Surgical Interventions in Rasmussen’s Encephalitis

Abstract

Rasmussen’s encephalitis (RE) is a rare, devastating neurological condition characterized by chronic unihemispheric brain inflammation, refractory epilepsy, and progressive neurological decline and hemiplegia with an etiology that remains elusive and a pediatric-skewed epidemiology. The deleterious nature of RE on the patient’s neurocognitive development necessitates early medical intervention. RE is diagnosed on the basis of clinical features, MRI, EEG and biopsy obtained after surgery. While medical management often proves ineffective, surgical intervention has shown durable seizure freedom and control. This review comprehensively explores the surgical techniques in the management of RE including the steps involved in modified functional hemispherectomy, peri-insular, vertical-parasagittal and endoscopic hemispherotomies. We have summarized how each procedure aims to disconnect the affected hemisphere from the healthy hemisphere through different corridors of approach. Hemidecortication procedures offer good functional and cognitive outcomes overall despite the expected hemianopia, hemiplegia and loss of fine motor skills. We also review the importance of selecting appropriate candidates for surgery, as well as comprehensive pre- and postoperative management, along with the most likely outcomes of surgery.