This week we added a reference library for RE to the website. The library has over 300 references to scholarly publications relating to RE categorized by relevant topics. Go to the home page of this website to learn more about how to obtain a copy of the library file. The reference library was created by my good friend, Deirdre Pinto who lives in Australia and whose niece has RE. Deirdre has been an invaluable contributor to the RE Children’s Project since our inception penning many important pieces on this site including the scientific notes from our Deer Valley Conference in 2010. We decided to create the library in the hopes it would spur additional interest in RE by investigators as well as to be a resource for families that are first learning about RE.
Here is what a reference looks like:
Reference Type: Journal Article Record Number: 4413
Author: S. Bittner, O. J. Simon, K. Göbel, C. G. Bien, S. G. Meuth and H. Wiendl
Year: 2013 Title: Rasmussen encephalitis treated with natalizumab
Short Title: Rasmussen encephalitis treated with natalizumab ISSN: 0028-3878
Abstract: Rasmussen encephalitis (RE) is characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable seizures. Inflammatory lesions consist of oligoclonally expanded cytotoxic CD8+ T cells attacking neurons and astrocytes.1 Immunotherapies may slow down tissue and function loss, whereas convincing effects on seizure activity have rarely been reported.2 Functional hemispherectomy (HE) is highly effective for elimination of seizures but can only be offered if no indispensable function resides in the affected hemisphere.1 We present a patient with such an HE contraindication who was treated with natalizumab, a blocker of T-cell entry into the CNS. Research Notes: Published ahead of print. URL: http://www.neurology.org/content/early/2013/06/21/WNL.0b013e31829c5ceb.extract# http://www.ncbi.nlm.nih.gov/pubmed/23794679
This week I attended a meeting of several individuals impacted by encephalitis. The meeting was organized Ava Easton of The Encephalitis Society, a wonderful organization located in the UK and interested in expanding its presence in the US. At the meeting I met individuals who have lost children to encephalitis as well as whose lives have been turned upside down by this disease. I met one couple whose daughter passed away due to auto-immune encephalitis and interestingly their initial diagnosis was rasmussens. The couple has funneled their tragedy into a greater good and they have founded the Auto-immune Encephalitis Alliance. Please check out their website.
It was interesting to learn about encephalitis, similar to epilepsy, encephalitis is used to describe a broad range of conditions all of which have inflammation of the brain as a defining trait. That is certainly a trait of RE. Inflammation can result from infections or from immune (allergic) reactions. Infectious encephalitis is most commonly caused by viruses, and more than 100 different viruses have been related to encephalitis. Seizures are also part of encephalitis occurring in up 50% of the cases. My thanks to Ava for inviting me to the meeting, we can learn a lot from the efforts of these encephalitis groups.
We shipped tissue this week from Portugal to UCLA. Our tissue transfer program is a success and we are well on our way to having a brain bank with enough tissue to advance research into RE. Here is a partial list of the hospitals that have participated in our research program:
Children’s National Medical Center – Washington, DC
The Johns Hopkins Hospital – Baltimore, MD
NYU Langone Medical Center BioRepository Center – New York, NY
The Hospital das Clínicas de Ribeirão Preto – São Paulo, Brazil
Children’s Hospital at Westmead – Westmead NSW, Australia
University of South Alabama Medical Center – Mobile, AL
University of Alberta Hospital – Edmonton, Alberta Canada
Duke University Medical Center – Durham, NC
Ann & Robert H. Lurie Children’s Hospital of Chicago – Chicago, IL
Fondazione I.R.C.C.S Istituto Neurologico Carlo Besta – Milan, Italy
Hospital de Santa Maria – Lisbon, Portugal
Ronald Regan UCLA Medical Center – Los Angeles, CA
My heartfelt thanks to these organizations as well to their doctors who are assisting us advance our knowledge of RE. If you do not see you hospital on the list download our protocol manual and ask them to get involved.
Please continue to support our efforts. We are making an impact. Thank you.