International Journal of Epilepsy 2016; 03(01): 42-62
DOI: 10.1016/j.ijep.2015.12.047
Thieme Medical and Scientific Publishers Private Ltd. 2017

A case of Rasmussen's encephalitis mimicking focal cortical dysplasia treated with focal cortical resection

Vamsi Krishna Yerramneni
1   Dept of Neurosurgery, NIZAMS, Hyderabad
S K Jabeen
1   Dept of Neurosurgery, NIZAMS, Hyderabad
› Institutsangaben

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12. Mai 2018 (online)

Introduction: Rasmussen's encephalitis (RE) is one of the important causes of refractory seizure presenting epilepsia partialis continua (EPC), progressive hemiparesis and neuro-psychological deterioration. Currently, the best approach to RE is hemispherectomy.

Case histories: A 8-year-old girl right handed with normal birth and development presented with 20–30 seizures per day since the past 2 years’ The semiology was in the form of auditory aura followed by behavioral arrest, excessive salivation from mouth and deviation of angle of mouth to left side. The child was responsive during the attack. Visual acuity normal with no motor or sensory deficits.

MRI brain revealed T2 and FLAIR hyperintensity in the right insula extending into right temporal pole. Caudate was normal. MRI was interpreted as focal cortical dysplasia involving right insula.

VEEG showed frequent right hemispheric discharges with temporal neocortical simple partial seizures.

Patient was operated with focal cortical resection of right insula lesion with right temporal lobectomy. Patient had postoperative right hemiparesie due to a subcortical infarct. Histopathology revealed the characteristic picture of rasmussens encephalitis.

Discussion: The follow-up brain MRI 1 year later showed diffuse atrophy of the brain with more atrophic change in right hemisphere. At 3 years follow up, EEG showed right hemispheric slowing with few electrographic seizure with no clinical seizures. She was treated with a short course of IV methyl prednisolone. Right hemiparesis improved.