Functional hemispherectomy in a 13 year old boy with Rasmussen’s encephalitis in Khatam-al-anbia Hospital Tehran Iran

Background: Rasmussen’s encephalitis is a rare chronic inflammatory neurodegenerative disease affecting a single cerebral hemisphere, causing progressive neurological deterioration and intractable seizure. Case report: Here I present a case of 13 year-old boy with left sided focal motor seizures since age 9 year. Gradually left motor seizures became continuous, accompanied with slowly progressive left side hemiparesis and mild cognitive impairment. His seizures were uncontrolled with antiepileptic medications, IVIG and plasma exchange. In view of the prolonged history of refractory left-sided partial motor seizures, left hemiparesis, and right-sided brain atrophy on imaging, a possibility of Rasmussen’s encephalitis (RE) was considered. Functional hemispherectomy was done at age 13. Now two months after the surgery he is seizure free and his motor functions got better, Brain DTI image showed good disconnection between the two hemispheres. In this case report I will compare the two video EEG monitoring and annual brain MRI images of the patient during the last three years of his illness. Also we could evaluate the EEG changes after the surgery. Conclusion: This case reveals in the late stage of Rasmussen encephalitis hemispherectomy is still good chose in control of intractable seizures which could control the seizures completely without noticeable complication. It seems disconnection procedures also could affect the electrophysiological properties of brain.

Keywords:

Rasmussen’s encephalitis , hemispherectomy