http://videos.simonandschuster.com/SusannahCahalans-Month-of-Madness/1937884129001
http://www.youtube.com/watch?v=Najj0aVLJwU
Autoimmune 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
Clinical Features of Patients with antiNMDAR encephalitis at 1 month (n=577)
Titulaer MJ et al. Lancet Neurol 2013; 12:157-65
May mimic psychiatric
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
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
Recommended Workup
• At the outset (DO NOT HAVE TO WAIT FOR
NEUROLOGY CONSULT!!)
Serum inflammatory markers (ESR, CRP)
Serum autoantibodies (NMDAR and paraneoplastic,
thyroid)
MRI with and without contrast
• EEG
• Strongly consider lumbar puncture
CSF autoantibodies (NMDAR and paraneoplastic)
OP, cell count, glucose, protein, oligoclonal bands
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!!).
Treatments
• The cornerstone of treatment is immunomodulatory
therapy
• Steroids
• Plasmapheresis
• IV immunoglobulin (IVIG)
• Oral immunosuppressents including Cellcept
• Rituximab
• 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 2nd line 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
Titulaer MJ et al. Lancet Neurol 2013; 12:157-65
Titulaer MJ et al. Lancet Neurol 2013;12:157-65
Take Home Points
• Initiate treatment as soon as possible
• Recovery occurs over months
• Average of 14 months for antiNMDAR encephalitis
• If first line treatment fails, move to
second line treatment
Overcoming Our Cognitive Biases
• ESSENTIALISM
• 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 KNOWLEDGE
If it is catatonia then it is a psychiatric disorder
• STEREOTYPICAL BIAS
Young female must be psychiatric…
Positive Family history of psych D/O must be same
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: www.florenceforth.org