Abstracts

Rasmussen[apos]s encephalitis (RE) is a relentlessly progressive disease with onset most commonly in childhood. Clinically, RE is characterized by the development of refractory partial seizures and progressive hemiparesis. The exact etiology of RE is not known, although recent scientific studies have implicated an underlying immune mechanism. These studies have led to trials of various immunomodulatory therapies, some of which have shown potential benefit, with improved seizure frequency and delayed progression of neurologic deficit. Yet, this benefit is variable and often short-lived. The only proven curative therapy for RE is functional hemispherectomy. This radical surgery is typically performed after significant neurologic deficit has occurred. Affected children may have the potential for recovery of the motor and language functions of the involved hemisphere. However, a dilemma is faced in deciding on the appropriate treatment course for adolescent or adult onset cases of RE which often have a more variable course and slower progression. In these individuals, a temporizing treatment is desirable as the clinical consequences of functional hemispherectomy may hasten the development of permanent neurologic deficit. There are few reports on the outcome in older RE patients after other, less radical, surgical interventions. We report a case of late-onset RE, in which the patient had become progressively disabled by her seizures, specifically the development of epilepsia partialis continua (EPC) and the adverse effects of high doses of multiple anti-epileptic medications. Despite the severity of her seizures, neurologically, she had no evidence of permanent motor deficit. Intracranial EEG monitoring identified the area involved in her focal motor seizures and focal resection of this area resulted in dramatic improvement of seizure frequency and allowed for the initiation of a medication taper. This resection resulted in minimal motor deficits of the corresponding side and overall the patient experienced a significant improvement in her quality of life. Based on this experience, it may be proposed that focal surgical resection should be considered in older RE patient[apos]s who are disabled primarily by their seizures and have relative preservation of neurologic function, thus avoiding the certain deficits from functional hemispherectomy. The duration of this benefit is unclear, although in our patient improvments have been sustained for a period of months. Long-term follow-up will be necesary to compare the benefits of focal surgical resection to other less radical therapies.