. (The World’s leading expert on AE).  This deck answers 52 of the most commonly asked questions about AE and doubles as a rehabilitation tool.  Increase your knowledge and become a strong advocate quickly and easily with AE trivia cards. 

We appreciate the National Organization of Rare Diseases’ (NORD) enthusiastic support in recommending this groundbreaking product. 

AE Warrior Store

Shop for AE Bling and Raise Awareness

Check out the two new t-shirt designs at the Autoimmune Encephalitis Warrior Store, the “Don’t Quit/Do IT #AEWarrior” and the colorful retro #AEWarrior design.  

AE Warrior Store products proclaim the bravery and fighting spirit it takes to fight AE and regain your health. Shirts, hoodies, hats, puzzles, mugs, and gift items make this your one-stop shop for patients, caregivers, and friends who have a vested interest in raising awareness for Autoimmune Encephalitis.  Designs can be transferred to any product you prefer. The proceeds of your purchase will immediately support Autoimmune Encephalitis patients, caregivers, and families who are walking this difficult journey.

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 

#GiveATCheckOut

Have you wanted to support IAES, Autoimmune Encephalitis awareness, and the AE Warrior that you love?

Here is an easy and affordable way to donate without feeling a thing when shopping with your Paypal account. Each time you check out you will be reminded of an opportunity to give $1 to IAES.

Simply go to your PayPal account and select “set favorite charity”, and choose International Autoimmune Encephalitis Society.  When you make a purchase and check out, an option comes up that asks if you would like to give $1 to IAES? 

Help create the positive change you envision for the AE Community by donating your change.

Are YOU an IAES angel? Do you love someone with AE? Do you want to raise AE awareness to not just support AE Warriors but lead researchers to finding a cure?  The IAES Angel is someone who lifted IAES upward by ensuring that comfort, guidance and improved health is brought into an AE patient’s life.

IAES Angels are motivated by their Spirit of giving.  They are Champions in raising AE awareness. Your devotion to supporting our mission and improving the lives of those who suffer from AE is felt mightily and immediately put to use. 

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

International Autoimmune Encephalitis Society is proud to be a platinum level participant on the Guide Star exchange, demonstrating or commitment to financial transparency.  IAES is a charitable non-profit 501 (c)(3) organization. Tax ID#81-3752344

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.

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”, 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.

On June 16th, 2022, the International Autoimmune Encephalitis Society and Tabitha Orth, IAES President, and Founder, officially became the 7,315th “point of light”. The award was founded by President George H.W. Bush in 1990.

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