RASMUSSENS ENCEPHALITIS - A CASE REPORT
Abstract
CASE HISTORY A 3 year old boy presented with status epilepticus, aphasia and right hemiparesis. On evaluation he was found to have atropy of left cerebral cortex on MRI and Unilateral slowing and epileptiform activity on left side in EEG. He was diagnosed to have RE as per EUROPEAN CONSENSUS STATEMENT. INTRODUCTION RE is a chronic progressive, inflammatory disease of the brain affecting one hemisphere. The characteristic feature inclue Epilepsia Partialis Continua, hemiparesis, congnitive disturbances and speech disturbances. ETIOLOGY It is of unknown etiology. The postulated hypothesis include direct viral insult, primary autoimmune injury and immune response secondary to viral infection. DIAGNOSIS The diagnostic criteria as proposed by European consensus statement 2005 has primary and secondary criteria. The primary criteria includes Clinical (partial seizures and unilateral neurological deficits), MRI(unilateral focal cortical atropy and T2FLAIR hyperintense signal or atropy of head of caudate nucleus) and EEG (Unilateral slowing with or without epileptiform activity, unilateral ictal onset).The Secondary criteria includes Clinical (EPC or progressive unilateral neurological deficit), MRI(progressive unilateral focal cortical atrophy) and Histopathology (T-lymphocyte dominated encephalitis with activated microglial cells and reactive astrogliosis.TREATMENT The various treatment modalities include anticonvulsants, physiotherapy, immunotherapy, plasmapheresis, Monoclonal antibodies and surgical procedures like functional hemispherectomy.
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