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Doctor’s Information

The most common type of Autoimmune Encephalitis, anti-NMDA receptor Autoimmune Encephalitis (ANMDARE) which accounts for 20% of cases, was identified in 2005.  Even though The California Encephalitis Project 2007-2011 showed this variant of autoimmune encephalitis occurred more commonly than other etiologies of encephalitis such as enterovirus encephalitis, HSV-1, West Nile Virus or Varicella zoster virus (VZV), many Emergency Department physicians are not familiar with it.  A new specialty, autoimmune neurology, has evolved for clinicians fascinated with this area of medicine. Emergency Departments do evaluate for encephalitis and meningitis but autoimmune encephalitis often goes unrecognized.

Given that Autoimmune Encephalitis is more common than originally thought, Emergency Department Staff, would be well advised to keep autoimmune encephalitis as a differential diagnosis for patients presenting with acute psychosis or encephalitis. This may be particularly important in patients with new onset psychosis being referred to a psychiatric facility, especially if they presented with any neurological symptoms. Psychiatrists and Emergency Room Physicians are often the first to see these patients, data shows that due to the wide spectrum of initial symptoms in AE patients, the correct diagnosis of these patients is often missed or delayed. Therefore, lumbar puncture should be offered to ALL patients with new onset psychosis as part of the broader evaluation for their mental and physical health. 

Autoimmune encephalitis is a treatable disease, and it responds better to early treatment. There are numerous co-hort studies and case reports of patients who demonstrated improvement immediately following immunosuppressant therapy or tumor removal.  Studies have found that favorable outcome is associated with early treatment.  To learn more about Autoimmune encephalitis masquerading as psychosis/psychiatric illness, we highly recommend Psychiatrists, ER physicians and those learning about these disorders review the research under AE -Psychiatric presentation.

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Recent advances in Psychiatry

Growing evidence derived from cerebrospinal fluid (CSF), neuropathological, imaging, genetic, and epidemiological studies link neuroinflammation and immune dysregulation to a subset of individuals with a variety of severe mental disorders (SMDs), including affective and non-affective psychotic disorders.

Standard Operating Procedure

This SOP ( From – SOP: antibody-associated autoimmune encephalitis), aims for facilitating the approach to patients with central nervous system symptoms suspicious of AE, for raising awareness of the clinical presentation of such patients, and for proposing the necessary diagnostic and therapeutic steps. 

Click to see the full size table of Most important antibodies and clinical syndromes. 

SOP_antibody-associated autoimmune encephalitis_Pruss_diagnosis_dx

Neuroimmunology: Updates and antibody test utilization

Mayo Clinic 2021

As more antibodies in autoimmune encephalitis have been identified and with the increased knowledge about this group of diseases, Sean Pittock, M.D., reviews how Mayo Clinic has updated their antibody panel evaluations (Aug-31-2021) and put them in phenotype-specific categories so more patients will be identified and testing will be more targeted, efficient and more cost effective for the patient. He also discusses the role paraneoplastic testing can play to avoid delays in diagnosing and improving efficiency for the laboratory. Proper utilization of the phenotype-specific test menu improves specificity, offers physicians a definitive diagnosis, and shortens the patient’s journey.

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.

These updates are being made on a solid foundation of research.

Commercial tests for autoantibodiesto NMDAR, LGI1, Caspr2, AMPAR (GluR1, GluR2 subunits), and GABA-B-R are widely available. Newer cell surface antigens like GABAA-R and DPPX are more difficult to test clinically.  Contact Mayo Clinic Rochester, MN to inquire about antibody testing through research resources. It is important to test both the serum and the Cerebral spinal fluid (CSF) because of false positives with these antibodies.

Novel Auto-antibody in Neuroimmunology

Recommended Reading for Clinicians

Autoimmune Encephalitis and Related Disorders of the Nervous System-book-Dalmau-Graus

The first medical text for Autoimmune Encephalitis every clinician should have in their library! By Cambridge press and available on Amazon.

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 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. Gain full HTML access of the whole book, including supplementary videos, via a scratch-off code inside the cover.

Neuroimmunology Piquet

This book provides a clinical focus on neuroinflammatory diseases as well as a review in pathophysiology and treatment approaches.

Organized into six parts, the book begins with a basic review of the immune system and concepts for learning and treating neuroimmune conditions. The next four sections cover specific subfields of neuroimmunology and autoimmune neurology – the clinical and diagnostic features of multiple sclerosis, other autoimmune conditions of the central nervous system, autoimmune conditions of the peripheral nervous system, and systemic autoimmune conditions that affect the nervous system. To conclude, Section six discusses various clinical approaches to specific presentations in neuroimmunology, including pediatric demyelinating diseases.  These sections provide practical clinical information to improve the reader’s knowledge in this complex field. The chapters are written by world renown authors with extensive knowledge to help provide up to date information. The full scope of autoimmune neurology is discussed, which is a unique feature of this book.

Neuroimmunology serves as a resource for those in training including residents and fellows to provide clear clinical reasoning and background in a rapidly advancing field.

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AI-Driven Antibody Search.  Let our AI decode the literature to provide antibody usage data that’s unbiased and experiment-specific

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Autoimmune encephalitis: proposed recommendations for symptomatic and long-term management Part 1

Autoimmune encephalitis: proposed best practice recommendations for diagnosis and acute management Part 2

One-Year Functional Status (NEOS) score

AE is refractory to Antipsychotics

Antibody Mediated Psychosis

Treatment of Autoimmune brain disorders

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Medical Alert ID Card

Emergency Medical Alert ID Card for AE Warriors (Click image to print)

Andrew McKeon, M.B., B.Ch., M.D.,

Mayo Clinic Webinar:
The Spectrum of Autoimmune CNS
Disorders June, 2017

#COVID19 'World vs Virus'

Dr Matt McCarthy, David Duong, Todd Pollack of Harvard and John Rafferty discuss life in lockdown.Clinical Approach to Diagnois of Autoimmune Encephalitis

Dr David Carr

Anti-NMDA receptor Encephalitis
A must know diagnosis for all
emergency medicine practitioners

Practice Current:

Autoimmune Encephalitis &
the Role of Antibody Testing
March, 2019

Lancet Podcast

Clinical Approach to Diagnois of Autoimmune Encephalitis

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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