Abstracts

Rasmussen encephalitis (RE) is an acquired neurological disease characterized by intractable partial seizures and symptoms of progressive dysfunction of one cerebral hemisphere. Laboratory findings suggested the possibility of a immune-mediate component in the pathogenesis of the disease implying. However, surgical treatment still remains the preferred treatment. We report our experience on the surgical treatment of 10 RE patients collected in the context of an Italian collaborative study., The series included 10 patients with RE followed in the centers of Bambino Ges[ugrave] Children[apos]s Hopsital (Roma), Neurological Institute [ldquo]C. Besta[rdquo] (Milano) and Ospedale Maggiore (Bologna). Pre- and post-operative evaluations included: EEG recordings with video monitoring, MRI, assessment of motor function and cognitive evaluation based on clinical observation and standardized tests. The surgical procedures was based on functional disconnection of the affected hemisphere (hemispherotomy), performed according to vertex or peri-insular approach., The series includes 10 patients (6 M,4 F), aged between 10 and 23 years (16.3[underline]+[/underline]3.4) observed between 1993 and 2000. The age at onset ranged between 3 and 11 years (6.5[underline]+[/underline]2.8); surgery was performed 8 months to 9 years from disease onset (4.1[underline]+[/underline]2.7). Post-surgical follow-up ranged between 5 and 10 years (6.9[underline]+[/underline]1.5). The disease onset was marked by partial seizures, epilepsia partialis continua and unilateral limb paresis followed by status epilepticus. Neurologic deficits appeared in all patients within 24 months. EEG recordings consistently showed slow and epileptic abnormalities over the affected hemisphere. At the time of surgery patients were treated with three or more AED. All, but one, patients became seizure-free after surgery, AE treatment has been stopped in 7 patients, and reduced in 3. Patients still treated are given a mean of 1.5[underline]+[/underline]1 drugs. The need for neurological evaluations and EEG monitoring reduced from monthly to yearly., After surgery seizures ceased in all patients, thus demonstrating the successfulness of the operative procedures. It is noteworthy that postural control improved in all patients and regained unassisted gait. Mental abilities, as evaluated by standardized scales, improved in all cases. None of them performed anymore habitual monthly EEG and neurological evaluations. Surgical treatment of RE appeared in our series as a gold standard because of its effectiveness in seizure control, dramatic improvement in cognitive evaluations and reduction in the general healthcare assistance.,