Utility of Ictal SPECT in the presurgical evaluation of Rasmussen's encephalitis

BACKGROUND: Rasmussen's encephalitis is a progressive disease characterized by severe progressive neurological and cognitive deficits. It is a difficult condition to treat with conventional medical treatment and requires, in most of the cases, surgical intervention. METHODS: We present the case of a patient with Rasmussen's encephalitis who presented with epilepsia partialis continua, who did not respond to antiepileptic treatment and was evaluated for possible surgical intervention. RESULTS: The electroencephalographic studies revealed bihemispheric involvement during seizures, although magnetic resonance imaging (MRI) showed a marked right hemispheric atrophy. Hyperperfusion over the right hemisphere was seen with the performance of ictal single photon emission computed tomography (SPECT), which finally guided a right functional hemispherectomy and subsequent seizure control. CONCLUSION: Ictal SPECT could be a useful tool in the armamentarium of tools used for the localization of the epileptogenic focus in patients with Rasmussen's encephalitis and other localization-related epilepsies.