Clinical characteristics and surgical outcomes of Rasmussen encephalitis: A retrospective dual-center cohort study

Rasmussen encephalitis (RE) is a rare progressive disorder causing drug-resistant epilepsy. Hemispheric surgery is an established treatment, but comprehensive data on postoperative seizure, motor, and cognitive outcomes are limited. We aimed to evaluate these outcomes and identify associated prognostic factors.

This dual-center retrospective study included RE patients who underwent hemispheric surgery at two tertiary epilepsy centers in China. Seizure outcomes were classified by Engel class, with Engel class I regarded as seizure-free. Motor outcomes were evaluated using the Motricity Index (MI), Gross Motor Function Classification System (GMFCS), and Manual Ability Classification System (MACS), with stable outcomes defined as improvement or no change between baseline and follow-up. Cognitive function was evaluated using standardized scales. Multivariable Cox regression identified factors associated with seizure and motor outcomes.

Eighty-five patients (52 female, 63%) with median follow-up of 2.75 years were included. At last follow-up, 79% were seizure-free after initial hemispheric surgery. Hemispheric disconnection showed better seizure control (92%) than anatomic (67%) or functional hemispherectomy (57%), with shorter surgery and less blood loss. Stable gross motor function was achieved in 94%, whereas 64% experienced worsening fine motor skills. Generalized seizures (hazard ratio [HR] 5.48, 95% confidence interval [CI] 1.63-18.42), contralateral magnetic resonance imaging (MRI) abnormalities (HR 6.43, 95% CI 1.14-36.28), contralateral interictal electroencephalography (EEG) discharges (HR 4.61, 95% CI 1.57-13.51), and type of hemispheric surgery (HR 5.25, 95% CI 1.57-13.51) were associated with seizure persistence. Postoperative seizure-free (HR 4.43, 95% CI 1.61-12.17) and baseline MI (HR .94, 95% CI .91-.96) predicted overall motor stability, whereas fine motor stability was related to epilepsia partialis continua (EPC) duration (HR .50, 95% CI .25-.98), preoperative immunotherapy (HR 3.30, 95% CI 1.13-9.59), and baseline MACS (HR 2.45, 95% CI 1.68-3.57).

This study suggests that hemispheric surgery is effective in achieving seizure-free and favorable gross motor recovery in RE. Early surgery, attention to contralateral abnormalities, and preoperative immunotherapy may further improve outcomes.