Rasmussen encephalitis. Case report

Rasmussen encephalitis is a neurodegenerative, inflammatory, chronic, rare and progressive disease that affects only one cerebral hemisphere, which usually occurs in childhood. It is characterized by presenting progressive unilateral focal neurological deterioration and focal refractory seizures to drugs, associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Continuous refractory partial epilepsy is the common denominator of the disease. Diagnosis is based on clinical, electroencephalographic and neuroimaging characteristics. Magnetic resonance imaging can demonstrate focal or unihemispheric involvement and helps exclude other pathologies. Specific therapeutic approach for these cases remains unclear. Several immunomodulatory treatments can delay, but not stop the progression of the disease. Treatment of choice is hemispheric surgical disconnection to reduce associated neurological deficiencies. A case of Rasmussen encephalitis is presented.