Abstracts

Rationale: Rasmussen’s encephalitis (RE) consists of unilateral inflammation of the cerebral cortex leading to drug-resistant epilepsy with neurological, neuropsychological, and cognitive deterioration. Recent advances suggest that immunomodulatory therapy slows progression, but hemispheric disconnection (HD) or resection remain the most effective treatment for long-term seizure control. Tradeoffs between permanent neurologic impairment and benefits of seizure control make surgical decision making difficult for both providers and caregivers. Thus, there is a crucial need for investigation of wholistic patient-centered outcomes after surgical intervention. We performed a retrospective cohort study to investigate clinical outcomes following HD for RE.

Methods: We used survey data pulled from the Pediatric Epilepsy Surgery Alliance Global Pediatric Epilepsy Surgery Registry for analysis. Patients diagnosed with RE who underwent HD were included. This yielded 37 records with comprehensive postoperative outcomes that included measures of seizure freedom, hydrocephalus, mobility, neurological deficits, language deficits, feeding difficulties, and quality of life (QoL). Descriptive statistics were used to compare target measures pre- and postoperatively, excluding records where the target measure was not reported.

Results: Demographics are reported in Table 1. The mean age of seizure onset was 5.57 years. The mean time from epilepsy onset to first surgery was 3.58 years. The most common procedure was a complete hemispheric surgery. Comprehensive outcomes are reported in Table 2. Compared to before surgery, 84.8% reported seizure freedom, 18.2% developed hydrocephalus, and 74.7% developed hemiparesis. For mobility, 35.8% were no longer running unassisted, 6.1% were no longer walking unassisted, 6.4% were no longer standing unassisted, and 31.2% were no longer rolling unassisted. 84.4% of patients who underwent surgery developed vision complaints, most commonly homonymous hemianopia (93.4%). Less common vision deficits included strabismus (14.4%) and cortical vision impairment (3.8%). Language deficits included apraxia (15.8%) and aphasia (17.4%). The most common learning issue after surgery was a specific learning disability in 31.6% of patients. 86% of caregivers reported improvement in their child’s QoL after surgery and 54.8% wished surgery was performed earlier. 12.5% did report some regret of having surgery.

Conclusions: We reported post-operative outcomes that will aid surgical decision-making for patients with RE. 85% reported seizure freedom, 86% reported improved QoL, and 54.8% reported wishing surgery was performed earlier. These benefits must be carefully weighed against known neurologic sequelae of HD, which our study confirms. Future studies with longitudinal data will assess interval changes in the reported post-operative outcomes. Additionally, specific factors pertaining to the surgical course such as time to surgery or total number of surgeries can be compared to determine if it affects the rate of post-operative deficits or seizures.

Funding: None