Rasmussen syndrome: An Argentinean experience in 32 patients

Abstract

PurposeThe aim of this study is to analyze the electroclinical features, treatment, and evolution of patients with Rasmussen syndrome (RS). Materials and methodsWe conducted a retrospective, descriptive study in 32 consecutive patients with RS followed between 1990 and 2012. ResultsTwenty boys and 12 girls were included in the study. The mean and median ages at onset of the seizures were 6.5 and 7 years, respectively. Twenty-eight cases had epilepsia partialis continua that had started at a mean age of 9.5 years. Fixed hemiparesis occurred within the first two years after seizure onset in 26 patients. The ictal EEG showed a multifocal origin, but confined to the affected hemisphere in all patients. Mild focal atrophy involved the temporo-insular region associated with enlargement of the ipsilateral horn and Sylvian fissure. An abnormal cortical and/or subcortical hyperintense signal was observed in T2 and Flair images in 25 and 17 patients, respectively. T2 hyperintensity and atrophy in the basal ganglia was documented in five patients. Corticosteroids associated with immunoglobulins were used in 25 patients. Surgical treatment was performed in 25 patients. After a mean follow-up of 13 years (range, 2–20) good surgical outcome – Engel class I – was observed in 23 of 25 patients operated. ConclusionCorticosteroid and intravenous immunoglobulin treatment should be considered in the early stages of the disease. Patients with RS had a good response to surgical excision of the affected hemisphere.