Clinical characteristics and factors associated with preoperative neurodevelopment in children with Rasmussen encephalitis: A single-centre retrospective study of 51 patients.

PURPOSE: To characterise clinical features of Rasmussen encephalitis (RE) and identify factors associated with preoperative neurodevelopmental status in affected children.

METHODS

This retrospective single-centre study included 51 paediatric patients diagnosed with RE who underwent surgical treatment. Demographic characteristics, seizure features, video-electroencephalography (VEEG) findings, magnetic resonance imaging (MRI) features, and neurodevelopmental assessments were systematically collected. Associations between full-scale developmental quotient/intelligence quotient (FSDQ/IQ) and clinical variables, including age at onset, disease duration, electroencephalography findings, MRI characteristics, and number of antiseizure medications (ASMs), were analysed.

RESULTS

All patients underwent hemispherotomy or hemispherectomy and were seizure-free at the latest follow-up, although one experienced postoperative seizures from the contralateral hemisphere. Epilepsia partialis continua occurred in 54.9% of patients, and 52.9% had contralateral independent epileptiform discharges. Early MRI showed hemispheric or focal atrophy in 49%. Preoperative neurodevelopmental assessment was completed in 45 patients, with a mean FSDQ/IQ of 70.5. Overall, 55.6% demonstrated normal or borderline development, and 26.7% had mild developmental delay. Motor function was the most affected domain, whereas cognitive and non-motor domains were generally borderline. Patients with contralateral epileptiform discharges had lower FSDQ/IQ than those without (p = 0.001), and this association remained significant after adjustment for age at seizure onset, disease duration, and number of ASMs.

CONCLUSION

Preoperative neurodevelopmental status in children with RE was heterogeneous, with overall scores in the borderline range. Contralateral independent epileptiform discharges were independently associated with lower FSDQ/IQ, although the underlying mechanisms and clinical significance of this association remain uncertain. These findings support close developmental monitoring and individualised preoperative assessment in RE.