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Autoimmune Encephalitis

What is Autoimmune Encephalitis?

AE is a serious medical condition in which the immune system attacks the brain, impairing function.  While AE may be triggered by an infection, many cases have an unknown cause. Depending on what part of the brain is affected, and which part of the immune system is activated, the symptoms can vary broadly. AE is a severe condition, with an approximately 6% mortality rate. While rapid diagnosis and appropriate treatment allows many patients to recover most or all functions, a significant percentage of patients have persistent symptoms that negatively impact their ability to function.

What are the most common symptoms of AE?

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.

Symptoms commonly associated with AE can include:

  • cognitive impairment

  • memory difficulties

  • seizures

  • involuntary movements

  • slowed or loss of ability to speak

  • behavioral changes such as agitation

  • loss of inhibition

  • hallucinations (visual or auditory) 

  • paranoid thoughts

  • severe anxiety

  • sleep disruption including severe insomnia

  • decreased level of consciousness – to the point of unresponsiveness, catatonia or coma

  • weakness or numbness of part of the body

  • loss of balance

  • vision changes

Although just one of these symptoms does not imply autoimmune encephalitis, an otherwise unexplained mixture of the above neuro-psychiatric symptoms may be a clue that the underlying cause is autoimmune encephalitis.

Are you more likely to be diagnosed with AE due to age or gender?

The disease occurs in men, women and children of all ages, but it has historically been diagnosed most frequently in young women. This is similar to other autoimmune diseases, where women are more commonly affected. An older study suggests that out of 100-plus known autoimmune diseases, 75% of people affected are female. However, it is important to recognize males also get autoimmune diseases and not overlook their presentations.

What are the most common treatments for AE?

As soon as a patient is diagnosed with AE, they should receive one or more of the four (4) first-line treatments.

  1. removal of a teratoma (if present) that could be triggering the autoimmune response

  2. steroids to reduce immune response and inflammation

  3. plasmapheresis/plasma exchange to remove harmful antibodies from blood

  4. intravenous immunoglobulin (IVIG), which works in many different ways to increase removal of antibodies, inhibit binding of the harmful antibodies, and decrease the inflammatory response to antibodies.

Is AE related to PANDAS, and if so, how?

PANDAS (Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcal infection) is a disease that results in psychiatric symptoms, including obsessive-compulsive behaviors, that is triggered by a strep infection. PANS (Pediatric Acute-onset Neuropsychiatric Syndrome) is the broader category of infection induced . Small clinical trials have not shown consistent response to immunotherapy for PANDAS, but ongoing studies are underway. How PANDAS/PANs relates to AE is an area of ongoing exploration. While some feel PANDAS/PANS is on a continuum with AE, others feel they are distinct entities. Currently, there are different diagnostic criteria and treatment recommendations for PANS/PANDAS and AE.

How can I determine whether someone I’m caring for has AE or not?

If you suspect that you or a family member may have AE, you should consult your primary care physician in addition to specialists in neurology, psychiatry, immunology, or rheumatology. Workup should include a thorough history and physical exam to determine if the clinical course could be consistent with AE. If presentation is consistent, then tests including MRI, EEG, spinal tap and blood tests for known antibodies, and in some cases scans for teratomas or other cancers should be strongly considered. At least fifteen different types of autoimmune encephalitis have been identified in the laboratory. 

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Content sighted from: https://aealliance.org/

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