Abstracts

Rationale: Rasmussen encephalitis (RE) is a severe pediatric inflammatory brain disease characterized by unilateral inflammation of cerebral cortex and unilateral brain atrophy, drug-resistant focal epilepsy and progressive neurological and cognitive deterioration. The aetiology and pathogenesis of RE remain unclear. Our previous results have demonstrated that adenosine A1 receptor (A1R) and adenosine kinase play an important role in the epileptogenesis of RE.  Recently, adenosine A2A receptor (A2AR) has been proved to play a crucial role in counteract the A1R-mediated inhibition of synaptic transmission, and act as a STOP signal of the immune-inflammatory system. We hypothesized that the epileptogenic mechanisms underlying RE is associated with alterations of A2AR expression.  Methods: Immunohistochemistry approach was used to examine the expression of A2AR in cortical specimens from RE (n=12), and compared that with control cortical tissue. The quantification of A2AR expression in RE versus controls was evaluated using Western blot. Results: Marked expression of A2AR was observed in the lesions of RE, with predominantly expressed in perinuclear of neurons astrocytes. Significantly increasing of A2AR expression in RE versus controls was demonstrated by Western blot.  Conclusions: These results suggest that upregulation of neuron and astrocyte A2AR in RE is a common pathologic component of RE. A2AR might, therefore, be a target in the treatment of RE. Funding: This project was supported by the grant from the BIBD-PXM2013_014226_07_000084, National Natural Science Foundation of China (81571275).