Cognitive Outcomes In Children With Infantile Spasms: Successful Treatment Response Is Beneficial Regardless Of Etiology

Objective: A prospective study to determine the cognitive outcomes in children with Infantile Spasms (IS) based on treatment response. Background: Infantile Spasms (IS) pose significant risks for poor developmental outcome linked to symptomatic seizure etiology. The association of successful treatment on cognitive outcomes based on treatment response remains unclear. Design/Methods: IS cases were prospectively enrolled for observational study at a tertiary center (2013–2017). IS etiology was categorized as known or unknown. Initial treatment choice was either ACTH or Prednisolone (PSL) or Vigabatrin (VGB). Non-responders (NRESP) to the first treatment were switched to a second medication. The Responder (RESP) group were spasm free patients with EEG confirmation at 3 months from diagnosis. The Vineland-II was administered at diagnosis, and at 12 and 24 months of age to measure cognitive outcomes. Seizure and cognitive outcomes were compared between RESP and NRESP and based on seizure etiology. Results: Sixty patients were enrolled with 23% having unknown etiology. Mean age was 6.6 months and age at final follow up was 24 months. Initial treatment choice was ACTH (34), PSL (4) and VGB (22). Response rate was 74% (86% unknown vs 72% known etiology, p=0.5). At 12 months, 54% were seizure free. At 24 months, 40% were off medication and seizure free for more than 1 year. Vineland scores between RESP and NRESP was 73 vs. 77 (p=0.4) at diagnosis, 72 vs. 61 (p= Conclusions: Cognitive outcome is influenced by responder category. Non-responders demonstrated cognitive decline over time with poorer outcomes in the known etiology group, supporting the negative prognostic role of the symptomatic etiology group. Disclosure: Dr. Mittal has nothing to disclose. Dr. Asato has nothing to disclose. Dr. Sogawa has nothing to disclose.