Highlighting the Demographics, Ethnic Distribution, and Post-Surgical Seizure Outcomes of Children with Rasmussen’s Encephalitis
Abstract number :
1.333
Submission category :
9. Surgery / 9B. Pediatrics
Year :
2023
Submission ID :
483
Source :
www.aesnet.org
Presentation date :
12/2/2023 12:00:00 AM
Published date :
Authors :
First Author: Jonathan Yarimi, MD – Baylor College of Medicine/Texas Children's Hospital
Presenting Author: Cristina Trandafir, MD – Baylor College of Medicine/Texas Children's Hospital
Howard Weiner, MD – Baylor College of Medicine/Texas Children's Hospital; Eyal Muscal, MD – Baylor College of Medicine/Texas Children's Hospital; Daniel Curry, MD – Baylor College of Medicine/Texas Children's Hospital; James Riviello, MD – Baylor College of Medicine/Texas Children's Hospital; Cristina Trandafir, MD – Baylor College of Medicine/Texas Children's Hospital
Rationale:
This study was a cohort characterization of pediatric patients with Rasmussen's encephalitis (RE).
Methods:
A retrospective chart review was performed during the period of January 2012 to June 2023. Charts with an encounter diagnosis of Autoimmune Encephalitis were identified. RE was considered in 47 patients. Twenty-nine of these met 2005 Bien Criteria (1) or had RE with atypical features and confirmatory biopsy (2). Two were excluded for incomplete documentation, leaving us with a cohort of 27 children. Surgery included focal resection and functional or anatomical hemispherectomy. Data review was conducted after approval by local IRB. Descriptive statistics were reported.
Results:
Twenty-seven patients met the inclusion criteria. A total of 70% of our cohort were male (n=19). Mean age at seizure onset was 6.7 years (IQR 1.2-15.8). The ethnic distribution was 55% Hispanic (n=15) and with average age at seizure onset 8.1 years (IQR 3-15.8). A total of 22% of the children were Caucasian (n=6), with an average age at seizure onset of 5.2 years (IQR 1.2-13.8). Finally, 22% were Black (n=6) with an average age at seizure onset of 4.7 years (IQR 2.9-8.3). Average length of follow up time was 8.6 years (IQR 1.5-17.9). 51% of our cohort (n=14) developed Epilepsia Partialis Continua (EPC). Average time to EPC from first seizure was 17.6 months (IQR 2-68). Caucasian children who developed RE were more likely to develop EPC (n=6) than Hispanic children (n=5). A total of 93% (n=25) of the children had surgery, with 72% (n=18) receiving hemispherectomy. Mean time from first seizure until first surgery was 455 days (IQR 20-2692) and mean time from first seizure until hemispherectomy was 627 days (IQR 20-2692). A total of 67% of children (n=12) who received hemispherectomy achieved complete seizure freedom. Only 14% (n=1) of children who received focal surgery without eventual hemispherectomy achieved complete seizure freedom. A total of 46 % (n=6) of children with focal resection had subsequent hemispherectomy due to inadequate seizure control.
Conclusions:
Our cohort of children with RE shows a predominance of Hispanic children who had a later age at seizure onset and were less likely to develop EPC than the Caucasian children, suggesting that ethnicity may affect the disease course. About half of the children with focal resections needed subsequent hemispherectomy due to inadequate seizure control. Our data demonstrate that hemispherectomy is the most effective surgery to achieve seizure freedom. However, patients received this surgery on average 1.7 years from seizure onset.
Funding: None
Surgery