Introduction of Autoimmune encephalitis for Non medical professionals and mental health professionals work in neurology. Reference provided in last slide and prepared of self learning purpose not for any commercial purpose.

1. Autoimmune Encephalitis
Presenter : Arun.S
Chairperson: Dr. Priya Treesa Thomas
15 JULY 2017

2. Introduction
Autoimmune encephalitis (AE) can produce a very wide range of neuro-
psychiatric symptoms. A major challenge in diagnosis is that different
symptoms may appear at different times and different levels of
intensity, so that the disease may mimic many other disorders. Some
patients initially present with either neurological or psychiatric
symptoms, further complicating diagnosis.
The disease occurs in men, women and children of all ages, but it has
historically been diagnosed most frequently in young women. An older
study suggests that out of 100-plus known autoimmune diseases, 75%
of people affected are female.

3. “autoimmune encephalitis” appears in the medical literature in the
1970’s and 1980’s, the first specific AE antibody was identified in 2005
when Dr. Josep Dalmua described the anti-NMDA-receptor encephalitis
type. The field of AE has expanded rapidly since then. Now there are
more than fifteen known types of AE, including auto-antibodies
directed against NMDA, LGI1, CASPR2, VGKC-complex antibodies,
AMPA, and GABA.

4. Symptoms associated with AE
• weakness or numbness of part of
the body
• loss of balance
• slowed or blurred speech or loss of
ability to speak
• Ataxia
• involuntary movements
• distorted vision
• cognitive impairment
• memory disturbance
• decreased level of consciousness –
to the point of unresponsiveness,
catatonia or coma
• seizures – (either self-evident, or
smaller seizures that show up on
an EEG reading)
• partial or complete loss of appetite
for long periods
• food and drink tasting inedible or
triggering nausea
• excessive eating without feeling
sated

5. Symptoms associated with AE
• agitation
• inability to sleep
• loss of inhibition
• rapid, pressured, or involuntary speech
• hallucinations (visual or auditory) and delirium
• paranoid thoughts
• severe anxiety

6. establish a diagnosis of AE
• This lead many doctors to rely on testing for the presence of antibodies (i.e. anti-
NMDA receptor antibodies) or diagnosing by process of elimination and
diagnosis-through-treatment. For example, if test results for all other diseases are
negative and the disease symptoms improve with immune-modulation therapy.
• In 2016, a Lancet Neurology article by Dalmau et al provides the first broadly
accepted diagnostic criteria for autoimmune encephalitis. The article was
followed by a precis that provides a series of panels for use in diagnosing AE.
Among the key findings of the new criteria:
• the diagnosis criteria does not rely on antibody status;
• response to immunotherapy not a focus of the diagnostic criteria;
• the initial diagnostic assessment should be conducted rapidly to allow early
initiation of immunotherapy; and
• the diagnostic criteria should be applied with caution for children, especially
those less than 5 years old

7. Anti-NMDA Receptor Encephalitis
• This disorder has become a leading cause of autoimmune encephalitis
in children and adolescents, with 40% of patients being younger than
age 18 years.
• The syndrome is highly predictable in adults and teenagers and
usually evolves in stages, including a prodromal phase of fever,
headache, or viral-like symptoms that often goes unnoticed.
• This is followed within a few days or weeks by the onset of psychiatric
and behavioral problems including anxiety, bizarre behaviors,
paranoid thoughts, grandiose or hyperreligious delusions, and
insomnia that progress to decreased level of consciousness, seizures,
dyskinesias, choreoathetoid movements or postures, and breathing
or autonomic instability.

8. Limbic Encephalitis
• This disorder refers to an inflammatory process of the limbic system,
including the medial temporal lobes, amygdala, and cingulate
gyrus.25,32 Despite being one of the best clinically and radiologically
characterized autoimmune encephalitis, it is also one of the most
frequently misdiagnosed

9. Hashimoto Encephalopathy
• This is an ill-defined disorder in which the syndrome and
immunological findings are unclear. There are approximately 30
pediatric cases reported in the English literature.51–55 In a review of
25 patients, 52% had hypothyroidism and 48% normal thyroid
function; however, some patients can develop hyperthyroidism.
• For this reason and because not all patients respond to steroids, the
term encephalopathy associated with autoimmune thyroid disease is
considered more accurate than the previous steroid-responsive
encephalopathy associated with autoimmune thyroiditis. 5

10. Rasmussen Encephalitis
• This is an inflammatory encephalopathy characterized by progressive
refractory partial seizures, cognitive deterioration, and focal deficits
that occur with gradual atrophy of one brain hemisphere.
• The disorder frequently presents in 6- to 8-year-old children, although
adolescents and adults can be affected.
• The etiology is unknown and, therefore, multiple theories have been
proposed

11. Other Encephalitis Associated With Epilepsy
or Status Epilepticus
• The discovery of treatment-responsive encephalitis associated with
antibodies against cell surface or synaptic proteins has resumed the
interest for a potential autoimmune basis of several devastating
encephalopathies with refractory seizures that are suspected to be
induced by fever or an inflammatory process.
• Some of the terms used to describe these disorders include “Acute
Encephalitis with Refractory Repetitive Partial Seizures (AERRPS),”
“Fever-Induced Refractory Epileptic Encephalopathy Syndrome
(FIRES),” and “Devastating Epilepsy of School-aged Children (DESC).

12. Causes of AE
• The direct cause of most cases of AE remain unknown. However the
following have been shown to trigger AE:
• a teratoma ( a type of tumor, generally found in the ovaries);
• the presence in the body of a cancer, that indirectly triggers an
autoimmune response (this is called a “paraneoplastic syndrome”)
• exposure to certain common bacteria, including, but not limited to,
streptococcus and mycoplasma pneumonia, with or without active
infection.

13. outcomes
• The 2013 Lancet Neurology article reviewed the study of 577
patients with AE reported that 53% of patients who received
immunomodulation therapy showed improvement within 4 weeks.
• 81% of patients showed substantial or complete recovery. On
average, patients continued to improve for 14 months after onset of
acute AE.
• 12% of patients who recovered from a first acute episode had at least
one relapse in the next two years.
• Overall mortality associated with the disease was approximately 6%.
[Note that this study is limited to one type of AE – anti-NMDA-
receptor antibody encephalitis].

14. most common treatments for AE
First-line treatments Second line” treatments
• “Second line” treatments—
immunosuppressant drugs—should be
started promptly if first-line treatments fail to
improve symptoms. The three most
commonly used drugs are:
• Rituximab
• CellCept
• Cytoxan (cyclophosphamide
• As soon as a patient is diagnosed with AE,
they should receive one or more of the four
(4)
• removal of a teratoma (if present) that could
be triggering the autoimmune response
• steroids to reduce immune response and
inflammation
• plasmapheresis to remove harmful antibodies
from blood
• intravenous immunoglobulin (IVIG), which is
believed to occupy the binding sites where
harmful antibodies attach to brain cells.

15. Reference
• https://aealliance.org/patient-support//symptoms/
• https://www.theatlantic.com/magazine/archive/2016/07/when-the-
body-attacks-the-mind/485564/
• https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3705178/pdf/nihms
484905.pdf
• https://www.encephalitis.info/support/information/practical-
resources-on-encephalitis/types-of-encephalitis/types-of-
autoimmune-encephalitis/acute-disseminated-encephalomyelitis-in-
children/