If the combination of symptoms and test results suggest that a person has “possible AE”, the current recommendations are to start first line medications that treat inflammation in the brain, such as steroids, IV immune globulin, and/or plasma exchange. Right now, there are no research studies that tell your health care providers which of these medications, or which combination of these medications, should be used first. As a result, treatment may differ in AE patients based on their ages, how severe their symptoms are and where they are being treated.
In addition to the medications that act on the immune system to decrease brain inflammation, many people with AE will need supportive medications to treat the AE symptoms that cause suffering and disability. This group of treatments includes anti-seizure medications to reduce or prevent seizures, medications that decrease uncontrolled movements, medications that treat anxiety or depression, antipsychotic medications for patients who hallucinate, medications that improve attention, and medications that help with sleep.
As the results return from the workup for AE, including anti-neuronal antibody testing, health care providers will be able to decide if a patient meets the criteria for “definite AE” or “probable AE” and further treatment decisions will be discussed. For example, additional medications that act on the immune system may be recommended in some patients with AE. If the test results do not fit with a diagnosis of AE and a different diagnosis is more likely, initial treatments (e.g. steroids, IVIG or plasma exchange) may be stopped.
Some people with AE improve within days of receiving their first line treatments and don’t need additional medications that act on the immune system. Steroids and/or IVIG may be continued for several weeks to months, slowly decreasing the dose, to ensure that the brain inflammation stops. Other people with AE will need more medications that act on the immune system in order to stop the brain inflammation. These medications include rituximab, cyclophosphamide, mycophenolate mofetil, or azathioprine. These medications may be started early (within weeks of diagnosis) in patients who are not improving with first line treatments. Or, these medications may be started later if there are signs of ongoing inflammation or if the doses of first line treatments cannot be decreased without symptoms coming back.
Physical therapy, occupational therapy and speech therapy may be very helpful to improve recovery and may be started in hospital and sometimes continued in rehabilitation centers.
Starting treatment early seems to decrease long-term complications and the risk of relapse, as well as lead to faster recovery. If untreated, AE can cause progressive neurologic decline and even death. Some people with AE respond very quickly to the first medications that are given and they do not require stronger medications or a prolonged treatment course. Others will need intense medication regimes and a long duration of treatment. Right now, there are no symptoms, signs or test results that tell the doctors in advance how a patient with AE will respond to treatment.
Sometimes, medications that treat the symptoms of AE don’t work well when a person is first diagnosed, but doctors may recommend trying them again later after the brain inflammation has decreased because they may work better. Also, medications and/or medication doses may need to be changed often when a patient is very ill because of AE.
Lancaster, The Diagnosis and Treatment of Autoimmune Encephalitis, J Clin Neurol, 2016 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4712273/
Shin et al., Treatment strategies for autoimmune encephalitis, Ther Adv Neurol Disord, 2017 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5784571/
Many patients need medications to help with behavioral and psychiatric symptoms of AE and they are an important part of AE treatment. However, there are key considerations that affect decisions about the timing and choice of medications. Early after diagnosis, especially in NMDA receptor encephalitis, antipsychotics may worsen symptoms. Also, antipsychotics need to be avoided in people with catatonia. But, antipsychotics may be helpful for patients later in their disease course who still have symptoms of psychosis or agitation. Other medications to help with mood, anxiety, agitation, and attention are frequently needed throughout the disease course.
It may be helpful for patients with AE to have a psychiatrist on their treatment team to discuss the different reasoning, risks and benefits of available psychiatric medications.
Some people are afraid to start a psychiatric medication because they worry that people will think they or their child has a psychiatric disease. Being on these medications does not change the diagnosis – AE is the reason that behavioral or psychiatric symptoms developed.
Patients can have psychiatric symptoms both in the active inflammatory and the recovery stages of AE. For some, these symptoms may never fully go away. If a person has psychiatric symptoms before they had AE (such as depression, anxiety, or attention deficit hyperactivity disorder), those symptoms are unlikely to get better with immunotherapy and the added stress on the brain from AE may even make those symptoms worse.
After initial diagnosis and treatments, both adults and children with AE enter the period of ongoing treatment and recovery. Some people with AE may respond quickly to treatment. But it can take several months for many patients to show improvements, especially if they were not responsive to first line treatments. For patients with more severe disease or disease that does not respond well to treatments, ongoing hospitalization, long stays in rehabilitation hospitals, or constant supervision at home may be needed.
This is a challenging stage of disease for patients with slow recoveries and those with major behavioral changes or psychiatric symptoms. AE patients with marked psychiatric symptoms may be deemed too “high risk” to go to rehabilitation centers, but “not sick enough” from a medical perspective to require ongoing hospital care. Caregiver fatigue, frustration with the slow improvement, and questions about what treatment changes may be needed can become overwhelming. Support systems become strained with the duration and intensity of this disease.
As patients improve and become more aware of their illness and their limitations, additional symptoms of frustration, anxiety, depression and aggression can develop. It can be hard to tell whether these new symptoms are because of a flare of disease or because of the significant stress experienced by patients and their caregivers. Close follow-up with health care providers is important since changes in treatments may be needed.
Slow re-introduction to activities and people is often preferred. Recovery often takes many months, and initial efforts should focus on daily activities and interacting with close family and friends before returning to work or school. Both mental and physical fatigue is common. Children and adults generally do best with a slow, steady increase in time and intensity of work or school. Frequent breaks are often helpful.