Rasmussen’s Encephalitis: Clinical Features and Mechanisms Advances

Rasmussen’s encephalitis (RE) is neurological disorder of childhood characterized by uni-hemispheric inflammation, intractable focal epilepsy and progressive cognitive and neurological deficits. Currently, hemispherectomy is the only effective method to control the seizures associated with RE. Although this disease has been heavily investigated, the pathogenesis of RE with unilateral cortex atrophy and focal seizure is still enigmatic. Neuropathological and immunological studies support the hypothesis that destruction of neurons and astrocytes by cytotoxic CD8 Tcells as a pathogenic mechanism underlying this enigmatic disorder. Recently data indicated that intrinsic activation of endogenous pro-inflammation high-mobility group box-1 (HMGB1) and Toll-like receptor (TLR) pathways, and dysregulation of adenosinergic mechanism are involved in the development of epilepsy, which suggest the specific targets in the treatment of epilepsy, inflammation and cognitive deterioration associated with epilepsy in RE patients.