Site icon Autoimmune Encephalitis

THE HERD May 2021 2nd edition

theherd - THE HERD May 2021 2nd edition

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

Selected Highlighted News in the field of Autoimmune Encephalitis~ May 2021 2nd Edition

In this Issue~

  • *Announcements: New program #TuesdayTries, AE Study in Australia seeks your participation, New antibody identified in autoimmune encephalitis
  • *Children’s Corner (for all ages): Clinical Features and Treatment Outcomes of Seronegative Pediatric Autoimmune Encephalitis, Meet 8-year-old AE Warrior Makenzie Shearer
  • ABI Rehabilitation: The Stroop Test, Maze-medium difficulty
  • Useful Tips for the AE Warrior:  Leave a ‘Love note’ to yourself, Medisafe Medication Management app
  • Most Popular Visual Downloads: Aphasia as a Symptom of Autoimmune Encephalitis
  • *COVID-19’s Impact on the AE Community:  Covid-19 Vaccines: The Ultimate 2021 Guide to Every Vaccine
  • *Featured AE Article: Autoimmune Encephalitis in Late-Onset Seizures: When to Suspect and How to Treat, When Get up and Go is Gone. Lack of Initiation and Motivation after Brain Injury 
  • *Clinician’s Corner: Important updates in Autoimmune Encephalitis Testing  from Mayo Clinic
  • *COVID-19 Clinician’s Corner: A Case Report of Simultaneous Autoimmune and COVID-19 Encephalitis
  • *Open Access: Autoimmune Encephalitis in Children: From Suspicion to Diagnosis
  • *AE Trivia Cards: The single most important educational/rehabilitation tool for AE
  • *AE Warrior Store: See our new AE mug and hat collections

IAES is launching “Tuesday Tries” on May 25th. This monthly zoom meet-up will take place the last Tuesday of each month and will celebrate your critical milestones in recovery and care and help you to build a more resilient network!

Your host, 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 Study Seek Your Participation

AE Patients Residing in Australia ~Your Participation is Needed

Dr. Mastura Monif, a member of the IAES Medical Advisory board who is located in Australia, is conducting a study on autoimmune encephalitis and seeks patient participation.

The Monif Group 

Leads The Australian Autoimmune Encephalitis Consortium Project that consists of up to 13 health and academic centers around the country bringing national experts together to tackle the issue of Autoimmune Encephalitis facing the Australian population.

The group has formed The Australian Autoimmune Encephalitis Consortium bringing together national experts from 4 states around the country to tackle the issue of AE facing the Australian Health sector. With this study, they hope to produce the largest cohort of retrospective and prospective cases of AE in Australia with the aim of gaining a better understanding of disease trajectory as well as identifying key clinical, electroencephalogram/seizure phenotype, cellular and biochemical, radiological & cognitive biomarkers of disease onset, progression & outcome. This study brings together a collaborative & multidisciplinary team of neurologists, neuroimmunologists, epilepsy experts, neuropsychologists, neuroradiologists, psychiatrists, and neuroscientists. The findings are hoped to generate extensive data regarding AE as well as the production of clinical guidelines for early identification, diagnosis & treatment of these devastating conditions.

E-mail Dr. Monif regarding Study Participation


New Antibody identified in Autoimmune Encephalitis

Encephalitis with autoantibodies against the glutamate kainate receptors GluK2

GluK2-abs associate with an encephalitis with prominent clinico-radiological cerebellar involvement. The antibody effects are predominantly mediated by internalization of GluK2.

Six of 8 patients developed acute encephalitis and clinical or MRI features of predominant cerebellar involvement (4 presenting as cerebellitis which in 2 caused obstructive hydrocephalus), and 2 had other syndromes (1 with cerebellar symptoms). One of the samples showed mild reactivity with non-kainate receptors (AMPAR and NMDAR) leading to identify 6 additional cases with GluK2-abs among patients with anti-AMPAR (5/71) or anti-NMDAR encephalitis (1/73).  GluK2-abs internalized GluK2 in HEK293 cells and neurons; these antibody-effects were reversible in neurons. 

Children’s Corner

Clinical Features and Treatment Outcomes of Seronegative Pediatric Autoimmune Encephalitis


Nine (19.6%) of the 46 patients were diagnosed as seropositive AIE. All of the patients with seropositive AIE had anti-N-methyl-D-aspartate receptor antibodies. Commonly identified neuropsychiatric symptoms were altered mental status, cognitive dysfunction, seizure, speech dysfunction, and psychotic disorder in both the seronegative and seropositive groups. Immunotherapy produced favorable treatment outcomes in both the seropositive (n=7, 77.8%) and seronegative (n=35, 94.6%) AIE patients. Treatment outcomes for first-line immunotherapy were better in seronegative AIE than seropositive AIE patients (p=0.003), and hence a smaller proportion of seronegative patients required second-line treatment (p=0.015).


Pediatric seronegative AIE patients showed clinical presentations similar to those of seropositive AIE patients, with favorable treatment outcomes after immunotherapy.

Clinical Features and Outcomes of Anti-N-Methyl-d-Aspartate Receptor Encephalitis in Infants and Toddlers

Results: A total of 41 patients (age range: six to 34 months; median age: 23 months; female: 19) were enrolled in this study. Nineteen (46%) patients exhibited classical anti-NMDAR encephalitis, whereas 22 (54%) patients exhibited anti-NMDAR encephalitis after viral encephalitis. There was a high presentation of movement disorders (100%), developmental regression (90%), abnormal behaviors (90%). All patients were administered first-line therapy, with only 17% of them being administered second-line immunotherapy. Two patients succumbed to the disease, whereas none of them relapsed. At the long-term follow-up (more than one year), 20 of 35 (57%) exhibited satisfactory outcomes (modified Rankin Scale ≤2). Compared with patients with classical anti-NMDAR encephalitis (n = 18), patients after viral encephalitis (n = 17) were more likely to have worse clinical outcomes. They exhibited a higher modified Rankin Scale/Pediatric Cerebral Performance Category score and more frequent seizures. A predictor of poor outcome was presentation after viral encephalitis (odds ratio 35.7, 95% confidence interval 4.64 to 275.03, P = 0.001).

Conclusion: Anti-NMDAR encephalitis in infants and toddlers clinically presents with movement disorders, developmental regression, and abnormal behaviors. Interestingly, this group had a higher proportion of patients after viral encephalitis, which is regarded as the only risk factor for poor outcomes.

Meet 8-year-old AE Warrior Makenzie Shearer

Makenzie Shearer is an otherwise healthy 8-year-old little girl who developed new onset refractory epilepsy syndrome (NORSE) likely secondary to autoimmune encephalitis. She had a prolonged hospitalization here which included the institution of pharmacologic comas to control her status epilepticus. During this time, there was ongoing collaboration between Pediatric Neurologist Dr. Anne Marie Morse,  and Dr. Brenda Banwell of CHOP. (Both on the IAES Doctor’s List). Both doctors believe their collaboration allowed them to maximize a therapeutic strategy which included a novel biological agent.

ABI Rehabilitation From AE

The Stroop Test: Great brain teaser to challenge your mental vitality and flexibility

Are you working on your cognitive rehabilitation from AE?

This task is called the Stroop Test, and is used in neuropsychological evaluations to measure mental vitality and flexibility, since performing well requires strong attention, inhibition and self-regulation capability (also called executive functions). You’ll find it’s not so easy. 

Visit the IAES website to find other exercises that will assist with your cognitive rehabilitation. 

Maze- Medium difficult

Word Search puzzles and puzzles of all types are a wonderful exercise to do for brain injury rehabilitation from AE.  They are a great activity to help your AE warrior develop their problem-solving and analytical skills.

IAES has created several word search puzzles that feature hidden keywords that will encourage and aid in your learning and understanding of autoimmune encephalitis.

Challenge yourself with this puzzle and others that you will find as free downloads on our website. Then look through the puzzles in the AE Warrior Store that have been created with art by fellow AE patients. You are sure to become inspired and challenged in your recovery efforts.

Useful Tips for Patients & Families

Leave a Love Note To Yourself To find at a later Date

Tessa McKenzie, IAES’ Chief Resilience Office and facilitator of our up-coming #AETuesdayTries Zoom meet-ups recently asked IAES members what advice they would give to their younger self or a young AE Warrior. We particularly enjoyed Oksana Sweeney’s response and wanted to share it with you. 

“Don’t get overly excited about the marked improvements and overly discouraged by nose dives – the recovery and relapse are basically a twine and on the good day you have to bottle up the feeling of superpowers, and leave a note to self to do two things: (1) analyze the journey and figure out what improved so you can better manage your care and clearly explain things to your doctor and (2) also leave a love note for yourself with some chocolate in a place you will forget but likely will stumble upon on a bad day for the next time you have a decline- to remind that this too shall pass. Overall the roping gets stronger.”

Medisafe Medication Management app

Never forget to take your meds and pills again with the MUST HAVE pill reminder ranked #1 by pharmacists and physicians in independent tests. (Apple)

Most Popular Download

What is Aphasia?


What is Aphasia?

Aphasia is a language disorder affecting at least 2 million Americans that impairs the ability to produce or comprehend speech, along with the ability to read or write. It can result from any injury or disease that damages the language centers of the brain, including autoimmune encephalitis (AE). To better understand aphasia in the context of AE, IAES in Partnership with PennNeuroKnow have created this handout we hope you will find helpful.

COVID-19’s Impact on the AE Community

Covid-19 Vaccines: The Ultimate 2021 Guide to Every Vaccine



Covid-19 vaccines have taken center stage in recent months. Mass vaccination programs are underway, and billions of people are now pinning their hopes for the future on the success of a global rollout.

In many ways, the world we live in now is very different to just last year.

Vaccination offers the best chance of a return to normal life, and a huge responsibility falls upon the shoulders of vaccine manufacturers. With this added pressure comes publicity, and scrutiny. Pharmaceutical companies are now household names, and data from coronavirus vaccine trials is publicly published, analyzed, and critiqued by health experts and journalists alike.

In stark contrast, an endless supply of fake news and rumors, fueled by a fear of the unknown, has diluted public belief in vaccination programs. This complete guide aims to cut through this fog of misinformation and provide you with all of the available facts on Covid-19 vaccines.

In this extensive guide, you’ll learn everything about Covid-19 vaccines– from high-profile vaccine candidates, to the wider issues and talking points surrounding immunization campaigns.


As more people get vaccinated, what will getting vaccinated mean for immunocompromised people, and how does immunocompromised status potentially impact COVID-19 vaccine immunity?

People who are immunocompromised have weakened immune systems. According to the Centers for Disease Control and Prevention (CDC), this includes people who have diseases that affect their immune system.

“These COVID vaccines are not live vaccines,” Gulik says. “And so, a patient that is immunocompromised—no matter what degree of compromise—could take the vaccine.”

 Key Takeaways

  • Doctors recommend immunocompromised people receive the COVID-19 vaccine.
  • The Pfizer and Moderna COVID-19 vaccines do not contain live traces of COVID-19.
  • Depending on the person’s immunocompromised level, some may receive lower levels of immunity from the vaccine.

Featured AE Article~

Autoimmune Encephalitis in Late-Onset Seizures: When to Suspect and How to Treat

AE is an important etiology in late-onset seizures, and seizures may be the first symptom of AE. 

The results of our study provide clinicians with additional information verified in a great cohort of 225 patients on the prevalence and outcomes of AE as well as predictors of when to suspect AE in patients with late-onset seizures. Although characteristic signs of inflammation in AE may be lacking especially in elderly patients, the presence of CSF and MRI signs of inflammation, mesial temporal neuropsychological alterations, younger age, a known malignancy and specific semiological features (such as FBDS) should suggest AE. An epileptic seizure may be the first symptom of AE. In short, AB testing in CSF and sera, cerebral MRI, lumbar puncture, and neuropsychological testing for mesial temporal deficits should be part of the diagnostic protocol for AE following late onset seizures.


When Get up and Go is Gone. Lack of Initiation and Motivation after Brain Injury

Many IAES members have issues around motivation after having autoimmune encephalitis. This can be part of a result of remaining brain injury as well as memory issues. This article discusses the lack of motivation due to brain injury and may be helpful to members who continue to deal with it. Some suggestions are developing a routine, as well as using reminders on your phone or calendar.


Clinician’s Corner

Important updates in Autoimmune Encephalitis Testing From Mayo Clinic

Evaluations have been updated to reflect the many new antibodies that have been identified in AE and separating testing out for movement disorders. Andrew McKeon, M.B., B.Ch., M.D. of Mayo Clinic updates clinicians about evaluations in leading to a diagnosis. He offers suggestions on test utilization, why clinicians should move away from the paraneoplastic evaluation.

 The primary changes to the autoimmune evaluations include complete removal of striational antibodies and anti-calcium channel antibodies. Although N-type calcium channel antibodies sometimes accompany P/Q-type, and can be associated with cancer, it was determined, overall, that including N-type does not add sufficient value. In addition, P/Q-type antibody will only be used in the Lambert-Eaton syndrome and movement disorders evaluations. Alpha-3 ganglionic acetylcholine receptor antibody will be included in the autonomic dysfunction evaluations only.

COVID-19 Clinician’s Corner


A Case Report of Simultaneous Autoimmune and COVID-19 Encephalitis

COVID-19 encephalitis is a rare condition usually presenting with altered mental status. Simultaneous presence of anti-NMDAR antibody and SARS-CoV-2 virus in CSF is a very rare condition described in a few case reports so far. On the other hand, brain edema is an unusual presentation of anti-NMDAR encephalitis. Herein, we reported a case with simultaneous detection of anti-NMDAR antibody and SARS-CoV-2 virus in her cerebrospinal fluid (CSF) presenting with brain edema, altered mental status, seizures, and respiratory symptoms.

In this case, we had simultaneous presence of SARS-CoV-2 RNA and NMDAR antibody in CSF accompanied by generalized brain edema which is rare, and to our knowledge, it has never been addressed so far. Although many new insights have been provided in regard to the pathogenesis of COVID-19, precise interactions between virus-host cells remain unclear.

Autoimmune Encephalitis in Children: From Suspicion to Diagnosis

Highly recommended overview ~

This article reviews types of AE, causes, diagnosis, differential diagnosis, treatment and prognosis.

Conclusion: Autoimmune encephalitis is an inflammatory, rapid, progressive disease that requires extensive and prompt workup. Patients should have neuroimaging, EEG, lumbar puncture, and antibody testing on serum and CSF at the same time that treatment is initiated based on a clinical diagnosis. Further investigation is necessary in search of cancer, central nervous system inflammation, infection, and other alternative etiologies. Early diagnosis and effective treatment might improve long-term outcomes.

AE Trivia Playing Cards

The perfect companion for patients, Caregivers & Therapist

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

New Arrivals~Mugs and Hats

New arrivals ~ The AE Warrior Store has two new collections you will not want to miss. Caregivers, Advocates and AE warriors will appreciate receiving a mug or hat that raises awareness for autoimmune encephalitis. 

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.

Ways to Shop & Support IAES

Have you wanted to support IAES, autoimmune encephalitis awareness, and the AE Warrior that you love?

Have you found our services helpful? Do you want to make sure patients are identified early and that they and their loved ones receive the best help possible during this difficult journey?

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 that you can give $1 to IAES.

During the COVID-19 pandemic, it is vitally important that you #StayHome and practice #SocialDistancing. Grocery shopping, as well as all shopping, should be done online.

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.

The need for our services has increased exponentially. This simple act of kindness, will support the work we do and advance our ability to service the community.

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.

Exit mobile version