The widely-read journal Nature Reviews, in its specialized Neurology publication recently featured an article with a summary of the current state of knowledge and research related to neuroimmune illnesses that occur in children, including autoimmune encephalitis. Over a dozen experts collaborated on this paper. A sample of interesting points, which also largely apply to adults includes the following:
- There are many different immune-mediated neurological diseases, all with various causes and manifestations. What they have in common is the immune system not functioning properly and interfering with the central nervous system. These diseases are probably more prevalent than previously thought.
- Most children or adolescents who get these diseases were previously healthy and developmentally on target; the onset is usually abrupt.
- There may be different mechanisms driving the disease at the acute stage vs chronic inflammation, if it occurs. In addition, patients may develop more than one disease. (Anti-NMDA receptor encephalitis patients may go on to develop a disease that involves demyelination due to different antibodies such as those to AQP4 or MOG.)
- Early diagnosis and treatment improves outcomes. A response to immunotherapy can be diagnostic when test results are still pending.
- New biomarkers are actively being investigated and there is much promise in this area.
- Immunotherapy is the mainstay of treatment; however, formal treatment protocols are not established yet. Doctors must tailor treatment for each patient and take many issues into consideration.
- Researchers are working hard to identify the mechanisms of disease at the molecular level to figure out exactly how the disease begins and progresses. There is a strong belief that this information will lead to better treatment strategies that target the particular biological pathways causing illness.
- “Comprehensive multidisciplinary care is essential.”
- Many children require inpatient rehabilitation after discharge from the hospital. After a return home, improvements may still be seen for greater than 2 years.
- For these rare diseases “multicenter collaborative research is essential” for progress that will ultimately lead to the development of precision medicine and targeted immune therapies. There often are not enough patients to do large-scale clinical trials in rare diseases.
- Both environmental and genetic factors are believed to play a role in many of these illnesses. There is hope that in the future, whole genome sequencing may provide insight about genetic contributions to these diseases.
- The authors also note the importance of the following to advance research in the field: consistent collection of patient data including outcomes and establishment of databases, cooperative investigation of treatments, and development of additional testing methods.
The abstract for this paper is available here.