2010

Innovator Awards

A New Experimental Model Of Rasmussen’s Encephalopathy: Understanding Its Pathophysiology

Lionel Carmant, MD
University of Montreal
 

Rasmussen encephalitis (RE) is a rare but severe brain disorder that starts in childhood with seizures that become more and more frequent, as well as a progressive weakness of the affected side. The mechanisms of RE are still not well understood, even if some experimental data suggest an immune mediated disorder. By injecting RE patients’ blood cells into severely immunologically-compromised mice, we are able to induce seizures and produce pathological findings in the mouse brain similar to pathological lesions observed in children with RE. We can also use frozen blood cells from individuals with RE, making our strategy to understand the pathophysiology of RE even more feasible. Preliminary data shows that RE is mediated by the immune system, and that seizures are due to the release of inflammation products followed by an infiltration of white blood cells in the brain. The model we will use for this research will allow us to identify the cells responsible for RE and to precisely determine the role of the immune system in this rare disorder. In the future, we plan to utilize this model test to new therapeutic strategies for RE.

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