LONGITUDINAL ELECTROENCEPHALO-CLINICAL EVOLUTION IN 4 PATIENTS WITH RASMUSSEN ENCEPHALITIS

• Published in: Neuropediatrics
• Main Authors: Amrom, D, Wayenberg, JL, De Borchgrave, V, Van Rijckevorsel, K
• Format: Conference Proceeding
• Language: English
• Published: 18.05.2006
• ISSN: 0174-304X; 1439-1899
• DOI: 10.1055/s-2006-945903

Objectives: To describe the evolution of electroencephalogram (EEG) over years, and after hemispherotomy , in Rasmussen encephalitis (RE) patients. Methods: The EEG recordings of 4 patients with documented RE were retrospectively compared between them and over time. Three patients had disease onset in early childhood and underwent hemispherotomy when intractable epilepsy had appeared. Postoperatively clinical seizures ceased and the 3 patients were medication free. One patient had disease onset when 11 years old, and was followed in our unit from the age of 29 years. Immuno-modulatory treatments had only temporary effects. Vagal nerve stimulation was unhelpful. He did not undergo hemispherotomy. Patient still suffers from refractory tonic seizures, spastic hemiparesia and severe cognitive impairment. Results: Before hemispherotomy, the EEG recorded at the beginning of the disease in the 3 infants showed a polymorphic delta activity over the fronto- or centro- temporal areas of the affected hemisphere, intermingled with epileptiform activities. Thereafter the EEG deteriorated progressively on both hemispheres. After hemispherotomy, electrical ictal discharges were observed in the affected hemisphere up to 3 years, but the background activity was faster or normalised on the unaffected side. Only small spikes were occasionally observed in the frontal area of the preserved side. In the non operated adult patient, EEG showed permanent slow spike waves in the right temporal region, on a diffusely slowed background rhythm. High voltage spike-waves were occasionally recorded in the unaffected region. Conclusion: In all patients, unilateral slow waves were recorded from the beginning of the disease. Hemispherotomy does not have power over ictal discharges in the affected hemisphere, but controls clinical seizures.