Abstract

Adenosine dysfunction in Rasmussens encephalitis

Author(s): Tianfu Li, Qing Gao, Guoming Luan

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. Overexpression of the ADK, the major adenosine removing enzyme, was observed in the lesions of RE. As the upper neuromodulator of the brain, adenosine is well known with anti-inflammtion, aniti-epilepsy as well as improving cognitive dysfunction associated with epilepsy. Overexpression of ADK and resulting adenosine deficiency is involved in the development of RE- pharmacoresistant seizures, inflammation, and deficits in cognitive function. Dysregulation of adenosine signaling is a common pathologic hallmark of RE, which suggest the specific targets in the treatment of epilepsy, inflammation and cognitive deterioration associated with epilepsy in RE patients.