Autoimmune Encephalitis: From Symptoms to Treatment
When the Immune System Attacks Brain Tissue
Medically reviewed by Smita Patel, DO
Autoimmune encephalitis occurs when the immune system becomes disrupted and attacks a person’s own brain tissue. Encephalitis is inflammation or infection of brain tissue. It causes symptoms such as confusion, loss of control of physical movements, and seizures.
All types of encephalitis are considered uncommon, including autoimmune encephalitis. Causes may include cancer or inflammatory disorders. This article describes the causes, symptoms, diagnosis, and treatment of autoimmune encephalitis.
Photo composite by Lecia Landis for Verywell Health; Getty Images
Autoimmune Encephalitis: Brain Symptoms and Sensations
Autoimmune encephalitis can cause a variety of symptoms, depending on which parts of the brain are affected by inflammation.
Symptoms may include:
Confusion
Disorientation
Movement problems, such as tremors
Ataxia, which is impaired coordination
Changes in mood and lack of motivation
Dysregulated body temperature
Difficulty breathing
Weakness of one or both sides of the body
Lethargy (extreme sleepiness and fatigue)
Loss of consciousness
Unresponsiveness
You can have one or more of these symptoms, and the symptoms of encephalitis can begin suddenly and progress quickly.
Related: Rasmussen's Encepahalitis—A Type of Autoimmune Encephalitis
Diagnostic Delays in Autoimmune Encephalitis
Because encephalitis is not as common as other neurological conditions, such as stroke or multiple sclerosis, the diagnosis may be delayed. Sometimes, if there are indications of inflammation affecting the brain, a diagnosis of infectious encephalitis might be considered.
A lumbar puncture (spinal tap) might be performed to analyze the cerebral spinal fluid (CSF) that surrounds the brain and spinal cord. During this procedure, a healthcare provider carefully places a small needle in your back, below the level of the spinal cord. Sometimes, this procedure is done with imaging guidance.
The procedure can be slightly uncomfortable, but it is safe. It takes about 10 minutes for the CSF to be collected and then sent to a lab for analysis.
The results of a lumbar puncture can be especially helpful in diagnosing autoimmune encephalitis. The pattern of immune cells and the presence of any other material (such as infectious material or protein) can help identify whether you have encephalitis and what type of encephalitis you might have.
Effects of Undiagnosed Autoimmune Encephalitis
Undiagnosed and untreated autoimmune encephalitis can be fatal. Inflammation may cause persistent seizures, and it can lead to permanent brain damage.
Consequences of untreated autoimmune encephalitis may include:
Growth or spread of undiagnosed cancer if the autoimmune process is caused by cancer
Epilepsy (seizure disorder)
Stroke (blocked blood flow or bleeding in the brain) due to inflammation in the brain
Brain damage due to inflammation or bleeding in the brain
Persistent disorientation, confusion, or symptoms of psychosis (losing touch with reality)
Infections, such as pneumonia (lung infection), due to prolonged periods of physical inactivity
Muscle contractions due to prolonged periods of physical inactivity
Typically, symptoms can be treated before a definitive diagnosis is reached, which may prevent complications.
Autoimmune Encephalitis Treatment
The treatment of autoimmune encephalitis includes therapies directed towards controlling the immune dysfunction, as well as therapies that can help manage the symptoms. Usually, the symptoms (such as seizures or loss of consciousness) require prompt medical attention.
Hospitalization
During hospitalization for autoimmune encephalitis, respiratory support (supplemental oxygen or mechanical breathing) may be necessary, and antiepileptic medications are used for managing seizures. A comprehensive diagnostic evaluation would take place during the hospitalization.
The diagnostic evaluation may include:
Complete blood count (CBC): Can identify signs of an infection or an underlying inflammatory disorder
Electrolyte (mineral) tests: Can detect signs of organ dysfunction, which may occur due to certain types of cancer
Urinalysis: Can identify signs of an infection or kidney disorder
Antibody tests: Can identify antibodies (immune proteins) that can attack the brain
Blood tests for inflammatory markers: Can detect signs of immune disease, such as lupus or rheumatoid arthritis
Chest X-ray or computerized tomography (CT): Might be necessary if you are having difficulty breathing or signs of an infection
Brain imaging: Brain CT scan or brain magnetic resonance imaging (MRI) scan for signs of inflammation in the brain
Screening for underlying cancer: May include blood tests, imaging tests, or a biopsy (sampling of tissue to be analyzed in the lab)
Lumbar puncture: Removes CSF to be examined for signs of inflammation or infection
Medications
Medications that may be used to treat autoimmune encephalitis may include anti-inflammatory treatments, such as corticosteroids. These treatments may reduce the dysfunctional action of the immune system, preventing the immune attack on the body’s tissues.
Treatments to reduce the immune dysfunction can include:
Immunoglobulins (IVIG): This treatment involves infusing antibodies to improve your immune function.
Plasma exchange: This process involves blood exchange to remove unhealthy antibodies.
Steroids: A high dose of steroids can be used to reduce the effects of the immune system, which may stop or decrease the inflammation in the brain.
Chemotherapeutic medication: If autoimmune encephalitis is caused by cancer, sometimes chemotherapy is used to shrink the tumor.
Some people who have autoimmune encephalitis due to cancer may benefit from surgical removal of the tumor. Sometimes, when a tumor causing autoimmune encephalitis is surgically removed, the symptoms of encephalitis may resolve.
Symptomatic treatment may include antiepileptic medication for those with seizures, management of hormonal dysfunction for people experiencing hormone disruptions due to the immune process, management of blood pressure, and respiratory support with supplemental oxygen or mechanical breathing.
Specialists Who Work With Autoimmune Encephalitis Patients
Many healthcare specialists can help manage autoimmune encephalitis. The type of specialist you may need would depend on your symptoms and the cause of your condition.
Some specialists who might be involved in the care of autoimmune encephalitis include:
Oncologist: A doctor who diagnosis and treats cancer
Neurologist: A doctor who diagnosis and treats brain and nerve disorders
Rheumatologist: A doctor who diagnosis and treats inflammatory conditions
Physical therapist or occupational therapist: Specialists who can help with improving motor skills and self-care skills
Why Does Autoimmune Encephalitis Happen?
Autoimmune encephalitis means the immune system attacks a person’s brain tissue, causing inflammatory damage.
Researchers have suggested that this may occur when the immune system responds to some type of infectious or environmental particle or cancer cell that may resemble brain cells or other brain tissue. Then the immune system begins to attack healthy structures in the brain.
Autoimmune encephalitis caused by cancer is often referred to as a type of paraneoplastic syndrome.
Areas of the brain that can be targeted in autoimmune encephalitis are the cerebellum, cerebral cortex, or regions of the brain stem.
Causes vs. Risk Factors
It’s unclear why some people develop autoimmune encephalitis while others do not. Not everybody who has underlying risk factors, such as cancer, will develop autoimmune encephalitis.
Risk factors for autoimmune encephalitis include:
Cancer
Dysfunctional immune system
Underlying inflammatory disease
In addition to these risk factors, some people may develop autoimmune encephalitis without any identifiable cause. This would be described as an idiopathic condition.
Complications, Long-Term Effects, and Management
Some people may experience complications of autoimmune encephalitis. Complications may occur due to brain damage caused by inflammation. People may experience strokes due to interrupted blood flow or may experience bleeding in the brain due to inflammation.
Complications and long-term effects may include:
Weakness on one side of the body
Persistent coordination difficulties
Impaired speech
Seizures
Vision changes
Hallucinations or distorted thinking
Mood disorders
Cognitive changes, such as difficulty with memory, attention, concentration, or problem-solving
Managing the long-term impact of autoimmune encephalitis may require medication or therapies, such as physical therapy or speech therapy. If you have recovered from autoimmune encephalitis, you can expect to follow up with your healthcare team, even after the inflammatory process has resolved.
Once you have stabilized, you may be referred to a specialist focused on managing your long-term symptoms.
Summary
Autoimmune encephalitis is a condition in which the body’s immune system attacks healthy brain tissue. There are several potential underlying triggers for this type of immune system dysfunction, including cancer and inflammatory disorders. This condition can also be idiopathic (without an identifiable underlying cause).
A variety of neurological symptoms may be seen in autoimmune encephalitis because of disruption of brain function. Sometimes, this condition can be treated with medications that reduce immune system function or with treatments targeting underlying cancer.
This condition is not common, and diagnosis can be delayed. Treatment to manage symptoms such as seizures may be started during the diagnostic process.
Autoimmune encephalitis can lead to serious and long-term consequences, and it may be fatal. However, this condition can be treatable, and it is possible to have a complete or almost complete recovery with treatment.
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