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.
Learn more about the different treatments