Abstracts

Rationale: Current guidelines for the treatment of infantile spasms (IS) recommend the use of either ACTH or vigabatrin as first-line therapies (1,2). However, 59% of children do not respond to their first IS treatment while 37% within that group respond to a second spasm treatment (3). More recently, the International Collaborative Infantile Spasms Study reported that initial treatment of infantile IS with hormonal therapy (synthetic ACTH or prednisolone) in combination with vigabatrin is significantly more effective at stopping IS than hormonal therapy alone, but further research exploring the outcomes of combined first-line treatments is needed (4). Methods: Consecutive patients with new onset infantile spasms at Lurie Children’s Hospital were enrolled and followed prospectively. We reviewed clinical and EEG data of subjects treated with a combination of natural ACTH and vigabatrin at the time of diagnosis to determine spasm response 3 months after treatment initiation. Results: Nine out of 66 (14%) consecutive patients with video-EEG confirmed IS displaying either hypsarrhythmia (78%) or modified hypsarrhythmia (22%), were treated with the ACTH-vigabatrin combination at diagnosis. Etiology was identified in 4 patients (44%), including hypoxic ischemic encephalopathy, lissencephaly, and agenesis of the corpus callosum. Mean age of onset of IS was 5.7 months (IQR: 4.5-6.8) and mean time between spasm onset and diagnosis was 0.6 months (IQR: 0-0.3). Seven out of 9 patients (78%) demonstrated a complete response with absence of clinical and electrographic spasms and resolution of hypsarhythmia on the 3-month EEG. One patient did not achieve spasm or hypsarhythmia resolution. One patient had a relapse of spasms but not hypsarhythmia by the 3-month EEG after initial response after 2 weeks of treatment. Five patients (56%) that had normal development prior to IS onset did not show any developmental delay at 3 months following combination therapy. No adverse events were attributed to the combination of the two medications. Conclusions: This case series demonstrates the combination of ACTH and vigabatrin was effective and well tolerated for the treatment of IS, for both patients of unknown and known etiology. References:1. Pellock, J. M., et al. Epilepsia 2010;51: 2175–2189. 2. Go CY, et al. Neurology 2012;78:1974-80.3. Knupp, K. G., et al. Epilepsia, 57: 1834–1842.4. O'Callaghan FJK, et al. The Lancet Neurology.16(1):33-42. Funding: The National Infantile Spasms Consortium database was funded by a grant from the Pediatric Epilepsy Research Foundation (PI: Dr Berg).