Steady progress: Review article on autoimmune encephalitis and how field is evolving

As difficult as diagnosis and treatment are for autoimmune encephalitis, fortunately for the community of patients, families, and caregivers, rapid progress has been made within only a decade. A group of physicians in Oxford, England has published a review for the journal Neuropharmacology that summarizes the various types of AE, including a classification of syndromes, scientific details concerning discoveries of the ways in which antibodies are pathogenic, and current treatments (Varley, Taylor and Irani, in press, 2017). Points that may be of interest for patients and caregivers include the following:

  • Anti-NMDA and anti-LGI1 are the most commonly diagnosed forms of AE, although the exact prevalence is unknown.
  • Anti-NMDA receptor AE usually presents with psychiatric symptoms and movement disorders and anti-LGI1 with seizures and memory loss, although there are variations in presentation.
  • Other less common but identifiable forms of AE include aquaporin-4, myelin-oligodnedrocye glycoprotein (MOG), CASPR2, GABAAR, GABABR, GAD65, glycine R, AMPAR, and DPPX, and others.
  • New antibodies are still being discovered but of rarer syndromes so new methods of exploration are evolving.
  • There is a lack of controlled studies so this area needs to be developed.
  • Important discoveries are being made about how B cells and plasma cells produce antibodies and where they may reside.
  • First-line treatments include steroids, plasma exchange, and IVIG or some combination. Second-line treatments include cyclophosphamide and rituximab. Physicians must tailor treatments according to the patient’s needs.
  • New treatments are being tried in small studies, including monoclonal antibodies that target the types of cells producing dysfunction and other medical methods of reducing plasma cells. Some of these studies show promising results.
  • The authors note that physicians and scientists are studying and discovering exactly how antibodies disrupt brain function with the hope that targeted therapies will become widely available in the next few years to enhance and personalize treatments.

To read in more detail, please see Science Direct.

Post by Lynn Chapman