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

Selected Highlighted News in the field of Autoimmune

Encephalitis October 2019 2nd edition

• Announcements: IAES represents Autoimmune Encephalitis at NORD Summit, Dr. Sarosh Irani Honored
• Most Popular Article of the Month: Tourist memory “Wiped”
  
• Most Popular Podcast of the Month:  Autoimmune Encephalitis & the role of Antibody testing
  
• STUDY Needs Your Participation: Understanding Consequences of Encephalitis
  
• Most Shared Post: FAQs Friday- My doctor found a teratoma. What is it and what should I expect?
  
• Most Popular Poem: Dr. Seuss on Autoimmune Encephalitis
• Clinician’s Corner: An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models
  
• Open Access:  Innate immunity in the Central Nervous System. A missing piece of the Autoimmune Encephalitis Puzzle.
  

We believe THE TIME IS NOW to drive innovation and collaboration to develop stronger resources, better outcomes and find cures for the rare disease community. 

IAES President, Tabitha Orth and Vice-President Gene Desotell attending the Washington D.C. conference to enhance our ability to fight for patients with autoimmune encephalitis and their loved ones. 

Congratulations to Dr. Sarosh Irani of Oxford Autoimmune Neurology Group. Dr. Irani was honored today by the Royal College of Physicians as the recipient of the Graham-Bull Prize in clinical science!
Sarosh is a dear friend and supporter of IAES and is a leading researcher in the field Autoimmune Encephalitis and Autoantibodies in Neurological diseases: discovery towards treatment and cause.

Most Popular Article of the Month~

Tourist’s Memory ‘Wiped’ Nataya fights for her future after rare disease robs her of her past

Read the story of a french backpacker, Nataya Damarchio age 29 who entered coma with a mysterious illness. She was diagnosed with autoimmune encephalitis.

This article is extremely well written and informative. A compelling story with Autoimmune Encephalitis well defined within.

It is estimated that 3,500 in Australia have the diagnosis.

Dr. Mastura Monif, who treated Nataya is heading research in Australia on Autoimmune Encephalitis. Learn more about that work.

Most Popular Podcast of the Month~

Autoimmune Encephalitis & the role of Antibody Testing

Dr. Luca Bartolini discusses autoimmune encephalitis and the role of antibody testing based on a Practice Current survey run between November 2017 and May 2018.

Study needs Your Participation: Understanding Consequences of Encephalitis

Most Liked and Shared IAES Post

FAQs Friday~ My doctor found a teratoma. What is it and what should I expect?

A teratoma is a tumour that may contain several types of body tissue. A person’s antibody attack on the brain can be a reaction to the presence of an ovarian or testicular teratoma.

The Anti-NMDA receptor encephalitis is associated in some cases with ovarian pathology, in particular teratomas. It is considered, that the antibodies that are attacking (the NR1-NR2 subunits of the NMDA subtype of glutamate receptors) the person’s healthy brain cells, have developed, in response to this abnormal tissue.

Ovarian tumors, primarily ovarian teratomas occur 59% cases in anti-NMDAr encephalitis. Teratomas are found most commonly in women between age 12 and 45 and in patients in Asian or African American descent. Testicular teratoma is common. Other germ-cell and rarely non-germ cell tumors have also been described in association with anti-NMDA-receptor encephalitis.

The removal of any identified teratoma takes place first and then first-line immunotherapy treatment typically corticosteroids, intravenous immunoglobulins or plasma exchange is administered. In one series of patients with anti-NMDA-receptor encephalitis, five out of six patients with an ovarian teratoma who did not undergo surgery died. In contrast removal of the tumor may be curative in approximately 80% of patients who undergo tumor removal and immunosuppressive treatment, substantial neurological improvement occurs. Symptoms of anti-NMDA-receptor encephalitis markedly improved within one month of tumor removal and immunosuppressive treatment, though recovery can continue for up to 24 months.

Treatment is generally thought to be more effective in patients who have underlying tumor removed. Cases of ovarian teratomas discovered years after initial onset of symptoms have been described particularly in patients who experienced a slow recovery. A case was reported where oophorectomy was performed despite negative scan results (MRI, abnormal Ultrasound, CT) and on postoperative biopsy an occult teratoma was revealed, was subsequent improvement in clinical symptoms. Patients may require prolonged monitoring for development of teratoma.
OVERVIEW
· Anti-NMDA Receptor Encephalitis is an under-recognised progressive neurological disorder caused by antibodies against NR1-NR2 NMDA receptors
· 60% of patients with anti-NMDA receptor encephalitis have the presence of a tumor (most commonly teratoma)
CLINICAL PRESENTATION
· Up to 90% of patients are female
· The disorder typically progresses over 1-2 weeks
· An initial non specific flu-like prodrome (sub-febrile temperature, headache, fatigue)
· Followed by a psychotic stage with bizarre behaviour, disorientation, confusion, paranoid thoughts, visual or auditory hallucinations and memory deficits
· In the following phase, decreased consciousness, hypoventilation, lethargy, seizures, autonomic instability and dyskinesias develop
· The evidence of a tumour can be present (e.g. pelvic teratoma)
DIFFERENTIAL DIAGNOSIS
Diagnosis is often delayed due to resemblance to other conditions such as:
· Infectious encephalitides (particularly HSV and HHV-6)
· Other autoimmune etiologies (e.g. limbic encephalitis due to autoantibodies against Hu, Ma2, CV2 and amphiphysin)
· Neuroleptic malignant syndrome
· Lethal catatonia
· Cerebral space-occupying lesions
· Metabolic disorders – hyper/hypothyroidism, Cushing syndrome, Addison disease
· Psychiatric disorders – schizophrenia, psychotic depression, pseudo-seizures
· Drugs, toxins or withdrawal (e.g. ketamine, PCP, dextromethorphan, antipyschotic-induced movement disorders, etc)
INVESTIGATIONS
· NR1 and NR2 antibodies in CSF combined with a characteristic clinical picture have 100% sensitivity and specificity
· pelvic ultrasound
· further tests to exclude other causes of encephalopathy
MANAGEMENT
· Early removal of tumor if present
· Immunotherapy includes consideration of corticosteroids, intravenous immunoglobulin and plasma exchange therapy in severe cases
· supportive care and monitoring
PROGNOSIS
· Early identification and removal of tumor is associated with better outcomes (<4 months from symptom onset)
· 47% Full Recovery
· 28% Mild stable deficits
· 18% Severe deficits
· 7% Death
Reference for this post is from Anti-N-methyl-D-aspartate receptor encephalitis: review of clinical presentation, diagnosis and treatment 2015. Anti-NMDA-receptor encephalitis: A neuropsychiatric syndrome associated with ovarian teratoma 2015. And Anti-NMDA Receptor Encephalitis by Dr Chris Nickson, last updated April 17, 2019 in Life in the Fast Lane.

Most Popular Poem

You have brains in your head, you most certainly do

100 billion neurons are there, give or take one or two.

One rainy day when the weather was shoddy,

My immune system rebelled and made a strange antibody.

As quick as a shot from my head to my toes, my thinking and feeling went haywire to ‘anything goes’.

My seeing, my walking, my talking, MY WORD,what I heard, what I saw, what I thought was absurd! 

That antibody doubled, it tri-multi-zooppled!

Making an army of friends ~ all uninvited~ who hitch rides on my neurons getting them over excited!

Zipping this way and that way around in my brain

those antibodies zapped me causing terrible pain.

My poor brain it was not feeling well. My poor brain felt insane and it started to swell!

To the Doctor I went (for my thinking was spent).

I got this test and that test and a Brain selfie too

As my Doctor, called another Doctor, who called another he knew.

They all came to see me, to try and figure out what was wrong in my head?

What was this fuss all about?

“Could it be something rare? Could it be something quite new?”

Came a voice from the doorway where all eyes suddenly flew.

It was Dr. Dalmau stopping in for a look

All the Doctors were quiet, as this man wrote the book.

“I agree when you say it is not meningitis, this patient has Autoimmune Encephalitis!

By: Tabitha Andrews Orth

Clinician’s Corner

An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models

This update by Dr. Dalmau and  colleagues is one IAES particularly favors as it is explicitly directed to psychiatrists as well as neurologists

The identification of anti-NMDA receptor (NMDAR) encephalitis about 12 years ago made it possible to recognise that some patients with rapidly progressive psychiatric symptoms or cognitive impairment, seizures, abnormal movements, or coma of unknown cause, had an autoimmune disease. In this disease, autoantibodies serve as a diagnostic marker and alter

NMDAR-related synaptic transmission. At symptom onset, distinguishing the disease from a primary psychiatric disorder is challenging. The severity of symptoms often requires intensive care. Other than clinical assessment, no specific prognostic biomarkers exist. The disease is more prevalent in women (with a female to male ratio of around 8:2) and about 37% of patients are younger than 18 years at presentation of the disease. Tumours, usually ovarian teratoma, and herpes simplex encephalitis are known triggers of NMDAR autoimmunity. About 80% of patients improve with immunotherapy and, if needed, tumor removal, but the recovery is slow.

Innate Immunity in the Central Nervous System: A Missing Piece of the Autoimmune Encephalitis Puzzle?

While a number of the important interactions between the innate, adaptive and neural components in CNS autoimmunity and neuroinflammation have been well-studied, there remains significant gaps in our knowledge. AE provides a unique disorder which can assist us in understanding the mechanisms of CNS autoimmunity and its genesis. In particular the role of dysregulated innate cell activity in driving autoreactive lymphocyte proliferation and maturation to immunoreactive lymphocytes. This will also provide us with potential improvements in diagnosis and treatment of AE, as well as other CNS autoimmune diseases.

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.

Your donations are greatly appreciated. Every dollar raised allows us to raise awareness and personally help Patients, Families and Caregivers through their Journey with AE so that best outcomes may 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.