Conservative management of Rasmussen's encephalitis in a Nigerian girl

Abstract

Rasmussen's encephalitis (RE) is a rare disease of the brain characterized by focal seizures, unilateral cortical deficits, hemiparesis, and hemiatrophy of the contralateral cerebral hemisphere. The affected brain tissue shows a chronic inflammatory histopathology and an autoimmune reaction is suspected. Therapeutic strategies include anticonvulsants, immunomodulation and surgery. We present the first report of RE in a Nigerian. A 4-year-old girl presented with a 15-month history of recurrent involuntary jerky movements of the right upper limb and tonic deviation of the neck to the right side often associated with loss of consciousness. Seizures lasted 2 to 3 min and occurred every 2 to 3 h. She later developed right-sided weakness. On examination, she had right-sided spastic hemiparesis with astereognosis. The electroencephalograph showed theta and delta waves on the left cerebral hemisphere with epileptiform sharp and slow waves and phase reversal pattern. Brain computerized tomogram showed left hemispheric hemiatrophy with non-enhancing hypodense lesions in the left hemisphere. This was confirmed by brain magnetic resonance imaging, which showed left cerebral atrophy with associated left white matter hyperintensities on fluid-attenuated inversion recovery sequences. There was no enhancement with gadolinium. She improved on anticonvulsants. She fulfilled the European Consensus criteria for the diagnosis of RE. Electroencephalography and magnetic resonance imaging are important for the diagnosis of RE, a condition worth considering in patients with focal seizures and unilateral neurological deficits.