Authors :
Presenting Author: Monika Jones, JD – Pediatric Epilepsy Surgery Alliance
Shaun A. Hussain, MD, MS – Division of Pediatric Neurology, Department of Pediatrics, UCLA Mattel Children's Hospital, David Geffen School of Medicine
Rationale:
Infantile Epileptic Spasms Syndrome (IESS) is the most common epilepsy syndrome in the first year of life and a severe form of epileptic encephalopathy. Diagnostic and treatment delays are often reported and have been linked to adverse long-term developmental and behavioral outcomes. Epilepsy surgery is a potentially curative—but underrecognized—treatment option for a significant minority of children with IESS. In this study, data from the Global Pediatric Epilepsy Surgery Registry was analyzed to explore patterns in diagnosis and treatment of children with IESS who have undergone epilepsy surgery.
Methods:
Parents/caregivers of children with IESS who are enrolled in the Global Pediatric Epilepsy Surgery Registry were surveyed via a secure web-based platform (REDCap). Data was anonymized prior to statistical analysis. Data included demographics and clinical attributes, including epilepsy syndrome classification, timing of IESS onset, diagnosis, treatment, and surgery, as well as developmental outcomes. Groupwise comparisons of continuous and dichotomous variables were accomplished using Wilcoxon rank-sum and Chi-Square tests, respectively. Results:
We identified 124 children (43% female, 83% white, 92% non-Hispanic, 80% from USA) with IESS who underwent epilepsy surgery. Median age at IESS onset and first surgery (in months) were 3.9 (IQR 1.9, 6.6) and 27.1 months (IQR 14.3, 46.3), respectively. Median latency from IESS onset to IESS diagnosis was 12 days (IQR 3, 35); 36% of respondents reported ‘significant’ diagnostic delay. Among children old enough for age-appropriate evaluation, respondents frequently reported moderate to severe impairment in all aspects of cognitive function, and in particular, expressive speech (74%). We observed trends such that moderate to severe speech impairment was more frequent among children with latency from IESS onset to diagnosis > 60 days (p = 0.06) and latency from IESS onset to first surgery > 12 months (p = 0.07).Conclusions:
Among children with IESS who undergo epilepsy surgery, significant impairments in speech are common and may be linked in part to both diagnostic delay and long latency to epilepsy surgery. Funding: None