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http:// William Gallentine, DO Assistant Professor, Pediatrics – Division of Child Neurology Heather Van Mater, MD MS Assistant Professor, Pediatric Rheumatology

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a utoimmune encephalitis
William Gallentine, DO

Assistant Professor, Pediatrics – Division of Child Neurology

Heather Van Mater, MD MS

Assistant Professor, Pediatric Rheumatology

Xavier Preud’homme, MD

Assistant Professor, Internal Medicine & Psychiatry

Autoimmune Encephalitis
clinical features of patients with a nti nmdar encephalitis at 1 month n 577
Clinical Features of Patients with anti-NMDAR encephalitis at 1 month (n=577)

Titulaer MJ et al. Lancet Neurol 2013; 12:157-65

may mimic p sychiatric disorders
Patients with NMDAR encephalitis (as well as many other types of autoimmune encephalitis) most commonly present with psychiatric symptoms which may mimic common disorders like schizophrenia and bipolar disease May mimic psychiatric disorders
psychiatric symptoms presenting along with symptoms inconsistent with primary psychiatric diagnosis
The presence of non-psychiatric signs such as seizures, movement disorder, cognitive impairment, language loss, or decreased level of consciousness should serve as red flags indicating further evaluation Psychiatric symptoms presenting along with symptoms inconsistent with primary psychiatric diagnosis
recommended workup

Serum inflammatory markers (ESR, CRP)

Serum autoantibodies (NMDAR and paraneoplastic, thyroid)

MRI with and without contrast


Strongly consider lumbar puncture

CSF autoantibodies (NMDAR and paraneoplastic)

OP, cell count, glucose, protein, oligoclonal bands

Recommended Workup
take home points
Providers should have a low threshold for further evaluation for these disorders

Because autoimmune encephalitis is highly responsive to immunotherapy (especially IF TREATED EARLY in the course!!).

Take home points
The cornerstone of treatment is immunomodulatorytherapy



IV immunoglobulin (IVIG)

Oral immunosuppressents including Cellcept


Cyclophosphomide (Cytoxan)

Removal of tumors – for example, ovarian teratoma in anti-NMDAR encephalitis

Management of symptoms while waiting for immunomodulatory therapy to work (e.g., for seizures, agitation)

prognosis of anti nmdar encephalitis
6% mortality

81% had good outcome at 24 month f/u

Initial signs of improvement 4 weeks in 53%

In non-responders to 1st line therapy, only 57% received 2ndline therapy, even though 2nd and 3rd line immunotherapy resulted in significantly better outcomes

Relapses: 12% relapse risk in 2 years

2/3 of relapses less severe than initial

Those that received 2nd tier agent on initial presentation had fewer relapses

Predictors of good outcome:

Early treatment

For first-line failures, administration of 2nd line agent

Prognosis of anti-NMDAR encephalitis

Titulaer MJ et al. Lancet Neurol 2013; 12:157-65

Titulaer MJ et al. Lancet Neurol 2013;12:157-65

take home points1
Initiate treatment as soon as possible

Recovery occurs over months

Average of 14 months for anti-NMDAR encephalitis

If first line treatment fails, move to second line treatment

Take Home Points
overcoming our cognitive biases

This is medical so it is not psychiatric…or

This is purely behavioral…

Must recognize and manage the cluster of signs constituting catatonia

Agitation, inappropriate laughter or crying, enuresis, drooling, movement disorder, autonomic instability, etc.

Benzodiazepine (Ativan) rather than antipsychotics

CURSE of KNOWLEDGEIf it is catatonia then it is a psychiatric disorder

STEREOTYPICAL BIASYoung female must be psychiatric…Positive Family history of psych D/O must be same

Overcoming Our Cognitive Biases
a call to action
Listen to patients, listen to parents

Evaluation and treatment requires multidisciplinary teams

Support Dr. Gallentine and Dr. Van Mater’s Duke Children’s Autoimmune Brain Disease Program to be a Center of Excellence for the treatment of autoimmune encephalitis

Develop a similar Duke program for adults

We have founded the Autoimmune Encephalitis Alliance and are launching a Autoimmune Encephalitis Centers of Excellence Campaign to

Establish clinical standards of care across medical disciplines

Coordinate basic and clinical research efforts

Raise awareness / connect families so nobody faces autoimmune encephalitis alone

First fund raising event: Florence Forth – Fundraising Run, March 2, 2013. Information at:

A Call to Action