HMGB1-TLR Signaling in Rasmussens Encephalitis

Abstract

Rasmussen’s encephalitis is neurological disorder of childhood characterized by uni-hemispheric inflammation, intractable focal epilepsy and progressive cognitive and neurological deficits. Currently, the pathogenesis of Rasmussen’s encephalitis is still enigmatic and hemispherectomy is the only effective method to control the seizures associated with Rasmussen’s encephalitis. Recently data indicated that intrinsic activation of endogenous proinflammation high-mobility group box-1 (HMGB1) and Toll-like receptor (TLR) is involved in the development of Rasmussen’s encephalitis. Activation of HMGB1-TLR signaling plays a critical role in brain inflammation, development of epilepsy and cognitive dysfunction. Targeted therapy on HMGB1-TLR signaling might be a novel strategy with anti-inflammation, anti-epilepsy as well as improving cognitive dysfunction associated with epilepsy in Rasmussen’s encephalitis.