One core mission of the Autoimmune Encephalitis Alliance is to share the latest research to improve the clinical diagnosis and treatment of AE. A list of symptoms and a brief discussion of diagnosis are provided in the Living with AE section of this website.
In January 2013, the AE Alliance coordinated a Grand Rounds at Duke University Medical Center. Our board members and science team have conducted similar events at other medical institutions to share knowledge about AE. We will be conducting addtional events in the coming months.
Pubmed identifies 155 newly published papers on “autoimmune encephalitis” in just the last 18 months. The following papers are a very small subset of the peer-reviewed literature. UPDATE: See recent AE Alliance blog post with updated literature review.
The first two papers reviewed here were published by Dr. Josep Dalmau and his research group.
A Lancet Neurology article by Dr. Titulaer, Dalmau and others provides the best understanding to date of anti-NMDA receptor encephalitis. This seminal paper is a must read for any clinician interested in AE and provides the basis for much of the Living with AE section of this website. The study presents data on treatment effects and outcomes for the largest cohort of patients with anti-NMDA receptor encephalitis: 577 patients ranging in age from 8 months to 85 years (mean age of 21). 211 of the patients were children. Key findings from this study include:
- most patients with anti-NMDAR encephalitis respond to immunotherapy,
- second-line immunotherapy (rituximab and cyclophosphamide) is usually effective when first-line treatments (steroids, IVIG, plasmaphoresis) fail, but only 58% of patients received second-line immunotherapy,
- the recovery of some patients took up to 18 months, and
- early and aggressive treatment predicted good outcomes.
A JAMA neurology paper focuses on the subset of patients whose initial onset or relapse of anti-NMDAR encephalitis presents with isolated psychiatric symptoms. Recognition of these episodes is important because they respond to immunotherapy. These findings suggest that patients with new-onset psychosis, having a history of encephalitis, subtle neurological symptoms, and/or abnormal results on ancillary tests should be assessed for NMDAR antibodies. These data have significant implications for psychiatric assessments, particularly of new-onset psychosis. Autoimmune encephalitis must be included in the differential diagnosis.
Two additional important papers from the Dalmau group should be reviewed:
- Encephalitis and antibodies to synaptic and neuronal cell surface proteins, Neurology. 2011 Jul 12;77(2):179-89
- Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol. 2011 Jan;10(1):63-74
The following peer-reviewed articles demonstrate the range of new research from neurology, psychiatry and other disciplines. Please share your knowledge of other studies that we should include or offer your perspective on clinical diagnosis and treatement of AE.
- Najjar et al propose a standarized classification system for autoimmune encephalitis that covers sero-positive (NMDAR, VGKC-complex, GAD autoantibodies) and zero-negative cases of AE. In a 2013 paper, Najjar et al.
- A 2013 paper by Suleiman et al suggest a set of proposed guidelines for the identification of autoimmune epilepsy in children. The paper also provides 13 case studies of children presenting to the author’s tertiary clinic. Of the 13 cases, 7 children tested positive for neuronal surface antibodies.
- A 2013 paper by Kotsenas et al provides new guidance on using MRI to detect Autoimmune Voltage-Gated Potassium Channel Complex Encephalitis.
- A 2012 paper by Rosenfeld et al offers “five new things” about AE and paraneoplastic syndromes. One of the five findings includes “the recognition of a characteristic EEG pattern (“extreme delta brush”) in 30% of patients…”
Careful case reports are also valuable in describing how AE presents. Below are links to some recent case reports that have contributed to our understanding of AE in children:
- Anti-NMDAR case in 11 year old girl
- Anti-NMDAR case in 16 year old boy
- Anti-NMDAR case in 16 year old girl
- Anti-NMDAR cases in 3 year old girl and 8 year old girl
The AE Alliance is committed to being a resource for new disseminating new knowledge. We are just getting started and look forward to working with researchers and clinicians from across the globe to build a shared understanding of the basic science and clinical care for AE. We hope you will join us.
With knowledge gathered and disseminated, new models of clinical care will be required to connect disciplines across institutions. The AE Alliance seeks to create Centers of Excellence that will serve as new models of care.